Declaration of potential conflict of interest
of authors/collaborators of Updated Geriatric Cardiology Guidelines of
the Brazilian Society of Cardiology - 2019If the last three
years the author/developer of the Updated:
Names Members of the Statement
Participated in clinical studies and/or experimental
trials supported by pharmaceutical or equipment related to the guideline
in question
Has spoken at events or activities sponsored by industry
related to the guideline in question
It was (is) advisory board member or director of a
pharmaceutical or equipment
Committees participated in completion of research
sponsored by industry
Personal or institutional aid received from industry
Produced scientific papers in journals sponsored by
industry
It shares the industry
Adenalva Lima de Souza Beck
No
No
No
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Ana Clara Tude Rodrigues
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André Luiz Cerqueira de Almeida
No
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No
Bruna Morhy Borges Leal Assunção
No
No
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Eliza de Almeida Gripp
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Fabio Villaça Guimarães Filho
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Henry Abensur
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José Maria Del Castillo
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Marcelo Haertel Miglioranza
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No
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Marcelo Luiz Campos Vieira
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No
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Márcio Vinicius Lins de Barros
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Maria do Carmo Pereira Nunes
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Maria Estefânia Bosco Otto
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Minna Moreira Dias Romano
No
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No
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Renato de Aguiar Hortegal
No
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Rodrigo Bellio de Mattos Barretto
No
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No
Samira Saady Morhy
No
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Silvio Henrique Barberato
No
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Thais Harada Campos
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Vicente Nicoliello de Siqueira
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1. Introduction
In accordance with the “Standards for the Elaboration of Guidelines, Positions and
Normations” sanctioned by the Brazilian Society of Cardiology, this document was
written to update the “Echocardiography Indication Guidelines” of 2009. The new
document is not intended to be a comprehensive review of echocardiography, but
rather an indispensable basic guide to support the rational clinical decision-making
of the physician requesting the exam for adult patients. Although it considers the
recent technological advances of echocardiography, its purpose is not to describe
in
detail echocardiography methods, but to summarize in a clear and concise way the
main situations in which echocardiography brings benefit to the diagnosis and/or
therapeutic orientation of the individual. This manuscript chose to highlight the
class of recommendation, as described below:
Class I: conditions for which there is conclusive evidence or, in the
absence thereof, general agreement that the examination procedure is
useful and safe.
Class II: conditions for which there is conflicting evidence and/or
divergence of opinion on the utility and/or safety of the
examination.
Class IIa: evidence or opinions favorable to the examination. Most
experts approve.
Class IIb: utility and/or safety less well established, with divergent
opinions.
Class III: conditions for which there is evidence or consensus that the
examination is not useful and, in some cases, may even be harmful.
In addition, the level of evidence was also described, as follows:
A: several concordant randomized clinical trials or robust
meta-analyses;
B: less robust meta-analysis data or single randomized clinical study or
observational studies;
C: expert opinion.
Thus, it was agreed that all the tables with recommendations for the use of
echocardiography in the different clinical scenarios include columns with class of
recommendation and level of evidence.
2. Evaluation of Heart Function and Structure
2.1. Left Ventricular Systolic Function
The analysis of left ventricular (LV) systolic function is a primary indication
of the use of echocardiography. Echocardiographic analysis of LV systolic
function can be performed using older techniques such as M-mode, 2D
echocardiography, and even more modern techniques, such as three-dimensional
(3D) echocardiography or research of myocardial deformation (strain). The M-mode
has been used since the 1950s for cardiac structural analysis and provides
widely standardized measurements
1,2
of the
dimensions of the cavity and the thickness of the LV. Thus, parameters of
ventricular systole analysis are derived as: (1) percentage of systolic
shortening of the left ventricular dimension, represented by the difference
between the final diastolic and the final systolic dimensions, divided by the
final diastolic one; (2) mean corrected circumferential shortening velocity,
corresponding to the ratio between the percentage of systolic shortening of the
left ventricular dimension divided by the ejection time corrected by the
preceding R-R interval (ejection time divided by the square root of the R-R
interval); (3) ventricular volumes at the end of systole and diastole,
calculated from the method by Teichholz et al.;
3
and (4) LV ejection fraction (LVEF), obtained
from the difference between the diastolic and systolic ventricular volumes
(volume ejected by systole), divided by the diastolic volume. The M-mode
analysis is highly reproducible and presents high temporal resolution for the
analysis of ventricles without spatial deformation.
4
However, M-mode measurements, in general,
adequately determine global systolic function only when there are no segmental
changes, remodeling and/or geometric alterations of the LV.
4
With the advent of 2D
echocardiography, a greater amplitude of the LV spatial observation was
acquired, allowing better analysis of the left ventricular systolic function,
when compared to the one-dimensional analysis. This occurs in situations where
there are changes in left ventricular geometry, such as apical aneurysm and
other segmental changes resulting from coronary artery disease. The analysis of
2D left ventricular systolic function can be performed qualitatively (visual
estimation) or quantitatively of LVEF. Visual estimation is highly dependent on
the training of the operator, which can result in inaccuracy of reproduction of
results. The quantitative method for volume and systolic function analysis of
the most widespread and widely valid 2D-LV is the biplanar disc technique
(modified Simpson’s rule), in which the total volume is calculated based on the
sum of the volumes of small cylindrical discs in apical cuts 4 and 2 LV
chambers, with the intention of minimizing the effects of modifying the
ventricular geometry in LVEF calculation.
4
Normal volume and LVEF values, calculated using 2D
echocardiography, show different values depending on gender. Thus, for men, the
final LV diastolic volume is between 34 and 74 mL/m2, the final LV
systolic volume is between 11 and 31 mL/m2 and LVEF is between 52 and
72%; LV final diastolic volume is between 29 and 61 mL/m2, LV final
systolic volume is between 8 and 24 mL/m2 and LVEF is between 54 and
74%.
4
The 2D LVEF
analysis may present inaccuracies during the shortening of LV (foreshortening)
or inadequate acoustic window, and when there are coexistent geometric changes
in the apical 4 and 2 LV chambers.
4
The analysis of LV segmental contractility by 2D
echocardiography represents a semiquantitative technique to assess regional
systolic function, which has shown good application in clinical practice,
especially for stress echocardiography (calculus of parietal motility index,
which integrates analysis of ventricular wall thickening and ventricular segment
contractility). Tissue Doppler, a technique used in the analysis of diastolic
function, can also be used to assess global and segmental LV systolic function.
The systolic velocity of the ventricular myocardium (s-wave), when measured in
the region of the mitral annulus, reflects the longitudinal myocardial systolic
shortening and may be reduced early in patients with diastolic dysfunction and
normal ejection fraction.
5
This
method may also be useful for ventricular synchrony analysis and as a complement
in stress echocardiography, although it does not allow adequate evaluation of
systolic function in the apical LV segments and depends on the angle of
incidence of the ultrasound beam.
3D echocardiography represented an improvement over 2D echocardiographic
observation of LV function, once it does not present the limitations of the 2D
analysis in ventricles with altered geometry, in addition to being more
reproducible and better correlated with the gold standard provided by nuclear
magnetic resonance.
4,6-11
Most recent algorithms allow the calculation of LVEF
and LV volumes in a semi-automatic way, with great correspondence with the
analysis performed by nuclear magnetic resonance.
12
However, 3D echocardiography presents
difficulties in relation to low temporal resolution and to the dependence on
transthoracic echocardiographic image quality.
4
The understanding and analysis of LV mechanics and systolic function can also be
determined by the measurement of ventricular strain. Strain is defined as the
modification of length of the myocardial segment (in %), considering the
different spatial arrangements of the myocardial fibers. In this way, the
longitudinal, circumferential and radial strain (for the respective
longitudinal, circumferential and radial myocardial fibers) is calculated. The
strain can be calculated for each of the LV segments or for all segments (global
LV strain).
13,14
The analysis of the cardiac mechanics can be
performed from parameters derived from myocardial deformation, such as twist,
torsion and LV rotation. In order to obtain ventricular strain, the most
commonly used technique takes into account the movement of gray points in the
myocardium during the cardiac cycle (speckle tracking technique).
13,14
Global 2D strain has the advantages of not depending on
the angle of the ultrasound beam (as in tissue Doppler) and of presenting an
independent prognostic LVEF value.
4
However, it does not yet present a standardized value of
normality among the different manufacturers of echocardiography
equipment.
4
The LV
global longitudinal strain (GLS) has been the one most commonly used in
practice, which is useful for the detection of subclinical myocardial
dysfunction, even when LVEF is preserved; for example: evaluation of
cardiotoxicity after the use of chemotherapy for antineoplastic treatment,
rejection after heart transplantation, severe aortic stenosis, hypertrophic
cardiomyopathy and myocardium infiltrative diseases.
15-19
2.2. Left Ventricular Diastolic Function
The evaluation of LV diastolic function is an integral part of routine
echocardiography analysis, especially in patients with dyspnea or suspected
heart insufficiency.
20,21
Furthermore, in several
cardiopathies, the diastolic dysfunction precedes the systolic one. Diastolic
dysfunction is usually the result of altered relaxation, with or without
reduction of restorative forces (early diastolic suction), and increased LV
stiffness, leading to elevated LV filling pressures.
20
When pulmonary capillary pressure exceeds 12
mmHg, or final diastolic LV pressure exceeds 16 mmHg, filling pressures are
considered high.
21
Elevation
of filling pressures occurs as a compensatory response to maintain adequate
cardiac output, and its estimation is important not only for the diagnosis of
cardiac insufficiency but also for the definition of its severity and response
to treatment.
21
It is
recommended that the non-invasive analysis of diastolic function be performed by
the integrated approach of several techniques, the most important ones being:
Pulsatile Doppler of the mitral flow, tissue Doppler of the mitral valve
annulus, left atrial volume (LA) indexed by body surface and tricuspid
regurgitation velocity.
20
Pulmonary venous flow and Valsalva maneuver can be used as additional parameters
in specific cases, which are useful in differentiating the degrees of diastolic
dysfunction.
20
While
pulsatile and tissue Doppler velocities reflect the instantaneous filling
pressures of the LV, the measurement of the LA volume reflects the cumulative
effect of filling pressures over time and, therefore, this index is the chronic
expression of diastolic dysfunction.
22
However, it is important that other causes of LA
enlargement are discarded and that this data is taken into consideration along
with the patient’s clinical condition, chamber size and Doppler indices for the
evaluation of diastolic function.
In individuals with preserved systolic function and without structural heart
disease, diastolic dysfunction is considered if there is a change of more than
50% of the following 4 parameters: relationship between the early diastolic
velocity of mitral inflow (E) and the early diastolic velocity of the mitral
annulus (e’) E/e’ mean > 14; septal e´ velocity’ < 7 cm/s or lateral <
10 cm/s; tricuspid regurgitation velocity > 2.8 cm/s and LA indexed volume
> 34 mL/m.
20,21
For the group of patients with
systolic dysfunction and those with preserved systolic function concomitant with
the presence of cardiac disease (clinical or echocardiographic manifestation),
the integrated use of the information allows us, in most cases, to estimate the
ventricular filling pressures and the graduation of diastolic
dysfunction.
20
Three
patterns of diastolic dysfunction are defined, in ascending order of severity:
grade I (abnormal ventricular relaxation without increase of filling pressures);
grade II (elevation of filling pressures coexisting with altered relaxation,
usually presenting “pseudonormal pattern” of the mitral flow); and grade III
(very high filling pressures, accompanied by a restrictive pattern of the mitral
flow). To define the presence of increased filling pressures in this group with
heart disease, we must first analyze the mitral flow, before other parameters.
The relationship between E and the atrial diastolic velocity of the mitral
inflow (A) E/A ≤ 0.8 (with E-wave ≤ 50 cm/s) is compatible with
normal filling pressures and isolated abnormal relaxation, while the relation
E/A ≥ 2 is consistent with elevated filling pressures. However, for cases
with an E/A > 0.8 and < 2, an abnormalityof at least 2 of the following 3
parameters is required: E/e’; tricuspid refurgitation velocity; and LA indexed
volume. In some cases, the definition criteria for diastolic dysfunction are not
completely fulfilled, and thus the degree of diastolic dysfunction can be
reported as indeterminate.
20
This algorithm for the evaluation of diastolic dysfunction from the
echocardiography has recently been validated in a multicenter study which
assessed patients with and without left ventricular systolic
dysfunction.
23
Non-invasive evaluation of filling pressures by echocardiography correlated with
the diastolic pressures measured by catheterization, showing greater accuracy
than isolated clinical parameters.
23
It should be noted that the parameters for evaluation of diastolic function may
present important limitations in specific situations, such as hypertrophic
cardiomyopathy, mitral annular calcification, severe mitral regurgitation,
cardiac transplantation and cardiac arrhythmias.
20
Some patients, even with grade I diastolic
dysfunction defined at rest, become symptomatic only during exercise and
therefore it may be useful to analyze filling pressures during physical stress
(diastolic stress echocardiography).
20,24
Patients with
diastolic dysfunction are unable to increase ventricular relaxation with
exercise, when compared to normal subjects, with increased filling pressures,
which can be identified by increased E/e’ ratio and tricuspid regurgitation
velocity.
24
In normal
patients, velocities of E and e’ increase proportionally and the index remains
constant. Finally, the evaluation of diastolic function using techniques derived
from strain and strain rate is promising, but requires further studies to
establish its additional clinical value.
20
2.3. Cardiomyopathies
Cardiomyopathies are a heterogeneous group of myocardial diseases associated with
mechanical and/or electrical dysfunction, which usually exhibit inappropriate
ventricular hypertrophy or dilatation, due to a variety of causes, often
genetic.
25
Cardiomyopathies are confined to the heart or are part of generalized systemic
disorders. The classification is based on functional or structural changes in
the following subtypes: dilated, hypertrophic, restrictive, and arrhythmogenic
cardiomyopathy (or dysplasia) of the right ventricle (RV), more recently
referred to as arrhythmogenic cardiomyopathy.
26
Subsequently, as the knowledge on the genetics
foundations of cardiomyopathies developed, other classifications have been
proposed, subdivided into genetic, acquired and mixed.
26
More recently, channelopathies and related
disorders, such as long and short QT syndrome, Brugada syndrome and
catecholaminergic polymorphic ventricular tachycardia, have been included in the
group of cardiomyopathies, since they are cardiomyocyte diseases characterized
by arrhythmogenic electrophysiological dysfunction.
25,26
2.3.1. Dilated Cardiomyopathy
It is characterized by LV dilation associated with global systolic
dysfunction, in the absence of volume or pressure overload. The prevalence
of dilated cardiomyopathy (DCM) is variable, reflecting the geographical and
ethnic differences, as well as the methodologies used. A prevalence of 1:250
is estimated, based on the frequency of the left ventricular dysfunction as
an expression of DCM.
27
The criterion to define LV dilation is the final diastolic diameter > 2.7
cm/m2. With increased gradual dilatation on the short axis,
the LV cavity becomes spherical, with sphericity index (long/short axis
dimension) close to 1 (normal value > 1.5).
28
Wall thickness is usually normal, but the
myocardial mass is increased. The degree of impairment of systolic function
is variable, and systolic dysfunction is often progressive. LV volumes are
calculated in a more reproducible and accurate way by using the 3D
echocardiography. Abnormalities associated with diastolic function may be
present, contributing to the variation in the clinical and hemodynamic
presentation of DCM. The involvement of the RV can be evidenced, but it is
not a criterion for the diagnosis of DCM.
29
Notably, DCM is associated with an
increased risk of severe arrhythmia, indicating the pathological involvement
of the cardiac conduction system. Complex remodeling of one or both
ventricles contributes to the secondary features of DCM, which include
functional mitral and tricuspid regurgitation, enlarged atria, intracavitary
thrombi, and evidence of low cardiac output.
28
In the context of DCM, the analysis of
diastolic function aims to estimate the filling pressures; and the mitral
flow pattern is usually enough to identify patients with increased LA
pressure. E-wave deceleration time is an important predictor of outcomes in
these patients.
30
Other
diastolic dysfunction parameters, including the E/e’ ratio, have good
correlation with pulmonary capillary pressure and have an additional
prognostic value for LVEF.
30
Echocardiography is the imaging method of choice for the evaluation of DCM
patients, providing key data not only for diagnosis, risk stratification and
treatment definition, but also plays a key role in the evaluation of family
members.
28
Key
echocardiography indications in the evaluation of DCM are displayed in table 1. Transthoracic
echocardiography
(TTE) is indicated in the initial evaluation of patients with heart failure
and suspected DCM. TTE is recommended in first-degree relatives of DCM
patients due to the high incidence (20 to 50%) of familial DCM.
28
Several echocardiographic
parameters were used to assess mechanical desynchrony in patients with DCM.
However, the broader role of echocardiography in the selection of patients
for cardiac resynchronization therapy remains undefined. Currently,
echocardiographies are limited to patients with borderline QRS duration (120
to 149 ms), whose presence of intra- or interventricular desynchrony may
provide additional information.
28
The echocardiography guiding the placement of
electrodes at the site of greater mechanical activation delay (evaluation by
speckle tracking) showed benefit in heart failure free survival, with a more
favorable impact on ischemic heart disease compared to DCM.
31
Table 1
Recommendation of echocardiographies in dilated cardiomyopathies
Recommendation
Class of recommendation
Level of evidence
Assessment of patients with suspected dilated
cardiomyopathy or heart failure
I
C
Assessment of signs and symptoms suggestive of
myocardial dysfunction
I
C
Reassessment of patients with cardiomyopathy
known to present worsening of symptoms or to require changes
in therapy
I
C
First-degree relatives of patients with dilated
cardiomyopathy
I
B
Assessment of candidate patients for cardiac
resynchronization therapy with LBBB and QRS duration between
120 and 149 ms
IIa
C
Reassessment of routine in patients with stable
dilated cardiomyopathy, without clinical or therapeutic
changes
III
C
Chagasic cardiomyopathy
Initial evaluation of patients with positive
serology for Chagas disease for diagnosis and risk
stratification of cardiomyopathy
I
C
Patients with the indeterminate form of Chagas
disease who present new electrocardiographic alterations
compatible with the development of cardiomyopathy
I
C
Patients who present worsening symptoms of
heart failure, syncope, arrhythmic or thromboembolic
events
I
C
Routine reassessment of clinically stable
patients with no changes in therapy
III
C
LBBB: left bundle branch block
2.3.2. Chagasic Dilated Cardiomyopathy
Chagasic dilated cardiomyopathy (CCM) presents similar characteristics to
idiopathic DCM, but with predominance of segmental changes in contractility,
especially in the basal segments of the inferior and inferolateral
walls.
32
Apical
aneurysm is a typical CCM finding and is useful in the differential
diagnosis of dilated cardiomyopathies.
33
The morphology of aneurysms is variable and
non-standard sections are often required for the identification of apical
contractile changes. The presence of thrombi within the aneurysms is
frequent and associated with cerebral thromboembolic events.
34
Diastolic dysfunction is
universally present in patients with CCM and heart failure.
35
The main echocardiographic
parameters previously studied with a prognostic value in CCM are LVEF, right
ventricular function, LA volume and E/e’ ratio.
33,36
The contractile function of the LA evaluated by the negative peak of the
global atrial strain was an independent predictor of clinical events in the
CCM.
35
The
heterogeneity of systolic contraction, quantified by mechanical dispersion
to speckle tracking, was associated with ventricular arrhythmias in patients
with CCM, regardless of LVEF.
37
Recommendations for performing TTE in CCM are set out
in table 1.
38
2.3.3. Cardiac Resynchronization Therapy and Pacemaker
Optimization
Cardiac resynchronization therapy is an established treatment option for
patients with heart failure with marked reduction of LVEF. The
echocardiography is fundamental in the indication, estimation of success and
evaluation of the results of this procedure and may also contribute to the
recovery of unfavorable results. This treatment is indicated as class I in
patients with heart failure (New York Heart Association (NYHA) functional
class II, III or IV), with LVEF lower than 35%, optimized medication and
left bundle branch block with QRS duration above 150 ms.
28
Also in class IIa and IIb
indications, it is necessary to recognize the reduction of the LVEF below
35%, being contraindicated when this value is not present. Therefore, in the
possibility of indication for cardiac resynchronization therapy,
transthoracic echocardiography is a class I indication, level of evidence C.
In this examination, it is mandatory that LVEF be obtained by the Simpson 2D
method, with a description, in the report, of their volumes. It is also
possible to use the 3D methodology, of less variability, although still
unproven in this clinical scenario. Approximately 30% of patients do not
present clinical improvement or significant reduction in LV final systolic
volume.
39
TTE can
provide information that helps identify a greater probability of successful
treatment response, such as the presence of mechanical, inter- and
intraventricular desynchrony, the presence of myocardial reserve and
determination of the last site of activation, which may be associated with
higher degree of fibrosis. To this end, the use of a variety of methods is
encouraged, from the visual evaluation of 2D echocardiography,
M-mode,
40
tissue
Doppler and, especially, the use of a technique that evaluates
longitudinal
41
or
radial myocardial deformation.
42,43
In the
evaluation of successful response to treatment, it is expected, in regards
to imaging, mainly negative remodeling to be observed, usually characterized
by reduction of 15% of the initial systolic volume, analyzed between 3 and 6
months of the implant.
39,44
In case the negative
remodeling and/or the clinical improvement of the patient is not met, one
possibility is to adjust the pacemaker, guided by TTE, to optimize the
atrial and ventricular stimulus intervals. The main correction in this case
seems to be adjustment of the atrial and ventricular stimulus intervals,
guided by the echocardiography, which allows for retrieval of the
results.
45,46
2.3.4. Assessment after Heart Transplantation
The echocardiography is the main noninvasive imaging modality as well as the
most versatile one in the evaluation and monitoring of patients after
cardiac transplantation, providing accurate information on morphology and
graft function. From the immediate postoperative period up to the moment of
hospital discharge, serial echocardiographic exams are recommended both to
identify and monitor surgical complications and early graft dysfunction,
whether due to primary or secondary causes (e.g., reperfusion injury,
non-responsive pulmonary hypertension (class I, level B).
47,48
In the presence of early graft dysfunction, the
echocardiography usually shows an overall reduction in myocardial function
(LVEF < 45%), loss of the contractile reserve, increase in RV volume with
systolic dysfunction (tricuspid annular plane systolic excursion - TAPSE
< 15 mm or RV ejection fraction < 45%).
47
A comprehensive echocardiographic
examination (class I, level B) is recommended in the sixth month after
cardiac transplantation), which will serve as the baseline for assessing
graft morphology and function during sequential and regular follow-up
examinations (interval and frequency of exams in figure 1).
47
Quantifications of cardiac chambers size and volumes,
RV systolic function, LV diastolic and systolic parameters, and pulmonary
arterial pressure should be performed on the sixth month and subsequent
echocardiographies.
47
It is recommended that such echocardiographic studies
also include advanced methodologies, such as the study of myocardial
deformation (strain) and 3D evaluation of the volumes and function of
cardiac chambers and tricuspid valve (frequently injured during the
endomyocardial biopsy procedure), for providing a more accurate and
comprehensive analysis (class I, level B).
47
Figure 1
Echocardiographic evaluation after cardiac transplantation.
*Graft dysfunction: confirmed on echocardiographic
examination by dropping the ejection fraction by more than 10%
to a value lower than 50%, compared to the baseline examination
of the sixth month; §patients with graft
dysfunction suspected or confirmed; clinical symptoms of a
possible new cardiac abnormality; alterations in the resting
electrocardiogram.
It should be noted that there is no single isolated echocardiographic
parameter that can be reliably used to diagnose acute rejection.
47
However, an
echocardiographic study with no change from the baseline study has a high
negative predictive value for acute rejection of the graft. On the other
hand, if several echocardiographic parameters are abnormal, the probability
of acute rejection of the graft increases considerably.
47
When an abnormality is
detected, a careful review of the images of the present study and the
baseline study (side-by-side) is highly recommended (class I, level
B).
47
GLS is an
adequate parameter to assist in the subclinical diagnosis of graft
dysfunction, regardless of etiology, in addition to an adverse event
predictor, when comparing the variations of values occurred during serial
evaluations (class IIb, level B).
47,49,50
The association of GLS
with endomyocardial biopsy helps to characterize and monitor an episode of
acute rejection or global dysfunction.
47
Pericardial effusion should be serially assessed
for extent, location and hemodynamic impact (class IIb, level B). In the
case of a recently detected pericardial effusion, the hypothesis of acute
rejection should be considered, taking into account the patient’s global and
clinical echocardiographic evaluation.
47,51
Cardiac
graft vascular disease is the main cause of late complication; and the
dobutamine stress echocardiography has proven to be a safe and accurate
method to identify the affected patients.
47,52-54
The evaluation of the
coronary reserve flow, as well as the sonographic contrast infusion to
highlight the borders and to evaluate the myocardial perfusion , when
combined with stress echocardiography, have been shown to increase the
accuracy of the diagnosis of graft vascular disease.
55-59
Thus, dobutamine stress echocardiography alone
(class IIA, level B) or in association with the evaluation of the flow of
coronary reserve and/or with the use of sonographic contrast (class I, level
B) may be an adequate noninvasive alternative to routine coronary
angiography to assess the presence of cardiac graft vasculopathy, provided
that the medical center has good experience with methodologies.
In addition to the role of cardiac graft monitoring, intraoperative
echocardiography can be used as an alternative to fluoroscopy to guide
endomyocardial biopsies, avoiding repeated exposure to X-rays, particularly
in children and young women (class I, level B). Whether in transthoracic or
transesophageal mode, the echocardiography allows a simultaneous
visualization of the soft tissues and the biotope, guaranteeing greater
biopsy safety in different regions of the RV with a reduction in the
complication rate.
47,60
Furthermore, the use of
echocardiography during the procedure allows immediate recognition and
management of a possible complication.
2.3.5. Monitoring of Cardiac Function During Chemotherapy with
Cardiotoxic Drugs
Current cancer therapy is quite effective in some types of tumors, though it
can induce cardiovascular complications. Cardiotoxicity (CT) induced by
cancer treatment is recognized as the major cause of morbidity and mortality
in cancer survivors.
61
Before starting anti-neoplastic treatment, it is essential to access the
risk of CT,
62
taking into
consideration: (a) the specific risk of the drug used in chemotherapy, as
some of them may affect the cardiac function (anthracyclines, trastuzumab),
while other ones, the vascular function (5-fluoracil, capecitabine), or both
(bevacizumab); (b) the use of radiotherapy, as it increases the risk of
heart failure when concomitant with anthracyclines, pericardial lesion
(constrictive pericarditis) and coronary artery disease; (c) the presence of
previous risk factors, such as age > 65 years, female gender,
hypertension, diabetes mellitus, coronary artery disease and history of
heart failure. All patients receiving potentially cardiotoxic drugs should
be periodically monitored for CT signs, which can be classified according to
the injury the drug used produces.
63
CT Type I, potentially irreversible,
anthracycline-related dose, is dose-dependent, mainly at > 250 to 300
mg/m2 (often used in the treatment of breast cancer,
lymphoma, leukemia, and sarcoma). It most commonly occurs in the first year
of chemotherapy, or even two to three decades after completion of treatment,
as progressive systolic dysfunction. It may rarely present as an acute
systolic dysfunction, immediately after dosing. Type II CT, which is
potentially reversible, mainly related to trastuzumab (used in the treatment
of breast cancer in patients with increased HER2 receptor expression), has
no relation to the cumulative dose.
63
Such information is the basis for the algorithms of
serial left ventricular function monitoring during and after treatment of
cancer patients, published by the European Association of Cardiovascular
Imaging (EACVI) and the American Society of Echocardiography
(ASE).
3
The most
historically used parameter is LVEF, calculated by TTE using the Simpson
biplanar 2D method.
4
LVEF
values between 53 and 73% should be considered normal in the evaluation.
The main advantages of 2D TTE in relation to other imaging modalities, such
as radioisotope ventriculography and magnetic resonance imaging (MRI), are:
greater availability, lower cost, possibility of serial re-evaluations and
greater safety (absence of radiation and limitation in patients with renal
insufficiency). 3D TTE, used in sequential and comparative MRI evaluations
for LVEF assessment, showed reproducibility comparable to MRI and better
accuracy than 2D TTE,
64
being more indicated, when available, in the serial evaluation of these
patients.
65
The definition of CT due to chemotherapy was defined by the consensus of
these two societies
3
as the
decrease of LVEF > 10 percentage points to values < 53% and should be
confirmed after 2 to 3 weeks of diagnosis by new imaging. This decrease may
or may not be accompanied by symptoms of heart failure and may or may not be
reversible. One of the major limitations of the use of LVEF for CT diagnosis
in the follow-up of these patients is that changes in LVEF occur later. In
order to minimize the risk of developing irreversible cardiomyopathy, it is
essential to identify early signs of CT, since the administration of
cardioprotective medication in this phase may result in an improvement in
cardiac function.
66
Thus,
the search for a technique that allows subclinical and early detection of CT
before LVEF decrease or the onset of clinical symptoms has been an area of
intense investigation. In this scenario, the use of GLS gained importance,
evaluating myocardial deformation. Such technique has inter- and
intraobserver reproducibilities smaller than the LVEF obtained by the 2D
TTE, but is limited by the variability of normal values according to the
brand of the equipment used, age and gender of the patients.
67
Systematic review
confirmed the prognostic value of the alterations in GLS for CT, preceding
the LVEF decrease obtained by 2D or 3D TTE.
15
The consensus recommends serial GLS
evaluation in patients at risk of CT, with subclinical left ventricular
dysfunction suggestive of a fall of > 15% of the baseline value, even
without LVEF change.
63
The
relative decrease between 8 and 15% suggests a more rigorous follow-up. GLS
Variation of < 8% is consistent with absence of subclinical
dysfunction.
63
Although some studies have drawn attention to changes in diastolic function
following chemotherapy,68 there is no current evidence to support such
parameters as indicative of CT.
63
A use of biomarkers in the assessment integrated
with imaging methods in chemotherapy patients evidenced the importance of
troponin I (TnI) with a high negative predictive value in the detection of
CT.
69
It is
probable that patients who do not evolve with TnI elevation have lower
probability of events and perhaps less need for imaging tests in subsequent
evaluations.
69,70
There is still no robust
scientific evidence based on randomized clinical trials to support the
algorithms proposed by the European Society of Oncology
71
and the consensus of EACVI
and ASE,
63
in the
follow-up of these patients; however, these documents represent current
knowledge in the area. The orientation of the EACVI - ASE
consensus
63
to the
present moment is:
Initial evaluation of left ventricular function before the start
of chemotherapy in patients who will use potentially cardiotoxic
chemotherapeutics. If it is not possible in all patients, it is
recommended in those at high risk for the development of CT: age
> 65 years, previous left ventricular dysfunction, predicted
use of high doses of anthracyclines (type I) or combination of
type I and II drugs. To perform the LVEF assessment by the 3D
TTE if available or, alternatively, by the 2D TTE (Simpson
method). It is desirable that the evaluation by GLS and TnI is
carried out. If not possible to perform GLS, report the S-wave
of the medial and lateral tissue Doppler of the mitral annulus.
Further monitoring of left ventricular function is recommended
after this initial evaluation, depending on the chemotherapy to
be initiated.
Type I drugs (anthracyclines): evaluate left ventricular function
(2D/3D LVEF and GLS) at the end of chemotherapy and after 6
months at dose < 240 mg/m2. For doses > 240
mg/m2, evaluate left ventricular function before
each additional 50 mg/m2 cycle at the end of
chemotherapy and after 6 months.
Type II drugs (trastuzumab): evaluate left ventricular function
every three months during chemotherapy.
Patients receiving trastuzumab following anthracycline treatment:
assess left ventricular function every three months during
chemotherapy and six months after its completion.
2.3.6. Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is a genetic cardiovascular disease
characterized by increased left ventricular wall thickness ≥ 15 mm in
adults, with non-dilated ventricular cavity not explained by abnormal
loading conditions, such as arterial hypertension or valve aortic
stenosis.
72
Minor
hypertrophy degrees (13 to 14 mm) may also diagnose HCM, particularly among
relatives of these patients. TTE is considered the initial imaging for
diagnosis, stratification of the risk of cardiac events and management of
patients with HCM. Among the parameters to be evaluated in HCM are: location
and hypertrophy degree; identification of obstruction and intraventricular
gradient at rest or intentionally caused; presence of magnitude of mitral
reflux; systolic and diastolic function; and LA size. Any hypertrophy
pattern may be found, though the asymmetric is the most frequent one (75% of
cases), and it is more common at the confluence of the anterior
interventricular septum with the LV free wall.
73
Other forms of hypertrophy are: basal,
concentric, apical and lateral wall. There is a linear association between
maximum myocardial thickness and sudden death, with greater risk in patients
with thickness ≥ 30 mm.
72,74
Gradual
identification of the LV outflow tract is important in the management o
symptoms and in the stratification of the risk of sudden death.
72
TTE evaluation generally
characterizes the presence of LV outflow tract obstruction (instantaneous
gradient ≥ 30 mmHg) at rest (one third of patients) of after
provocative maneuvers (one third), such as exercises (echocardiography under
physical stress) or Valsalva maneuver. Echocardiography under physical
stress can be very useful in patients with HCM, since in addition to
detecting the presence and degree of obstruction during effort, it allows
the objective evaluation of symptoms, functional capacity, systolic blood
pressure response and the presence of secondary mitral regurgitation.
Approximately 25% of HCM patients have abnormal blood pressure response
during exercise, characterized by a drop in systolic pressure or by failure
to increase in > 20 mmHg. This finding has been interpreted as a risk
factor for unfavorable prognosis and sudden death.
75
Stress echocardiography with dobutamine is
not recommended. The cut-off value of the intraventricular gradient ≥
50 mmHg at rest or after provocative maneuvers is considered when indicating
surgical treatment or percutaneous intervention in symptomatic patients,
despite therapy with optimized medication.
72
Patients with HCM in general have
diastolic dysfunction, commonly with altered relaxation (grade I), though
without significant correlation between mitral flow data and LV filling
pressures. Thus, the integrated approach of mitral Doppler data, tissue
Doppler, pulmonary vein flow and LA volume is recommended in these
patients.
76
LVEF
is normal or increased in most patients, giving the false impression of
preserved systolic function. However, longitudinal strain assessment
invariably shows a global and regional decrease (coincident with sites of
greater hypertrophy) of contractility.
77
Estimating the size of the LA is fundamental, as
there is a significant correlation between the dilation of the chamber and
an increased risk of cardiovascular events, such as atrial fibrillation and
sudden death. The main complication of HCM is sudden cardiac death (SCD),
especially in young and apparently healthy individuals.
78,79
Cardiac defibrillator implantation for primary or
secondary prophylaxis may reduce mortality from this complication and is the
only therapy with evidence of life-saving potential.
80
TTE has a relevant role in
the two most commonly used risk stratification scores form SCD in HCM, which
determined the relationship between come clinical risk factors and
prognosis. In the American model of primary prevention, one of the risk
factors among 5 variables is the presence of interventricular septum
thickness ≥ 30 mm.
75
In the European model, of the seven variables analyzed,
three of them are provided by TTE: septum thickness, LA and left ventricular
outflow tract (LVOT) gradient at rest or after Valsalva maneuver.
72
Family screening of
first-degree relatives of HCM subjects should be performed periodically due
to their risk of developing the disease. Recommendations for the use of
echocardiography in HCM are summarized in table 2.
Table 2
Recommendations of transthoracic echocardiography, echocardiography
under physical stress and transesophageal echocardiography in
hypertrophic cardiomyopathy
72,75
Recommendation
Class of recommendation
Level of evidence
TTE in the initial assessment of all patients
with suspected HCM, at rest and during Valsalva
maneuver
I
B
EPS in symptomatic patients with resting or
Valsalva intraventricular gradient < 50 mmHg to assess
the degree of dynamic obstruction and mitral regurgitation
during exercise
I
B
Reassessment by TTE when there are changes in
symptoms or a new cardiovascular event
I
B
TTE in the assessment of therapeutic results of
pharmacological, surgical (myomectomy), interventional
(alcoholic septal artery occlusion) and pacemaker
treatments
I
C
TTE in the screening of first-degree relatives
with HCM diagnosis
I
B
Serial TTE (every 12 to 18 months) in children
of HCM patients, starting at age 12 (or earlier, if there is
intention to take on competitive sports or sudden death
among relatives)
I
C
TTE during alcohol septal artery ablation
I
B
TEE in intraoperative myectomy monitoring and
during alcoholic occlusion of the septal artery with
inadequate TTE
I
B
Serial TTE every one to two years may be useful
in stable symptomatic patients to reassess myocardial
hypertrophy, dynamic obstruction, and ventricular
function
IIa
C
TEE may be useful when TTE is inconclusive, in
the planning of myomectomy or in the evaluation of mitral
regurgitation secondary to mitral valve abnormalities
IIa
C
Serial TTE (every five years) is reasonable in
periodic reassessment in first-degree relatives of adult HCM
patients
IIa
C
TTE combined with intravenous contrast
injection is reasonable if the diagnosis of apical HCM
and/or apical infarction are doubtful, or the quantification
of hypertrophy is inadequate, especially if MRI is
unavailable, non-diagnostic or contraindicated
IIa
C
EPS may be useful in asymptomatic HC patients,
with no dynamic obstruction at rest when gradient detection
in LVOT is relevant for lifestyle or career change
orientation, or decision making on medical treatment
IIb
C
TTE should not be performed in less than 12
months in HCM patients when there is no change in symptoms
or predicted conduct change
III
C
TTE: transthoracic echocardiography; HCM: hypertrophic
cardiomyopathy; EPS: echocardiography under physical stress;
TEE: transesophageal echocardiography; LVOT: left ventricular
outflow tract
2.3.7. Restrictive Cardiomyopathies
Restrictive cardiomyopathies (RCM) are a group of entities characterized by
abnormalities in the ventricular filling pattern, which may be associated
with thickened and rigid walls and generally preserved systolic function.
RCMs comprise various entities, including, endomyocardial fibrosis (EMF),
endomyocardial fibroelastosis, Löefler parietal endocarditis,
infiltrative (such as amyloidosis and sarcoidosis), storage (such as
hemochromatosis and Fabry disease), idiopathic and other forms secondary to
different processes (scleroderma, carcinoid syndrome, metastases of systemic
neoplasms, anthracycline toxicity and irradiation heart disease).
81
Diagnosis by
echocardiography is based on common anatomical and functional changes:
ventricular cavities of normal or reduced size, usually with Doppler degree
III diastolic function (restrictive type), generally preserved overall
systolic function, and dilated atria. Tissue Doppler analysis shows velocity
e’ obtained in the septal mitral annulus usually below 7.0 cm/s, a useful
measure in constrictive pericarditis differentiation.
82
In amyloidosis, there is
thickening of the atrioventricular valves, myocardial walls, and eventually
the atrial septum, with a more intense (ecorrefringence) reflection and
“granular and sparkling” myocardial aspect.
83
The GLS analysis of the LV with 2D
echocardiography in amyloidosis observes very low values, especially in the
mid and basal segments with relative “apical sparing” (it aids in the
differential diagnosis with other diseases).
84
In EMF, one may observe: obliteration of
the apex by fibrosis, signs of ventricular restriction, and involvement of
the atrioventricular valves. The fibrosis of the apical thrombi is
differentiated due to absence of akinesia or dyskinesia in the left EMF.
Another differential diagnosis is apical HCM, which presents no endocardial
thickening or restrictive pattern and displays specific electrocardiographic
changes. Cardiac sarcoidosis may present with regional contractile
abnormalities and non-ischemic distribution aneurysms. GLS measure
represents an early marker of myocardial involvement in sarcoidosis and the
magnitude of the reduction is associated with poor prognosis.
85
The recommendations for
TTE in RCM are set out in table 3.
Transesophageal echocardiography (TEE) is indicated when there are technical
difficulties to TTE and in the transoperative monitoring of fibrosis and
apical correction of valve defects.
Table 3
Recommendations of echocardiography in restrictive
cardiomyopathies
Recommendation
Class of recommendation
Level of evidence
Diagnostic investigation of patients with heart
failure without clear etiology
I
C
Differential diagnosis of patients with
restrictive syndrome
I
C
Symptomatic patients with systemic diseases
potentially causing RCM
I
C
Patients with hypereosinophilic syndrome,
ascites and distended jugular veins
I
C
Patients with ascites and lower limb edema,
without established diagnosis
I
C
Patients submitted to radiotherapy with signs
of systemic venous hypertension
I
C
Reassessment of patients with previous RCM
diagnosis when there is a change in the clinical course
of the disease
I
C
Patients with EMF for therapeutic planning and
prognostic evaluation
IIa
C
Patients with edema and ascites, with evidence
of normal systemic venous pressure and no evidence
of cardiopathy
III
C
RCM: restrictive cardiomyopathies; EMF: endomyocardial
fibrosis.
2.3.8. Arrhythmogenic Cardiomyopathy (Right Ventricle Arrhythmogenic
Dysplasia)
Arrhythmogenic cardiomyopathy (AC) is considered an inherited cardiomyopathy
with autosomal dominant transmission, predisposing to the emergence of
ventricular arrythmias, sudden death in young people, ventricular
dysfunction and heart failure. Due to the frequent involvement of the LV,
the use of the term CA is currently recommended, which comprises both
ventricles, replacing the term “arrhythmogenic dysplasia of the
RV”.
86
The disease
is characterized by a progressive replacement of the ventricular myocardium
by fibrous and adipose tissue, which can lead to thinning of the wall and
aneurysm formation. In the RV, the process is typically located in the
inferior, apical and infundibular walls (dysplasia triangle), and may be
diffuse or segmental. LV involvement occurs in more than half of the cases,
typically located in the subepicardium or mesocardium, and often confined to
the inferolateral segment. Echocardiography is the imaging modality of
choice in the initial assessment of AC (Table 4) and the most commonly used propaedeutic
method for
patient follow-up.
87
Typical morphologic features in patients with CA include regional
contractile abnormalities and/or dilation and right ventricular dysfunction.
Among the traditional echocardiographic criteria, derived from 2D
echocardiography, proposed for the diagnosis of AC,
88
are: presence of akinesia,
dyskinesia or right ventricular aneurysm; increased ventricular outflow
tract diameter (measured on long and short parasternal axis); and reduction
of the fractional variation of the RV area. Recently, the routine and
systematic addition of other echocardiographic measurements and techniques
was recommended in order to improve evaluation:
89
Table 4
Recommendations of the echocardiography in arrhythmogenic
cardiomyopathy
Recommendation
Class of recommendation
Level of evidence
Assessment of patients with suspected AC
I
B
Reassessment of patients with known AC when
there is change of symptoms or new cardiovascular event
I
C
Family screening in first-degree relatives of
AC patients
I
C
Routine re-evaluation of clinically stable
patients with no changes in therapy
III
C
AC: arrhythmogenic cardiomyopathy
Conventional parameters: basal RV diameter (normal ≤ 41
mm); systolic excursion of the annular tricuspid plane (TAPSE -
normal ≥ 17 mm).
Advanced parameters: wave s’ to the tissue Doppler of the RV’s
free wall (normal ≥ 9.5 cm/s); longitudinal strain of the
RV’s free wall (normal ≥ -20%); LV’s GLS (normal ≥
-18%); RV ejection fraction to 3D echocardiography (normal
≥ 45%).
In short, TTE, preferably with analysis of conventional and advanced
parameters, is indicated in patients with suspected or established AC
(evaluation of disease progression), as well as in family screening of
first-degree relatives.
2.3.9. Non-compaction Cardiomyopathy
Non-compaction cardiomyopathy (NCC) is considered a distinct cardiomyopathy,
marked by genetic heterogeneity, with an overlapping of different phenotypes
and great variability of clinical presentation. As a consequence, there is
still controversy in the literature regarding its nomenclature: whereas for
the American Heart Association (AHA)
90
it is considered a primary cardiomyopathy, the
European Society of Cardiology (ESC)
81
considers it an unclassified disorder. Its
pathogenesis implies early interruption of compaction of the trabecular
meshwork of the LV during embryogenesis, resulting in the formation of two
layers: a thin compacted epicardial layer and a thick endocardial one
(similar to a “spongy” mesh) with marked trabeculations and deep
intertrabecular recesses. The 2D echocardiography is the basis for
diagnosis, follow-up, and better delineation of NCC phenotypic
expressions.
91
Several criteria have been employed in diagnosis, taking into account the
increase in the proportion of the non compacted layer (for example, the non
compacted/compacted ratio at the end of systole > 2), presence of
excessive trabeculation, hypokinesia of non compacted areas (commonly
located at the apex and lateral wall) and visualization of flow in the
recess (via color Doppler). New techniques have recently been incorporated
to aid in diagnosis, such as the use of echocardiographic contrast, 3D
echocardiography and myocardial strain for the analysis of regional
deformation and rotation (which assumes a characteristic pattern in this
nosological entity).
92
Therefore, it should be noted that the diagnosis of suspected cases has
increased in recent years, due to advances and improvements in imaging
methods, as well as the perception of the need to actively investigate
first-degree relatives affected by the disease (described in 13 to 50% of
this specific group).
93
On
the other hand, increasingly frequent “exam findings” (physiological versus
pathological variants) have been reported in clinical practice, leading to
the worrisome excess of diagnoses.
7
Therefore, it is recommended to carry out a
comprehensive evaluation, including clinical, electrocardiographic data and
careful analysis of the findings in complementary imaging studies.
94
The recommendations for
performing the echocardiography in NCC are set out in table 5.
Table 5
Recommendations of echocardiography in non-compaction
cardiomyopathy
Recommendation
Class of recommendation
Level of evidence
NCC clinical suspicion
I
C
Reassessment of patients with known NCC when
there is change of symptoms or new cardiovascular
event
I
C
Screening in first-degree relatives of NCC
patients
I
C
Carriers of muscular diseases and/or other
clinical syndromes that may be related
I
C
Use of new techniques such as strain, 3D
echocardiography and echocardiographic contrast for
complementary evaluation and aid in differential
diagnosis
IIa
B
Routine reassessment of clinically stable
patients with no change in therapy
III
C
NCC: non-compaction cardiomyopathy
2.4. Arterial Hypertension and Myocardial Hypertrophy
The elevation of systolic stress in the LV wall, secondary to systemic arterial
hypertension (SAH), can produce myocardial hypertrophy by increasing ventricular
mass.
95
Unlike
physiological hypertrophy (growth, pregnancy and physical activity),
characterized by preserved cardiac structure and function, left ventricular
hypertrophy (LVH), secondary to SAH, is commonly associated with fibrosis,
myocardial dysfunction and increased mortality.
96
The echocardiography is the clinical choice
exam to detect LVH, due to its being more accurate than the
electrocardiogram
97,98
and allowing estimation of LV
mass (LVM). The methodology to measure LVM and to define its cut-off points and
index form (body surface, height, weight) varies between studies. Most
echocardiography authors and laboratories follow the recommendations published
by ASE and EACVI.
95,99
LVM indexing to the body
surface area in g/m2 is the most used one,
100
and normality values are different for men
and women (Table 6).
95,99
Table 6
Degree of abnormalities of left ventricular mass
95,99
Linear method
Female
Male
Normal
Slight increase
Moderate increase
Severe increase
Normal
Mild increase
Moderate increase
Severe increase
LV mass, g
67 to 162
163 to 186
187 to 210
≥ 211
88 to 224
225 to 258
259 to 292
≥ 293
Mass/BS, g/m2
43 to 95
96 to 108
109 to 121
≥ 122
49 to 115
116 to 131
132 to 148
≥ 149
Mass/height, g/m
41 to 99
100 to 115
116 to 128
≥ 129
52 to 126
127 to 144
145 to 162
≥ 163
Mass/height2,7, g/m2,7
18 to 44
45 to 51
52 to 58
≥ 59
20 to 48
49 to 55
56 to 63
≥ 64
RWT (2 x LVPW/LVDD)
0.22 to 0.42
0.43 to 0.47
0.48 to 0.52
≥ 0.53
0.24 to 0.42
0.43 to 0.46
0.47 to 0.51
≥ 0.52
Septum thickness, cm
0.6 to 0.9
1.0 to 1.2
1.3 to 1.5
≥ 1.6
0.6 to 1.0
1.1 to 1.3
1.4 to 1.6
≥ 1.7
LVPW thickness, cm
0.6 to 0.9
1.0 to 1.2
1.3 to 1.5
≥ 1.6
0.6 to 1.0
1.1 to 1.3
1.4 to 1.6
≥ 1.7
2D Method
LV mass, g
66 to 150
151 to 171
172 to 182
≥ 193
96 to 200
201 to 227
228 to 254
≥ 255
Mass/BS, g/m2
44 to 88
89 to 100
101 to 112
≥ 113
50 to 102
103 to 116
117 to 130
≥ 131
LV: left ventricle; BS: body surface; RWT: relative wall thickness;
LVPW: left ventricle posterior wall; LVDD: left ventricle diastolic
diameter; 2D: two-dimensional
Cumulative exposure to elevated blood pressure levels among young adults is
associated with LV systolic dysfunction in mid-life.
101
The presence of LVH is considered as
evidence of target organ damage in hypertensive patients, and its association
with cardiovascular diseases and mortality is well documented.
102-104
Such an increase in cardiovascular risk in
hypertensive patients is directly related to LVM, regardless of blood pressure
values.
103
In
addition to LVM, the geometric pattern of LVH is also seen as an important
variable related to cardiovascular risk. Four patterns of LV geometry
99
are described in (Table 7). The altered geometric patterns
(concentric LVH, eccentric LVH and concentric remodeling) are predictors of
cardiovascular complications in hypertensive patients, with concentric LVH being
associated with higher risk of events.
103
Table 7
Geometric patterns of the left ventricle
99
Left ventricle geometry
Left ventricle mass /body surface
(g/m2)
Left ventricle mass /body surface
(g/m2)
Normal
≤ 115 (men) or≤ 95
(women)
≤ 0.42
Concentric hypertrophy
> 115 (men) or> 95 (women)
> 0.42
Eccentric hypertrophy
> 115 (men) or> 95 (women)
≤ 0.42
Concentric remodeling
≤ 115 (men) or≤ 95
(women)
> 0.42
*Measures taken by the linear method
Another frequent finding in SAH is the presence of LV diastolic
dysfunction.
101
Hypertensive individuals with heart failure commonly present with LVH,
abnormalities in diastolic function and preserved ejection fraction. In these
cases, diastolic dysfunction alone may be responsible for the signs and symptoms
of heart failure.
105
In
addition, E/e’ ratio > 13 is associated with high cardiac risk in
hypertensive patients, regardless of LVM.
106
The use of GLS, obtained by 2D speckle tracking,
allows for the early identification of subclinical systolic dysfunction in
several scenarios, including hypertensive patients without LVH.
107
GLS decline was related to
hospitalization by heart failure, infarction, stroke, and death in patients with
asymptomatic hypertensive heart disease.
108
The regression of LVH in hypertensive patients,
evaluated by serial echocardiographies after therapeutic interventions, is
associated with decreased risk of fatal and non-fatal cardiovascular events,
even in those cases where LVH has not been detected by the
electrocardiogram.
109
This benefit is directly related to the degree of reduction of LVM indexed to
body surface, regardless of ambulatory blood pressure. LVH regression is also
associated with an improvement in LV systolic
110
and diastolic function
111
in hypertensive patients.
The thoracic aorta is more frequently affected by dilatation in hypertensive
patients without adequate blood pressure control than in normotensive and
controlled hypertensive ones.
112
Long-term follow-up has shown that blood pressure levels
are one of the main modifiable factors of adult aortic root
dilatation.
113
The
recommendations for performing the echocardiography in SAH are listed in table 8.
Table 8
Recommendations of the echocardiography in the evaluation of hypertensive
patients
Recommendation
Class of recommendation
Level of evidence
LVH detection
I
A
Assessment of systolic and diastolic function in
hypertensive patients with clinical suspicion of heart
failure
I
A
Hypertensive patients with left bundle branch
block
I
C
Assessment of the aortic diameter in hypertension
without adequate blood pressure control
I
B
Hypertensive patients with LVH on ECG for
quantification of LVH and definition of LV geometric
pattern
IIa
B
Global longitudinal strain evaluation in patients
with hypertensive cardiopathy
IIa
C
Reassessment of patients with hypertensive heart
disease without alteration of their clinical status
IIb
B
Assessment of first-degree relatives of
hypertensive patients
III
C
Selection of antihypertensive therapy
III
C
Monitoring of antihypertensive therapy in
controlled and asymptomatic individuals
III
C
LVH: left ventricular hypertrophy; ECG: electrocardiogram; LV: left
ventricle
2.5. Athletes
The clinical entity called “athlete’s heart” has been recognized for more than
two decades
114
and is
characterized by cardiac morphological alterations, mainly of increased
ventricular mass, secondary to physical training stimulus. These alterations are
not accompanied by changes in myocardial function, not only by conventional
echocardiographic methods but also by techniques such as tissue Doppler and
strain.
115,116
Still, as a result of intact
ventricular function, there is no significant increase in atrial
cavities
117
and
reversibility of morphological alterations after discontinuation of training may
be a decisive diagnostic factor in doubtful cases. The use of TTE, therefore,
can elucidate cases of doubtful diagnosis between this situation and
hypertrophies or pathological ventricular remodeling, such as HCM or even
hypertrophy secondary to SAH.
115
However, the use of echocardiography as a routine method
in the follow-up of athletes lacks robust scientific evidence.
Events of sudden death in athletes constitute an important clinical scenario and
the potential prevention of some situations through clinical cardiological
evaluation raises the discussion about the need to use complementary methods in
this evaluation. Although not all deaths in athletes are cardiovascular,
pathologies such as hypertrophic cardiomyopathy and coronary anomalies are among
the most frequent causes of sudden death during exertion in this
population.
118,119
Although the usefulness of
anamnesis and physical examination is consensual, the need for TTE and even for
the electrocardiogram in population screening of athletes is not a matter of
general agreement among cardiology associations.
120
However, if clinical evaluation suggests the
likelihood of hypertrophic cardiomyopathy (or others of genetic origin),
valvular heart disease or other structural cardiac changes, it becomes an
essential investigative method (Table
9).
Table 9
Recommendations of transthoracic echocardiography in the evaluation of
athletes of competitive and/or professional physical activities
Recommendation
Class of recommendation
Level of evidence
In the differentiation of "athlete's heart" from
conditions of pathological hypertrophy
I
B
Assessment for release of competitive physical
activity, when clinical consultation demonstrates the
possibility of hypertrophic cardiomyopathy or other genetically
transmissible ones
I
B
Assessment for release of competitive physical
activity, when clinical consultation shows signs of valvular
heart disease or other structural cardiac changes
I
C
In routine assessment of athletes when there is no
suggestion of ventricular overload or hypertrophy by clinical
consultation or ECG
IIb
C
ECG: electrocardiogram
3. Heart Murmurs, Valvular Heart Disease, Valvar Prostheses and
Endocarditis
3.1. Heart Murmurs
Heart murmurs are common findings, with prevalence between 5 and 52%.
121
They are produced when the
laminar blood flow becomes turbulent, such as in stenoses or valve refluxes,
emitting sound waves that can be detected with the aid of the stethoscope. It is
important that during physical examination, even in asymptomatic patients,
careful auscultation is performed in order to define its
characteristics.
122
An innocent murmur can be defined as a short, smooth ejective noise (1 to 2++ in
4), audible at the left sternal border, followed by a second normal sound, in
the absence of other abnormalities.
123
This finding, associated with normal chest x-ray and
electrocardiogram, estimates a low probability of cardiac disease and, in this
case, there is no need for complementary echocardiography.
124
However, due to inadequate
training or maintenance of knowledge, characteristics of the murmur or patient’s
anatomy, the auscultation may leave doubts about the existence of underlying
organic causes. In such situations, the use of electronic stethoscope
125
and performing directed
cardiac ultrasound,
126
if
available, may be useful. In case of persisting doubt or suspicion of cardiac
alteration, the echocardiography should be performed (Table 10). This systematic approach
and investment in
medical training allows for a rational use of resources, avoiding excessive
diagnosis and unnecessary exams.
124
Table 10
Recommendations of transthoracic echocardiography in patients with
murmur
Recommendation
Class of recommendation
Level of evidence
Asymptomatic patients, with murmur suggestive of
cardiopathy
I
C
Asymptomatic patients with signs or exams (e.g.,
electrocardiogram) suggestive of cardiac disease
I
C
Patients with murmur and low probability of heart
disease that cannot be ruled out by clinical investigation,
electrocardiogram, chest X-ray or directed ultrasound
IIa
C
Patients without signs or symptoms suggestive of
cardiopathy
III
C
3.2. Native Valves
Echocardiography is the standard diagnostic method for assessing heart valves.
TTE should be performed in suspected and in diagnosed valvular heart disease,
for evolutionary follow-up of moderate and important lesions, and changes in
clinical status.
124
The
examination identifies the mechanisms involved, quantifies hemodynamic severity
and repercussion, estimates prognosis, and assists treatment decision.
127,128
In addition, physical exertion echocardiography can be
performed to evaluate the behavior of echocardiographic parameters in
asymptomatic patients and in cases of divergence between symptoms and the
severity of the lesions estimated in the exams performed at rest.
24,127,129
Besides
traditional echocardiographic techniques, recent applications, such as the
strain and the 3D, have provided new anatomical and functional
information.
130-132
3.2.1. Mitral Regurgitation
TTE, in addition to diagnostic confirmation, provides information necessary
for follow-up and decision making in mitral regurgitation(MR).
127
TTE identifies dilation
of cardiac cavities and dysfunction of both ventricles, in addition to
allowing the classification of regurgitation into primary (due to valve
lesions) or secondary (caused by changes in LV geometry). The 3D
echocardiography is more accurate in volumetric measurements and left
ventricular function; it can be useful in the evaluation of RV
4
and allows better
visualization of the valve apparatus and planning of
interventions.
12,133
The evaluation of the
regurgitation degree can be made by the integrated approach of multiple
qualitative and quantitative parameters: cavity dilations, pulmonary artery
pressure, mitral inflow velocity, pulmonary vein flow pattern, mitral
regurgitation density and duration analysis, calculus of the jet area or
regurgitant volume, vena contracta measurement and
regurgitant orifice measurement (flow convergence method, or proximal
isovelocity surface area - PISA).
133
Challenging situations for echocardiography are
the presence of multiple and/or eccentric jets, cardiac arrhythmias and
acute MR. In these cases, special emphasis should be given to integrated
analysis relating anatomical and hemodynamic parameters. The improvement of
quantitative measures, such as PISA and vena contracta,
through the 3D echocardiography, can aid in the evaluation of eccentric
reflux.
132,134
Another important point
is the measurement of left ventricular function, especially in asymptomatic
patients, which can be overestimated by LVEF measurement, with implications
for deciding the best time for intervention and for postoperative outcomes.
Recently, measurement of myocardial deformation (strain) has been studied to
more sensitively identify ventricular dysfunction, but despite good
prospects, it still requires more studies and standardization.
131,135,136
TEE, whether 2D or 3D, is indicated for evaluation of the regurgitation
mechanism in the care of inappropriate transthoracic images or in
discrepancies between echocardiographic and clinical parameters.
4,133
The general recommendations for the use of the
various modalities of echocardiography in MR are contained in tables 11, 12 and 13.
Table 11
Recommendations of transthoracic and stress echocardiography in
mitral regurgitation
Recommendation
Class of recommendation
Level of evidence
Initial assessment of severity and MR
mechanism
I
C
Periodic assessment of left ventricular
dimensions and function in patients with moderate to severe
MR without symptom changes
I
B
Patients with MR and modifications of signs or
symptoms
I
B
Assessment in the first postoperative
month
I
C
Assessment of hemodynamic changes and
ventricular adaptation during pregnancy
I
C
Stress echocardiography in asymptomatic
patients with severe MI to assess tolerance to physical
efforts and hemodynamic changes
IIa
B
Stress echocardiography to assess discrepancy
between severity of valve disease and symptoms
IIa
B
Stress echocardiography to evaluate left
ventricular reserve
IIb
B
Assessment of ventricular mechanics (strain)
for patients with borderline left ventricular function
IIb
B
3D TTE to assess preoperative anatomy and left
ventricular function
IIb
C
Routine assessment of slight MR with LV normal
function and dimensions
III
C
MR: mitral regurgitation; TTE: transthoracic echocardiography;
LV: left ventricle
Table 12
Recommendations of transesophageal echocardiography in mitral
regurgitation
Recommendation
Class of recommendation
Level of evidence
Intraoperative assessment to define the
mechanism and to assist in valve repair
I
C
Unsatisfactory TTE for determination of
severity and/or insufficiency mechanism, or for the
assessment of LV function
I
B
Asymptomatic patients with severe MR to assess
the possibility of valve repair
IIa
C
3D TEE to assess preoperative anatomy and left
ventricular function
IIb
B
Assessment of patients with slight MR
III
C
TTE: transthoracic echocardiography; LV: left ventricle; MI:
mitral regurgitation; TEE: transesophageal echocardiography
Table 13
Recommendations of echocardiography in patients with mitral valve
prolapse
Recommendation
Class of recommendation
Level of evidence
Diagnosis, anatomical and functional evaluation
of patients with physical signs of MVP
I
C
Confirmation of MVP in patients with previous
diagnosis, but without clinical evidence to support it
I
C
Risk stratification in patients with clinic
features or diagnosis of MVP
IIa
C
Exclusion of MVP in first-degree relatives of
patients with myxomatous valve disease
IIb
C
Exclusion of MVP in patients with no suggestive
physical signs or family history
III
C
Periodic echocardiographies in patients with
MVP without insufficiency or with slight insufficiency,
without alterations of symptoms or clinical signs
III
C
MVP: mitral valve prolapse.
3.2.2. Mitral Stenosis
The diagnosis of mitral stenosis (MS) using echocardiography makes it
possible to define its probable etiology as a consequence of a wide
evaluation of the valve anatomy.
137
The hemodynamic characterization of the gradients
and valvular area, together with the description of thickening, leaflet
mobility, subvalvar involvement and degree of calcification of the
commissures, determines the progression stage of the disease and defines the
most appropriate type of treatment when the disease is symptomatic. The
joint interpretation of the echocardiography and the clinical symptoms
determines the indication of surgical intervention or balloon catheter
valvuloplasty.
137
Recently, MS has been grouped into four distinct categories, based on
anatomy, Doppler evaluation, presence of pulmonary hypertension,
repercussions on LA, and symptoms: stage A (patients at risk for MS); stages
B and C (asymptomatic patients, but with hemodynamic changes); and stage D
(symptomatic patients with hemodynamic changes). Table 14 describes the parameters
that must be included
in the echocardiography to make this evaluation complete.
138,139
The use of TTE usually defines the
anatomy and severity of the lesion (Table
15), but there are indications for the use of TEE, such as in
situations of technically difficult echocardiographic window or 24 hours
before balloon catheter valvuloplasty to rule out the presence of thrombi in
the LA (Table 16).
138,140,141
3D echocardiography, in the TTE or TEE modalities, has
been shown to allow better anatomical analysis and more accuracy in the
valvar area calculated by planimetry.
142,143
Physical or pharmacological stress echocardiography (dobutamine) may be used
in the discordance between symptoms and resting echocardiography
data.
138
Such
phenomenon of incompatibility between symptoms and hemodynamic repercussion
can result from the disproportion between the valve area and the patient’s
body size, or the lack of complacency of the valve orifice (which should
increase during exercise).
144
On a low-dose dobutamine echocardiography, the mean
mitral transvalvular gradient should increase above 18 mmHg
145
in order for MS to be
considered the cause of the symptoms, while on exercise echocardiography
(treadmill), the significant cut-off value is one elevation above 15
mmHg.
138,144
The increase in systolic
pressure in the pulmonary artery is considered of clinical value only during
the exercise echocardiography and should reach at least 60 mmHg so that
pulmonary hypertension is considered secondary to MS. Other less frequent
indications of a stress echocardiography may be found in asymptomatic
patients with marked stenosis (Table
17).
144
Care
should be taken to diagnose associated lesions in MS, whether it is a
significant MI (which imposes a limitation on balloon catheter
valvuloplasty) or lesions on other heart valves.
Table 14
Elements of echocardiographic evaluation of mitral stenosis
Parameter
Description
Valve anatomy
Presence of dome, commissural fusion
Doppler
PHT value
Two-dimensional or three-dimensional
Planimetry of the mitral valve area
Left atrium
Indexed volume
Pulmonary artery pressure
Assessment of tricuspid or pulmonary
insufficiency
PHT: pressure half-time.
Table 15
Recommendations of transthoracic echocardiography in mitral
stenosis
Recommendation
Class of recommendation
Level of evidence
Establish diagnosis of patients with signs and
symptoms of MS
I
B
Quantification of severity (PHT, gradients,
valve area and pulmonary artery pressure)
I
B
Assessment of concomitant valve lesions
I
B
Determination of score for valvotomy by balloon
catheter. Block Wilkins: thickening, mobility, subvalvar and
calcification
I
B
Reassessment of stable MS with area < 1
cm2 each yearReassessment of stable
MS with area between 1 and 1.5 cm2 every 2
yearsReassessment of stable MS with area > 1.5
cm2 in 3 to 5 yearsImmediate
reassessment with change of symptoms
I
B
Follow-up of balloon catheter valvuloplasty
after dilatation
I
B
Assessment of hemodynamic alterations and
adaptation during pregnancy
I
B
MS: mitral stenosis; PHT: pressure half-time
Table 16
Recommendations of transesophageal echocardiography in mitral
stenosis
Recommendation
Class of recommendation
Level of evidence
Inconclusive transthoracic
echocardiography
I
B
Assessment of thrombus preceding balloon
catheter valvuloplasty
I
B
Assessment of the degree of mitral
regurgitation preceding balloon catheter valvuloplasty (when
there is doubt about transthoracic)
I
B
Table 17
Recommendations of stress echocardiography in mitral stenosis
Recommendation
Class of recommendation
Level of evidence
Discordance between symptoms and valvar
area/gradient (mitral area > 1.5 cm2)
I
C
Assessment of asymptomatic patients with area
< 1 cm2
IIa
C
Assessment of asymptomatic patients with an
area between 1 and 1.5 cm2 in pregnancy or major
surgery planning
IIb
C
3.2.3. Aortic Stenosis
TTE is the first-line method (Table
18) for the diagnosis and assessment of the severity of aortic
valve stenosis (AVS).
128,146-148
The definition of the moment of surgical
or percutaneous intervention depends on the integrated analysis of clinical
and echocardiographic parameters (valvular anatomy, Doppler valvular
hemodynamics and repercussion on cavity size and pulmonary artery pressure)
that allow to classify aortic stenosis into four stages: stage A (risk of
AVS); stage B (mild and moderate asymptomatic AVS); stage C (asymptomatic
marked AVS), subdivided into C1 (with LVEF ≥ 50%) and C2 (LVEF <
50%); and stage D (classical symptomatic marked AVS).
149
In some AVS subgroups,
valve area is reduced in the low gradient and low flow periods, either due
to the concomitance of left ventricular dysfunction (LVEF < 50%) or the
presence of small and hypertrophied LV, despite preserved LVEF). These
subgroups are designated as stage D2 (with decreased LVEF) or D3 (with
normal LVEF).
146,147,150
In these discrepancy situations, in
which valve area is ≤ 1.0 cm2, the gradient is < 40
mmHg and LVEF is preserved (AVS with low paradoxical gradient or with low
gradient and normal flow), additional methods such as TEE (3D, if possible),
computed tomography or cardiac resonance may be necessary to confirm the
severity of AVS.
147
TEE
allows a better evaluation of the aortic valve anatomy (valvular
calcification), etiology (degenerative, congenital or rheumatic), LV exit
path (diameter and geometry, mainly 3D) and greater accuracy in the
calculation of valvular area, either by continuity equation or by direct
planimetry.
147,150
The absence of
significant calcification should alert to the possibility of sub- or
supravalvar obstruction. In good-quality images, 3D TTE also allows for a
more accurate LVEF assessment and a calculation of ejection volume (aortic
transvalvular flow) by subtraction of final diastolic and systolic volumes,
without the need to use LV exit pathway measurement and Doppler. This
calculation, however, should be analyzed along with the other parameters,
once it may also underestimate aortic transvalvular flow.
147,150
If the calculation of the valve area is
made during hypertension (blood pressure ≥ 140 x 90 mmHg), it should
be repeated after blood pressure control because it may underestimate the
transvalvar flow. Reduction in LV systolic function by GLS measurement, with
no other explanation, in the presence of preserved LVEF, favors the
diagnosis of severe AVS with low paradoxical gradient. Stress
echocardiography with low dose of dobutamine, with calculation of the
projected area of the valve, if necessary,
144,147
should be performed if there is marked aortic stenosis
with low-flow/low gradient and left ventricular dysfunction (stage D2) to
distinguish truly marked stenosis from pseudostenosis and to evaluate the
contractile reserve (Table 19).
Echocardiography under physical stress is recommended to unmask symptoms or
to provide prognostic information on moderate or marked asymptomatic AVS
with preserved LVEF (stages B or C) (Table
19). An increase in the mean pressure gradient (> 18 to 20
mmHg), the absence of contractile reserve and the increase of pulmonary
artery systolic pressure (PASP) > 60 mmHg during exercise are parameters
of worse prognosis and require follow-up at shorter intervals.
144
Stress echocardiography
may also be useful in low-flow/low paradoxical gradient (with preserved
LVEF), asymptomatic or with mild or doubtful symptoms, to confirm the
severity of AVS using the same criteria.
144
In suboptimal images, the evaluation of
LVEF can be improved by the use of myocardial contrast, to better delineate
endocardial borders.
147
The invasive hemodynamic study is restricted to situations in which
noninvasive imaging tests are inconclusive.
146,149
The follow-up interval with TTE depends on the stage
of the disease and predictors of poor prognosis. Accentuated aortic valve
calcification is another predictor of more severe stenosis and worse
clinical progression.
151
Marked AVS with low paradoxal gradient has worse prognosis when compared to
classical AVS and the remaining AVS subgroups.
150
The paradoxical low gradient and normal
flow (LV > 35 mL/m2) seems to have a prognosis similar to that
of moderate AVS, but should be accompanied by shorter intervals,
particularly if symptomatic.
147
In classical AVS, the maximum velocity ≥
5m/s
152
and an
annual increase in maximum velocity ≥ 0.3cm/s
151
in serial examinations
(recorded in the same incidence and with the same quality) are predictors of
worse prognosis and of faster progression.
146,147,149
Table 18
Recommendations of transthoracic echocardiography in aortic valve
stenosis
Recommendation
Class of recommendation
Level of evidence
Diagnosis and assessment of the severity of AVS
in the presence of suspicious murmur or symptoms potentially
related to AVS, such as: chest pain, dyspnea, palpitations,
syncope, stroke or peripheral embolic event
I
B
Syncope
I
B
History of bicuspid AV in first degree
relatives
I
B
Patients with AVS to assess wall thickness, LV
size and function
I
B
Reassessment of patients with the AVS diagnosis
with change of symptoms or signs
I
B
Suboptimum transthoracic contrast
echocardiography (≥ 4 contiguous LV segments not
seen), for assessment of LV function and calculation of the
ejected volume
I
B
Annual reassessment of asymptomatic patients
with marked AVS (maximal velocity ≥ 4 m/s) (stage
C1), with reduction of the interval to 6 months if there are
predictors of greater severity at rest (AV marked
calcification, maximum velocity > 5.5 m/s, increase in
maximal velocity ≥ 0.3 m/s/year and low-flow/low
paradoxical gradient) or effort echocardiography*
I
B
Reassessment every 1 to 2 years of asymptomatic
patients with moderate AVS (maximal velocity 3 to 3.9 m/s)
(stage B), with reduction of the interval to 1 year if there
are predictors of greater severity on echocardiography at
rest or effort echocardiography*
I
B
Reassessment of asymptomatic patients with
discrete AVS (maximal velocity 2 to 2.9 m/s) (stage B),
every 3 to 5 years, with reduction for 1 year in the
presence of marked calcification
I
B
Reassessment after hypertension control in
patients with accentuated AVS with low-flow/low gradient and
preserved LVEF
I
B
Monitoring of percutaneous implantation of
aortic valve prostheses and results immediately after
implantation (catheter, position, prosthesis function,
regurgitation)
I
B
Assessment of complications immediately after
percutaneous implantation of the aortic prosthesis
(hypotension, coronary occlusion, LV dysfunction, LVOT
obstruction, marked mitral insufficiency, prosthesis
displacement, tamponade, right ventricular perforation, gas
embolism, aortic dissection)
I
B
Early assessment (within 30 days) after
percutaneous implantation of aortic prosthesis as to the
degree of valve regurgitation (or paravalvarization) in the
presence of suspected valve dysfunction
I
B
Reassessment in less than one year of changes
in hemodynamic severity and LV function in patients
diagnosed with moderate AVS, before or during pregnancy, or
who will be submitted to situations of increased demand
(non-cardiac surgery)
IIa
C
Good quality transthoracic 3D echocardiography
for better assessment of valve morphology (especially in
suspected bicuspid AV) and the degree of calcification
IIb
B
3D echocardiography in good-quality
transthoracic image in symptomatic acute AVS with low
gradient and preserved LVEF (D3), to reassess the diameter
and geometry of the LVOT, to calculate the valvular area by
planimetry or to calculate the valvular area by the
continuity equation using the ejected volume measured
directly by 3D (instead of the ejected volume derived from
Doppler or two-dimensional Simpson)
IIb
B
AVS: aortic valve stenosis; AV: aortic valve; LV: left ventricle;
LVEF: left ventricle ejection fraction; LVOT: left ventricular
outflow tract; 3D: three-dimensional.
*
Predictors of worse prognosis on resting echocardiography: marked
aortic valve calcification and maximal velocity increase
≥ 0.3 m/s/year; on exercise echocardiography: increased
mean pressure gradient (> 18 to 20 mmHg), absence of
contractile reserve, and increased PASP (> 60 mmHg).
Table 19
Recommendations of stress echocardiography in aortic valve
stenosis
Recommendation
Class of recommendation
Level of evidence
Low-dose dobutamine stress echocardiography to
confirm symptomatic low-flow/low gradient AVS and reduced
LVEF and to assess the presence of contractile reserve
(stage D2)
I
B
Stress echocardiography in asymptomatic
patients with moderate or marked AVS (stages B and C1) to
assess exercise-induced symptoms, abnormal responses to
systemic or pulmonary arterial pressure, and behavior of
gradients and left ventricular function
IIa
B
Stress echocardiography in asymptomatic
patients (or with mild or doubtful symptoms) with
low-flow/low gradient AVS and preserved LVEF to
differentiate true stenosis from aortic pseudostenosis
IIb
B
Stress or dobutamine echocardiography in
symptomatic marked AVS
III
C
AVS: aortic valve stenosis; LVEF: left ventricle ejection
fraction
In patients who are candidates for percutaneous implantation of aortic
prosthesis for the treatment of AVS, 3D TEE can be used to evaluate the
diameter of the ring, but it depends on the operator and image quality and
should be used only when there is contraindication to computed tomography.
On the other hand, 3D TEE is recommended to monitor the procedure and to
evaluate outcomes or complications (Table
20).
148
Table 20
Recommendations of transesophageal echocardiography in aortic valve
stenosis*
Recommendation
Class of recommendation
Level of evidence
High acuity AVS with low-flow/low gradient and
preserved LVEF (D3), for assessment of valve area
(reassessment of LVOT measurement) and assessment of valve
morphology, including degree of calcification
I
B
Acute AVS with low flow/low gradient and
reduced LVEF (D2) for assessment of valve morphology,
including degree of calcification
IIb
B
Disagreement between the severity of AVS and
transthoracic examination and clinical evaluation
I
B
Difficulty assessing AVS at transthoracic
examination due to inadequate acoustic window
I
B
Assessment of aortic valve annulus size and
geometry in patients candidates for percutaneous aortic
valve prosthesis implantation
I
B
Monitoring of percutaneous implantation of
aortic valve prostheses and results immediately after
implantation (catheter, position, prosthesis function,
regurgitation)
I
B
Assessment of complications immediately after
percutaneous implantation of the aortic prosthesis
(hypotension, coronary occlusion, LV dysfunction, LVOT
obstruction, marked mitral insufficiency, prosthesis
displacement, tamponade, right ventricular perforation, gas
embolism, aortic dissection)
I
B
Early assessment (within 30 days) after
percutaneous implantation of aortic prosthesis as to the
degree of valvular or paravalvular regurgitation ) in the
presence of suspected valve dysfunction
I
B
Stroke after percutaneous implantation of
aortic prosthesis in case of suspected valve
dysfunction
I
B
Assessment of the distance of the aortic valve
annulus to the coronary sinus in patients candidates for
percutaneous implantation of aortic valve prosthesis
IIb
B
*
3D, if available. AVS: aortic valve stenosis; LVEF: left
ventricular ejection fraction; LVOT: left ventricular outflow
tract; LV: left ventricle
3.2.4. Aortic Regurgitation
Echocardiography is the first-choice method to confirm diagnosis and etiology
and to assess the severity and hemodynamic consequences of aortic
regurgitation (AR).
133,153
AR can be observed due
to primary diseases of the aortic valve (AV) or to abnormalities of the
aortic root and ascending aorta. Degeneration of AV and bicuspid aortic
valve are the most common etiologies. Other causes include rheumatic fever,
fibrosis or infection, alteration of the valvular apparatus support or
dilatation of the valve ring. The integrated analysis of clinical and
echocardiographic parameters (valve anatomy, aortic root and ascending
aortic root diameters, Doppler valvular hemodynamics and repercussions on
cavity size and pulmonary artery pressure) allows the classification of AR
in four stages: stage A (risk of AR), stage B (mild to moderate asymptomatic
AR), stage C [asymptomatic acute AR without (C1) or with
dysfunction/dilation of LV (C2)]; and stage D (symptomatic acute
AR).
149
In
suboptimal images, LVEF measurement may be more accurate with the use of
myocardial contrast to delineate the endocardial borders.
133
TEE (with 3D if
available), tomography or cardiac resonance may be necessary to better
assess the aortic root and ascending aorta (especially in the case of a
bicuspid aortic valve), the severity of AR, or the quantification of LV
ejection volumes and ejection fraction.
133
The appearance of symptoms in AR drastically
changes prognosis. Effortless echocardiography may be indicated to reveal
the presence of symptoms or to investigate other causes not related to AR
(diastolic dysfunction, pulmonary hypertension or dynamic MI).
144
However, it should not
be used to assess severity, once that increased heart rate shortens
diastole, limiting quantification.
144
The follow-up interval with TTE depends on the
stage of the disease and the presence of aortic dilatation associated with
bicuspid aortic valvopathy.
148,154
Recommendations for the use of the various modalities of echocardiography in
AR are set out in tables 21 to 24.
Table 21
Recommendations of transthoracic echocardiography in aortic
regurgitation
153
Recommendation
Class of recommendation
Level of evidence
Confirm the presence, etiology, and severity of
acute or chronic AR
I
B
In patients with dilation of the aortic root to
assess the degree of AR and the magnitude of aortic
dilatation
I
B
Reassessment of patients with prior AR and
change of symptoms or signs
I
B
Annual reassessment of LV size and function in
marked asymptomatic AR, with reduction of the interval to
six months for the first examination, or if there are
significant changes in diameters or LVEF on subsequent
examination (stage C)
I
B
Reassessment every one to two years in moderate
asymptomatic AR (stage B)
I
C
Reassessment every three to five years in
asymptomatic mild AR (stage B)
I
C
Reassessment in less than one year of
hemodynamic severity and LV function in patients diagnosed
with AR before or during pregnancy, or who will undergo
situations that increase demand (non-cardiac surgery)
IIa
C
AR: aortic regurgitation; LV: left ventricle; LVEF: left
ventricle ejection fraction
Table 24
Recommendations of transesophageal echocardiography in aortic
regurgitation*
153
Recommendation
Class of recommendation
Level of evidence
Discrepancy between qualitative and
quantitative parameters of transthoracic echocardiography
and/or between echocardiography and clinical evaluation
regarding the severity of AR
I
B
Confirm the presence, etiology and severity of
acute AR if the transthoracic echocardiography is of
limited, doubtful or inconclusive quality
I
B
In patients with a bicuspid aortic valve to
assess the diameter of the aortic root and ascending aorta
when the transthoracic image is suboptimal
I
B
*
3D, if available. AR: aortic regurgitation
3.2.5. Tricuspid Valvulopathy
TTE is the first-line method for evaluating tricuspid valve abnormalities
(Table 25).
146,148,149
In most cases, tricuspid regurgitation (TR) is
secondary to tricuspid ring dilatation and leaflet pull due to distortion
and right ventricular remodeling, which occur due to volume or pressure
overload caused by diseases of the left side of the heart , pulmonary
hypertension, pulmonary valve stenosis, among others. In this context,
leaflets are structurally normal. Primary causes of TR are rarer and may be
due to infective endocarditis (mainly drug users), rheumatic heart disease,
carcinoid syndrome, myxomatous disease, endomyocardial fibrosis, corneal
rupture related to endomyocardial biopsy, Ebstein’s anomaly, and congenital
dysplasia, among others.
133
Similar to mitral and aortic valve disease, it can be
classified into four stages (A to D).
149
A thorough analysis of valvular anatomy by TTE is
fundamental for the diagnosis of the etiology and mechanisms involved. It is
necessary to measure the diameter of the ring and the use of all indexes of
RV systolic size and function.
133
These measures help in decision making regarding
the moment of the surgery and in the surgical planning. In situations of
doubt regarding the RV, 3D TTE can be used, although it still requires more
validation. Cardiac resonance remains the gold standard.
146
In this context, TEE is
not recommended due to the anterior location of the RV, which makes it
difficult to see through the transesophageal route.148 Significant primary
TR requires intervention before RV impairment.
146,149
Secondary TR is usually treated when left side valve
disease is corrected. As in the other valvopathies, the echocardiographic
follow-up interval depends on the stage of the disease, but the etiology of
the disease must also be considered. In the case of secondary TR, it is
appropriate to follow the recommendations described for left heart valve
dysfunctions. Significant annular dilatation (≥ 40 or > 21
mm/m2) and dilatation or progressive RV dysfunction should
alert for earlier follow-up.
133
Tricuspid stenosis (TS) is an uncommon condition that,
if present, is frequently associated with TR of rheumatic origin.
146,149
In this case, the presence of associated
mitral stenosis is common, which is usually the predominant lesion. Other
causes are rare, such as congenital diseases, drugs, Whipple’s disease,
endocarditis, and large right atrial tumor.
146
TS diagnosis of is often neglected.
Careful analysis of the subvalvular apparatus is essential to predict valve
repair.
146
The
integration of clinical and echocardiographic parameters related to TS (mean
gradient > 5 to 10 mmHg, valve area ≤ 1.0 cm2 and mean
time of pressure drop ≥ 190 ms) classifies severity in stages C
(marked asymptomatic) and D (marked symptomatic).
149
Table 25
Recommendations of the echocardiography in tricuspid valvulopathy
Recommendation
Class of recommendation
Level of evidence
2D TTE is recommended to confirm diagnosis, to
assist in identifying the etiology and mechanisms of
tricuspid lesions, to determine severity, to assess
pulmonary pressure, as well as the dimensions of the cardiac
cavities and the right ventricle function and to
characterize any associated cardiac disease on the left
side
I
B
TEE (with 3D if available) may be used for more
detailed assessment of valve morphology, mechanisms and
Doppler quantification if the TTE is of limited, doubtful or
inconclusive quality, or there is a discrepancy between the
clinical data and the echocardiographic findings
I
B
3D TTE (in optimal windows) can be used to
assess systolic and diastolic volumes and RV systolic
function in patients with marked TR (stages C and D)
IIb
B
TEE (2D or 3D) for assessing the systolic
function of the RV in marked TR
III
C
TTE: transthoracic echocardiography; 2D: two dimensional; TEE:
transesophageal echocardiography; 3D: three dimensional; RV:
right ventricle; TR: tricuspid regurgitation
3.2.6. Pulmonary Valvulopathy
TTE is the initial recommended method to diagnose and evaluate the severity
of pulmonary stenosis (PS) or regurgitation (PR), its etiology and effects
on cardiac structure and right ventricular function (class I).
133,149
In addition to assessing the valvular
anatomy, investigation of the etiology requires a thorough evaluation of the
RV, pulmonary ring, pulmonary artery trunk and its branches. Primary PS or
PR (with leaflet involvement) are more often due to congenital diseases than
acquired ones. Secondary PR occurs in situations of pulmonary hypertension.
There is little literature on the quantification of the severity of PR on
the echocardiography, but there is a consensus that it should be done in an
integrated way with pulsed, continuous and color Doppler parameters; and
graded as mild, moderate, or marked.
133
PS and PR are classified, from the
clinical-echocardiographic point of view, as stage C (marked asymptomatic)
and D (marked symptomatic).
149
Evaluation of the pulmonary valve may be difficult for
TTE. In this situation, however, the TEE does not provide additional
information and is not recommended (class III). There is little data on the
value of 3D echocardiography. In cases of limited transthoracic imaging or
severity parameters inconclusive or discordant with clinical data, cardiac
resonance is recommended as the best method.
133
3.2.7. Associated Valvar Lesions
Associated valvular lesions (AVL) in our setting are frequent due to the high
prevalence of rheumatic fever (RF), which reaches 70% of valvular heart
disease in Brazil.
141
In
the EuroHeart Survey, 51% of AVL patients had RF and 40% had degenerative
valve disease.
155
Pathophysiology is complex, because it depends on the specific combination
of each valve lesion, and its diagnosis is challenging because the
guidelines provide us with specific valvular parameters alone. AVL may
result from two primary valvular diseases
156
or from the combination of primary and
secondary valvular disease.
157
Despite the high prevalence of AVL, there is little
evidence of the best course of action to be taken in each combination. The
most common combinations and their most frequent changes are reported
below:
156
AS and MI: Increased LV pressure caused by aortic stenosis may
increase the regurgitant orifice and decrease aortic
transvalvular gradients, mimicking a low-flow state.
156,158
In some AS
cases, there may be MR secondary to dilation and left
ventricular dysfunction (tethering). Less frequent, but possible
in these patients, is the presence of primary MR.
AS and MS: are cases of difficult clinical control, in which the
patient rapidly evolves to low throughput states. The gradients
of both valves may be underestimated, and if the patient is
inadvertently submitted to balloon catheter valvotomy of the
mitral valve, acute pulmonary edema may occur due to the lack of
LV compliance as a consequence of AS.
141,156
AR and MS: the presence of MS limits the increase in ventricular
volumes frequently observed in AR; which may underestimate the
severity of AR.
141,156
MR and AR: as a consequence of the volume overload imposed by
both valvopathies, these patients usually have earlier
contractile deficit than with each isolated valvopathy and
progress more rapidly to the symptomatic phase of the
disease.
141,156,158
Recommendations for TTE and TEE in AVL are listed in tables 26 and 27, respectively.
The frequency at which TTE should be performed
is debatable and depends on the type of AVL and symptomatology; in general,
the examination should be repeated according to the predominant valve lesion
guideline.
156
In
the case of balanced lesions, TTE should be repeated with a shorter interval
than the one suggested for a single valve lesion.
156
Table 26
Recommendations of transthoracic echocardiography in associated valve
lesions
Recommendation
Class of recommendation
Level of evidence
Establish diagnosis of patients with multiple
murmurs
I
C
Quantification of the severity of stenoses and
associated insufficiencies
I
C
Immediate reassessment with change of
symptoms
I
C
Annual reassessment of asymptomatic patients
with AVL
IIa
C
AVL: associated valvular lesions
Table 27
Recommendations of transesophageal echocardiography in associated
valve lesions
Recommendation
Class of recommendation
Level of evidence
Inconclusive transthoracic
echocardiography
I
C
Doubts in the quantification of valvular
lesions
I
C
Monitoring of invasive procedures for injuries
that can be treated percutaneously
I
C
3.3. Valvular Prostheses
TTE is recommended as a first line examination for the analysis of valvular
prostheses. TEE may be necessary when it is necessary to better evaluate the
structure and complications of valvular prostheses, recommended in cases of
dysfunction (Table 28). When performing
the echocardiographic examination of valvular prostheses, it is necessary to
know and document the reason for the investigation, the patient’s
symptomatology, the type and size of the prosthesis, the date of surgery, blood
pressure, heart rate, height, weight and the patient’s body surface area.24 A
detailed postoperative TTE is recommended four to six weeks after surgery, when
the thoracic surgical incision is healed, thoracic wall edema resolved, and left
ventricular function recovered. In this examination, it is important to record:
cavitary dimensions, ventricular function, prosthetic gradients, valvular areas,
presence of functional or pathological refluxes, pulmonary pressure and
alterations of other valves; defining the basal conditions of valve prostheses,
since the examination will be taken as reference for serial assessments.
Regarding the periodicity of TTE in patients with prosthesis, a frequent
assessment in asymptomatic patients with supposedly normal mechanical prosthesis
is not recommended. For biologic prostheses considered to be normal, exams after
five (ESC)
159
or ten years
(ACC/AHA) are recommended).
127
However, annual examinations are recommended in patients
with new design prostheses that have not had their proven durability in patients
with aortic dilatation at the time of surgery and in patients with mitral
prostheses to evaluate the evolution of tricuspid regurgitation and RV function.
Echocardiographic investigation (TTE and TEE) is recommended when changes in
cardiac auscultation, onset of symptoms or suspicion of prosthesis dysfunction
occur. In cases where there is clinical suspicion of infective endocarditis or
thrombosis, the analysis should be more thorough.
123
In cases of significant reflux of
prostheses, it is recommended to perform evolutionary TTEs every three to six
months.
128
TEE, due
to its proximity and posterior approach to the heart, achieves better diagnostic
precision in valvular prosthesis dysfunctions. In fact, TTE and TEE complement
each other, since the TTE better evaluates the changes in flow and TEE, the
morphological changes. It is always advisable to carry out the full and careful
TTE before recommending the TEE. The 3D TEE
160
provides additional information about the 2D image,
particularly regarding the spatial relationship of the structures around the
prosthesis, the direction and extent of regurgitant jets, the location of
paravalvular leaking and the identification, position and number of larger
anomalous prosthetic or periprosthetic echoes, potentially more
embolinogenic.
161
The
diagnosis of prosthesis stenosis should always be performed with the extensive
use of echocardiography. The transprosthetic gradients are variable in each
model and size, and there may be high gradients in cases of small, even normal,
prostheses when implanted in large body surface patients, a finding known as
mismatch.
162
Patients
who remain with significant LVH in the late postoperative period may also
present elevated gradients after aortic prosthesis implantation. Thus, the
comparison with the basal echocardiography is always important. In biological
prostheses, the most frequent cause of stenosis is the degeneration and
calcification of the leaflets, usually a late complication. In mechanical
prostheses, the growth of fibrous tissue into the ring, known as pannus, is also
a late complication that can cause stenosis, reflux, or double prosthetic
dysfunction. The detection and quantification of the reflux of the prostheses
are usually hampered by the acoustic shadow caused by the mechanical prostheses,
mainly in the mitral position. In such cases, TEE can aid in the detection and
quantification, and determine if the insufficiency is prosthetic or
periprosthetic, functional or pathological. We must be careful in
differentiating the “physiological” refluxes, which are common in prostheses,
from the pathological ones.
163
In general, physiological reflux presents laminar flow at
color Doppler and pathological reflux presents a turbulent, color mosaic flow.
In cases of suspicion of infective endocarditis in prosthetics, the diagnosis is
made difficult by the presence of shadows and reverberations, allowing the TTE
to identify only the large vegetation. Given the clinical suspicion of
endocarditis, it is always advisable to perform the TEE, which has greater
sensitivity, detecting smaller vegetations and possible complications, such as
annular abscesses (Table 29). 3D TEE
allows a more precise spatial localization, in relation to the adjacent
prosthetic and anatomical structures, of potentially emboligenic vegetations. In
cases of embolic phenomena or acute stenosis of the prosthesis, especially in
the mitral position, the presence of valve thrombosis or strands (fibrin) should
be suspected, with TTE and TEE being indicated (3D TEE, if possible) in order to
overcome the acoustic shadow and better observe the LA and the atrial face of
the prosthesis. In these cases, in addition to looking for thrombi or fibrin in
the valve or the LA, the prosthesis’ mobile structures and the emboligenic
potential of the thrombi should be functionally evaluated (Table 30).
Table 28
Recommendations of echocardiography in valvular prostheses
Recommendation
Class of recommendation
Level of evidence
TTE in patients with valve prostheses with changes
in clinical signs or symptoms, suggesting prosthetic dysfunction
(stenosis or insufficiency)
I
A
TEE in patients with prosthetic dysfunction to TTE,
for confirmation and better quantification of dysfunction
IIa
B
Periodic reassessment in patients with prosthesis,
with ventricular dysfunction without modification of symptoms or
clinical signs
IIa
B
Periodic reassessment in biological valve
prostheses without signs or symptoms of prosthetic
dysfunction
IIb
B
TTE: transthoracic echocardiography; TEE: transesophageal
echocardiography
Table 29
Recommendations of echocardiography in infective endocarditis in patients
with valve prostheses
Recommendation
Class of recommendation
Level of evidence
Detection and characterization of the valve lesion
and evaluation of the hemodynamic repercussion*
I
B
Detection of complications such as abscesses,
ruptures and fistulas*
I
B
Reevaluation in cases with poor clinical
evolution*
I
B
Suspected endocarditis in patient with negative
cultures*
I
B
Bacteremia of unknown etiology*
I
B
Persistent fever with no evidence of bacteremia or
new murmurs*
IIa
B
Routine assessment during treatment of
uncomplicated endocarditis*
IIb
B
Transient fever without evidence of bacteremia or
new murmur*
III
B
*
Transesophageal echocardiography may give additional information to
those obtained with transthoracic echocardiography
Table 30
Recommendations of echocardiography in patients with clinical suspicion
of valvular prosthesis thrombosis
Recommendation
Class of recommendation
Level of evidence
Carrier of mechanical prosthesis with embolic
phenomenon and/or acute heart failure
I
B
Assessment to determine the hemodynamic changes
caused by thrombosis
IIb
B
TEE to complement the TTE, to evaluate the mobility
and emboligenic potential of the thrombi and functional study of
the prosthesis
IIb
B
TEE: transesophageal echocardiography; TTE: transthoracic
echocardiography
3.4. Infective Endocarditis
Infective endocarditis (IE) is vascular or cardiac endocardial infection
resulting from invasion of microorganisms. Despite the advances in diagnostic
techniques and treatment, mortality by IE remains high.
164
The profile of the disease
presentation changed, with the emergence of new risk groups and more virulent
microorganisms, with staphylococci emerging as the main etiological agents.
Echocardiography is fundamental in the IE approach (Table 31).
165-168
The best
resolution of the devices and, especially, the use of TEE are responsible for
the high accuracy of the method in the diagnosis and evaluation of
complications. The additional value of TEE when TTE is not diagnostic is well
defined in the strong clinical suspicion of IE and/or the presence of valvular
prostheses. However, the indication of TEE as an initial examination needs to be
validated by new studies.
168
The definitive diagnosis of IE is based on positive blood cultures and/or
characteristic echocardiographic findings. The findings following the
echocardiography are major diagnosis criteria: vegetation defined by a mobile
condensed mass, adhered to the valvar endocardium, mural or implanted prosthetic
material; abscesses or fistulas; new prosthesis dehiscence (especially when it
occurs late after its implantation) and new valve regurgitation.
169
Table 31
Recommendations of transthoracic echocardiography and transesophageal
echocardiography in infectious endocarditis
Recommendation
Class of recommendation
Level of evidence
TTE is indicated as the first examination in the
clinical suspicion of IE
I
B
TEE is indicated on clinical suspicion of IE and
negative or non-diagnostic TTE
I
B
TEE indicated in the diagnostic suspicion of IE in
patients with valvular prostheses and intracardiac devices
I
B
Indicated to repeat TTE or TEE within five to seven
days in the face of high clinical suspicion and initial negative
TEE
I
C
Echocardiography indicated for the assessment of
staphylococcal bacteremia of unknown source
IIa
B
TEE may be indicated for suspected IE, even in
cases with positive TTE with good quality and reliable findings
(except isolated IE)
IIa
C
New TTE or TEE indicated for suspected new
complications (abscesses, perforations, embolisms, persistence
of fever, heart failure)
I
B
New TTE or TEE indicated for the follow-up of
uncomplicated IE, for vegetation size monitoring or detection of
silent complications. The type (TEE or TTE) and the date of the
new examination will depend on the initial findings, type of
microorganism and response to therapy
IIa
B
Intraoperative TEE in all cases of valve surgery by
IE
I
B
At the end of the treatment to establish new
parameters of cardiac and valvular morphology and function
I
C
TTE: transthoracic echocardiography; IE: infective endocarditis; TEE:
transesophageal echocardiography
4. Hypertension and Pulmonary Thromboembolism
Pulmonary hypertension (PH) is a clinical condition associated with high morbidity
and mortality, the prevalence of which is unknown due to different presentation
groups. From the knowledge of the various pathophysiological mechanisms, the current
classification divides PH into five groups.
170
Regardless of the mechanism, it is defined as mean
pulmonary arterial pressure greater than or equal to 25 mmHg, at rest, documented
by
cardiac catheterization.
170
Currently, TTE is considered a method of fundamental importance in the initial
evaluation of patients with clinical suspicion of PH (Table 32), since it offers information
related to: diagnosis,
hemodynamic status, therapeutic response and prognosis.
171
Hemodynamic data, such as pulmonary artery
systolic pressure, mean arterial pressure, pulmonary artery occlusion pressure and
blood volume (assessed by varying the size of the inferior vena cava), can be
measured by this method.
172
The
presence of RV hypertrophy, enlargement of the right cavities, anomalous movement
of
the septum and pericardial effusion suggest the diagnosis. The analysis of the
contractile function of the RV is performed through the TAPSE, s-wave (systolic) of
the tissue Doppler, RV area fractional variation and ejection fraction to the
3D.
4
Right cardiac
catheterization remains the gold standard for diagnosis, since it allows the direct
measurement of hemodynamic data in the pulmonary circulation and evaluates the
capacity of response to vasodilator therapy through the pulmonary vasoreactivity
test.
Table 32
Recommendations of the echocardiography in pulmonary hypertension and
thromboembolism
170,174
Recommendation
Class of recommendation
Level of evidence
TTE recommended as a first line examination for
noninvasive diagnostic investigation of suspected pulmonary
hypertension
I
C
TTE recommended in the assessment of signs of pulmonary
hypertension in symptomatic patients with portal hypertension or
liver disease and in all indicated to hepatic transplantation
I
B
TTE recommended as an initial examination for the
assessment of pulmonary hypertension in patients with systemic
sclerosis and annually
I
C
TTE recommended for noninvasive diagnostic assessment
of patients with pulmonary disease with suspected pulmonary
hypertension
I
C
High-risk pulmonary embolism, in the presence of shock
or hypotension, TTE at the bedside or angiotomography (depending on
the patient's clinical conditions or availability)
I
C
High-risk pulmonary embolism with signs of right
ventricular dysfunction, unstable for angiography (TTE at the
bedside with Doppler of lower limbs and/or TEE to assess pulmonary
artery thrombus)
IIb
C
Not recommended in asymptomatic HIV positive patients
for the detection of pulmonary hypertension
III
C
TTE: transthoracic echocardiography; TEE: transesophageal
echocardiography; HIV: human immunodeficiency virus
Pulmonary thromboembolism (PTE) is another clinical condition of high mortality,
which can cause complications such as chronic thromboembolic pulmonary hypertension
(PH group IV).
170
Clinical
suspicion, progress in diagnosis, and effective therapy are critical in reducing
mortality in the acute event. The sensitivity and specificity of TTE for the
diagnosis of PTE are 50 to 60% and 80 to 90%, respectively. In critical patients,
TEE may increase this sensitivity. The visualization of the thrombus in the right
atrium (RA), in the RV or in the trunk of the pulmonary artery ratifies the
diagnosis. However, indirect signs are more commonly found, such as dilatation of
the right cavities, RV contractile dysfunction, interventricular septal flattening,
McConnel’s signal (apical region with preserved contractility and akinetic mean free
wall segment, with sensitivity of 77% and specificity of 94%) and dilation of the
inferior vena cava. The pulmonary artery acceleration time is a parameter with good
sensitivity, since it is altered (< 100 ms) in cases of small pulmonary
embolism.173 RV strain is an important tool because it shows the segment that
presents reduced value and evaluates its deformity after reperfusion therapy.
Patients who develop contractile dysfunction of the RV or patent foramen ovale
present a reserved prognosis.
5. Coronary Artery Disease
5.1. Introduction
Coronary artery disease has a wide clinical spectrum, ranging from asymptomatic
severe disease,
175
long-term
stable angina or acute coronary syndrome (ACS)/acute myocardial infarction (AMI)
with hemodynamic instability as the first manifestation of the
disease.
176,177
The echocardiography has
applications in its diagnostic recognition, stratification of risk in the acute
phase, follow-up and determination prognosis in the long term.
178,179
5.2. Acute Coronary Syndrome
5.2.1. Transthoracic Echocardiography
In the scenario of a patient with acute chest pain and suspected coronary
artery disease, echocardiography may be useful (Table 33) and should be routinely
available in the
emergency department and thoracic pain units.
180
Evidence of new or presumably
contractile change from LV to TTE is one of the parameters in the third
universal definition of myocardial infarction
181
and may in fact assist in the
diagnosis/prognostic determination of an ACS. In addition, TTE may aid in
the differential diagnosis of chest pain and/or associated conditions, such
as acute aortic dissection, aortic stenosis, hypertrophic cardiomyopathy,
and pulmonary embolism.
180
By dividing LV into 16 or 17 segments, contractile
segmental function is visually quantified based on systolic thickening (ST):
hyperkinetic = 0 (ST > 70%); normal = 1 (ST = 50 to 70%); hypokinetic = 2
(ST < 40%); akinetic = 3 (ST < 10%); “dyskinetic = 4 (paradoxical
movement/systolic expansion)”. The wall motion score index (WMSI) is the
reference parameter to express the LV segmental function and its normality
value is 1; values between 1 and 1.6 show a contractile alteration of mild
degree; while WMSI values above 1.6 indicate greater involvement and worse
prognosis.
182
Obviously, the absence of alterations in segmental contractility in resting
TTE does not exclude the presence of coronary artery disease.
180
It should also be
considered that the contractile alteration may occur in other conditions
such as myocarditis, RV overload (volume/pressure), ventricular
pre-excitation, Takotsubo type cardiomyopathy, left bundle branch block,
chagasic cardiomyopathy or presence of pacemaker.
180
TTE is the exam of choice in cases of
hemodynamic instability with suspected cardiac origin, as well as in the
identification of mechanical complications of AMI.
179,180
However, it is necessary to avoid requesting the
examination for evaluation of patients with chest pain with a confirmed
diagnosis of myocardial ischemia (ACS/AMI), since TTE should not delay the
immediate onset of treatment.
176,177,180
On the other hand, in
the screening of symptomatic patients suspected of having coronary artery
disease in the emergency room, recent evidence indicates the potential
usefulness of GLS calculated by 2D speckle tracking. In the absence of
preexisting structural heart disease, previous infarction or left bundle
branch block, GLS (when < 16.5%) may complement existing diagnostic
algorithms and act as an early adjunct marker of ischemia.
183
Table 33
Recommendations of transthoracic echocardiography in acute coronary
syndrome
Recommendation
Class of recommendation
Level of evidence
Assessment of global and segmental ventricular
function
I
C
Differential diagnosis of alternative causes of
chest pain: severe aortic stenosis, hypertrophic
cardiomyopathy, pulmonary embolism, aortic dissection*, pericarditis
and the presence of cardiac tumors
I
C
Chest pain with hemodynamic instability and
suspected cardiac origin
I
C
Suspected mechanical complications in
myocardial infarction: left ventricular aneurysm, rupture of
free wall or papillary muscle, ventricular septal defect,
pericardial effusion
I
C
Assessment of right ventricular impairment in
the presence of inferior wall myocardial infarction
I
B
During chest pain of possible ischemic origin,
with electrocardiogram and non-conclusive cardiac
enzymes
IIa
B
Calculation of global longitudinal strain using
speckle tracking as an adjunct to existing diagnostic
algorithms and risk classification in patients with
suspected coronary disease&
IIa
B
Assessment of patients in the presence of chest
pain with a confirmed diagnosis of myocardial
ischemia/infarction
III
C
Evaluation of chest pain in patients whose
non-cardiac etiology is evident
III
C
*
Complementation with transesophageal echocardiography increases
accuracy and can provide additional information to the
transthoracic one; &in the absence of preexisting structural
heart disease, prior myocardial infarction, or left bundle
branch block.
5.2.2. Stress Echocardiography
The evidence of a new alteration of the segmental contractility at rest or
its appearance before the induction of stress (exercise or pharmacological)
suggests ischemic etiology.
184
Stress echocardiography is an independent predictor of
cardiovascular death, of additional value to the other methods and can avoid
coronary angiography.
175,179
Its use may be
recommended for risk stratification of patients in chest pain units (Table 34), especially
when the
electrocardiogram does not define the diagnosis and the exercise test is
submaximal, non-feasible or inconclusive.
179
Traditionally, stress echocardiography
is performed after 24 hours of chest pain relief in low to moderate risk
patients with no evident ischemic changes on the electrocardiogram and
normal cardiac enzymes.
Table 34
Recommendations of stress echocardiography in acute coronary
syndrome
Recommendation
Class of recommendation
Level of evidence
Patients with clinically controlled low risk
unstable angina* before deciding the invasive strategy
IIa
A
To assess the functional significance of
moderate coronary obstruction at angiography, as long as the
result interferes with the procedure
IIa
C
Risk stratification after uncomplicated
myocardial infarction
IIa
A
Investigation of patients with suspected
microvascular disease& to establish whether segmental
change occurs in conjunction with angina and
electrocardiographic abnormalities
IIa
C
Strain and strain rate parameters derived from
speckle tracking as an adjunct tool to wall motion score
index for diagnosis and/or prognosis of acute coronary
disease
IIa
B
High risk unstable angina or acute myocardial
infarction
III
C
*
No recurrence of angina, no signs of heart failure, no
abnormalities on the initial/serial electrocardiogram and normal
troponin; &typical angina pain with electrocardiogram change
or functional test, in the presence of normal coronary
angiography
5.2.3. Contrast Echocardiography
This echocardiographic modality allows the immediate and simultaneous access
of LV segmental contraction and myocardial perfusion.
179,180
In patients with acute chest pain and
non-diagnostic electrocardiogram, the use of contrast echocardiography
increases sensitivity for the diagnosis of ACS (Table 35).180,185 Patients with normal
perfusion and
myocardial function at rest have good prognosis, while the presence of
perfusion defects at rest identifies a subgroup at high risk for
ACS.
185
Table 35
Recommendations of contrast echocardiography in acute coronary
syndrome
Recommendation
Class of recommendation
Level of evidence
Use of echocardiographic contrast for better
definition of the endocardial border and to access left
ventricular structure/function when two or more adjacent
segments are not visible in the standard resting or stress
tests
I
B
Assessment of patients with acute chest pain
and non-diagnostic electrocardiogram
IIb
B
Assessment of myocardial perfusion in all types
of ACS
III
C
Routine use of echocardiographic contrast in
all patients with chest pain and suspected CAD
III
C
ACS: acute coronary syndrome; CAD: coronary artery disease
5.3. Chronic Coronary Artery Disease
5.3.1. Transthoracic Echocardiography
TTE, while providing important information on segmental contractility when
performed at the time of acute chest pain, is limited in the investigation
of patients with chronic coronary disease.
175
Two situations of indication should be
valued: the first one when there is a need for a differential diagnosis of
chest pain with non-ischemic causes, such as pericarditis, valvular diseases
(such as aortic stenosis) or cardiomyopathies that may occur with chest
pain; the second is based on the knowledge of global left ventricular
function as a prognostic factor in stable patients with chronic coronary
disease (Table 36).
175
Table 36
Recommendations of transthoracic echocardiography in chronic coronary
disease
Recommendation
Class of recommendation
Level of evidence
Differential diagnosis of precordial pain
I
B
Initial assessment of left ventricular function
as a prognostic indicator, even in patients with no evidence
of heart failure
I
B
Assessment of left ventricular function when
there is evidence of heart failure or change in clinical
status
I
B
Periodic reassessment of stable patients
without clinical change
III
C
5.3.2. Stress Echocardiography
Stress echocardiography is a useful investigative method for both suspected
patients and those with an established diagnosis of stable coronary disease
(Table 37). The method offers
good accuracy in the ischemic investigation of patients from moderate to
high risk, with a slight predominance of specificity compared to other
non-invasive imaging methods, such as myocardial scintigraphy.
179,184,186,187
The
stress-induced modality, whether physical, with treadmill or bicycle, or
pharmacological, with dobutamine sensitized with atropine or even with
dipyridamole, does not significantly change the diagnostic performance of
the test.
179
In general,
in the investigation of stable coronary disease, stress echocardiography
should be indicated for those patients with limited exercise performance,
either by functional class or by non-interpretable electrocardiogram, such
as in the presence of left bundle branch block.
188
Thus, the method should not be
considered as a substitute for the ergometric test. However, if available,
it can be used as the first examination in the investigation of selected
patients, with intermediate or high pretest probability. Furthermore, in the
investigation of the risk of chronic coronary disease, it can be used as a
sequential method to others such as coronary tomography, when the calcium
score shows levels above 400.
184,186
Another important clinical situation for indication of stress
echocardiography is the preoperative evaluation of patients undergoing
intermediate-risk surgery.
179,187,189
Even in this situation,
the method can be used but it should not generally replace the exercise test
when this is possible. However, in vascular surgeries, where there are one
or more risk factors, the investigation may start from the echocardiographic
examination under stress.
184
Situations in which there is a need for the
topographical definition of ischemia, such as those of a functional
significance investigation of already known lesions, also induce the image
examination under stress, which may be the echocardiography. However, the
availability of the method in the region of medical practice and the
technical ability and experience of the echocardiography laboratory for
individualized application of the levels of recommendation suggested here
should be considered.
Table 37
Recommendations of stress echocardiography in patients with known or
suspected chronic coronary disease
Recommendation
Class of recommendation
Level of evidence
As an initial method in the investigation of
chronic coronary disease in patients with intermediate or
high pre-test probability
I
B
As an initial method in the investigation of
chronic coronary disease in patients with low pretest
probability, but unable to perform an exercise test or with
electrocardiogram not interpretable
I
B
As a sequential method in the investigation of
patients submitted to an ergometric test with intermediate
or non-diagnostic result
I
B
As a sequential method in the investigation of
patients submitted to coronary tomography with a calcium
score (Agatston) > 400
I
B
As a sequential method in the investigation of
patients submitted to coronary angiography with identified
lesions of uncertain functional significance
I
B
In the preoperative evaluation of non-cardiac
vascular surgery in a patient with one or more risk factors
for chronic coronary disease
I
B
In the assessment of viability in patients with
ventricular dysfunction and chronic coronary disease and
eligible for revascularization
I
B
In the preoperative assessment of non-cardiac
intermediate risk surgery in a patient with one or more risk
factors for chronic coronary disease with functional class
< 4 METs or indeterminate
IIa
B
In the sequential investigation of patients
with moderate to high pre-test risk with previous testing
for ischemia for more than two years
IIa
B
In the assessment of asymptomatic patients
after incomplete revascularization
IIa
C
In the evaluation of symptomatic patients after
revascularization
IIa
B
Routine reassessment (every five years) in
asymptomatic patients after revascularization
IIb
C
As an initial method in the assessment of
patients with low pre-test probability and with clinical
conditions and interpretable electrocardiogram to perform an
ergometric test
III
C
In the preoperative assessment of non-cardiac
intermediate-risk surgery in patients with functional class
≥ 4 METS
III
B
Routine ergometric test substitution in
patients with physical capacity and interpretable
electrocardiogram for the performance of the test
III
C
MET: metabolic equivalent of risk.
5.3.3. Contrast Echocardiography
The use of echocardiographic contrast agents consisting of microbubbles
capable of overcoming the pulmonary barrier and remaining intact has become
a powerful weapon for adequate visualization of the endocardium of all LV
segments.
185,190
Thus, in the presence of
two or more contiguous segments with limited technical quality, the use of
any of these agents is indicated (Table
38).
191,192
The use of contrast
agents for myocardial perfusion, although it is part of the same procedure,
only with modifications in the acquisition and analysis of the images,
remains considered off-label by the US and European health agencies.
Guidelines on stable coronary disease already recognize the use of contrast
agents to delineate endocardial borders, but they are still not based on
myocardial perfusion evaluation.
175
However, robust evidence supports the use of these
agents for assessing myocardial perfusion both in the diagnosis of coronary
disease in acute and chronic coronary syndromes,
193
even showing its superiority over
conventional stress echocardiography in predicting cardiovascular
events.
194
The
accuracy of contrast agents has been compared with other methods such as
myocardial scintigraphy and, in contrast, showed similarity, with greater
sensitivity, mainly in the detection of uni-coronary lesions.
195
However, the use of
contrast agents for perfusion analysis is facilitated when stress is
performed with dipyridamole; and the low use of dipyridamole in most
Brazilian and worldwide echocardiography laboratories
192
may be a barrier to the
implementation of myocardial perfusion analysis.
Table 38
Recommendations for the use of echocardiographic contrast agents in
chronic coronary disease
Recommendation
Class of recommendation
Level of evidence
Improvement of endocardial border delineation
and analysis of global or regional ventricular function when
endocardial visibility in two or more segments is
limited
I
B
Myocardial perfusion analysis in the diagnosis
of chronic coronary disease, both in the assessment of
ischemia and viability as an adjunct to the modalities of
stress echocardiography
IIa
B
Assessment of the coronary flow reserve in the
study of the functional repercussion of already known or
viable coronary lesions
IIa
B
Use in the presence or suspected of significant
intracardiac shunts
III
B
Routine use of contrast in patients whose image
and endocardial edge delineation of the left ventricle are
of adequate quality
III
C
Coronary flow reserve can also be assessed by contrast echocardiography under
stress. Reduced values of coronary flow reserve are indicative of functional
repercussion in lesions that anatomically have dubious expression. In
addition, some studies have shown the role of this index in assessing
viability and in predicting myocardial functional recovery in patients with
stable coronary artery disease.
196
6. Evaluation of Emboligenic Sources and Cardioembolic Diseases
Stroke is the major cause of disability and the second leading cause of death in the
world.
197
Brazil is the
Latin American country with the highest mortality rates due to stroke, being the
main cause of female death.
198,199
Although the death rate from
stroke has declined in recent decades, the values remain very high.
200
It is estimated that
cardioembolic disease is responsible for 15% to 40% of all causes of ischemic
stroke,
201
whereas
indeterminate (cryptogenic) causes account for 30 to 40% of these ischemic
neurological events.
202-204
Other causes of ischemic stroke
include large artery atherosclerosis, small vessel occlusion (lacunar), and other
etiologies.
205
In
patients who are at risk or who have already had embolic neurologic events, the main
role of echocardiography is to identify the presence of an emboligenic source, to
determine the probability of such a source being a possible cause of ischemic stroke
or systemic embolism and to guide the therapy of these patients. We can classify
cardiac diseases as for their emboligenic potential under high and low risk
conditions (Table 39). The main causes of
ischemic stroke of cardioembolic origin are: atrial fibrillation, associated or not
with rheumatic MS (five-fold risk of stroke); left ventricular dysfunction (two to
three times greater risk of ischemic stroke than the general population);
206
acute myocardial infarction (the
risk is possibly decreasing by the implementation of early reperfusion
therapies);
207
mechanical
valvular prostheses (annual risk of ischemic stroke at 4.0%);
208
and infectious endocarditis (one
in five cases are complicated by ischemic stroke).
209
Variable rates of annual recurrence of ischemic
stroke have been reported in patients with aortic arch atheroma (less than 3 to
12%).
210
The patent
foramen ovale (PFO) can serve as a passageway from a paradoxical embolism of the
venous to the arterial circulation. Although patients with an ischemic stroke of
indeterminate (cryptogenic) etiology have a higher incidence of FOP than those with
known ischemic stroke cause,
211
a
large study reported that the presence of a PFO was not associated with an increased
risk of recurrence of ischemic stroke.
212
Other more rare causes of embolism include papillary
fibroelastoma, myxoma, and mitral calcification.
Table 39
Classification of cardiac diseases regarding their emboligenic potential
High risk
Low risk
Intracavitary thrombus
Patent foramen ovale
Atrial fibrillation
Interatrial septum aneurysm
Acute myocardial infarctation
Interatrial communication
Dilated cardiomyopathies
Spontaneous contrast
Infectious endocarditis
Lambl excrescences
Valve prostheses
Mitral valvular calcification
Rheumatic mitral stenosis
Aortic valve calcification
Left atrial myxoma
Endocarditis marantica
Papillary fibroelastoma
Mitral valve prolapse
Ulcerated plaques in the aorta
The cardioembolic etiology of ischemic stroke should be suspected in the presence
of
severe onset of early neurologic deficit without prodromes, multiple brain lesions
in multiple vascular territories, and recurrent ischemic stroke in a short period
of
time.
213
Systemic
embolization to other organs such as spleen and kidneys at the time of the ischemic
stroke increases the suspicion of cardioembolic etiology.
213
TTE and/or TEE should be recommended in patients
with suspected cardiac embolic source, including ischemic stroke and transient
ischemic attack (TIA) or systemic embolism. TTE is more suitable for evaluation of
embolic sources present in previous cardiac structures, such as the apical thrombus
investigation of the left ventricle. In contrast, during the TEE, the transducer is
positioned in the esophagus, and the probe is closer to the posterior heart
structures. The esophagus is also adjacent to the LA, so TEE corresponds to the gold
standard examination for thrombus screening in the left atrial appendix, with
sensitivity and specificity approaching 99%. TEE should be recommended as an initial
diagnostic tool in the assessment of cardiac embolic source in patients with
ischemic stroke, especially in those where the therapeutic decision (anticoagulation
or cardioversion) will depend on the echocardiographic findings. TEE should also be
recommended when TTE imaging is of poor quality in young patients with ischemic
stroke, in patients with ischemic stroke of undetermined etiology, and in those with
non-lacunar ischemic stroke. TTE may not be useful when TEE is already programmed
for TEE, such as in the evaluation of intracardiac masses, prosthetic heart valves,
the thoracic aorta, or to guide percutaneous procedures. TEE should not be
recommended when TTE findings are compatible with the embolic cardiac source. Both
TTE and TEE should not be recommended in patients whose results will not guide the
therapeutic decision. Table 40 lists the
main recommendations of TTE and/or TEE in patients with TIA, ischemic stroke or
systemic embolism.
Table 40
Recommendations of the echocardiography in the evaluation of emboligenic
sources and cardioembolic diseases
Recommendation
Class of recommendation
Level of evidence
Suspected cardiac embolic source, including ischemic
stroke and TIA or systemic embolism
I
C
Young patient (< 45 years) with TIA or acute
ischemic stroke
I
C
Elderly patient with evidence of non-lacunar ischemic
stroke
I
C
TIA or cryptogenic ischemic stroke
I
C
TEE as the initial test to facilitate clinical
decision-making regarding treatment (anticoagulation or
cardioversion)
I
B
Assessment of cardiac emboligenic source when
non-cardiac origin has been previously identified
IIb
C
TTE when TEE is already programmed (e.g. in the
evaluation of intracardiac masses, prosthetic heart valves, thoracic
aorta, or to guide percutaneous procedures)
IIb
C
TEE when TTE findings are diagnostic of cardiac embolic
sources
III
C
TTE and/or TEE results do not guide the therapeutic
decision
III
C
TIA: transient ischemic attack; TEE: transesophageal echocardiography;
TTE: transthoracic echocardiography.
7. Atrial Fibrillation
Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia, whose
prevalence increases with advancing age.
214,215
In the United
States, it is estimated that the prevalence of atrial fibrillation will double from
5.2 million cases in 2010 to 12.1 million cases in 2030.
216
In addition to population aging, the increased
prevalence of AF can be explained by the comorbidities and associated cardiovascular
risks such as hypertension, heart failure, coronary artery disease, valve diseases,
obesity and diabetes mellitus.
217
The risk of developing AF is 1 in 4 individuals from 40 years of age on.
218
Recent national and
international guidelines have reported the classification of AF based on its
presentation, duration and spontaneous termination of AF episodes.
214,219,220
Paroxysmal AF
is defined as that which is reversed spontaneously or with medical intervention
until seven days after its initiation. Episodes lasting longer than seven days are
referred to as persistent AF. Persistent long-term AF represents cases lasting more
than one year. Permanent AF corresponds to the cases in which attempts to revert to
sinus rhythm will no longer be instituted. Finally, non-valvular AF is defined as
AF
in the absence of rheumatic MS, mechanical or biological valve or previous mitral
valve repair. As part of the initial evaluation, all AF patients should have a TTE
to identify structural heart diseases, including valvular heart disease, assess RA
and LA size, LV and RV size and function.
184
TEE is the most sensitive and specific technique for
detecting intracavitary thrombi, especially in the left atrial appendage, as a
potential source of systemic embolism in AF, and can be used to guide early
cardioversion or catheter ablation procedures.
214,220,221
TEE can also identify features
associated with an increased risk of thrombus formation in LA, including reduced
flow velocity in the left atrial appendage, spontaneous contrast in LA, and aortic
atheroma.
214
Table 41 lists the main recommendations of
TTE and/or TEE in patients with AF. Although the echocardiography provides important
information for assessing the likelihood of achieving successful rhythm control
after cardioversion, including atrial size, left ventricular systolic function, and
severity of valve disease, randomized trials with a larger sample size are still
lacking to understand the real prognostic value of imaging techniques in patients
with AF.
222
New echocardiographic
techniques such as LA evaluation by strain and 3D echocardiography are promising
tools for future clinical practice.
222
Table 41
Recommendations of echocardiography in patients with atrial fibrillation
Recommendation
Class of recommendation
Level of evidence
TTE in the initial assessment of all patients with AF
to identify structural heart disease and guide treatment
I
C
TTE in patients with AF lasting ≥ 48 hours to
decide early cardioversion with brief heparinization, without
previous oral anticoagulation
I
B
TEE in the assessment of patients before ablation or
percutaneous occlusion of the left atrial appendage
I
B
Patient with AF requiring emergency cardioversion due
to hemodynamic instability
III
C
TTE: transthoracic echocardiography; AF: atrial fibrillation; TEE:
transesophageal echocardiography.
8. Heart Tumors and Masses
The cardiac masses comprise a broad set of lesions which may be neoplastic and
non-neoplastic in nature. As regards incidence, the most frequent causes of cardiac
masses are thrombi and vegetation, and rarely are tumors and pseudotumors (intrinsic
and extrinsic structures that mimic a cardiac tumor).
223
Cardiac tumors are extremely rare, with
secondary tumors (metastatic neoplasms) being 20 times more frequent than primary
tumors.
224,225
Although the classification of
these lesions for benignity or malignancy is an important predictor of prognosis,
any cardiac tumor may have substantial hemodynamic or electrical consequences
depending on size and location.
226
Most are detected incidentally during cardiac imaging tests or
after complementary evaluation of specific clinical situations, such as after
embolic event, suspected endocarditis and the possibility of malignancy involving
the heart. Myxomas are the most frequent benign primary tumors in adults, followed
by fibroelastomas and, finally, fibromas (much more common in the pediatric
population). Primary malignant tumors, however, represent a much smaller portion of
primary cardiac neoplasms, with sarcomas and lymphomas being more common. Much more
frequent, as mentioned above, are secondary tumors, represented by metastases, which
can occur by various forms of dissemination (hematogenous, contiguous, venous and
lymphatic), associated mainly to tumors of the breast, lung, esophagus, mediastinum
and melanoma. In these cases, the involvement of the pericardium occurs most of the
time.
227
Echocardiography, because of its availability and applicability, is the imaging
technique commonly chosen for diagnosis (Table
42). The examination may delineate the multiple cardiac structures and
characteristics of a mass, such as location, mobility, morphology, size, insertion
site, and potential hemodynamic consequences. It also allows for serial images over
time without the need for contrast agents (iodine or gadolinium) or radiation. New
techniques, such as the 3D modality, by providing additional anatomical data are
capable of increasing the diagnostic accuracy of the method, assisting in the
surgical strategy, as well as monitoring immediate and late results of the
procedure.
228,229
The contrasted echocardiographic
study represents a very useful tool, offering greater anatomical detail and
assisting in the differentiation of the masses through the analysis of its
vascularization (hypervascularization is frequently associated with the presence of
malignancy).
230
Table 42
Recommendations of the echocardiography in patients with intracardiac masses
and tumors
Recommendation
Class of recommendation
Level of evidence
Assessment of individuals with clinical suspicion
(signs and symptoms) or patients with conditions predisposing to
cardiac tumors
I
C
Carriers of malignant neoplasia with high risk of
cardiac involvement
I
C
Evolutionary follow-up after surgical removal of
cardiac tumors with high potential for recurrence (such as
myxomas)
I
C
TTE for complementary anatomical and functional
assessment in cases in which TTE was not definitive
I
C
TEE for complementary intraoperative assessment
I
C
3D echocardiography to search for additional anatomical
information not seen in 2D TTE
I
C
Use of echocardiographic contrast for differential
diagnosis and vascularization analysis
IIa
B
Patients with direct relatives with family history of
myxoma
IIa
B
Patients whose results of the examination findings will
not imply in therapeutic decision
III
C
TEE: transesophageal echocardiography; TTE: transthoracic
echocardiography; 3D: three dimensional; 2D: two dimensional.
9. Pericardial Diseases
Echocardiography should be indicated in the suspicion of pericardial affections,
including (but not only) pericardial effusion, pericardial mass, constrictive
pericarditis, effusive-constrictive pericarditis, patients after cardiac surgery and
suspicion of cardiac tamponade (Table
43).
231,232
It contributes decisively to the
semiquantitative evaluation of the pericardial effusion and its hemodynamic
repercussion (depending on the volume and the velocity of the collected fluid), as
well as exploring the underlying etiology, whether primary (e.g. pericarditis,
chylothorax) or secondary (e.g. bleeding, metastasis, myxedema, hydropericardium).
The method provides information about the nature of the fluid, suggesting the
presence of fibrin, clot, tumor, air, and calcium. The size of the effusion may be
classified by the diastolic measurement of the echo-free pericardial space , as of
small (< 10 mm), moderate (10 to 20 mm) and large (> 20 mm).
233
Findings indicative of cardiac
compression may precede the clinical manifestations of the tamponade and configure
an emergency situation. In this context, pericardial puncture guided by
echocardiography may alleviate hemodynamic impairment and save lives.
233
Such procedure can be performed
safely in centers with experience, avoiding radiation associated with fluoroscopy
and/or cost of surgery, which makes pericardiocentesis guided by echocardiography
the procedure of choice.
234
Individuals with chronic or recurrent pericardial effusion, not responsive to the
proposed clinical treatment, may be referred for elective pericardial drainage after
serial evaluation. The spectrum of echocardiography utilization in pericardial
disease also includes congenital defects, trauma, neoplasia, cysts, CT after
radiotherapy and the differential diagnosis between constrictive pericarditis and
restrictive cardiomyopathy. In this differentiation, findings compatible with
constriction are: exacerbated decrease (> 25%) in the E-wave velocity of the
mitral flow in the first beat after inspiration, normal tissue Doppler mitral
annular velocity (e’ > 7 cm/s) and annulus paradoxus (e’ septal > e’
lateral).
233
Table 43
Recommendations of the echocardiography in pericardial diseases
Recommendation
Class of recommendation
Level of evidence
Clinical suspicion of pericardial effusion
I
C
Serial studies for evaluation of recurrent stroke
I
C
Assessment after radiotherapy (five years in patients
at high risk for cardiotoxicity and ten years in others)
I
C
Suspicion of constrictive pericarditis, early detection
of constriction or differential diagnosis with restriction
I
B
Suspected cardiac tamponade (chest trauma, cardiac
surgery, iatrogenic perforation in cardiac catheterization or
electrophysiological study, rupture of the ventricular wall after
myocardial infarction and aortic dissection)
I
C
Suspected pericardial cyst, pericardial mass or
pericardial agenesis
I
C
Monitoring of pericardiocentesis
I
B
Serial studies to assess the effect of treatment on
stroke
IIa
C
Routine examination for small effusions in
hemodynamically stable patients
III
C
Investigation of pericardial thickening without
repercussion
III
C
10. Systemic Diseases
10.1. Introduction
The indication of echocardiography in systemic diseases depends on the prevalence
of associated heart disease, the characteristics peculiar to cardiac involvement
in each situation and the clinical suspicion of cardiac involvement.
235
For example, examination is
mandatory in individuals with systemic diseases potentially causing restrictive
cardiomyopathy that show signs and symptoms of heart failure in clinical
evolution. Some systemic diseases for which the indication of the examination
should be considered are as follows.
10.2. Chronic Renal Failure
Morphophysiological changes in LV (such as hypertrophy, dilatation, systolic and
diastolic dysfunction) are common in patients with end-stage renal disease and
predict a worse prognosis.
236-238
International guidelines
recommend TTE for all dialysis patients one to three months after the initiation
of renal replacement therapy and at three-year intervals thereafter, despite the
symptoms.
239
10.3. Amyloidosis
It is a common cause of restrictive cardiomyopathy and may be familial
(transthyretin) or nonfamiliar (primary or light chain). Cardiac involvement due
to amyloid deposition may lead to some suggestive echocardiographic findings:
thickening and increased echogenicity (“granular” appearance) of the LV walls
(especially in the absence of arterial hypertension), biatrial dilatation,
thickening of the valves and interatrial septum, diastolic dysfunction (grade II
and III), small pericardial effusion, prominent decrease in longitudinal strain
in the basal and mid LV segments (“sparing” the apical segments), and later
systolic dysfunction with LVEF.
240
10.4. Sarcoidosis
It is important to investigate the presence of cardiac involvement in sarcoidosis
(granulomatous disease of unknown origin), as this is a potentially fatal
condition. Among the various echocardiographic findings that may be found, we
have: dilated cardiomyopathy, restrictive cardiomyopathy, segmental
contractility alterations that do not obey the classic coronary territorial
distribution, basal septum akinesia, inferolateral aneurysm and abnormal
thickness of the septum (thickening or thinning).
241
10.5. Neoplasias
The echocardiography can detect silent pericardial metastases in some types of
neoplasia (such as breast and lung) and monitor the cardiotoxic effect of
chemotherapeutic agents.
242
10.6. Autoimmune Diseases
The test may diagnose lupus-associated cardiac manifestations, such as
pericardial effusion and sterile vegetations, systemic sclerosis, such as
pulmonary hypertension, or rheumatoid arthritis, such as valve
abnormalities.
235
11. Diseases of The Aorta, Pulmonary Artery and Veins
11.1. Aorta
The evaluation of the aorta is routine in the TTE, since it allows to examine
some of its segments, mainly the aortic root and the proximal portion of the
ascending aorta, affected in numerous affections. The root of the aorta is
formed by the aortic ring, the sinuses of Valsalva and the synotubular junction.
The descending aorta and the proximal abdominal aorta can be evaluated at the
suprasternal and subxiphoid sections, respectively.
154,243
However, the TTE should be considered a screening test, with limitations, since
it does not allow the analysis of all segments of the aorta, such as the aortic
arch and the distal descending. In this case, it is necessary to use other
imaging methods such as TEE, computed tomography or magnetic resonance
imaging.
154,243
The TEE allows the
realization of excellent resolution images due to the proximity of the esophagus
to the thoracic aorta. Despite the multiplanar sections offered, a small segment
of the ascending aortic junction with the aortic arch is not visualized due to
the interposition of the trachea.
154
The precise diagnosis of acute aortic syndromes, such
as aortic dissection, intramural hematoma, penetrating ulcer and rupture of
aortic aneurysms, is fundamental in the therapeutic strategy to be adopted. In
unstable patients with suspected acute aortic syndrome, the imaging modality
chosen will depend on local availability and expertise. In general, TTE is
performed as an initial investigation (class I), complemented with TEE and/or
tomography (both class I). The TEE shows good accuracy, mainly in the exclusion
of artifacts caused by reverberations of the anterior wall of the LA and the
pulmonary artery.
244
Depending on the clinical suspicion, the diagnostic investigation should proceed
with two or more imaging examinations due to the possibility of false
negatives.
245
11.2. Pulmonary Artery
The trunk of the pulmonary artery and the initial portion of the pulmonary
branches can be assessed to TTE. TEE is more accurate, allowing a greater
examination of the pulmonary artery and its branches, which allows a better
appreciation of thrombi in the proximal territory of the pulmonary artery.
Dilations of these vessels can also be diagnosed. Pulmonary artery dilatations
are uncommon lesions and may be associated with different etiologies, such as
congenital heart diseases, systemic vasculitis, collagenosis, infections and
traumas.
11.3. Veins
Anomalies of the superior and inferior vena cava can be diagnosed by TTE and/or
TEE. The presence of thrombi in these pathways and the extension of tumors into
the right cavities can be evaluated. TEE is particularly useful for the
identification of thrombus or vegetation in the superior vena cava in patients
with long-stay catheters and in cases of pulmonary vein stenosis after AF or
atrial flutter ablation procedures. The persistence of the left superior vena
cava should be suspected in the presence of dilated coronary sinus and the
diagnosis can be made using intravenous injection of agitated saline solution,
which will first contrast the coronary sinus and then the right cavities. In
this case, it is important to emphasize the need to exclude anomalous drainage
of the left pulmonary vein via the vertical vein.
246
12. Intraoperative Echocardiography in Cardiac and Non-Cardiac Surgeries
12.1. Introduction
Intraoperative echocardiography is a technique for monitoring cardiac and
non-cardiac surgeries that allows a rapid and real-time assessment of anatomic
and functional cardiac features (global and segmental function, valvular
function, volume and vascular resistance), aortic and phenomena with embolic
potential.
247,248
In non-cardiac surgeries,
clinical information obtained by intraoperative echocardiography is often
complementary to data provided by other hemodynamic monitoring devices (e.g.,
central venous catheter, pulmonary artery catheter, or arterial line).
247,249
In the case of cardiovascular surgeries, the
intraoperative echocardiography can also contribute with real-time dynamic
information and images of the cardiac structures to plan, guide and evaluate the
immediate result of the surgical intervention.
250
12.2. Modalities of Intraoperative Echocardiography
Transesophageal: most widely used in open, minimally invasive or
percutaneous cardiac surgeries, as well as in non-cardiac surgeries.
It has the advantage of not entering the sterile field and of not
disturbing the surgical procedure, allowing continuous monitoring.
It is a relatively safe modality when performed by properly trained
professionals. The contraindications are the same as for the
conventional TEE. In young children, the use of intraoperative TEE
should be considered on a case-by-case basis, based on the unique
risks of these patients (e.g., bronchial obstruction).
248
Epicardial or epiaortic: are an alternative for monitoring open heart
surgeries in which there is absolute or relative contraindication of
manipulation of the esophagus, or blood dyscrasia. In these
embodiments, the linear or sectoral transducer is wrapped in a
sterile cap and applied directly over the heart or aorta. The
epiaortic technique is a very important tool in patients with
advanced atheromatous disease, since it allows the choice of a
suitable site for cannulation and aortic clamping.
249
Transthoracic: may be considered as a monitoring alternative for
percutaneous or non-cardiac procedures that are performed with
superficial sedation, or in cases in which the patient has absolute
or relative contraindication of esophageal manipulation. In this
modality, the examination can be performed serially during the
procedure, or at specific times as needed (e.g., in cases of
hemodynamic instability, to guide endomyocardial biopsy).
Intracavitary: little used, more restricted to percutaneous
procedures.
12.3. Recommendations in Cardiac and Thoracic Aorta Surgery
The main objectives of the use of intraoperative echocardiography in cardiac and
thoracic aorta surgeries are: to confirm and refine the preoperative diagnosis;
to detect new or unsuspected morphophysiological alteration; adjusting the
surgical or anesthetic plane according to the findings; to guide the positioning
of cannulae or devices; to evaluate the presence of air, masses and thrombi in
cardiac cavities and their embolic potential; to evaluate segmental and global
left ventricular function and cavitary pressures; and to evaluate the immediate
outcome of the intervention.
244,245,249
The main recommendations in
this scenario are in the Table 44.
Table 44
Recommendations of the intraoperative echocardiography in cardiac and
thoracic aorta surgeries
Recommendation
Class of recommendation
Level of evidence
Adults submitted to cardiac or thoracic aorta
open-chest surgery (e.g., valve plasty replacement, structural,
mass resection, correction of aortic dissection or aortic
aneurysm)
I
B
Adults undergoing minimally invasive cardiac
surgery
I
B
In young children undergoing open or minimally
invasive cardiac surgery, the indication should be considered
case by case according to the risks of intraoperative
echocardiography in the pediatric population (e.g., bronchial
obstruction)
I
B
Placement and adjustment of ventricular assist
devices
I
B
Myocardial revascularization surgery when there is
left ventricular systolic dysfunction
IIa
B
In case there is a trained professional available,
three-dimensional echocardiography should always be used for
valvular (aortic and mitral regurgitation), structural and mass
resection procedures
I
B
12.4. Recommendations in Non-Cardiac Surgeries
The main objectives of intraoperative echocardiography in non-cardiac surgeries
are: to assess volume status and fluid response; to estimate vascular resistance
and cavitary pressures; to evaluate function of the ventricles (global and
segmental) and valves; and to investigate special issues according to the
clinical status and the type of intervention.
244,245,249
The main recommendations in
this scenario are found in Table 45.
Table 45
Recommendations of the intraoperative echocardiography in non-cardiac
surgeries
Recommendation
Class of recommendation
Level of evidence
Intraoperative echocardiography may be indicated
for the monitoring of non-cardiac surgeries if the patient is
suffering from severe cardiovascular disease and/or the planned
procedure may result in severe hemodynamic, myocardial,
pulmonary or neurological impairment
I
B
Large vascular surgery (usually in open abdominal
aortic repair surgeries)
I
B
Liver transplantation
IIa
B
Pulmonary transplantation
IIa
B
Renal tumor resection
IIb
B
Surgery of trauma
IIb
B
Neurosurgery
IIa
B
Orthopedic Surgeries
IIb
B
Laparoscopic surgeries
IIb
B
13. Echocardiography in Percutaneous Interventions
13.1. Introduction
In general, cardiac interventions through cardiac catheterization have developed
with the support of the echocardiographic image, especially by the
transesophageal route. Its use in daily practice is increasingly frequent, in
view of the development of techniques and technologies for the treatment of
diseases previously only corrected by the conventional surgical procedure. In
addition, there was remarkable progress in the diagnostic capacity of
echocardiography, mainly due to the improvement of the image quality and the
advent of the 3D image, obtained by TTE and TEE. The examination has practically
no contraindication and allows the early identification of potential
complications. There is no randomized study for non-use of echocardiography
during procedures, and some are limited to the use of thoracic, esophageal or
intracardiac modalities. Therefore, in relation to interventional procedures,
indication of echocardiography use is in principle class I, level of evidence C,
a fact already recognized in the literature.
234
In some examinations, it becomes necessary
as well as essential, since the X-ray (XR) image is insufficient to perform the
procedure, and sometimes even expendable. The role of echocardiography in major
interventional procedures follows below.
13.2. Follow-up of Interventions in Congenital Heart Diseases
Atrial septostomy: also known as Rashkind procedure. The septostomy
is performed by balloon in a population in which the TTE allows an
optimal visualization of the atrial septum and of the catheter,
guiding in an appropriate way and evaluating the immediate result
and eventual complications. The use of TEE is usually unnecessary,
and also impracticable, due to the lack of adequate probes. In this
case, even the XR scoping can be discarded.
Procedures for occlusion of atrial septal defects: there are devices
for occlusion of defects of the atrial septal defect, ostium
secundum, as well as patent foramen ovale. The examination can be
performed in both, mainly TEE or intracardiac echocardiography,
where it is possible to assist in the choice of the device, its
positioning, immediate result and rapid identification of
complications. TTE may be preferable to TEE in selected
patients.
251
Procedures for occlusion of ventricular septal defects: TEE should be
performed to better understand the anatomical aspects, the procedure
itself and possible complications. Acquired ventricular septum
defects, such as those after trauma or after acute infarction, may
also be treated with the aid of echocardiography, presenting the
same value in the procedure.
Procedures for occlusion of ductus arteriosus persistence: in this
situation, echocardiography is disregarded, since catheterization
during the procedure is usually sufficient for success.
13.3. Electrophysiology Procedures
The echocardiography mainly supports the procedure of puncture of the atrial
septum, usually through TEE, or alternatively by intracardiac echocardiography.
In the procedure of ablation or implantation of pacemaker the echocardiography
is dispensable, since the electrical mapping provides the necessary information.
In the presence of complications, such as perforation of a chamber with
pericardial effusion and tamponade, the echocardiography is normally
requested.
13.4. Alcoholic Ablation in Hypertrophic Cardiomyopathy
One of the treatments for symptomatic patients consists of alcohol ablation of
the segment where there is a greater degree of hypertrophy and related to
intraventricular obstruction in its exit pathway. TTE is more used, and there is
no restriction on the use of TEE. Prior to alcohol infusion, the septal artery
is catheterized and a solution is infused. At this time, the echocardiography
should assess whether the contrast-enhanced myocardial segment corresponds to
the portion of the myocardium associated with the obstruction and if it does not
occur in all transmurality of the segment, which is undesirable. The Doppler
study estimates the gradient of obstruction and also the degree of mitral
regurgitation. After alcoholic ablation, we repeat the measurement of gradients
and mitral regurgitation, whose falls indicate successful treatment, and
possible complications are investigated.
13.5. Left Atrial Appendage Occlusion
An alternative, when it is impossible to carry out adequate anticoagulation in
patients at high risk for atrial arrhythmia embolism, is occlusion of the left
atrial appendage. TEE is mandatory in this treatment the, as it not only helps
the transseptal puncture, but also allows adequate appenddage measurements,
which select the dimensions of the occluder device. Still in the room, the TEE
guides the procedure, confers its result and makes it possible to diagnose
complications.
13.6. Treatment of Heart Valves
Percutaneous treatment of mitral regurgitation: among the several
treatments proposed, the only one that is commercially available is
Mitraclip®. In this treatment there is a mimicry of the
Alfieri surgery, where there is the formation of a double mitral
orifice. To do so, a metallic clip is inserted through the vein,
which advances to the LA after puncture of the atrial septum and is
positioned so as to reduce severe mitral regurgitation. In this
case, no step is performed without the TEE, considering that the 3D
image provides better understanding of the procedure.
Balloon catheter mitral valvotomy: the use of balloons for the
treatment of severe rheumatic mitral stenosis is a safe and
efficient alternative. The echocardiography prior to the procedure
provides information that can predict the chance of
success.
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In the intervention, the TEE is preferable,
and the morphological aspects of the mitral valve should be
reviewed. During the inflation of the balloon (or balloons), the
echocardiography allows to detect proper positioning. Immediately
after the procedure, measures are performed with the objective of
evaluating the success of the procedure and the complications that
may have occurred.
Balloon catheter aortic valvotomy: the use of this therapy is
currently safeguarded as the last alternative for the treatment of
aortic stenosis or as a bridge to compensate for the clinical
condition and subsequent implantation of a prosthesis via cardiac
catheterization or surgery. The echocardiography can be used to
evaluate immediate results and complications.
Transcatheter implant of aortic valve prosthesis: the indication of
the percutaneous implantation of aortic valve prosthesis is
conditioned to clinical and morphological aspects of the aortic
valve. It is necessary to diagnose severe aortic stenosis prior to
the procedure, as well as the analysis of the aortic complex, which
selects the size of the most appropriate device for the procedure,
directly related to its success. The preference is for the use of
TEE, especially with 3D image. During the procedure, it is necessary
to review the severity of valve stenosis by measuring the gradients
and estimating the effective flow orifice. Still, measurement of the
aortic complex and especially of the area/perimeter of the aortic
ring, performed only through the 3D echocardiography is necessary.
The value obtained is optimally related to tomography measurements
that are usually used to select the device.
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Whether femoral
or transapical, monitoring of the arrival of the prosthesis to the
aortic valve is performed, as well as the aid of the ideal
positioning, prior to its opening. After implantation, the TEE
should provide data on adequate prosthesis expansion, presence and
degree of prosthetic and/or paraprosthetic regurgitation.
Complications of the procedure are part of the echocardiography
investigation.
13.7. Treatment of Prosthetic Dysfunction
Valve in valve: The term “valve in valve” means the implant of a
prosthesis via a catheter over a dysfunctioning bioprosthesis. It
can be made in prostheses in aortic and mitral position, the latter
only by transapical way. The monitoring during the process of
arrival of the prosthesis, its implant and the identification of
complications must be done by the TEE.
Occlusion of paraprosthetic regurgitation orifices: one of the
possibilities of regurgitation in a valve prosthesis is the presence
of paraprosthetic orifices. TEE, especially 3D, should be used to
identify these orifices, locate them accurately, and measure their
area and diameter to select the most appropriate occlusion device.
In the procedure, the echocardiographic image helps to visualize the
passage of the guiding catheters through the paraprosthetic site, to
open the device and to measure the success of the treatment.