RFA, male, 67 years old, admitted for decreased level of consciousness and respiratory
insufficiency (May 13th 2011).
Patient under follow-up at InCor for a double aortic lesion since 2003. The patient
had
progressed asymptomatic in regards to dyspnea and chest pains. Many years ago, the
patient underwent surgery for peptic ulcer.
He had presented with at least three episodes of hemiparesis with complete regression
during follow up and, cognitive deficit, preventing him from correctly following medical
prescriptions, occurred as a sequela. Atrial fibrillation was found during outpatient
care. There was no use of oral anticoagulant due to the reported cognitive deficit.
During follow-up, the patient underwent several echocardiographic evaluations that
showed
dilatation and mild dysfunction of the left ventricle (Table 1).
Table 1
Weight, height and evolutionary echocardiographic data
2003
2009
2010
May 2011
May 2011
Weight (kg)
75
58
56
60
65
Aorta (mm)
35
43
42
44
44
LA (mm)
43
47
46
39
39
RV (mm)
Normal
Normal
Normal
Normal
Normal
Septum (mm)
12
12
12
10
11
Posterior wall (mm)
13
12
10
9
10
LV (diast/syst) (mm)
70/56
65/50
68/41
50/-
62/52
LVEF (%)
48
45
69
40
35
Degree of aortic insufficiency
Severe
Severe
Severe
Moderate
Severe
Gradient LV- Ao (mmHg)
22
-
20
Ao sinuses (mm)
42
-
- Sinotubular junction (mm)
33
-
- Ascending (mm)
34
-
- Arc (mm)
26
-
Systolic pressure of the PT (mm Hg)
33
-
Mitral Valve
Insuf
Insuf+
Insuf +
Insuf +
Insuf +
LV kinesis
Hypo
Diffuse hypo
Diffuse hypo +
Inferior and inferolateral and anterolateral
akinesia
Inferior and inferolateral and anterolateral
akinesia
LA: left atrium; LV: left ventricle; RV: right ventricle; EF: ejection
fraction; Ao: aorta; PT: pulmonary trunk; diast/syst: diastolic/systolic
diameterratio.
In November of 2010, during outpatient care, the patient complained of adynamia, weakness
and weight loss in the previous six months and, about one month before the appointment,
he had presented gastrointestinal bleeding, for which he needed a blood transfusion.
On
that occasion, his red blood cell count was 6 g/dL (Table 2). The patient was on acetylsalicylic
acid 100 mg, enalapril 40 mg,
digoxin 0.25 mg, furosemide 40 mg, potassium chloride 600 mg, ferrous sulfate 2x/day
and
amitriptyline 25 mg daily.
Table 2
Laboratory exams
2006
2009
2010
May 13th 2011
May 15th 2011
May 16th 2011
Erythrocytes: million/mm3
4.9
4.7
3.3
3.9
4.0
3.8
Hemoglobin (g/dL)
12.6
12.9
6.0
11.4
11.5
11.1
Leukocytes /mm3
7900
4400
3200
7460*
10280**
12890***
Platelets /mrr3
252000
206000
284000
77000
105000
94000
Potassium (mEq/L)
4.4
4.9
4.5
3.6
Sodium (mEq/L)
140
136
137
152
Cholesterol (mg/dL)
162
188
130
HDL- C (mg/dL)
33
36
33
LDL-C (mg/dL)
102
123
84
Triglycerides (mg/dL)
133
146
66
Glucose (mg/dL)
92
95
TSH (µUI/mL)
0.848
0.952
0.562
Free T4 (ng/dL)
2
0.95
Creatinine (mg/dL) FG (ml/min/1.73 m2)
1.7
1.37 (55)
1.26 (> 60)
4.74 (13)
3.4 (18)
6.41 (9)
Urea (mg/dL)
55
42
50
247
147
218
Uric acid (mg/dL)
6.7
5.5
CRP (mg/L)
2.09
272
312
Urine I
Density
1.019
Proteins (g/L)
0.29
Leukocytes (/mL)
104000
RBC (/mL)
16000
CK MB (ng/mL)
32.8
13.0
Troponine I (ng/mL)
> 100
> 100
PT (INR)
1.4
1.40
2.6
APTT (rat.)
1.24
1.32
Incoagulable
Venous blood gas
pH
7.29
7.11
pCO2 (mmHg)
48.4
47.6
pO2 (mm Hg)
48.4
46.6
O2 saturation (%)
72.4
83.2
Bicarbonate (mEq/L)
22.7
14.6
Base excess (mEq/L)
(-) 3.4
(-) 14.2
*
25% band; 63% segmented; 8% lymphocytes e 4% monocytes.
**
16% band; 81% segmented; 2% lymphocytes e 1% monocytes.
***
91% neutrophils; 6% lymphocytes e 3% monocytes.
TSH: thyroid stimulating hormone; GF: glomerular filtration; CRP:
C-reactive protein; CK MB: isoenzyme of creatine kinase; PT: pulmonary
trunk; APTT: Activated Partial Thromboplastin Time.
ECG showed atrial fibrillation rhythm, left bundle branch block, and left ventricular
overload.
Echocardiogram showed left ventricle dilatation and hypertrophy and severe aortic
insufficiency (Table 1).
Upper digestive endoscopy revealed gastritis and anastomotic mouth ulcer from a Billroth
II reconstruction gastrectomy surgery.
Chest X-Ray (Sep. 2010) showed free diaphragmatic domes and sinuses, lung parenchyma
with
normal transparency. Normal pulmonary vascularization, hilar configuration, topography
and dimensions. The aorta was elongated with wall calcification and presence of
cardiomegaly with normal mean arch.
The patient remained on follow-up and a new upper digestive endoscopy was prescribed.
The
endoscopy was done on March of 2011 and showed partial Billroth II reconstruction
gastrectomy and enanthematic gastritis of the stump. Biopsy showed moderate chronic
active gastritis with regenerative foveolar hyperplasia, lymphoid aggregates, and
complete intestinal metaplasia in the pyloric mucosa. Helicobacter
pylori culture was negative.
In May of 2011, the patient was brought to the Emergency Room of the Hospital with
syncopal episode followed by a decrease of consciousness level and dyspnea six days
prior to admission.
Physical examination (at 20 h and 45 m on May 12th 2011) revealed decreased
level of consciousness, dehydration, uremic breath, and axillary temperature of 37.8°C.
Heart rate was 121 bpm, blood pressure 135 x 66 mmHg. The patient required orotracheal
intubation for respiratory support, and 500 mL of 0.9% sodium chloride was
administered.
ECG (May 13th 2011) showed atrial fibrillation rhythm with mean heart rate of
124 bpm, QRS duration of 108 ms, corrected QT duration of 437 ms, ÂQRS (-)60°,
left bundle branch block, left ventricle overload, ventricular repolarization
alterations with ST depression and inverted T waves in V4 to V6.
(Figure 1).
Figure 1
ECG. Atrial fibrillation rhythm, left bundle branch block.
Chest X-Ray (May 12th 2013) showed cardiomegaly and pulmonary congestion
(Figure 2).
Figure 2
Chest X-Ray. Cardiomegaly and pulmonary congestion.
Labs (May 13th 2011) revealed white blood cell count with a left shift,
without leukocytosis; thrombocytopenia, renal insufficiency with uremia, mixed acidosis,
and elevated myocardial injury markers, troponin I and CK-MB (Table 2).
Echocardiogram (May 13th 2011) showed left ventricle with eccentric
hypertrophy and decreased systolic function due to akinesia in the basal segments
of the
inferior and inferolateral walls and in the basal and mean segments of the anterolateral
wall, as well as moderate aortic insufficiency (Table
1).
Head CT (May 13th 2011) revealed attenuation of nonspecific periventricular
white matter; right occipital hypoattenuating area, with right lateral ventricle
dilatation, hypoattenuating areas in semi-oval center bilaterally.
Blood cultures collected on May 13th 2011 were positive for methicillin
sensitive Staphylococcus epidermidis and coagulase-negative
Staphylococcus (Staphylococcus lugdunensis). Urine culture was
positive for Proteus mirabilis and in the tracheal secretion there was
growth of one million colonies of Escherichia coli, Klebsiella
pneumoniae and Staphylococcus aureus. Initially,
ceftraixona, clarithromycin and oxacillin were administered, and were later substituted
for imipenem, vancomycin and an association of piperacillin and tazobactam.
After the first day of hospital admission, the patient progressed with hypotension
and
required the use of noradrenaline. He underwent dialysis for four hours on May
14th 2011, presenting with hypotension in the last hour.
The patient presented an episode of hypoglycemia (22 mg/dL) and hypotension, which
was
reverted with intravenous glucose. Another complication was a right pneumothorax,
which
was drained (May 16th).
The patient maintained a decreased level of consciousness, even with no sedation,
persistent fever and hemodynamic worsening, impeding hemodialysis on May
15th.
Lab exams revealed worsening of kidney function and acidemia (Table 2).
Transesophageal echocardiogram (May 15th) was not indicative of infective
endocarditis.
A new chest X-Ray (May 15th) showed condensation of apical segments of the
lower lobe (Figure 3). X-Ray of the abdomen
revealed urinary retention.
Figure 3
Chest X-Ray. Cardiomegaly and pulmonary condensation in the right inferior
lobe.
Blood cultures collected on May 16th 2011 were negative.
Presented asystolic cardiorespiratory arrest and died (May 16th 2011).
Clinical aspects
Patient with aortic insufficiency diagnosed at 57 years of age, presenting with
several transient ischemic attacks simultaneous to atrial fibrillation. Presented
with upper digestive bleeding and, finally, syncope followed by decreased
consciousness and complex mixed shock with acute renal failure.
The patient showed normal aortic insufficiency clinical evolution, that is,
progressive dilatation of the left ventricle with few cardiovascular symptoms.
Generally, aortic insufficiency progression is slow and the left ventricle has time
to dilate and handle high regurgitating volumes with normal filling pressure. This
good compliance maintenance and low end-diastolic pressure has little repercussion
on the left atrium and on the pulmonary venocapillary territory, thus there is no
increase in liquid content in the lungs and dyspnea appears as a late symptom.
With the advancement and progression of myocardial fibrosis and other myocardial
mechanic alterations, ventricular dysfunction occurs and symptoms as dyspnea, heart
failure and, more rarely, angina, appear.
1
Due to a lack of symptoms, the best moment for the recommendation of surgery is
widely discussed. In a series of 246 patients at Mayo Clinic, followed for 10 years,
there was an occurrence of cardiovascular events in 83%, surgical repair in 62%,
heart failure in 47%, and vascular complications in 15%. The functional class of
heart failure greatly influenced 10-year survival of patients - 75% for those in
Class I NYHA, 59% for those in Class II, and 28% for those in Classes III and IV.
Among echocardiographic indices, end-systolic volume corrected for body surface was
a good prognosis indicator: 81% survival for patients with systolic volume under 25
L/m
2
, and 34% for those
with systolic volume of 25 L/m
2
or
more. Ejection fraction was also a mortality indicator: for those with an ejection
fraction under 50%, there was 74% mortality; and for patients with an ejection
fraction over 50%, there was 35% mortality.
2
Another widely used index is the left ventricle systolic diameter with cut-off value
of 55 mm, because, when it is higher, even with valve replacement surgery, survival
is approximately 30% in five years. However, when the cut-off value is under 55 mm,
survival is around 90%. The use of these indices determines a symptom-independent
prognosis.
3-5
The most likely prognosis of the valve disease does not seem to be rheumatic disease,
because the diagnosis was very late and there was no previous history of rheumatic
fever. It also does not seem to be related to Marfan's syndrome because of the
patient's age and lack of involvement of the aorta and the mitral valve, with no
valve prolapse in the latter.
The appearance of atrial fibrillation may contribute to the decompensation of heart
failure, because atrial contraction may be responsible for up to 30% of the systolic
volume in senior patients, patients with myocardial hypertrophy, or ventricular
dysfunction, all of whom present with reduced ventricular compliance and decreased
protodiastolic filling.
6,7
Ejection fraction and tolerance to effort greatly decrease with the appearance of
atrial fibrillation, even in patients without valvulopathy.
8
In regards to syncope followed by low cardiac output presented by the patient, they
may be secondary to some events that notoriously decompensate individuals with
cardiac dysfunction, such as pulmonary thromboembolism, bradyarrhythmias or
tachyarrhythmias, or even acute ischemic syndrome with malignant arrhythmias.
The occurrence of syncope in aortic valve disease is more common in aortic stenosis,
which, along with angina and dyspnea, combine the classic symptoms of this
disease.
9
In aortic
stenosis, it occurs more frequently during effort; however, it can occur at rest if
there is intermittent total atrioventricular block. Nevertheless, syncope is not a
common symptom of aortic insufficiency, except for acute aortic insufficiency
secondary to ascending aortic dissection, which does not appear to have occurred in
this current case.
10
Massive pulmonary thromboembolism may present with syncope and shock. However,
echocardiograms did not show right ventricular dysfunction or dilatation as would
be
expected in this case.
11
Acute coronary condition was favored by the appearance of inferior, inferolateral
and
anterolateral wall akinesia in the echocardiograms during the last hospital
admission and by the significant increase of troponin, even though there was no
major increase of CK-MB or Q waves in the electrocardiogram.
Duke criteria for diagnosing infectious endocarditis are divided into bigger criteria
(blood culture with typical germ and echocardiographic findings of vegetation or
perivalvular abscess) and six smaller criteria (predisposition, fever, vascular
phenomena, indicative echocardiogram, and suggestive microbiological
findings).
12
Endocarditis cannot be ruled out because the patient, who had valvulopathy, presented
with fever, worsened hemodynamics and growth of Gram positive cocci in the blood
culture. However, no worsening of aortic regurgitation degree was detected and there
was no presence of vegetations on heart valves in the transesophageal
echocardiogram.
Mansur et al.
13
showed that the
best predictors of hospital death in cases of endocarditis were previous cardiac
state, causative microorganism, complication occurrence, and white blood cell
count.
Pneumonic condition was probably of bacterium-origin and may have aggravated the
patient's condition. (Dr. Desiderio Favarato)
Differential diagnoses: Aortic insufficiency of undetermined etiology,
ischemic heart disease with acute myocardial infarction and sepsis of pulmonary
origin. (Dr. Desiderio Favarato)
Necropsy
The heart weighed 684 g. The left ventricle showed severe hypertrophy with mild
dilation of the cavity, focal area of fibrous myocardial replacement of the
posterior wall (infarction scar) and recent transmural infarction of the
posterolateral wall (Figure 4). The atrial
septum was intact and the foramen ovale was closed. Histologic exam confirmed acute
infarction with histological dating compatible to 2-5 days of evolution. Coronary
arteries presented complex atherosclerosis, with areas of calcification; there was
80% obstruction of the distal segment of the right coronary, and 60% of the initial
segment of the anterior interventricular artery. Circumflex and left marginal
arteries presented occlusive recent thrombosis, as well as atherosclerotic plaque
(Figure 5). Aortic valve was trivulvular,
with collapsed semilunars, showing fibrous retraction of the free rim and
calcification points. There were no thrombi, vegetations or infectious endocarditis
(Figure 6). In the lungs, we detected
recent, bilateral, thromboemboli, with areas of recent hemorrhagic infarction in the
superior and right-inferior lobes (Figure 7).
There was bronchopneumonia in the inferior lobe of the right lung, along with
fibrinopurulent pleuritis, with presence of Gram positive cocci colonies and food
debris in alveolar spaces, characterizing aspiration etiology (Figure 8)
Figure 4
Cross section of the left ventricle showing area of healed infarction,
with fibrous replacement of the myocardium on the posterior wall
(asterisk) and recent transmural infarction on the posterolateral wall
(double asterisk).
Figure 5
Histological section of the initial segment of the left marginal artery
showing calcified atherosclerotic fibrous plaque (asterisk) and recent
luminal thrombosis (double asterisk). Hematoxylin-eosin, X 15.
Figure 6
Trivalve aortic valve with collapsed semilunar, showing retraction and
thickening of the free edge (arrow). Note the milky focal thickening of
the endocardium of the left ventricular outflow tract (asterisk),
secondary to regurgitant jet of aortic insufficiency.
Figure 7
Microscopic detail of the right lung inferior lobe showing arterial
occlusion by thromboembolus (arrow) and area of recent hemorrhagic
infarction (asterisk).
Figure 8
Histological section of the lung showing bronchopneumonia with presence
of remnants of vegetable cells (arrows), characterizing etiology of
aspiration. Hematoxylin-eosin, X 50.
The encephalon presented old infarction area, healed and cavitated, in the right
occipital pole. There was partial gastrectomy, with Billroth II reconstruction, with
no abnormalities. We found horseshoe kidney and the aorta presented complex
atherosclerosis, with calcified fibrofatty plaques. (Dr. Luiz Alberto
Benvenuti)
Anatomopathological diagnosis - Atherosclerotic ischemic heart disease
and acute myocardial infarction; degenerative aortic valve disease, with functional
insufficiency; thromboembolism and recent pulmonary infarction; aspiration
bronchopneumonia; previous partial gastrectomy; horseshoe kidney. (Dr. Luiz
Alberto Benvenuti)
Comments
Case of 67 year-old man, on follow up for many years at InCor for aortic valve
disease with insufficiency. Presented with a history of partial gastrectomy and
previous strokes, with cognitive deficit. In the period of outpatient monitoring,
the patient presented episodes of digestive bleeding, adynamia, progressive weakness
and weight loss. Digestive biopsy revealed chronic gastritis with no evidence of
neoplasia. After admission to the ER with a history of syncope followed by decreased
level of consciousness and dyspnea, the patient remained in the hospital for three
days, having died at the end of the period. During his hospital stay, acute
myocardial infarction and probable infection with non-defined focus were
diagnosed.
Necropsy confirmed the presence of acute myocardial infarction on the posterolateral
wall of the left ventricle. The infarction was based on the recent thrombotic
occlusion of the circumflex and left marginal coronary arteries, previously affected
by atherosclerotic disease - main cause of ischemic heart disease.
14
The patient also had bilateral
pulmonary thromboembolism with presence of recent hemorrhagic infarction areas in
the right lung. Interestingly, the simultaneous occurrence of acute myocardial
infarction and pulmonary thromboembolism is unusual, leading to diagnostic
difficulties since both conditions present with similar signs and
symptoms.
15
Necropsy
confirmed the presence of chronic aortic valve disease with insufficiency, of
degenerative etiology. There was no infectious endocarditis, consistently with the
clinical diagnosis. On the other hand, we confirmed the presence of infectious
disease, also clinically suspected, established in the right lung as aspiration
bronchopneumonia. Such infection, probably pre-existent, was related to the previous
history of syncope and decreased level of consciousness, because the patient already
presented infectious evidence at admission. There was no neoplastic lesion in the
gastrectomy stump and horseshoe kidney was found during necropsy. Death was caused
by mixed hemodynamic cardiogenic infectious shock. (Dr. Luiz Alberto
Benvenuti)
Section editor: Alfredo José Mansur
(ajmansur@incor.usp.br)
Associated editors: Desidério Favarato
(dclfavarato@incor.usp.br), Vera Demarchi Aiello
(anpvera@incor.usp.br)