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      Speckle tracking echocardiography to assess regional ventricular function in patients with apical hypertrophic cardiomyopathy

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          Abstract

          AIM

          To explore regional systolic strain of midwall and endocardial segments using speckle tracking echocardiography in patients with apical hypertrophic cardiomyopathy (HCM).

          METHODS

          We prospectively assessed 20 patients (mean age 53 ± 16 years, range: 18-81 years, 10 were male), with apical HCM. We measured global longitudinal peak systolic strain (GLPSS) in the midwall and endocardium of the left ventricle.

          RESULTS

          The diastolic thickness of the 4 apical segments was 16.25 ± 2.75 mm. All patients had a normal global systolic function with a fractional shortening of 50% ± 8%. In spite of supernormal left ventricular (LV) systolic function, midwall GLPSS was decreased in all patients, more in the apical (-7.3% ± -8.8%) than in basal segments (-15.5% ± -6.93%), while endocardial GLPPS was significantly greater and reached normal values (apical: -22.8% ± -7.8%, basal: -17.9% ± -7.5%).

          CONCLUSION

          This study shows that two-dimensional strain was decreased mainly confined to the mesocardium, while endocardium myocardial deformation was preserved in HCM and allowed to identify subclinical LV dysfunction. This transmural heterogeneity in systolic strain had not been previously described in HCM and could be explained by the distribution of myofibrillar disarray in deep myocardial areas. The clinical application of this novel finding may help further understanding of the pathophysiology of HCM.

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          Most cited references20

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          Two-dimensional strain-a novel software for real-time quantitative echocardiographic assessment of myocardial function.

          We sought to assess the feasibility of 2-dimensional strain, a novel software for real-time quantitative echocardiographic assessment of myocardial function. Conventional and a novel non-Doppler-based echocardiography technique for advanced wall-motion analysis were performed in 20 patients with myocardial infarction and 10 healthy volunteers from the apical views. Two-dimensional strain is on the basis of the estimation that a discrete set of tissue velocities are present per each of many small elements on the ultrasound image. This software permits real-time assessment of myocardial velocities, strain, and strain rate. These parameters were also compared with Doppler tissue imaging measurements in 10 additional patients. In all, 80.3% of infarct and 97.8% of normal segments could be adequately tracked by the software. Peak systolic strain, strain rate, and peak systolic myocardial velocities, calculated from the software, were significantly higher in the normal than in the infarct segments. In the 10 additional patients, velocities, strain, and strain rate obtained with the novel software were not significantly different from those obtained with Doppler tissue imaging. Two-dimensional strain can accomplish real-time wall-motion analysis, and has the potential to become a standard for real-time automatic echocardiographic assessment of cardiac function.
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            Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy.

            We sought to ascertain whether myocardial scarring occurs in living unselected patients with hypertrophic cardiomyopathy (HCM). Myocardial scarring is known to occur in select HCM patients, who were highly symptomatic prior to death or who died suddenly. The majority of HCM patients, however, are minimally symptomatic and have not suffered sudden death. Cine and gadolinium-enhanced magnetic resonance imaging was performed in 21 HCM patients who were predominantly asymptomatic. Gadolinium hyperenhancement was assumed to represent myocardial scar, and the extent of scar was compared to left ventricular (LV) morphology and function. Scarring was present in 17 patients (81%). Scarring occurred only in hypertrophied regions (> or =10 mm), was patchy with multiple foci, and predominantly involved the middle third of the ventricular wall. All 17 patients had scarring at the junction of the interventricular septum and the right ventricular (RV) free wall. On a regional basis, the extent of scarring correlated positively with wall thickness (r = 0.36, p < 0.0001), and inversely with wall thickening (r = -0.21, p < 0.0001). On a per patient basis, the extent of scarring (mean, 8 +/- 9% of LV mass) was minimally related to maximum wall thickness (r = 0.40, p = 0.07) and LV mass (r = 0.33, p = 0.15), and correlated inversely with ejection fraction (r = -0.46, p = 0.04). Myocardial scarring is common in asymptomatic or mildly symptomatic HCM patients who have not suffered sudden death. When present, scarring occurs in hypertrophied regions, is consistently localized to the junctions of the septum and RV free wall, and correlates positively with regional hypertrophy and inversely with regional contraction.
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              Expression profiling of cardiac genes in human hypertrophic cardiomyopathy: insight into the pathogenesis of phenotypes.

              The goal of this study was to identify genes upregulated in the heart in human patients with hypertrophic cardiomyopathy (HCM). Hypertrophic cardiomyopathy is a genetic disease caused by mutations in contractile sarcomeric proteins. The molecular basis of diverse clinical and pathologic phenotypes in HCM remains unknown. We performed polymerase chain reaction-select complementary DNA subtraction between normal hearts and hearts with HCM and screened subtracted libraries by Southern blotting. We sequenced the differentially expressed clones and performed Northern blotting to detect increased expression levels. We screened 288 independent clones, and 76 clones had less than twofold increase in the signal intensity and were considered upregulated. Sequence analysis identified 36 genes including those encoding the markers of pressure overload-induced ("secondary") cardiac hypertrophy, cytoskeletal proteins, protein synthesis, redox system, ion channels and those with unknown function. Northern blotting confirmed increased expression of skeletal muscle alpha-actin (ACTA1), myosin light chain 2a (MLC2a), GTP-binding protein Gs-alpha subunit (GNAS1), NADH ubiquinone oxidoreductase (NDUFB10), voltage-dependent anion channel 1 (VDAC1), four-and-a-half LIM domain protein 1 (FHL1) (also known as SLIM1), sarcosin (SARCOSIN) and heat shock 70kD protein 8 (HSPA8) by less than twofold. Expression levels of ACTA1, MLC2a and GNAS1 were increased in six additional and FHL1 in four additional hearts with HCM. A diverse array of genes is upregulated in the heart in human patients with HCM, which could account for the diversity of clinical and pathologic phenotypes. Markers of secondary hypertrophy are also upregulated, suggesting commonality of pathways involved in HCM and the acquired forms of cardiac hypertrophy. Elucidation of the role of differentially expressed genes in HCM could provide for new therapeutic targets.
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                Author and article information

                Journal
                World J Cardiol
                WJC
                World Journal of Cardiology
                Baishideng Publishing Group Inc
                1949-8462
                26 April 2017
                26 April 2017
                : 9
                : 4
                : 363-370
                Affiliations
                María Cristina Saccheri, Tomás Francisco Cianciulli, Luis Alberto Morita, Ricardo José Méndez, Martín Alejandro Beck, Juan Enrique Guerra, Alberto Cozzarin, Luciana Jimena Puente, Lorena Romina Balletti, Jorge Alberto Lax, Division of Cardiology, Echocardiography Laboratory, Hospital “Dr. Cosme Argerich”, C1155AHD Buenos Aires, Argentina
                Tomás Francisco Cianciulli, Researcher of the Ministry of Health, Government of the City of Buenos Aires, Buenos Aires C1155AHD, Argentina
                Author notes

                Author contributions: Saccheri MC, Cozzarin A and Puente LJ attended the patient; Cianciulli TF, Beck MA and Lax JA prepared the manuscript and figures; Saccheri MC, Cianciulli TF and Lax JA performed the echocardiographic images and participated in the manuscript description; Morita LA, Méndez RJ, Guerra JE and Balletti LR participated in the design and review of the manuscript; all authors read and approved the final manuscript.

                Correspondence to: Tomás Francisco Cianciulli, MD, FACC, FASE, Division of Cardiology, Echocardiography Laboratory, Hospital “Dr. Cosme Argerich”, Pi y Margall 750, Ciudad Autó­noma de Buenos Aires, Buenos Aires C1155AHD, Argentina. tcianciulli@ 123456gmail.com

                Telephone: +54-11-41210879 Fax: +54-11-41210875

                Article
                jWJC.v9.i4.pg363
                10.4330/wjc.v9.i4.363
                5411971
                28515855
                0b094ade-d2cb-4af1-bb5a-13ba52511b8f
                ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.

                Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

                History
                : 25 September 2016
                : 30 December 2016
                : 14 January 2017
                Categories
                Observational Study

                apical hypertrophic cardiomyopathy,two-dimensional strain,speckle tracking,endocardium,midwall,regional myocardial systolic function

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