肥厚性心肌病的超声心动图特征-一项单中心观察研究

A. Islam, D. Adhikary, Shovan Rahman, Mohsin Ahmed, Md. Toufiqur Rahman, M. Ullah, A. Majumder
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摘要

背景:肥厚性心肌病(HCM)是左心室肥厚(LVH)的常染色体显性遗传病。表型表达差异很大,从亚临床肥厚到引起左心室流出道(LVOT)阻塞的严重不对称间隔肥厚。除了遗传和表型异质性外,不同类型HCM的患病率可能具有地理和种族差异。方法:本观察性研究于2010年至2020年期间进行,以确定孟加拉国人群HCM的超声心动图特征。所有在孟加拉国达卡一家私人咨询中心接受经胸超声心动图(TTE)检查的患者均被纳入研究。HCM被定义为在没有其他肥厚原因的情况下,在左心室(LV)任何部位存在最大舒张末期壁厚e”15mm。HCM根据心肌肥厚、有无LVOT或左室中腔梗阻进一步分类。结果:76例HCM中,以非梗阻性HCM最常见(65.8%),其次为左室梗阻(13.2%)、左室中腔梗阻(10.5%)和根尖型HCM(10.5%)。不对称室间隔肥厚(ASH)占42.1%,二尖瓣前小叶收缩前运动(SAM)占14.5%,二尖瓣反流(MR)占50%,左室收缩功能障碍占5.3%,肺动脉收缩压升高(PASP)占15.8%。76例中66例最大左室壁厚≥30 mm。结论:该研究强调了基于常规超声心动图的孟加拉国人群HCM的临床有用概况。进一步的临床研究,新的超声心动图模式和遗传分析是必要的,以发现该种族的其他信息。心血管病杂志2021;第5 - 11 (1):14
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Echocardiographic Profile of Hypertrophic Cardiomyopathy – A Single-Centre, Observational study
Background: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease of left ventricular hypertrophy (LVH). Phenotypic expression varies widely from subclinical hypertrophy to gross asymmetric septal hypertrophy causing left ventricular outflow tract (LVOT) obstruction. On top of genetic and phenotypic heterogeneity, the prevalence of different types of HCM may have geographical, as well as, ethnic variation. Methods: This observational study was carried out during 2010 to 2020 to determine the echocardiographic profile of HCM in Bangladeshi population. All patients undergoing transthoracic echocardiography (TTE) in a private consultation centre of Dhaka, Bangladesh were included. HCM was defined as the presence of a maximal end-diastolic wall thickness of e”15 mm anywhere in the left ventricle (LV), in the absence of another cause of hypertrophy in adults. HCM was further classified according to the pattern of myocardial hypertrophy and presence or absence of LVOT, or mid-left ventricular cavity obstruction. Results: Out of 76 cases, non-obstructive HCM was the commonest type (65.8%), followed by HCM causing LVOT obstruction (13.2%), HCM causing mid-LV cavity obstruction (10.5%), and the apical variety ( 10.5%). Asymmetric septal hypertrophy (ASH) was found in 42.1%, systolic anterior motion (SAM) of anterior mitral leaflet (AML) in 14.5%, mitral regurgitation (MR) in 50%, left ventricular systolic dysfunction in 5.3%, and raised pulmonary artery systolic pressure (PASP) in 15.8% of cases. Maximum LV wall thickness ≥30 mm was found in 66 out of 76 cases. Conclusion: The study highlights the clinically useful profile of HCM in Bangladeshi population based on conventional echocardiography. Further studies involving clinical, newer echocardiographic modalities and genetic analyses are warranted to discover the additional information in this ethnicity. Cardiovasc j 2021; 14(1): 5-11
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