超声心动图诊断阻塞性肥厚性心肌病的应用

Jennifer Taylor
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摘要

来自德国柏林Sana Klinikum的Fabian Knebel和德国柏林慈善校园Mitte的心脏病和血管学部门以肥厚性心肌病(HCM)的定义开启了播客,其特征是左心室肥厚。然后,Knebel描述了这种情况的症状,如呼吸急促和体力消耗后的晕厥。每400人中就有一人携带可导致HCM的基因突变,如果不及时诊断,这是一种危险的疾病,可能导致室性心动过速和心源性猝死,或终末期心力衰竭。HCM是一种遗传疾病,在不明原因死亡的情况下应进行基因检测,以便在诊断出HCM时对家庭进行检测。超声心动图是HCM患者首选的成像方法,因为其相对较低的成本和广泛的可用性,但心脏MRI也可用于测量左心室壁厚度,纤维化和左心室流出道(LVOT)阻塞,并评估治疗的成功,如室间隔肌切除术。当使用超声心动图时,提示HCM的第一个明显发现是左心室壁增厚,通常在室间隔。欧洲和美国的指南一致认为舒张末期左室壁厚度≥15mm应考虑为HCM。Knebel就如何有效地进行超声心动图提供了建议,包括使用尖顶切面,以及在Valsalva操作期间测量LVOT的压力梯度。一个病例研究提出了病人的症状和体征提示心肌梗死,显示诊断HCM的潜在复杂性。Knebel总结了目前的治疗选择,如室间隔缩小手术和室间隔分支消融,并指出在不久的将来会有药物来减少LVOT阻塞和缓解症状。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Use of Echocardiography to Diagnose Obstructive Hypertrophic Cardiomyopathy
Fabian Knebel, Sana Klinikum, Berlin, Germany, and Department of Cardiology and Angiology, Charité Campus Mitte, Berlin, Germany, opened the podcast with a definition of hypertrophic cardiomyopathy (HCM), which is characterised by left ventricular hypertrophy. Knebel then described the symptoms of the condition, such as shortness of breath and syncope after physical exertion. Up to one in 400 persons carry the genetic mutations that can lead to HCM, which is a dangerous condition if left undiagnosed, potentially leading to ventricular tachyarrhythmias and sudden cardiac death, or end-stage heart failure. HCM is a genetic condition, and genetic testing should be performed in cases of unexplained death so that families can be tested if HCM is diagnosed. Echocardiography is the first imaging method of choice for patients with HCM due to its relatively low cost and wide availability, but cardiac MRI may also be performed to measure left ventricular wall thickness, fibrosis, and left ventricular outflow tract (LVOT) obstruction, and to evaluate the success of therapies such as septal myectomy. When using echocardiography, the first obvious finding to indicate HCM is a thickened left ventricular wall, usually in the interventricular septum. European and American guidelines agree that an end-diastolic left ventricular wall thickness of ≥15 mm should be considered HCM. Knebel provided advice on how to effectively perform echocardiography, including using apical cut planes, and measuring the pressure gradient in the LVOT during the Valsalva manoeuvre. A case study was presented of a patient presenting with signs and symptoms suggestive of myocardial infarction, demonstrating the potential complexity of diagnosing HCM. Knebel concluded with a summary of current therapeutic options, such as septal reduction surgery and septal branch ablation, and pointed out that in the near future there will be medications to reduce LVOT obstruction and alleviate symptoms.
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