1st Cardiology department, Laboratory of Cardiomyopathies and Inherited Cardiac Diseases, AHEPA University hospital, Thessaloniki 54636, Greece.
1 Cardiology department, Laboratory of Cardiomyopathies and Inherited Cardiac Diseases, AHEPA University hospital, Thessaloniki 54636, Greece.
Future Cardiol. 2021 Nov;17(8):1395-1405. doi: 10.2217/fca-2020-0230. Epub 2021 Feb 22.
Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease with an autosomal dominant pattern and a reported prevalence of about 0.2%. In this review, we present a simple algorithm for the management of first diagnosed HCM patients. Initially, the clinical examination, medical and detailed family history and the ECG are essential. The etiological diagnosis of left ventricular hypertrophy is important in order to differentiate HCM due to sarcomeric genes mutation from other phenocopies, such as cardiac amyloidosis. The next step consists of the cardiovascular imaging and ambulatory electrocardiography. Cardiopulmonary exercise testing may also be considered if available. All of the above provide evidence for the critical step of the risk stratification of patients for sudden cardiac death. The therapeutic strategy, with respect to obstructive and nonobstructive disease, arrhythmias and end-stage HCM is also described.
肥厚型心肌病（HCM）是最常见的常染色体显性遗传心脏病，其发病率约为 0.2%。在这篇综述中，我们提出了一个用于管理首次诊断为 HCM 患者的简单算法。首先，临床检查、医学和详细的家族史以及心电图是必不可少的。左心室肥厚的病因诊断很重要，以便将肌节基因突变引起的 HCM 与其他表型（如心脏淀粉样变性）区分开来。下一步包括心血管成像和动态心电图监测。如果有条件，也可以考虑进行心肺运动试验。所有这些都为患者发生心源性猝死的风险分层提供了依据。此外，还描述了针对梗阻性和非梗阻性疾病、心律失常和终末期 HCM 的治疗策略。
