A simple algorithm for a clinical step-by-step approach in the management of hypertrophic cardiomyopathy

Abstract

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. Lay Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disease characterized by the unexplained increase and growth of muscle cells of the walls of the primary cardiac chamber (left ventricle of the heart). It is a common cardiac condition that affects one in 500 individuals. Its symptoms vary from a completely asymptomatic patient to its most devastating event, sudden death (SD). In this review, we summarize a simple approach for the evaluation and management of patients with HCM. The clinical examination (i.e., heart and lung auscultation), medical history and the ECG are essential. The patient's family history should be carefully assessed in order to identify relatives with cardiac diseases and/or SD. The next step consists of the imaging of the heart and the identification of significant arrhythmias (problems with the rate or rhythm of the heartbeat). All of the above provide evidence for the separation of patient populations into different risk groups for SD. The medical and interventional management of patients with HCM is also described.