Hypertrophic Cardiomyopathy

The Role of Cardiac MRI in the Diagnosis and Risk Stratification of Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM), the most common genetic cardiomyopathy, is present in one in 500 of the general population and is caused by over 1,400 mutations in at least 11 genes encoding the cardiac sarcomere.1–4 Although the majority of patients with HCM remain asymptomatic with near-normal longevity, a small, but important, subset of patients are at increased risk for a wide range of clinical outcomes including development of advanced heart failure symptoms, atrial and ventricular arrhythmias, thromboembolic events, and even sudden death.5–8

ICD Therapy for Primary Prevention in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM), a genetic sarcomeric disorder associated with myocyte disarray and scar deposition, is intimately linked to sudden cardiac death (SCD) due to malignant ventricular arrhythmias. In the first modern published description of the disease in 1958, Dr Donald Teare describes the case of a 14-year-old male who collapsed while being chased around his school’s playground.1 He was reported to have been having ‘blackout episodes’ for months.

Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes

Arrhythmogenic cardiomyopathy (ACM) is usually referred to as arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).1A first historical description was made in 1736, whereas its first modern description dates back to 1982.2 Initially, ACM was thought to be an embryological malformation.3 Yet in recent years it became evident that the pathophysiology of an ongoing genetically determined myocardial atrophy did not fit the theory of a congenital myocardial aplasia.

Subclinical Atrial Fibrillation in Patients with Hypertrophic Cardiomyopathy

Atrial fibrillation (AF) is the most common arrhythmia in patients with hypertrophic cardiomyopathy (HCM), with a reported prevalence of AF in HCM of about 25 %.1 Patients with AF in HCM tend to be more symptomatic and have an increased stroke risk compared with patients without HCM.2 While the occurrence and treatment of (symptomatic) AF in HCM have been extensively studied, there is little data on the significance, prevalence, and management of subclinical AF in HCM.