Hypertrophic cardiomyopathy (HCM), a sarcomeric disease, is defined as myocardial left ventricular hypertrophy (LVH) in the absence of other cardiac disease. It may be a primary disorder or secondary to metabolic or genetic conditions. HCM is the most common cardiomyopathy responsible for sudden cardiac death in children and young adults. Echocardiography is the primary diagnostic modality and may demonstrate asymmetric septal hypertrophy, concentric LVH, apical hypertrophy or isolated posterior wall hypertrophy. Other diagnostic modalities including tissue Doppler imaging, strain and strain rate imaging, CMR and stress echocardiography may provide additional diagnostic and prognostic information. Differentiation of HCM from athletic heart is important and challenging. Familial screening of the nuclear family is warranted in HCM given the genetic predisposition to the disease.
Authors
McMahon CJ; Ganame J
Book title
Echocardiography in Pediatric and Congenital Heart Disease