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Management strategies in hypertrophic cardiomyopathy: influence of age and morphology

W.J. McKenna*, S. Sharma and P.M. Elliott

Department of Cardiological Sciences, St George's Hospital Medical School, London, U.K.

* Correspondence: William J. McKenna, MD, Department of Cardiological Sciences, St George's Hospital Medical School, Cranmer Terrace, London SW17 ORE, U.K.

Abstract

Management in hypertrophic cardiomyopathy is aimed at improving symptoms and exercise capacity and preventing complications, particularly sudden death. The strategy for management of symptoms is to identify potentially important mechanisms at which treatment can be targeted. Assessment is feasible with non-invasive evaluation. All patients with hypertrophic cardiomyopathy should undergo risk assessment for sudden death, regardless of their symptomatic, morphological and haemodynamic status. Recent prospective studies have examined five risk markers: family history of sudden death (two or more members aged <45 years), unexplained syncope, non-sustained ventricular tachycardia on Holler monitoring, abnormal exercise blood pressure response and severe left ventricular hypertrophy (≥3 cm). The majority of patients had none of these risk factors and did not die suddenly. Annual sudden death rates were at least 2% among patients with two or more of these risk markers, which warrants consideration of prophylactic treatment (amiodarone, implantable cardioverter-defibrillator) in such patients.

Key Words: children • hypertrophic cardiomyopathy • hypertrophy • infants • sudden death


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