Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death

摘要: Hypertrophic cardiomyopathy (HCM) is an inherited cardiac muscle disorder disease that affects sarcomeric proteins, resulting in small vessel disease, myocyte and myofibrillar disorganisation, fibrosis with or without myocardial hypertrophy. These features may result significant symptoms are a potential substrate for arrhythmias. Before the identification of causing genes World Health Organization defined HCM as presence left biventricular hypertrophy absence any systemic cause.w1 When these criteria applied to western population estimated prevalence approximately 1 500.1w2 Morphological evidence ventricular hypertrophy, however, be absent up 20% gene carriers.w3 Adults often asymptomatic but their mortality rate nonetheless high 1–2% per annum.2w4 This article will present natural history relate it need medical intervention alleviate prevent sudden death. The expression age related, occurring during soon after periods rapid somatic growth. Detectable cardiovascular abnormalities usually develop adolescence.w5 For this reason regular evaluation offspring carriers puberty early adulthood necessary diagnosis risk stratification. has been described infants young children data limited. Children diagnosed before 14 years have worse prognosis once they reach adolescence 2–4% annual incidence death.3 The development clinical elderly associated myosin binding protein C (MyBPC) mutations.w6 Although MyBPC appears benign presentation later decades, develops patients at all recognised complications including arrhythmia, stroke, …