PubMed2002
BACKGROUND: The clinical course of hypertrophic cardiomyopathy (HCM) is highly variable. Patients may remain asymptomatic throughout life, develop progressive and ultimately fatal heart failure, or die prematurely due to sudden death. This article reviews Western and Asian literature on HCM, including recent studies of large, regionally based cohorts that reflect the full spectrum of disease. The intent is to estimate HCM-related morbidity and mortality rates in the general population and identify risk factors for life and living benefits insurance. RESULTS: Clinical studies have identified risk factors that are associated with a higher incidence of sudden death, such as non-sustained ventricular tachycardia, left ventricular wall thickness, and family history of sudden death. For many of these factors, there was a nonlinear relationship between mortality risk and the number of risk factors (ie, each additional risk factor was associated with a disproportionately greater risk of sudden death). Natural history studies of patients with HCM reported total HCM-related mortality rates of 1.8% per year for ages 5 to 15 years, 1.0% to 1.5% for ages 16 to 65, 3.9% for ages 66 to 75, and 4.7% for ages above 75 years. Sudden death rates varied from 0.5% to 1.5% per year for most age groups, with children and adolescents experiencing sudden death rates near the top of this range. With regard to morbidity, patients progressed from New York Heart Association functional class I or II to III/IV at a rate of 1% to 2% per year, with higher progression rates in people aged 65 years or older. Risk varied with genotype and was generally similar in Western and Asian populations. CONCLUSION: Despite improvements in diagnosis and treatment, HCM is still associated with considerable morbidity and mortality.