Clinical course of hypertrophiccardiomyopathy with survival to advanced age

Objectives This study was designed to clarify and resolve the clinical profile of older patients with hypertrophic cardiomyopathy (HCM). Background Adverse consequences of HCM such as sudden death and incapacitating symptoms have been emphasized for the young and middle-aged. Methods Long-term outcome of HCM was assessed in a community-based cohort not subject to tertiary center referral bias. Results Of 312 patients, 73 (23%) achieved normal life expectancy (≥75 years; range to 96); 44 (14%) were ≥80 years old. Most patients ≥75 years (47; 64%) experienced no or only mild limiting symptoms and lived virtually their entire lives with few HCM-related clinical consequences; 26 patients (36%) experienced severe progressive symptoms. In elderly patients with HCM, diagnosis and symptom onset were considerably delayed to 74 ± 8 and 70 ± 11 years, respectively. For patients ≥50 years at diagnosis, the probability of survival for 5, 10, and 15 years was 85 ± 3%, 74 ± 4%, and 57 ± 6%, respectively, and did not significantly differ from a matched general population (p = 0.20). Patients ≥75 years were predominantly women, and had less marked wall thickness and more frequently showed basal outflow obstruction ≥30 mm Hg (compared with those <75 years; p < 0.01 and 0.001, respectively). Conclusions Hypertrophic cardiomyopathy is frequently well tolerated and compatible with normal life expectancy, and may remain clinically dormant for long periods of time with symptoms and initial diagnosis deferred until late in life. These observations afford a measure of reassurance to many patients with HCM, a disease for which clinical course is often unfavorable and unpredictable.