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CLINICAL RESEARCH

Clinical course of hypertrophiccardiomyopathy with survival to advanced age

Barry J. Maron, MD, FACC^*,*, Susan A. Casey, RN^*, Robert G. Hauser, MD, FACC^* and Dorothee M. Aeppli, PhD

^* Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota, USA
Division of Biostatistics, School of Public Health, University of Minnesota, Minneapolis, Minnesota, USA

Manuscript received January 20, 2003; revised manuscript received April 11, 2003, accepted May 21, 2003.

^* Reprint requests and correspondence: Dr. Barry J. Maron, Minneapolis Heart Institute Foundation, 920 E. 28th Street, Suite 60, Minneapolis, Minnesota 55407, USA.
hcm.maron{at}mhif.org

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.

Abbreviations and Acronyms   HCM   hypertrophic cardiomyopathy   HF   heart failure   LV   left ventricle

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