TY - JOUR
T1 - Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies
AU - Maron, Barry J.
AU - Rowin, Ethan J.
AU - Casey, Susan A.
AU - Link, Mark S.
AU - Lesser, John R.
AU - Chan, Raymond H.M.
AU - Garberich, Ross F.
AU - Udelson, James E.
AU - Maron, Martin S.
N1 - Publisher Copyright:
© 2015 American College of Cardiology Foundation.
PY - 2015/5/12
Y1 - 2015/5/12
N2 - Background Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%. Objectives This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients. Methods We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45 ± 8 years) over 7.2 ± 5.2 years of follow-up. Results Of 1,000 patients, 918 (92%) survived to 53 ± 9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50 ± 10 years from the following events: progressive heart failure (n = 17); arrhythmic sudden death (SD) (n = 17); and embolic stroke (n = 2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n = 33) or heart transplantation for advanced heart failure (n = 18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p = 0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension. Conclusions In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity.
AB - Background Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%. Objectives This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients. Methods We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45 ± 8 years) over 7.2 ± 5.2 years of follow-up. Results Of 1,000 patients, 918 (92%) survived to 53 ± 9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50 ± 10 years from the following events: progressive heart failure (n = 17); arrhythmic sudden death (SD) (n = 17); and embolic stroke (n = 2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n = 33) or heart transplantation for advanced heart failure (n = 18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p = 0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension. Conclusions In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity.
KW - heart failure
KW - heart transplant
KW - implantable defibrillators
KW - sudden death
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U2 - 10.1016/j.jacc.2015.02.061
DO - 10.1016/j.jacc.2015.02.061
M3 - Article
C2 - 25953744
AN - SCOPUS:84928969105
SN - 0735-1097
VL - 65
SP - 1915
EP - 1928
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 18
ER -