Underlying Causes and Long-Term Survival in Patients with Initially Unexplained Cardiomyopathy
G. Michael Felker, M.D., Richard E. Thompson, Ph.D., Joshua M. Hare, M.D., Ralph H. Hruban, M.D., Diedre E. Clemetson, David L. Howard, Kenneth L. Baughman, M.D., and Edward K. Kasper, M.D.
Background Previous studies of the prognosis of patients withheart failure due to cardiomyopathy categorized patients accordingto whether they had ischemic or nonischemic disease. The prognosticvalue of identifying more specific underlying causes of cardiomyopathyis unknown.
Methods We evaluated the outcomes of 1230 patients with cardiomyopathy.The patients were grouped into the following categories accordingto underlying cause: idiopathic cardiomyopathy (616 patients);peripartum cardiomyopathy (51); and cardiomyopathy due to myocarditis(111), ischemic heart disease (91), infiltrative myocardialdisease (59), hypertension (49), human immunodeficiency virus(HIV) infection (45), connective-tissue disease (39), substanceabuse (37), therapy with doxorubicin (15), and other causes(117). Cox proportional-hazards analysis was used to assessthe association between the underlying cause of cardiomyopathyand survival.
Results During a mean follow-up of 4.4 years, 417 patients diedand 57 underwent cardiac transplantation. As compared with thepatients with idiopathic cardiomyopathy, the patients with peripartumcardiomyopathy had better survival (adjusted hazard ratio fordeath, 0.31; 95 percent confidence interval, 0.09 to 0.98),and survival was significantly worse among the patients withcardiomyopathy due to infiltrative myocardial disease (adjustedhazard ratio, 4.40; 95 percent confidence interval, 3.04 to6.39), HIV infection (adjusted hazard ratio, 5.86; 95 percentconfidence interval, 3.92 to 8.77), therapy with doxorubicin(adjusted hazard ratio, 3.46; 95 percent confidence interval,1.67 to 7.18), and ischemic heart disease (adjusted hazard ratio,1.52; 95 percent confidence interval, 1.07 to 2.17).
Conclusions The underlying cause of heart failure has prognosticvalue in patients with unexplained cardiomyopathy. Patientswith peripartum cardiomyopathy appear to have a better prognosisthan those with other forms of cardiomyopathy. Patients withcardiomyopathy due to infiltrative myocardial diseases, HIVinfection, or doxorubicin therapy have an especially poor prognosis.
Source Information
From the Division of Cardiology, Department of Medicine (G.M.F., J.M.H., D.E.C., D.L.H., K.L.B., E.K.K.), and the Department of Pathology (R.H.H.), Johns Hopkins University School of Medicine; and the Department of Biostatistics, Johns Hopkins School of Public Health and Hygiene (R.E.T.) both in Baltimore.
Address reprint requests to Dr. Kasper at the Division of Cardiology, Johns Hopkins Hospital, Carnegie 568, 600 N. Wolfe St., Baltimore, MD 21287-6568, or at ekasper{at}jhmi.edu.
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