The variable natural history of idiopathic dilated cardiomyopathy

Kapil Parakh, Michelle M. Kittleson, Bettina Heidecker, Ilan S. Wittstein, Daniel P. Judge, Hunter C. Champion, Lili A. Barouch, Kenneth L. Baughman, Stuart D. Russell, Edward K. Kasper, Kranthi K. Sitammagari, Joshua M. Hare

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


Background: Determining the prognosis of patients with heart failure is essential for patient management and clinical trial conduct. The relative value of traditional prognostic criteria remains unclear and the assessment of long-term prognosis for individual patients is problematic. Objectives: To determine the ability of clinical, hemodynamic and echocardiographic parameters to predict the long-term prognosis of patients with idiopathic dilated cardiomyopathy. methods: We investigated the ability of clinical, hemo-dynamic and echocardiographic parameters to predict the long-term prognosis of individual patients in a large, representative, contemporary cohort of idiopathic dilated cardiomyopathy (IDCM) patients referred to Johns Hopkins from 1997 to 2004 for evaluation of cardiomyopathy. In all patients a baseline history was taken, and physical examination, laboratory studies, echocardiogram, right heart catheterization and endomyocardial biopsy were performed. results: In 171 IDCM patients followed for a median 3.5 years, there were 50 long-term event-free survivors (LTS) (median survival 6.4 years) and 34 patients died or underwent ventricular assist device placement or transplantation within 5 years (NLTS; non-long-term survivors) (median time to event 1.83 years. Established risk factors (gender, race, presence of diabetes, serum creatinine, sodium) and the use of accepted heart failure medications (angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta blockers) were similar between the two groups. Although LTS had younger age, higher ejection fraction (EF) and lower New York Heart Association (NYHA) class at presentation, the positive predictive value of an EF < 25% was 64% (95% CI 41%-79%) and that of NYHA class > 2 was 53% (95% CI 36-69%). A logistic model incorporating these three variables incorrectly classifed 29% of patients. conclusions: IDCM exhibits a highly variable natural history and standard clinical predictors have limited ability to classify IDCM patients into broad prognostic categories. These findings suggest that there are important host-environmental factors still unappreciated in the biology of IDCM.

Original languageEnglish (US)
Pages (from-to)666-671
Number of pages6
JournalIsrael Medical Association Journal
Issue number11
StatePublished - Dec 13 2012


  • Cardiomyopathy
  • Congestive heart failure
  • Epidemiology
  • Prognosis

ASJC Scopus subject areas

  • Medicine(all)


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