PMID- 23240370 OWN - NLM STAT- MEDLINE DCOM- 20130108 LR - 20131121 IS - 1565-1088 (Print) VI - 14 IP - 11 DP - 2012 Nov TI - The variable natural history of idiopathic dilated cardiomyopathy. PG - 666-71 AB - 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, hemodynamic 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 classified 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. FAU - Parakh, Kapil AU - Parakh K AD - Department of Medicine, Johns Hopkins Bayview Medical Center, Baltimore, MD, USA. FAU - Kittleson, Michelle M AU - Kittleson MM FAU - Heidecker, Bettina AU - Heidecker B FAU - Wittstein, Ilan S AU - Wittstein IS FAU - Judge, Daniel P AU - Judge DP FAU - Champion, Hunter C AU - Champion HC FAU - Barouch, Lili A AU - Barouch LA FAU - Baughman, Kenneth L AU - Baughman KL FAU - Russell, Stuart D AU - Russell SD FAU - Kasper, Edward K AU - Kasper EK FAU - Sitammagari, Kranthi K AU - Sitammagari KK FAU - Hare, Joshua M AU - Hare JM LA - eng PT - Comparative Study PT - Journal Article PL - Israel TA - Isr Med Assoc J JT - The Israel Medical Association journal : IMAJ JID - 100930740 RN - AYI8EX34EU (Creatinine) SB - IM MH - Biopsy MH - Cardiac Catheterization MH - Cardiomyopathy, Dilated/blood/*diagnosis/physiopathology MH - Creatinine/blood MH - Diagnosis, Differential MH - Echocardiography MH - Female MH - Follow-Up Studies MH - *Hemodynamics MH - Humans MH - Male MH - Middle Aged MH - Myocardium/*pathology MH - Prognosis MH - Retrospective Studies MH - Severity of Illness Index EDAT- 2012/12/18 06:00 MHDA- 2013/01/09 06:00 CRDT- 2012/12/18 06:00 PHST- 2012/12/18 06:00 [entrez] PHST- 2012/12/18 06:00 [pubmed] PHST- 2013/01/09 06:00 [medline] PST - ppublish SO - Isr Med Assoc J. 2012 Nov;14(11):666-71.