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(Circulation. 2006;114:2671-2678.)
© 2006 American Heart Association, Inc.

Pediatric Cardiology

Clinical Features and Outcomes of Childhood Dilated Cardiomyopathy

Results From a National Population-Based Study

Piers E. F. Daubeney, MBBS; Alan W. Nugent, MBBS; Patty Chondros, MSc; John B. Carlin, PhD; Steven D. Colan, MD; Michael Cheung, MB, ChB; Andrew M. Davis, MD; C.W. Chow, MD; Robert G. Weintraub, MBBS, on behalf of the National Australian Childhood Cardiomyopathy Study

From the Departments of Cardiology (P.E.F.D., A.W.N., M.C., A.M.D., R.G.W.) and Anatomic Pathology (C.W.C.), Royal Children’s Hospital, Melbourne, Australia; Clinical Epidemiology and Biostatistics Unit (P.C., J.B.C.), Murdoch Children’s Research Institute, Melbourne, Australia; Departments of General Practice (P.C.) and Paediatrics (J.B.C., C.W.C.), University of Melbourne, Melbourne, Australia; and Department of Cardiology, Children’s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Mass (S.D.C.).

Correspondence to Dr Robert Weintraub, Department of Cardiology, Royal Children’s Hospital, Flemington Rd, Parkville, VIC 3052, Australia. E-mail robert.weintraub{at}rch.org.au

Received April 20, 2006; revision received September 17, 2006; accepted October 11, 2006.

Background— Despite considerable mortality, population-based prognostic factors for childhood dilated cardiomyopathy are lacking.

Methods and Results— A population-based cohort study was undertaken of all children in Australia who presented with cardiomyopathy at age 0 to 10 years between January 1, 1987, and December 31, 1996. A single cardiologist analyzed all cardiac investigations, and a single pathologist analyzed histopathological material. There were 184 subjects with dilated cardiomyopathy. Positive viral identification or lymphocytic myocarditis was found in 30 (68.2%) of 44 cases with available early histology and 8 of 9 cases presenting with sudden death. Freedom from death or transplantation was 72% (95% CI, 65% to 78%) 1 year after presentation and 63% (95% CI, 55% to 70%) at 5 years. By proportional hazards regression analysis, risk factors for death or transplantation comprised age >5 years at presentation (hazard ratio 5.6, 95% CI, 2.6 to 12.0), familial dilated cardiomyopathy (hazard ratio, 2.9; 95% CI, 1.5 to 5.6), lower initial fractional shortening z score (hazard ratio per z-score unit, 0.75; 95% CI, 0.65 to 0.87), and failure to increase fractional shortening z score during follow-up (hazard ratio per unit increase, 0.68; 95% CI, 0.58 to 0.79). At follow-up, 78 (44.6%) of 175 cases diagnosed during life have no symptoms and are not taking any cardiac medication.

Conclusions— Early mortality is high in childhood dilated cardiomyopathy, but the clinical status of long-term survivors is good. This population-based study identifies children at risk of adverse events.

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