Published Online
on November 20, 2006

A more recent version of this article appeared on December 12, 2006

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Submitted on April 20, 2006
Revised on September 17, 2006
Accepted on October 11, 2006

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.).

^* To whom correspondence should be addressed. E-mail: robert.weintraub{at}rch.org.au.

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.

Key words: cardiomyopathy • heart failure • myocarditis • pediatrics

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