Published Online
on August 22, 2005

A more recent version of this article appeared on August 30, 2005

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Submitted on December 16, 2004
Revised on May 1, 2005
Accepted on May 31, 2005

Clinical Features and Outcomes of Childhood Hypertrophic Cardiomyopathy. Results From a National Population-Based Study

Alan W. Nugent MBBS, Piers E.F. Daubeney MBBS, Patty Chondros MSc, John B. Carlin PhD, Steven D. Colan MD, Michael Cheung MBChB, Andrew M. Davis MD, C. W. Chow MD, Robert G. Weintraub MBBS^*, for the National Australian Childhood Cardiomyopathy Study

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

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

Background--Population-based studies have provided insight into the natural history of adult hypertrophic cardiomyopathy, but comparable information for affected children is lacking.

Methods and Results--All Australian children who presented with primary cardiomyopathy at 0 to 10 years of age between January 1, 1987, and December 31, 1996, were enrolled in a longitudinal cohort study. A single cardiologist reviewed serial cardiac investigations on each subject. A total of 80 subjects with hypertrophic cardiomyopathy were identified. An underlying syndromal, genetic, or metabolic condition was identified in 46 subjects (57.5%). There were no cases of sudden death at presentation. Left ventricular outflow tract obstruction was present in 32 subjects (40%); right ventricular outflow obstruction was present in 10 (12.5%). Freedom from death or transplantation was 83% (95% CI, 73 to 90) 5 years after presentation and 76% (95% CI, 62 to 86) 10 years after presentation. By proportional-hazards regression analysis, risk factors for death or transplantation included concentric left ventricular hypertrophy, age at presentation <1 year, lower initial fractional shortening Z score, and increasing left ventricular posterior wall thickness relative to body surface area. At the latest follow-up, 54 of 65 surviving subjects had no symptoms, and 46 were receiving no regular medication.

Conclusions--Syndromal, genetic, and metabolic causes predominate in children with hypertrophic cardiomyopathy. Ventricular outflow tract obstruction is common. The clinical status of long-term survivors is good. This population-based study identifies children with hypertrophic cardiomyopathy who are at risk of adverse events.

Key words: cardiomyopathy • pediatrics • sudden death

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