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(Circulation. 2008;117:79-84.)
© 2008 American Heart Association, Inc.

Pediatric Cardiology

New-Onset Heart Failure Due to Heart Muscle Disease in Childhood

A Prospective Study in the United Kingdom and Ireland

Rachel E. Andrews, MRCPCH; Matthew J. Fenton, MRCPCH; Deborah A. Ridout, MSc; Michael Burch, MD, FRCP, FRCPCH, on Behalf of the British Congenital Cardiac Association

From the Department of Congenital Heart Disease, Evelina Children’s Hospital, Guys’ and St Thomas’ NHS Trust (R.E.A.); the Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust (M.J.F., M.B.); and the Centre for Paediatric Epidemiology and Biostatistics, Institute of Child Health (D.A.R.); all in London, United Kingdom.

Correspondence to Dr Michael Burch, Cardiothoracic Unit, Great Ormond Street Hospital for Children, Great Ormond St, London WC1N, United Kingdom. E-mail burchm{at}gosh.nhs.uk

Received October 21, 2006; accepted October 19, 2007.

Background— We undertook the first prospective, national, multicenter study to describe the incidence and outcome of heart muscle disease–induced heart failure in children.

Methods and Results— Data were collected on patients admitted to a hospital through 2003 with a first episode of heart failure in the absence of congenital heart disease. All 17 pediatric cardiac centers in the United Kingdom and Ireland participated. Follow-up data were obtained to a minimum of 1 year. The incidence was 0.87/100 000 population <16 years (n=104; 53 girls; 95% confidence interval 0.71 to 1.05 per 100 000). Median age at presentation was 1 year, with 82% in New York Heart Association class III to IV. Causes of heart failure included dilated cardiomyopathy (50 idiopathic, 8 familial), probable myocarditis (23), occult arrhythmia (7), anthracycline toxicity (5), metabolic disease (4), left ventricular noncompaction (3), and other (4). Overall 1-year survival was 82%, and event (death or transplantation)-free survival was 66%. Regression analysis showed older age and reduced systolic function on admission echocardiogram increased the event risk. Only 8% of event-free survivors (n=69) remained in New York Heart Association class III to IV, but 35 required readmission during the study period, and all but 8 remained on medication.

Conclusions— This first national prospective study of new-onset heart failure in children has shown an incidence of 0.87/100 000. Multivariable analysis of survival data indicates a better outcome for younger children and for those with better systolic function at presentation, but overall, one third of children die or require transplantation within 1 year of presentation.

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CLINICAL PERSPECTIVE

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