Published Online
on December 17, 2007

A more recent version of this article appeared on January 1, 2008

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Submitted on October 21, 2006
Accepted on October 19, 2007

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.

^* To whom correspondence should be addressed. E-mail: burchm{at}gosh.nhs.uk.

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.

Key words: cardiomyopathy • myocarditis • morbidity • mortality • transplantation

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