New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland.
BACKGROUND
We undertook the first prospective, national, multicenter study to describe the incidence and outcome of heart muscle disease-induced heart failure in children.


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.
Andrews
Rachel E
RE
Department of Congenital Heart Disease, Evelina Children's Hospital, Guys' and St Thomas' NHS Trust, London, United Kingdom.
Fenton
Matthew J
MJ
Ridout
Deborah A
DA
Burch
Michael
M
D000293:Adolescent; D000367:Age Factors; D009202:Cardiomyopathies / Q000150:complications* / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003625:Data Collection; D006333:Heart Failure / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D016027:Heart Transplantation; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007494:Ireland; D009205:Myocarditis / Q000150:complications; D009206:Myocardium / Q000473:pathology*; D011446:Prospective Studies; D016019:Survival Analysis; D013599:Systole; D016896:Treatment Outcome; D006113:United Kingdom