Heart failure from heart muscle disease in childhood: a 5–10 year follow‐up study in the UK and Ireland

AIMS: Our original study, the first national prospective study of new‐onset heart failure from heart muscle disease in children, showed overall 1‐year survival of 82%, and event (death or transplantation)‐free survival of 66%. This study aimed to evaluate 5 + year outcomes of this important cohort. METHODS AND RESULTS: All centres in the UK and Ireland with 1‐year event‐free survivors participated (n = 14). Anonymised data based on last hospital attendance and echocardiograms were reviewed. The investigator was blinded to outcome at the time of echo review. Of sixty‐nine 1‐year event‐free survivors, data were obtained on 64, with three lost to follow‐up and two moved abroad. There were three deaths at 2.2, 3.3 and 9.0 years after presentation and one transplant, at 5.2 years. Overall/event‐free survival was 77%/62% at 5 years and 73%/59% at 10 years, respectively. Overall and event‐free survival conditional on 1‐year survival was 94% at 5 years, and 89% at 10 years. For the 60 event‐free survivors, median (range) follow‐up duration was 9.04 (5.0–10.33) years for those still under review (n = 45), or time to discharge 5.25 (0.67–10.0) years (n = 15). Fifty‐eight were in New York Heart Association (NYHA) Class 1, and two in Class 2. Forty‐one out of sixty had normal echocardiograms at last follow‐up. Predictors of better longer‐term outcome were the same as for the original 1‐year follow‐up study, namely, younger age and higher fractional shortening measurement at presentation. CONCLUSIONS: Children who survive the first year following their first presentation with significant heart failure from heart muscle disease have a good longer‐term outcome although there remains a small attrition rate.