Background The effect of severity of presentation on transplant-free survival and recovery of function in paediatric dilated cardiomyopathy (DCM) is unknown.
Methods and Results We report a large single centre experience of 209 new DCM referrals from 2008–2013 (table 1). 82 children median age 1.0 years (IQR 3.4) required intensive care (ICU) and their survival without death or transplant was 51% to one year and 45% to five years. 127 children presented to the paediatric heart failure clinic. Excluding 58 with neuromuscular disease, median age was 4.1 years (IQR 11.3) and survival without death or transplant 85% to 1 year and 50% to 5 years. NT-proBNP normalised in survivors before echocardiographic parameters (Figure 1). Predictors of recovery included younger age, lower NT-proBNP and higher fractional shortening measurement on echocardiogram at presentation.
Conclusion Transplant-free survival to one year is significantly better for patients presenting to clinic, but longer-term survival is better amongst those presenting to ICU due to a late attrition in those with less severe heart failure at presentation (Figure 2). Falling NT- proBNP is the earliest marker of recovery.
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