Abstract

Adrenergic receptor (ADR) genotypes are associated with heart failure (HF) and ß-blocker response in adults. We assessed the influence of ADR genotypes in children with dilated cardiomyopathy (DCM).Ninety-one children with advanced DCM and 44 with stable DCM were genotyped for three ADR genotypes associated with HF risk in adults: a2cdel322-325, ß1Arg389, and ß2Arg16. Data were analyzed by genotype and ß-blocker use. Mean age at enrollment was 8.5 y.One-year event-free survival was 51% in advanced and 80% in stable DCM. High-risk genotypes were associated with higher left ventricular (LV) filling pressures, higher systemic and pulmonary vascular resistance, greater decline in LV ejection fraction (P < 0.05), and a higher frequency of mechanical circulatory support while awaiting transplant (P = 0.05). While ß-blockers did not reduce HF severity in the overall cohort, in the subset with multiple high-risk genotypes, those receiving ß-blockers showed better preservation of cardiac function and hemodynamics compared with those not receiving ß-blockers (interaction P < 0.05).Our study identifies genetic risk markers that may help in the identification of patients at risk for developing decompensated HF and who may benefit from early institution of ß-blocker therapy before progression to decompensated HF.

View details for DOI 10.1038/pr.2014.183

View details for PubMedID 25406899

View details for PubMedCentralID PMC4298011