Clinical course and risk profile in adolescents with idiopathic dilated cardiomyopathy

Abstract

We sought to analyze the prevalence, clinical course, and risk profile of left ventricular systolic dysfunction in adolescents with idiopathic dilated cardiomyopathy. Patients with clinical onset at <13 years (n = 18) or >19 years (n = 14) of age and/or patients followed up for <12 months were excluded. Clinical and biochemical markers were evaluated during 4 +/- 3 years of follow-up to determinate predictors of adverse outcome. A composite end point (hospitalization for worsening heart failure, cardiac transplantation, and death) was investigated. The final cohort consisted of 48 patients (median age 17 years) with idiopathic dilated cardiomyopathy. During follow-up, 11 patients required hospitalization for heart failure (21%) and 6 patients entered the transplantation list (4 underwent orthotopic cardiac transplantation, 8%). A Cox multivariate model evidenced N-terminus pro-brain natriuretic peptide (NT-pro-BNP; odds ratio 1, confidence interval 1 to 1.1, p = 0.001), New York Heart Association (NYHA) classes III to IV (odds ratio 2.5, confidence interval 1 to 5.9, p = 0.04), and electrocardiographic atrial enlargement (odd ratios 6.7, confidence interval 1.8 to 25, p = 0.005) as predictors of adverse events (composite end point) at 60 months. The association of NYHA classes III to IV, electrocardiographic atrial enlargement, and NT-pro-BNP value > or =250 pg/ml (are under the curve 0.88) showed 100% sensitivity, 77% specificity, 62% positive predictive value, and 100% negative predictive value. In conclusion, compared to other clinical, echocardiographic, and biochemical parameters, the combination of advanced NYHA class, electrocardiographic atrial enlargement, and NT-pro-BNP > or =250 pg/ml seem to better predict the risk of adverse events in adolescent with long-term systolic dysfunction.