PROGNOSTIC FEATURES OF CHILDREN WITH IDIOPATHIC DILATED CARDIOMYOPATHY

The presenting features and long-term outcome of 39 children (median age 6.5 months, range 1 day to 16 years) with idiopathic dilated cardiomyopathy (IDC) were reviewed to help determine the appropriate management of these patients. Four outcome groups were identified: those who died, improved, had IDC resolved or received transplants. Presenting clinical features of age, sex, race, congestive heart failure, cardiomegaly, and degree of systolic ventricular dysfunction did not predict final outcome. Left ventricular hypertrophy on the electrocardiogram was seen significantly more often in children who improved than in those who died or in whom IDC resolved (p = 0.002). A rhythm disturbance was also see more often in those who died than in those who survived (p = 0.025). Of 36 patients treated medically, 12 (33%) died, 15 (42%) improved and 9 (25%) resolved. Eighteen of 26 (69%) patients presenting at age less-than-or-equal-to 2 years survived, whereas 6 of 10 patients > 2 years survived. There were no differences based on age at presentation, in the time to death or time of follow-up. Three patients received orthotopic heart transplants, 1 of whom died from graft failure. Thus, no clinical feature including age at presentation consistently predicts ultimate outcome in children with IDC.