Dilated cardiomyopathy in children: Clinical course and prognosis

Summary The clinical profile of 19 patients with dilated cardiomyopathy ages 2–18 years (mean 13.4±4 years) was reviewed to detect any factors that might be predictive for their survival. Follow-up ranged from 5 to 105 months (mean 39±33 months). Routine treatment consisted of digitalis and diuretics: 14 patients received antiarrhythmics, 6 received vasodilators, and 12 were managed with immunosuppression. There were 12 survivors and 7 nonsurvivors: The 1-year mortality was 21.2% and the 2-year mortality 35.8%. All deaths were within first 2 years. Of the 12 patients who survived 2 years, a significant improvement was noticed in 9. In 3 patients tachycardia-induced cardiomyopathy was diagnosed, and abolition of supraventricular tachycardia was followed by improvement and regression of cardiomegaly. Endomyocardial boopsy was performed in 16 patients. Four with a histologic diagnosis of active myocarditis survived, and in 3 of them a considerable improvement was noticed. Of the 12 patients with nonspecific histologic findings, 6 died (p<0.05). There were no significant differences between survivors and nonsurvivors for any of the following parameters: incidence of severe heart failure (NYHA class III–IV) and severe ventricular arrhythmias (Lown class III–V), relative heart volume, echocardiographic left ventricular diastolic diameter and shortening fraction, and the hemodynamic parameters of cardiac index, left ventricular ejection fraction, left ventricular end-diastolic pressure, and left ventricular end-diastolic volume index.