INFANTILE DILATED CARDIOMYOPATHY - RELATION OF OUTCOME TO LEFT-VENTRICULAR MECHANICS, HEMODYNAMICS, AND HISTOLOGY AT THE TIME OF PRESENTATION

Background For patients with acute dilated cardiomyopathy, definition of prognosis and of clinical features predictive of outcome is particu larly important due to the availability of cardiac transplantation and other innovative treatment strategies. Methods and Results We reviewe d our experience with 24 children under 2 years of age with dilated co ngestive cardiomyopathy to determine outcome and potential predictive variables. Clinical, serological, ECG, echocardiographic, hemodynamic, and histological findings were analyzed. Idiopathic cardiomyopathy or myocarditis constituted 29% of the patients presenting with congestiv e heart failure without structural heart disease. Among these patients , 45% recovered completely, 25% survived with persistent left ventricu lar dysfunction, and 30% died. All except one of the deaths occurred d uring the first 2 months after presentation. Poorer outcome and higher mortality were associated with a more severely depressed left ventric ular ejection fraction and/or a more spherical left ventricular shape at presentation. Histological evidence of myocardial inflammation was a favorable prognostic indicator, whereas histological evidence of end ocardial fibroelastosis was associated with a poor outcome. During the recovery phase, diastolic volume fell rapidly. Ventricular mass was e levated from the earliest observations and fell more slowly, with pers istent elevation of the mass-to-volume ratio up to 2 years. Function a nd contractility improved over the first several months in most patien ts who recovered, although in occasional patients continued improvemen t was seen for as long as 2 years after presentation. Conclusions Hist ological and echocardiographic features can be used to identify patien ts at particularly high risk for death. To have any impact on outcome, decisions about cardiac transplantation must be reached rapidly, sinc e almost all deaths occurred within the first 2 months after presentat ion. Recovery of function is often rapid, but continued improvement ma y be seen for as long as 2 years.