Background-We hypothesized that children with dilated cardiomyopathy who require hospital admission are at increased risk for death or transplantation during their first hospitalization and in the first year that follows. We also assessed the value of routine data collected during that time to predict death or the need for transplantation prior to discharge and within 1 year of admission. Methods and Results-We conducted a retrospective review of 83 pediatric patients with dilated cardiomyopathy whose initial hospitalization fell between 2004 and 2009. The mean age at hospitalization was 7 years. The majority of patients demonstrated moderate or severe left ventricular dysfunction on initial echocardiogram (80%) and/or the need for intravenous inotropes within 7 days of hospital admission (69%). Five patients (6%) died, and 15 (18%) were transplanted in the initial hospitalization. At 1 year, 11/71 (15%) had died, and 27/71 (38%) were transplanted. The overall freedom from death, transplantation, or rehospitalization at 1 year following admission was 21%. Fractional shortening, left ventricular ejection fraction, serum cholesterol, uric acid, mixed venous saturation , and atrial filling pressures were all predictive of death or transplantation during the initial hospitalization. Left ventricular ejection fraction was predictive of death or transplantation at 1 year. Conclusions-The first hospitalization for dilated cardiomyopathy marks a period of high risk for clinical decline, end stage heart failure, and the need for cardiac transplantation. Echocardiographic function and hemodynamic and serum measurements may aid in predicting outcomes. Despite medical management, most patients will be rehospitalized and/or require cardiac transplantation within 1 year of admission. freedom from death or transplantation during the index hospitalization and at 1 year of follow-up. To our knowledge, this contemporary and homogenous cohort is the largest singleinstitution experience reported for hospitalized pediatric patients with DCM published to date.
Methods
Study Cohort and Data SourceWe identified and performed a retrospective chart review on 83 patients with DCM who had at least 1 hospitalization at Lucile Packard Children's Hospital at the time of, or subsequent to, their diagnosis between January 1, 2004, and December 31, 2009, of which 71 had reached the end point of death, transplantation, or had at least 1 year of follow-up. The diagnosis of DCM was based on the echocardiographic appearance of left ventricular dilatation and reduced left ventricular ejection fraction (LVEF), consistent with the approach taken in large pediatric registries. 3,7,8 Secondary diagnoses included DCM identified as primary idiopathic/familial or secondary to myocarditis, anthracycline toxicity, arrhythmias leading to heart failure, or genetic, neuromuscular, metabolic, or autoimmune disease. DCM was defined as idiopathic if no specific etiology of DCM was discovered and there were no affected family members. Myocarditis was either biopsy-p...