Long-Term Outcomes of Dilated Cardiomyopathy Diagnosed During Childhood: Results from a National Population-Based Study of Childhood Cardiomyopathy
Background—Existing studies of childhood dilated cardiomyopathy (DCM) deal mainly with early survival. This population-based study examines long-term outcomes for children with DCM.
Methods and Results—The diagnosis of DCM was based on clinical, echocardiographic and pathological findings. The primary study end-point included time to the combined outcome of death or cardiac transplantation. There were 175 patients aged 0-<10 years at the time of diagnosis. Survival free from death/transplant (95% confidence interval) was 74% (67-80) 1 year after diagnosis, 62% (55-69) at 10 years and 56% (46-65) at 20 years. By multivariable analysis, age at diagnosis <4 weeks or >5 years, familial cardiomyopathy and lower baseline left ventricular fractional shortening (LVFS) Z-score were associated with increased risk of death/transplant, as was lower LVFS Z-score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline LVFS Z-score, higher LVFS Z-score during follow up and greater improvement in LVFS Z-score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At latest follow-up, 100 of 104 (96%) of survivors were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy.
Conclusions—Death or transplantation occurred in 26% of childhood DCM subjects within one year of diagnosis and approximately 1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy and severity of LV dysfunction. The majority of surviving subjects are well and free of cardiac medication.
- Received March 21, 2013.
- Revision received August 26, 2013.
- Accepted August 29, 2013.