Survival in Pediatric Dilated Cardiomyopathy

Study Questions:

What is the transplant-free survival rate for children with dilated cardiomyopathy (DCM) over a 20-year time period?

Methods:

The study made use of the Pediatric Cardiomyopathy Registry to identify children <18 years old with DCM. Subjects were divided by year of diagnosis into an early cohort (1990-1999) and a late cohort (2000-2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort, and to identify the factors associated with death.

Results:

A total of 1,953 children with DCM were identified, of which 1,199 were in the early cohort and 754 were in the late cohort. The most common etiology of DCM in both cohorts was idiopathic, occurring in 64% of children in the early cohort and 63% of children in the late cohort. Baseline characteristics including median age (1.6 vs. 1.7 years), left ventricular (LV) end-diastolic z-scores (+4.4 vs. +4.2), and LV fractional shortening (16% vs. 17%) were similar between the early and late cohorts. The rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar as well. However, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being part of the early cohort was an independent predictor of death (hazard ratio, 1.4; 95% confidence interval, 1.04-1.9; p = 0.03).

Conclusions:

The authors concluded that children with DCM have improved survival in the more recent era, which appears to be associated with factors other than heart transplantation.

Perspective:

This study assessed the survival of pediatric patients after a diagnosis of DCM, specifically focusing on changes in outcomes over time. Although the percentage of patients undergoing cardiac transplantation remained steady over time, survival was improved in the later cohort (patients diagnosed from 2000-2009). The most likely reasons for this improvement would be improved medical therapy or increased use of mechanical support devices. Over the study period, there was likely increased application of therapies validated in the adult population such as angiotensin-converting enzyme inhibitors and beta-blockers. Additionally, there has been a significant increase in the use of mechanical circulatory support in recent years. Further research including documentation of medical therapy and use of mechanical circulatory support will be necessary to understand the changes in survival over time seen in this study.

Clinical Topics: Cardiac Surgery, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and Heart Failure, Acute Heart Failure, Heart Transplant, Interventions and Imaging, Echocardiography/Ultrasound

Keywords: Cardiomyopathies, Cardiomyopathy, Dilated, Echocardiography, Heart Failure, Heart Transplantation, Medication Therapy Management, Pediatrics, Survival Rate


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