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Incidence, causes, and outcomes of dilated cardiomyopathy in children
Jeffrey A Towbin1, April M Lowe, Steven D Colan
1Texas Children's Hospital, Baylor College of Medicine, Houston, USA.
Insights
Dilated cardiomyopathy (DCM) affects children, with incidence varying by age, sex, and race. Outcomes depend on the cause, age, and heart failure status at diagnosis, with many cases having unknown causes.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Epidemiology
- Genetics of Heart Disease
Background:
- Dilated cardiomyopathy (DCM) is the leading cause of heart transplantation in children, yet its epidemiology and clinical course remain poorly understood.
- Establishing a clear understanding of DCM in pediatric populations is crucial for improving patient outcomes and guiding treatment strategies.
Purpose of the Study:
- To comprehensively describe the incidence, etiologies, outcomes, and risk factors associated with DCM in children.
- To provide a detailed epidemiological profile of pediatric DCM.
Main Methods:
- A longitudinal study utilizing a prospective, population-based cohort from 89 pediatric cardiac centers, supplemented by a retrospective cohort from tertiary care centers.
- Included 1426 children diagnosed with DCM before age 18 in North America, excluding secondary causes of the disease.
- Primary DCM was confirmed using strict echocardiographic and/or pathological criteria.
Main Results:
- The overall annual incidence of pediatric DCM was 0.57 per 100,000 children, with higher rates in boys, Black children, and infants under 1 year.
- Idiopathic DCM accounted for 66% of cases; myocarditis and neuromuscular diseases were the most common known causes.
- One- and 5-year rates of death or cardiac transplantation were 31% and 46%, respectively. Independent risk factors for adverse outcomes included older age, congestive heart failure, and reduced left ventricular function.
Conclusions:
- Pediatric DCM is a heterogeneous condition where outcomes are significantly influenced by the underlying cause, patient age, and severity of heart failure at presentation.
- Incidence is affected by race, sex, and age, highlighting demographic disparities.
- The high proportion of idiopathic cases underscores a need for further research into underlying mechanisms to develop targeted therapies.
Context:
Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and cause of cardiac transplantation in children. However, the epidemiology and clinical course of DCM in children are not well established.
Objective:
To provide a detailed description of the incidence, causes, outcomes, and related risk factors for DCM in children.
Design And Setting:
Longitudinal study based on a population-based, prospective cohort of children diagnosed as having DCM since January 1, 1996, at 89 pediatric cardiac centers and a retrospectively collected cohort of patients seen primarily at large tertiary care centers in North America and who had diagnoses between January 1, 1990, and December 31, 1995, and were enrolled through February 2003.
Participants:
A total of 1426 children from the United States and Canada diagnosed as having DCM at younger than 18 years. Primary DCM was determined by strict echocardiographic and/or pathologic criteria. Patients with disease due to endocrine, immunologic, drug toxicity, and other causes were excluded.
Main Outcome Measures:
Annual incidence per 100,000 children; mortality; cardiac transplantation.
Results:
The annual incidence of DCM in children younger than 18 years was 0.57 cases per 100,000 per year overall. The annual incidence was higher in boys than in girls (0.66 vs 0.47 cases per 100,000; P<.001), in blacks than in whites (0.98 vs 0.46 cases per 100,000; P<.001), and in infants (<1 year) than in children (4.40 vs 0.34 cases per 100,000; P<.001). The majority of children (66%) had idiopathic disease. The most common known causes were myocarditis (46%) and neuromuscular disease (26%). The 1- and 5-year rates of death or transplantation were 31% and 46%, respectively. Independent risk factors at DCM diagnosis for subsequent death or transplantation were older age, congestive heart failure, lower left ventricular fractional shortening Z score, and cause of DCM (P<.001 for all).
Conclusions:
In children, DCM is a diverse disorder with outcomes that depend largely on cause, age, and heart failure status at presentation. Race, sex, and age affect the incidence of disease. Most children do not have a known cause of DCM, which limits the potential for disease-specific therapies.
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