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[Dilated cardiomyopathy in childhood]
1Abt. Pädiatrische Kardiologie Universitäts-Kinderklinik Hufelandstr, 55 45122 Essen. achim.schmaltz@uni-essen.de
Insights
Childhood dilative cardiomyopathy (DCM) affects young children, often diagnosed late with heart failure. Despite treatment, prognosis is poor, highlighting the need for better understanding and therapies for this serious heart condition.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Genetics and Rare Diseases
Context:
- Dilative cardiomyopathy (DCM) in children presents unique challenges.
- Prevalence is 2.6 per 100,000, with 75% manifesting in the first two years of life.
- Diagnosis is often delayed until congestive heart failure occurs.
Purpose:
- To summarize the current understanding of childhood dilative cardiomyopathy.
- To highlight diagnostic challenges and critical prognosis.
- To review known and unknown etiologies.
Summary:
- Childhood DCM has a poor prognosis, with 1-year and 5-year survival rates of 75% and 60% respectively.
- Idiopathic causes are most common, followed by inflammatory pathogenesis (approx. 40%).
- Infantile cardiomyopathies can stem from metabolic disorders, neuromuscular diseases, or malformation syndromes.
Impact:
- DCM is a leading indication for pediatric heart transplantation.
- Current therapies offer limited success, underscoring the need for novel treatment strategies.
- Understanding diverse etiologies is crucial for developing targeted therapies.
Abstract:
In childhood, dilative cardiomyopathy (DCM) has a prevalence of 2.6 patients in 100,000 inhabitants. Manifestation age is, in 75%, the first two years of life. There are no specific symptoms. Diagnosis is often made, when congestive heart failure occurs. Despite intensive therapy with digitalis, diuretics and ACE inhibitors, DCM is the main indication for heart transplantation. The prognosis is critical: 11 studies with approximately 450 children showed a mean 1-year survival rate of 75% and a 5-year survival rate of 60%. In most cases etiology of DCM remains unclear ("idiopathic"). In the second position (approximately 40%) is inflammatory pathogenesis. While familiar DCM is manifested mainly during the 3rd and 4th decade, specific cardiomyopathy is often found during infancy: inborn errors of metabolism, neuromuscular diseases or malformation syndromes are the causes of a cardiomyopathy, which can appear as dilative or hypertrophic cardiomyopathy. A causal therapy exists only in a few cases.