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Idiopathic dilated cardiomyopathy in the young: clinical profile and natural history
Insights
Idiopathic dilated cardiomyopathy in children often presents as congestive heart failure, with a poor prognosis and low survival rates. Some survivors show improved cardiac function, and viral illness may be linked to better outcomes.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Pediatrics
Background:
- Idiopathic dilated cardiomyopathy (IDC) in children is not well-understood.
- Limited data exists on the clinical presentation and long-term outcomes of pediatric IDC.
Purpose of the Study:
- To characterize the clinical profile and course of idiopathic dilated cardiomyopathy in pediatric patients.
- To analyze survival rates, causes of death, and factors influencing outcomes in children with IDC.
Main Methods:
- Retrospective review of 24 pediatric patients diagnosed with IDC at Mayo Clinic (1973-1982).
- Analysis of clinical presentations, echocardiographic findings, myocardial biopsies, and follow-up data.
- Evaluation of survival rates, causes of mortality, and changes in cardiac function over time.
Main Results:
- Congestive heart failure was the most common presentation (92%).
- Echocardiography showed dilated left ventricles with reduced function; left ventricular thrombus was noted in 23%.
- Overall survival was 63% at 1 year and 34% at 5 years; congestive heart failure was the leading cause of death.
Conclusions:
- Pediatric idiopathic dilated cardiomyopathy has a high mortality rate, primarily due to heart failure.
- Improved left ventricular systolic function was observed in some survivors.
- Severe mitral insufficiency correlated with mortality, while a preceding viral syndrome was more common in survivors.
Abstract:
The clinical profile and course of documented cases of idiopathic dilated cardiomyopathy in children have been poorly characterized. Twenty-four patients (median age 2 years, range less than 1 month to 18 years) with idiopathic dilated cardiomyopathy were identified from Mayo Clinic records from 1973 to 1982. The most common presentation was congestive heart failure (92% of patients). Echocardiography (22 patients) generally revealed a dilated left ventricle with reduced fractional shortening (mean 14%) and ejection fraction (mean 26%). Two-dimensional echocardiographic evidence of left ventricular thrombus was present in 3 (23%) of 13 patients. Median cardiac index and left ventricular end-diastolic pressure (19 patients) were 2.5 liters/min per m2 and 22 mm Hg, respectively. Myocardial biopsy in eight patients showed nonspecific findings without active inflammation or evidence of endocardial fibroelastosis. On follow-up (mean duration 33 months, range 0 to 149), systemic arterial embolism had occurred in 2 (8%) of 24 patients. Fifteen of 24 patients had died (63% survival at 1 year and 34% survival at 5 years of follow-up). The cause of death was congestive heart failure in 11, complications after cardiac transplantation in 3 and sudden cardiac death in 1. Nine patients are alive at a mean follow-up time of 65 months (range 26 to 149); five are asymptomatic. Serial determination of left ventricular systolic function, available in all survivors, showed improvement in six patients and no significant change in three. Severe mitral insufficiency was present only in patients who ultimately died. A recent viral syndrome was noted more frequently in patients who survived.(ABSTRACT TRUNCATED AT 250 WORDS)