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The clinical course of primary myocardial disease in infants and children
Insights
Primary myocardial disease in children is serious, with 75% experiencing death or ongoing cardiac issues. Early signs like congestive heart failure and specific EKG changes predict poor outcomes.
Area of Science:
- Pediatrics
- Cardiology
- Pathology
Background:
- Primary myocardial disease affects infants and children, encompassing conditions like idiopathic myocarditis and cardiomyopathy.
- This retrospective study analyzes a cohort of 161 pediatric patients diagnosed over 30 years.
Purpose of the Study:
- To investigate the clinical presentation, outcomes, and predictors of mortality in pediatric primary myocardial disease.
- To characterize the long-term course and resolution rates of cardiac disease in affected children.
Main Methods:
- Retrospective chart review of 161 pediatric patients with primary myocardial disease.
- Analysis of clinical data, electrocardiographic findings, and cardiac imaging.
- Identification of significant clinical predictors associated with fatal outcomes.
Main Results:
- Cardiac disease resolved in 27%, resulted in death in 35%, and persisted in 38% of patients.
- Congestive heart failure, ST-T changes, and cardiomegaly were common initial findings.
- Pulmonary vascular congestion, "northwest" axis deviation, and low cardiac index predicted fatal outcomes.
Conclusions:
- Primary myocardial disease poses a significant threat to pediatric health, with a high rate of mortality and long-term morbidity.
- Early identification of clinical predictors is crucial for managing and improving outcomes in affected children.
- Myocarditis cases showed a particularly high early mortality rate.
Abstract:
A total of 161 infants and children, ranging in age from 1 day to 17 years at initial encounter (mean, 3.7 years), was seen over a 30 year period with primary myocardial disease (idiopathic myocarditis, nonobstructive cardiomyopathy, endocardial fibroelastosis, and an anatomically unknown category). These patients were observed from 1 hour to 23 years after initial encounter and cardiac disease has resolved in 27 per cent, resulted in death in 35 per cent, and continues in 38 per cent. The majority were first referred to us with congestive heart failure; all exhibited ST-T changes and cardiomegaly, 67 of 150 had left ventricular hypertrophy, 23 of 151 arrhythmias, and 55 of 153 pulmonary vascular congestion. Initial ventricular depolarization abnormalities were very frequent. Significant clinical predictors of fatal outcome included pulmonary vascular congestion, "northwest" axis deviation, and a cardiac index less than three L./min./M.2. Death occurred during the first year after initial encounter in 44 of 57 who died, and in all 13 with proved myocarditis. Primary myocardial disease is a serious disease of infancy and childhood, resulting in death or residual cardiac disease in three fourths of those affected.