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[Endomyocardial fibrosis in children]
M Brigui1, F Remadi, N Belkhiria
1Service de Cardiologie, Centre Hospitalo-Universitaire Sahloul, Sousse, Tunisie.
Archives Francaises De Pediatrie
|January 1, 1993
Summary
Two children with endomyocardial fibrosis presented with severe heart failure. Two-dimensional echocardiography effectively diagnosed this rare condition, revealing characteristic endocardial changes.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Endomyocardial fibrosis (EMF) is a rare infiltrative disease affecting the subendocardium.
- Advances in imaging modalities are crucial for early diagnosis and management.
- This report details two pediatric cases of EMF in Tunisia.
Observation:
- Two pediatric patients, aged 3 and 12 years, presented with severe cardiac failure, primarily impacting the right ventricle.
- Two-dimensional echocardiography revealed apical amputation with echogenic material and thickened, dense endocardium, particularly around the papillary muscles.
- Cardiac catheterization was performed on the older child.
Findings:
- Post-mortem examinations confirmed fibroelastic thickening of the endocardium and myocardial fibrosis in both children.
- The younger child survived for 4 years post-diagnosis, while the older child survived for 2 years.
- Echocardiography demonstrated significant endocardial abnormalities consistent with EMF.
Implications:
- Two-dimensional echocardiography is a valuable tool for diagnosing endomyocardial fibrosis.
- This imaging technique can accurately assess the extent and severity of endocardial fibrosis.
- Early diagnosis through echocardiography can aid in managing pediatric cases of endomyocardial fibrosis.