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Endomyocardial fibrosis in the child
I Oberhaensli1, B Friedli, J Cox
1Unité de Cardiologie Pédiatrique, Hôpital Cantonal Universitaire, Genève, Suisse.
Insights
Endomyocardial fibrosis, a rare pediatric heart condition, presents with impaired ventricular filling. Surgical interventions offer palliative relief, improving symptoms but not resolving the underlying restrictive cardiomyopathy.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Rare Diseases
Background:
- Endomyocardial fibrosis (EMF) is a rare pediatric cardiac condition, primarily seen in tropical regions.
- The exact cause of EMF remains unknown, posing diagnostic and therapeutic challenges.
- Recent advancements in surgical techniques have improved survival rates for affected children.
Purpose of the Study:
- To report on four pediatric cases of endomyocardial fibrosis, detailing their presentation, diagnostic findings, and surgical management.
- To evaluate the efficacy of surgical interventions in improving clinical outcomes for children with EMF.
- To highlight the diagnostic capabilities of echocardiography in identifying EMF.
Main Methods:
- Case series involving four pediatric patients (one Swiss boy, three African girls) with advanced heart failure (NYHA Class III-IV).
- Diagnostic workup included echocardiography (bidimensional and Doppler) and cardiac catheterization.
- Surgical procedures included endocardectomy and valve repair/replacement.
Main Results:
- Echocardiography revealed typical findings of decreased ventricular distensibility and impaired filling.
- Three patients underwent surgical intervention (endocardectomy, valve repair/replacement), with two showing significant clinical improvement (NYHA Class II).
- One patient died; surviving patients exhibited persistent restrictive cardiomyopathy despite surgical palliation.
Conclusions:
- Surgical intervention for pediatric endomyocardial fibrosis provides palliative benefits, improving symptoms and functional class.
- Bidimensional and Doppler echocardiography are crucial for accurate diagnosis of EMF.
- EMF remains a challenging condition, with surgery offering temporary relief rather than a cure.
Abstract:
Endomyocardial fibrosis is a rare disease in children and has been mainly observed in tropical Africa, seldom in Europe. Its precise aetiology remains unknown. New surgical procedures have recently led to better survival. Four children, a 5 year old Swiss boy and 3 African girls, aged 12 to 14 years, were submitted with global heart failure class III to IV of the NYHA classification. Two patients had eosinophilia on admission. A third had a history of transient eosinophilia. The echocardiographic examination showed a very typical picture. In 3 patients (2 f and 1 m) both ventricles were involved; in one girl only the left ventricle. Decreased ventricular distensibility with impaired filling of the left and/or right ventricle was present in all. Diastolic pressures ranged between 24 and 35 mmHg; the systolic function was satisfactory in 3 children (ejection fraction [EF] of 44 to 61%) and severely decreased in the fourth (EF 10%). Three patients underwent endocardectomy: both ventricles in 1 case, only the left in 2 cases. Mitral valve repair by means of the Carpentier ring was done once, valve replacement once (Starr-Edwards prothesis). One child died; the 2 surviving patients showed a distinct improvement in their clinical state and are now in class II of the NYHA classification. Echocardiographic and catheter investigations show, however, persistence of the restrictive cardiomyopathy. Thus, surgical intervention must be considered a palliative procedure in these cases. The diagnosis can clearly be made nowadays with bidimensional and Doppler echocardiography.