Endomyocardial fibrosis in the child.

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.