Mitral and aortic valve incompetence in endocardial fibroelastosis diagnostic and hemodynamic significance

Abstract

Hemodynamic and angiocardiographic studies were performed in 20 infants and children whose initial clinical, electrocardiographic and roentgenographic findings were compatible with the clinical diagnosis of endocardial fibroelastosis. On the basis of right and left heart studies it was possible to separate these patients into 2 distinct clinical groups whose subsequent clinical courses differed strikingly. Patients in group 1, all of whom had a positive reaction to the mumps intradermal skin test, demonstrated moderate to marked mitral valve incompetency, mild to moderate aortic valve insufficiency, a thick left ventricular free wall showing poor systolic excursions, a dilated left ventricular cavity, increased left ventricular end-diastolic pressure and a noncompliant left ventricle. Their subsequent clinical courses were unfavorable and characterized by recurring bouts of cardiac decompensation and a short life expectancy. Prosthetic mitral valve replacement, successful in 2 patients, resulted in marked clinical improvement. On the other hand, patients in group 2 maintained cardiac compensation after initial treatment with digitalis and left and right heart studies were normal, the only finding being an equivocal increase in left ventricular wall thickness. All of these patients are living and all have normal electrocardiographic and roentgenographic findings. It is suggested that patients in group 1 had endocardial fibroelastosis (confirmed by necropsy observation and surgical observation in most) whereas patients in group 2 had a nonspecific or aseptic myocarditis that presented an initial clinical picture indistinguishable from that of endocardial fibroelastosis. The prognosis of these latter patients is good; the prognosis of patients with endocardial fibroelastosis is guarded. If the findings of this study are confirmed, left heart studies should be of value in confirming the antemortem diagnosis of endocardial fibroelastosis.