Cardiac valve replacement for congenital mitral valvular disease in children

Abstract

Certain congenital defects of the mitral valve causing severe disability during childhood can be managed only by valve excision and prosthetic substitution. Mitral valve replacement was performed in 21 children for correction of congenital mitral stenosis in 6, isolated congenital mitral insufficiency in 10, and complete atrioventricular canal defects in 5. Three patients underwent concomitant aortic valvulotomy. Fourteen patients had pulmonary hypertension and 3 had undergone previous repair of coarctation. Nine patients were in congestive heart failure. Three hospital deaths occurred in patients operated upon for intractable failure; one had associated endocardial fibroelastosis, another acute mitral insufficiency from Marfan's syndrome, and another complete atrioventricular canal. Two late deaths occurred; one due to recurrent thrombus formation on the prosthesis, and the other from bacterial endocarditis and cardiac failure. The low-profile discoid valve with minimal protrusion into the left ventricle is particularly suited for mitral replacement in children. Small adult-sized prostheses were used permitting growth and development of the children. Cloth covering of the seating ring permits incorporation by ingrowth of autologous tissue and decreased thromboembolic complications. Although the long-term fate of prosthetic heart valves is uncertain, the management of severe congenital mitral deformities by valve replacement has provided remarkable clinical improvement in these children.