The surgical management of pulmonary valve atresia

Abstract

Isolated atresia of the pulmonary valve is a rare congenital malformation. Two types are recognized; one in which the right ventricle is small and its cavity slit-like in character, and another in which this ventricle is large. Pulmonary blood flow is dependent upon a patent ductus. If the lives of the infants are to be salvaged, the lesion must be diagnosed, and operation performed before the ductus closes. Pulmonary valvotomy is recommended when a large right ventricle is present. When this ventricle is small, a palliative superior vena cava to pulmonary artery anastomosis is advocated. Seven infants with pulmonary atresia are reported. Three died before operation could be undertaken and four had pulmonary valvotomy performed. Of these four, one died postoperatively and three survived to be discharged from hospital. One of these died at six months with reclosure of the pulmonary valve orifice; one died following a repeat operation; and the lone survivor at eighteen months continues to do well, but has some residual pulmonary stenosis.