The syndrome of congenital absence of the pulmonary valve: pathological, clinical and surgical considerations.

Four cases of congenital absence of the pulmonary valve are described. Three of them underwent successful operation. The fourth case, operated on in the first days of life, died because of severe respiratory distress. After examining the embryological aspects of this malformation, its treatment is discussed in relation to particular respiratory problems in infants and to the possibility of inserting prosthetic valves in children undergoing elective surgical treatment.