Pulmonary hypertension accompanying ventricular septal defect and patent ductus arteriosus. Management in infancy and early childhood.

Forty-one infants and children with the combination of patent ductus arteriosus (PDA) and ventricular septal defect (VSD) were encountered over 20 years. Twenty-four presented in infancy with congestive cardiac failure. Pulmonary hypertension was present in 32, the cause in 19 being increased pulmonary blood flow. Increased pulmonary vascular resistance (PVR) was detected in 13 (indicated by a pulmonary to systemic resistance ratio (Rp : RS) greater than 0.24:1 and PVR greater than 4 units). Thus 22% had a pulmonary artery systolic pressure less than 30 mmHg and 68% had a pulmonary vascular resistance below four units, indicating an unusually mild form of the combined condition in these patients. Surgical management is discussed, and in particular the question of simultaneous closure of the defects during infancy. Cardiac failure, resistant to drug treatment, and increased PVR are indications of operation. The PDA should be closed and only if there is no substantial fall in pulmonary artery pressure is the VSD repaired.