The pulmonary hemodynamic response to perioperative anesthesia in the treatment of high-risk infants with congenital diaphragmatic hernia.

The continuing high mortality in congenital diaphragmatic hernia led us to study the cardiopulmonary disturbances associated with this lesion. Since these infants infrequently have right-to-left shunting in the operating room, we adopted a treatment protocol of: continuing general anesthesia in the postoperative period using fentanyl and pancuronium; cardiac catheterization postoperatively, including placement of a pulmonary artery line and a pulmonary angiogram; rapid frequency ventilation; moderate fluid restriction; and avoidance of vasodilators until other means of management had clearly failed. Fourteen high-risk infants, presenting within 6 hours of birth, were studied and compared to 17 high-risk infants, who served as historical controls. As revealed by the physiologic data acquired in the catheterization laboratory, high-risk infants divided into "Responder" and "Nonresponder" groups. Seven of 10 "Responders" actually shunted left to right during the catheterization, demonstrating a low pulmonary vascular resistance. Seven of 10 subsequently demonstrated significant right-to-left shunting at the level of the ductus and the foramen ovale, indicating the hyperreactivity of the pulmonary vascular bed. All but one was managed successfully by ventilatory adjustments and deepening of the level of anesthesia. "Nonresponders" had a fixed right-to-left shunt unresponsive to any medical or ventilatory manipulation. All "Nonresponders" died. Pulmonary angiography suggested a smaller diameter of the affected pulmonary artery compared to the main pulmonary artery in the "Nonresponders." This implies true hypoplasia resulting in a vasculature too small to accept a full cardiac output. Survival in the treatment group "Responders" was eight of 10 (80%) v seven of 14 (50%) in the control group.(ABSTRACT TRUNCATED AT 250 WORDS)