Congenital Diaphragmatic Hernia: Determination of the Optimal Time for Operation by Echocardiographic Monitoring of the Pulmonary Arterial Pressure

Preoperative stabilization and delayed operation rather than emergency repair of congenital diaphragmatic hernia (CDH) may improve survival, but there are no clear criteria for how long operation should be delayed. Because increased pulmonary vascular resistance (PVR) may be an important risk factor, we used Doppler echocardiography to study patients with CDH presenting with respiratory distress immediately after birth. During the study period 10 patients were admitted, but 2 were moribund on admission and died shortly thereafter. In the remaining patients the pulmonary arterial pressure (PAP) and direction of ductal shunt were estimated by Doppler echocardiography. Initial PAPs were in the range of 45 to 90 mm Hg, with bidirectional or right-to-left shunt through the ductus arteriosus. Reduction of pressure to 25 to 55 mm Hg, or reversal of shunt to left-to-right, reflecting decreased PVR, occurred after ventilation for 3 to 20 days (mean, 8 days). Patients underwent operation after there was evidence of reduced PVR. None developed persistent fetal circulation, and all 8 patients survived. We conclude that postponing operation until PVR has decreased seems to improve survival in patients with CDH presenting within hours of birth.