Persistent pulmonary hypertension complicating diagnosis and treatment of total anomalous pulmonary venous return in the neonate
Derek Fyfe, M.D.
Department of Pediatrics and Adolescent Medicine
Douglas S. Moodie, M.D.
Department of Cardiology, Department of Pediatrics and Adolescent Medicine
Carl C. Gill, M.D.
Department of Thoracic and Cardiovascular Surgery
Persistent fetal circulation (PFC) or persistent pulmonary hypertension of the newborn1–4 must be differentiated from cyanotic congenital heart disease as soon as possible after birth to provide appropriate therapy. Clinical and echocardiographic features of pulmonary hypertension with right-to-left shunting at atrial and/or ductal levels in the absence of obvious congenital heart disease may suggest PFC, but do not rule out cardiac causes of pulmonary hypertension, specifically total anomalous pulmonary venous return (TAPVR).4
We report 4 cases of obstructed infradiaphragmatic TAPVR in newborn infants, each initially diagnosed as PFC. Severe pulmonary vasospasm was present in each case and was clinically responsive to mechanical hyperventilation before correction of TAPVR in 2 patients. This pulmonary vasospasm persisted and complicated the operative course and postoperative management.
From this clinical experience it is apparent that severe pulmonary vasospasm associated with obstructed TAPVR may obscure diagnosis of this lesion and increase the intraoperative and postoperative complications of such patients.
Case 1. An 1870-g baby girl, twin B, product of a 37-week gestation, born to a 25-year-old gravida 1 (Table 1), was delivered by cesarean section because of fetal bradycardia. Twin A weighed 2600 g with no medical problems. Twin B had Apgar scores of 7 and 9, and was well until 24 hours of age when she became tachypneic and cyanotic. A chest radiograph revealed minimally increased vascularity and normal cardiac size (Fig. 1). A capillary gas on room air had a PaO2 of 30 torr, and on an FIO2 of 0.9 remained . . .