Maintenance of Ductal Patency after Repair of Obstructed Total Anomalous Pulmonary Venous Connection
James Hammel, Ali Ibrahimiye, Kim Duncan.
Children's Hospital and Medical Center, Omaha, NE, USA.
Despite effective repair, newborns with critically obstructed total anomalous pulmonary venous connection (TAPVC) may experience important postoperative morbidity and mortality, sometimes associated with severely elevated pulmonary vascular resistance (PVR), despite the use of inhaled nitric oxide, concentrated oxygen, and optimal medical management. In three recent cases, we managed postoperatively with continued ductal patency until PVR became sub-systemic.
Retrospective review case series.
Three neonates underwent repair of severely obstructed TAPVC. In each case, ductal flow was right-to-left preoperatively. Repair occurred emergently upon diagnosis, at 1-2 days of age. In the first case, the ductus was ligated at initiation of bypass. In the second and third, the ductus was snared. In each case, an unobstructed pulmonary venous connection was made (Vmax 1.4, 1.4, and 1.5 m/s), however pulmonary artery pressures were measured as suprasystemic, with depressed systemic pressure and pulsatility. The ductus ligature was removed in the first case, and the snare opened in the second and third, resulting in improvement of systemic pressure. Prostaglandin infusion was restarted. The sternum was left open. The ductus was snared shut on postoperative day 2, 3, and 5 when echo demonstrated mostly left-to-right ductal flow, and was permanently ligated on day 9, 5, and 7. The first two patients survived to discharge. The third, recent case has been extubated and continues recovering in hospital.
Deliberate maintenance of ductal patency after repair of TAPVC is an effective strategy for preservation of systemic perfusion in the face of temporarily suprasystemic PVR.
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