Elective Stent Implant in the Obstructed Vertical Vein of Supracardiac Total Anomalous Pulmonary Venous Connection Prior to Operative Repair

Abstract

Background: Total anomalous pulmonary venous connection (TAPVC) comprises 2% of congenital heart disease cases. Obstructed TAPVC typically presents with respiratory distress secondary to pulmonary congestion. We report a case of an infant patient who was electively referred to catheterization for stent placement to relieve vertical vein (VV) stenosis. Our objective was to prevent the emergent need for surgical intervention while allowing additional growth before surgery.

Case Presentation: A 7-day-old, late pre-term, small for gestational age male infant was transferred from an outside institution. He was initially placed on nasal cannula due to oxygen saturation around 80% but progressed to continuous positive airway pressure and had a chest X-ray suggestive of pulmonary edema. Echocardiography revealed supracardiac TAPVC, a small apical muscular ventricular septal defect, and a moderate secundum atrial septal defect. On admission, the patient was clinically stable with a baseline oxygen saturation of 72% on 40% oxygen. Echocardiography confirmed supracardiac TAPVC and also showed an obstruction with a mean gradient of 22 mmHg in the VV. The desire to optimize the patient’s clinical stability led to the decision to undergo cardiac catheterization for stent implantation in the VV. Immediately following the procedure, the patient’s hemodynamics improved, with a pressure gradient between the pulmonary venous confluence and the left innominate vein of 4 mmHg.

Conclusions: Over the last decade, surgical outcomes of TAPVC repair have improved with better control of pulmonary hypertension and preoperative clinical stabilization due to more aggressive medical management. This case presents an opportunity to consider an elective interventional strategy that palliates the disease to prevent an urgent need for definitive repair.