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 IntroductionTotal anomalous pulmonary venous connection (TAPVC) is a rare cardiac defect that comprises 2% of congenital heart disease cases [1]. TAPVC encompasses different anatomic subtypes in which pulmonary veins fail to connect directly to the left atrium and drain to the right atrium via an anomalous venous connection [2,3]. Supracardiac TAPVC is the most common type, comprising about 45% of cases [2]. A left-sided vertical vein (VV) accounts for 70% of the connections between the pulmonary confluence and the right atrium, and stenosis occurs in approximately 40% of cases [3].Obstructed TAPVC typically presents with respiratory distress secondary to pulmonary congestion,

Successful rapid staged hybrid approach for obstructed total anomalous pulmonary venous connection

“…To our knowledge this is the first report of stenting of both VV and DV. This approach should not be considered only for LBW and premature neonates, 4 but this may present a bridge to a safer repair in all obstructed TAPVC to allow repair under less critical circumstances when the initial pathophysiologic process is reversed, which may improve outcomes in these challenging population 5 …”

Palliative vertical vein stenting in a critical neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection

“…2,3 Relief of pulmonary venous obstruction via emergent vertical vein stenting to stabilise a critical neonate prior to surgery can decrease morbidity and mortality. 4,5 We report the successful relief of severe pulmonary venous obstruction in a neonate with infradiaphragmatic total anomalous pulmonary venous connection, whom we felt was unfit for emergent surgery.…”