A hybrid approach to stabilization and repair of obstructed total anomalous pulmonary venous connection in a critically ill newborn infant

Abstract

D T h e o e i s s g o d a a h s e r s v m linical Summary 3.9-kg male child was born at full term following an unremarkble prenatal course. Although initially vigorous he quickly develped respiratory distress and was brought to the neonatal intensive are unit where, upon arrival, he developed profound hypoxemia nd respiratory failure. He was intubated and placed on convenional mechanical ventilation. A chest radiograph was interpreted s mild cardiomegaly with diffuse hyaline membrane disease. mbilical arterial and venous catheters (UVC) were placed and his linical status rapidly deteriorated. Due to refractory hypoxemia, dministration of high-frequency ventilation and nitric oxide was ttempted without clinical response. Metabolic acidosis and hypoension ensued and high-dose dopamine and prostaglandin were nitiated. An echocardiogram was obtained and demonstrated seere biventricular dysfunction. The left atrium appeared small and he pulmonary veins were not visualized. The patient was transerred to this hospital for further evaluation and care. Upon arrival to this institution the infant was profoundly hyoxemic (peripheral saturation 44%), acidotic, and hemodynamially labile, with evidence of early end-organ dysfunction. He was mmediately cannulated for veno-arterial extracorporeal memrane oxygenation (ECMO) from the right neck. Repeat echocariography after stabilization on ECMO failed to demonstrate the ulmonary veins and he was brought to the cardiac catheterization aboratory for anatomic assessment. At cardiac catheterization arterial and venous access was obained from the right femoral vessels. Hemodynamic evaluation evealed systemic pulmonary artery (PA) pressures despite full CMO support and evidence of good left ventricular contractility. ngiography demonstrated total anomalous pulmonary venous onnection, with near complete infradiaphragmatic drainage (Figre 1, a). The majority of pulmonary venous return occurred hrough a single large descending vein that terminated in a venous onfluence within the liver before draining to the inferior vena