A 3.9-kg male child was born at full term following an unremarkable prenatal course. Although initially vigorous he quickly developed respiratory distress and was brought to the neonatal intensive care unit where, upon arrival, he developed profound hypoxemia and respiratory failure. He was intubated and placed on conventional mechanical ventilation. A chest radiograph was interpreted as mild cardiomegaly with diffuse hyaline membrane disease. Umbilical arterial and venous catheters (UVC) were placed and his clinical status rapidly deteriorated. Due to refractory hypoxemia, administration of high-frequency ventilation and nitric oxide was attempted without clinical response. Metabolic acidosis and hypotension ensued and high-dose dopamine and prostaglandin were initiated. An echocardiogram was obtained and demonstrated severe biventricular dysfunction. The left atrium appeared small and the pulmonary veins were not visualized. The patient was transferred to this hospital for further evaluation and care.Upon arrival to this institution the infant was profoundly hypoxemic (peripheral saturation 44%), acidotic, and hemodynamically labile, with evidence of early end-organ dysfunction. He was immediately cannulated for veno-arterial extracorporeal membrane oxygenation (ECMO) from the right neck. Repeat echocardiography after stabilization on ECMO failed to demonstrate the pulmonary veins and he was brought to the cardiac catheterization laboratory for anatomic assessment.At cardiac catheterization arterial and venous access was obtained from the right femoral vessels. Hemodynamic evaluation revealed systemic pulmonary artery (PA) pressures despite full ECMO support and evidence of good left ventricular contractility. Angiography demonstrated total anomalous pulmonary venous connection, with near complete infradiaphragmatic drainage (Figure 1, a). The majority of pulmonary venous return occurred through a single large descending vein that terminated in a venous confluence within the liver before draining to the inferior vena cava via the ductus venosus. The previously placed UVC had entered the pulmonary venous confluence and descending vein and was obstructing pulmonary venous return. The UVC was exchanged over a wire for a standard sheath and PA pressures decreased slightly. Further evaluation revealed that the descending vein was obstructed at the crux of the diaphragm and the ductus venosus was small, compounding the obstruction (Figure 1, b). Premounted stents were placed across both the descending vein and ductus venosus and greatly improved pulmonary venous return (Figure 1, c).Over the course of the next several days the patient's hemodynamic status stabilized and there was return of end-organ function. Diuresis was obtained and ionotropic support was weaned. He was decannulated from ECMO support on his 8th day of life, 5 days after his catheterization. He was taken to the operative suite where he underwent primary sutureless repair of his total anomalous pulmonary venous connection (TAPVC) and ligation of a s...