A case of infracardiac total anomalous pulmonary venous return

Abstract

A term baby boy weighing 2590 g was born via spontaneous vaginal delivery to a primigravida mother with an uncomplicated antenatal course. Her morphology scan at 21 weeks gestation was reported as normal. The neonate had Apgar scores of 9 and 9 at 1 and 5 min, respectively. He developed persistent cyanosis and respiratory distress at 4 h of age. On examination, he was tachypnoeic to 100 breaths per minute with increased work of breathing. He was desaturating to 60% on room air. On auscultation, he had a clear chest with no audible murmurs. There were no signs of heart failure or shock. He had mixed respiratory and metabolic acidosis (capillary blood gas at room air showed a pH of 7.20, a partial pressure of carbon dioxide (pCO2) of 59 mmHg, bicarbonate of 19 mmol/L and base excess of −5 mmol/L). An initial chest X-ray showed widespread ground-glass opacification (Fig. 1). A Clinician Performed Ultrasound performed at 22 h of age showed an infracardiac total anomalous pulmonary venous return (TAPVR), with the confluence of pulmonary veins draining inferiorly (via an abnormal vein) into the portal vein instead of the left atrium (Figs 2 and 3). He had a pure right-toleft shunt through a patent foramen ovale/atrial septal defect and patent ductus arteriosus, as well as pulmonary hypertension with a gradient of more than 80 mmHg measured across the tricuspid valve (right ventricle systolic pressure 80 mmHg plus right atrial pressure). Fig. 1 X-ray of neonate taken at 4 h of life showing widespread bilateral ground-glass lung parenchymal opacity as a result of pulmonary venous congestion.