Scimitar syndrome presenting as respiratory distress in a term neonate: A rare case report

Scimitar syndrome is a rare congenital cardiopulmonary anomaly characterized by partial anomalous pulmonary venous return (PAPVR) from the right lung to the inferior vena cava, often associated with right-lung hypoplasia and dextroposition of the heart. The infantile form usually presents early with respiratory distress and pulmonary hypertension, posing diagnostic and management challenges. We report a term male neonate born at 38 weeks of gestation, who developed severe respiratory distress soon after birth and was initially managed for meconium aspiration syndrome. Echocardiography revealed dextroposition of the heart, right atrial and ventricular dilatation, moderate pulmonary hypertension, and a small secundum-type atrial septal defect. Despite supportive care, oxygen dependency persisted, prompting CT angiography, which demonstrated an anomalous right pulmonary venous drainage into the infra-diaphragmatic inferior vena cava confirming scimitar syndrome. The patient was managed conservatively with continuous positive airway pressure (CPAP), diuretics, and pulmonary vasodilators, leading to gradual clinical improvement and successful weaning off oxygen by day 40 of life. Surgical repair was deferred until pulmonary function optimization. Scimitar syndrome should be suspected in neonates with unexplained respiratory distress and right-sided cardiac silhouette on chest radiograph. Early multidisciplinary evaluation and timely management can significantly improve clinical outcomes.