Abstract

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart defect (1–2% of congenital heart diseases). The obstructed form, particularly infracardiac or mixed types, often presents as a neonatal emergency with hypoxemia and rapid deterioration, mimicking pneumonia or respiratory distress syndrome (RDS). Case Presentation: A late-preterm male neonate (35 + 5 weeks, 2.77 kg) developed early tachypnea and hypoxemia (SpO2 93% on room air). Chest X-ray showed a left upper-zone opacity, and congenital pneumonia was suspected. Despite oxygen, surfactant, and antibiotics, the infant progressed to severe mixed acidosis (pH 7.10, pCO2 139 mmHg, lactate 11 mmol/L), refractory shock, polycythemia (PCV 71%), and acute kidney injury. Echocardiography initially revealed only an atrial septal defect. The neonate died at 42 hours of life. Retrospective review suggested a missed obstructed TAPVC with ASD. Conclusion: Obstructed TAPVC should be suspected in neonates with persistent hypoxemia and acidosis unresponsive to routine management. Early repeat echocardiography focusing on pulmonary venous drainage is crucial. This case emphasises that clinical vigilance and timely reassessment remain vital for detecting critical congenital heart disease.

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