Congenital pulmonary venous return anomaly

Congenital pulmonary venous return anomaly, also known as anomalous pulmonary venous connection or anomalous pulmonary venous drainage, is a group of congenital heart defects in which the pulmonary veins — the blood vessels that carry oxygen-rich blood from the lungs back to the heart — do not connect normally to the left atrium. Instead, they drain abnormally into the right atrium or into systemic veins such as the superior vena cava, inferior vena cava, or coronary sinus. This results in a left-to-right shunt, meaning oxygenated blood is recirculated through the lungs rather than being delivered to the body. The condition can be total (TAPVR/TAPVC), where all four pulmonary veins are anomalously connected, or partial (PAPVR/PAPVC), where only one or more veins are affected. In total anomalous pulmonary venous return, symptoms typically present in the neonatal period and can be severe, including cyanosis (bluish discoloration of the skin), respiratory distress, poor feeding, failure to thrive, and signs of right heart failure. An atrial septal defect or patent foramen ovale is necessary for survival, as it allows some oxygenated blood to reach the left side of the heart and systemic circulation. If the anomalous venous drainage is obstructed, the condition constitutes a surgical emergency with rapid clinical deterioration. Partial forms may be milder and sometimes remain undiagnosed until later in childhood or adulthood, presenting with symptoms of volume overload of the right heart such as fatigue, exercise intolerance, and recurrent respiratory infections. Diagnosis is made through echocardiography, cardiac catheterization, CT angiography, or cardiac MRI. The primary treatment is surgical correction, which involves redirecting the pulmonary venous flow to the left atrium. For total anomalous pulmonary venous return, surgery is typically performed in the neonatal period and is considered urgent, especially in obstructed forms. Outcomes have improved significantly with advances in pediatric cardiac surgery, though postoperative complications such as pulmonary vein stenosis can occur and may require further intervention. Long-term follow-up with a cardiologist is essential.

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