TAPVC

Total Anomalous Pulmonary Venous Connection (TAPVC) is a rare congenital heart defect characterized by the abnormal drainage of all or most pulmonary veins into the right atrium or systemic venous circulation rather than the left atrium. This condition disrupts normal blood flow, leading to increased pressure in the pulmonary veins and potential complications such as heart failure or cyanosis.

In TAPVC, the pulmonary veins fail to connect properly to the left atrium during fetal development. Instead, they drain into the right atrium directly or through an intermediary vessel, such as the coronary sinus or a vertical vein. The defect can be classified into four subtypes based on the location of venous drainage: supracardiac, intracardiac, infradiaphragmatic, and mixed. Supracardiac TAPVC is the most common form, where veins drain into a vertical vein that ascends to the superior vena cava or right atrium. Infradiaphragmatic TAPVC involves drainage below the diaphragm, often through the portal or hepatic veins, which can cause liver congestion.

Symptoms of TAPVC typically appear shortly after birth and may include tachypnea (rapid breathing), poor feeding, cyanosis (bluish skin), and signs of heart failure such as rapid heartbeat and fatigue. Severe cases may lead to respiratory distress or shock. Diagnosis is confirmed through imaging studies like echocardiography, chest X-ray, or magnetic resonance imaging (MRI), which reveal abnormal venous drainage patterns.

Treatment for TAPVC is primarily surgical, aiming to reroute the pulmonary veins to the left atrium to restore normal blood flow. Surgical correction is typically performed in the first few weeks of life to prevent complications. Postoperative care involves monitoring for complications such as bleeding, infection, or residual shunts. With timely intervention, most infants with TAPVC achieve normal cardiac function and growth. Long-term follow-up is essential to address any potential recurrence or late complications.