Total anomalous pulmonary venous connection

This code represents a congenital heart defect where all four pulmonary veins drain abnormally into the systemic venous circulation, rather than directly into the left atrium. This results in oxygenated blood mixing with deoxygenated blood, leading to cyanosis and pulmonary hypertension. The connection can be supracardiac, cardiac, infracardiac, or mixed.

Use this code for documented cases of total anomalous pulmonary venous return (TAPVR) identified through echocardiography, cardiac catheterization, or surgical findings. This diagnosis is typically made in neonates or infants presenting with cyanosis, respiratory distress, and heart murmur. Documentation should clearly specify the anomalous connection.

• Differentiate from partial anomalous pulmonary venous connection (PAPVC), which has a separate code.
• Always sequence this code as a primary diagnosis when it is the reason for the encounter.
• Look for specific anatomical details of the connection (e.g., supracardiac, cardiac) in the documentation, though this code covers all types.