Congenital Heart Defects - Anomalous Pulmonary Venous Connection
 

What’s wrong?

  • Total: Connection drains abnormally
  • Pulmonary veins from the lung drain into the RA instead of LA. The veins generally drain into a confluence which may empty into the RA directly (cardiac), the coronary sinus (cardiac), into the SVC (supracardiac) or IVC (infracardiac). There may be obstruction to the pulmonary venous return producing the 'obstructed' type.
  • Partial: Some veins drain abnormally, some are normal


Total Anomalous Pulmonary Venous Connection To Left Vertical Vein (Supracardiac), with Atrial Septal Defect Total Anomalous Pulmonary Venous Connection To Ductus Venosus (Infradiaphragmatic), with Atrial Septal Defect
   
Partial Anomalous Pulmonary Venous Connection (Right Pulmonary Veins to Junction of Superior Vena Cava & Right Atrium)  
 

Corrective Procedures?

This condition can only be corrected with open heart surgery.

  • Partial:

    Repaired by creating a tunnel within the RA using tissue from the pericardium, the sac-like membrane around the heart. This tunnel is connected to the ASD, to allow oxygen-rich pink blood from the pulmonary veins to pass to the left atrium.


  • Total:

    Opening the back of the LA, sewing the common veins directly to the wall of the LA, and closing off the abnormal blood vessel to the right side of the heart.

What are the possible complications after the procedures?

  • Narrowing of pulmonary vein anastomosis to LA
  • Arrhythmias - Atrial/Ventricular rhythm disturbances