Total Anomalous Pulmonary Venous Return (TAPVR)

Total Anomalous Pulmonary Venous Return (TAPVR)

Total Anomalous Pulmonary Venous Return (TAPVR) is a rare congenital malformation in which all four pulmonary veins (that normally transport oxygen-rich blood from the lungs to the left atrium of the heart) do not connect normally to the left atrium. Instead all four drain abnormally to the right atrium by way of an abnormal connection. TAPVR causes cyanosis (blue discoloration of skin and lips) and difficulty breathing. Severe cases will develop severe cyanosis, difficulty breathing, low blood pressure and excessive acidity in the blood (acidosis).

Classifications of TAPVR

TAPVR can occur with varying degrees of severity:

  • Obstructed TAPVR: This is the most severe form of TAPVR and occurs when the anomalous veins are also obstructed (narrowed) prior to entering the right atrium. This makes it difficult for blood to return from the lungs to the heart. With obstructed TAPVR, the baby is usually critically ill and will need emergency surgery.
  • Unobstructed TAPVR: In this form, a baby may be only mildly cyanotic and could go a few weeks or even longer before being diagnosed. Once an infant is diagnosed, corrective surgery is performed.
  • Partial anomalous pulmonary venous return (PAPVR): With PAPVR, a child may only present with mild signs, such as tiring easily, or may not show signs or symptoms until later in life.

Treatment of TAPVR

When TAPVR is detected and surgically corrected in early infancy, the long-term outlook is very good. However, anyone with TAPVR will need life-long follow-up with a cardiologist who has had special training in congenital heart defects. Children with repaired TAPVC may be advised to limit their physical activities. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, or if the child has a heart rhythm problem.