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Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return (TAPVR) is a rare condition where the pulmonary veins do not connect normally to the left side of the heart.

The normal heart has four chambers, two atria (collecting chambers) and two ventricles (pumping chambers). In the normal heart, unoxygenated blood from the body returns to the right atrium. This blood is then pumped by the right ventricle through the lungs to receive oxygen. This newly oxygenated blood returns to the left atrium through four pulmonary veins before being pumped to the rest of the body by the left ventricle, completing the cardiac cycle.

Normally there are four pulmonary veins (2 on the right, 2 on the left) which take newly oxygenated blood from the lungs to the left atrium. In TAPVR, the veins carrying the oxygenated blood from the lungs do not connect to the left atrium; that connection which should have formed early in fetal life never develops. Instead, the blood returns to the right atrium via one of several abnormal pathways.

There are four types of TAPVR - how the blood returns from the pulmonary veins to the right atrium determines what type of TAPVR:

Supracardiac (45%)

Pulmonary veins connect to a vein above the heart which then directs the blood to the right atrium

Cardiac (25%)

Pulmonary veins connect directly to the right atrium

Infracardiac (25%)

Pulmonary veins drain to a vein below the diaphragm before draining into the right atrium

Mixed (5%)

Pulmonary veins connect randomly to the heart

 

All patients with TAPVR must have an atrial septal defect connecting the right atrium with the left atrium.  This is the only way for oxygenated blood from the pulmonary veins to reach the left side of the heart, which can then be pumped to the rest of the body.  Since both unoxygenated and oxygenated blood will enter the right atrium, the blood which will be pumped into the rest of the body will not be fully oxygenated.

Some patients with TAPVR also have pulmonary venous obstruction, which is a narrowing between the pulmonary veins and right atrium, resulting in high pressure in the pulmonary veins and leakage of fluid into the lungs. This is a severe, life-threatening problem which requires emergent surgery.  In addition, it can cause lung problems in newborns which can greatly complicate recovery from surgery.

Surgical Treatment for TAPVR

Treatment of TAPVR is surgery, which consists of placing the patient on the heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium. Fortunately, in most cases of TAPVR, all four pulmonary veins come to a central place (called a confluence) just behind the left atrium and a full anatomical repair can be achieved.  However, after surgery, scar tissue may form around the connection between the pulmonary veins and the left atrium.  If the scar tissue formation is severe enough, additional surgery may be required in the future.

Recovery from surgery is most closely related to the health of the lungs going into surgery. Although surgery for TAPVR has improved over the years, mortality from TAPVR repair is still fairly high, approximately 5-8% overall. This will vary greatly from child to child depending on the kind of TAPVR and the amount of pulmonary venous obstruction before surgery.

Contact Us

Pediatric Cardiology Clinic
University of Texas Health Science Center Professional Building
6410 Fannin, Suite 500
Houston, TX 77030

Phone: (832) 325-6516

Pediatric Cardiovascular Surgery Clinic
University of Texas Health Science Center Professional Building
6410 Fannin, Suite 950
Houston, TX 77030

Phone: (713) 500-7339

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Note: This web page is meant to be a helpful, informative introduction on the subject of the Total Anomalous Pulmonary Venous Return. The information may not be applicable to all cases, especially if there are additional defects. It is not meant to replace the opinion of a personal physician.