In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle through the tricuspid valve. The blood travels to the lungs through the pulmonary valve to pick up fresh oxygen. Next, the blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body.

With TAPVC, the pulmonary veins that return oxygen rich blood from the lungs connect to the right side of the heart instead of the left atrium. This leads to the mixing of oxygenated and de-oxygenated blood. The body tissue does not get as much oxygen as it is supposed to. TAPVC can be mild to severe. There can be a range of connection problems. Other heart problems may also be present.

Heart Chambers and Valves

Blood Flow Through the Heart

Causes

TAPVC is caused by a congenital defect. This means that the problem develops in the womb and a baby is born with it. It is not known exactly why this happens.

Risk Factors

The risk factors are not well known. Things that may play a role are:

A family history of heart defects
Having other heart defects
Environmental exposures

Symptoms

Problems may be:

Blue or pale grayish skin color
Trouble breathing
Poor growth

Diagnosis

The doctor will ask about your child’s symptoms and health history. A physical exam will be done. It will focus on the heart. An irregular heart rate may be detected.

Blood tests will be done.

Images may be taken of your child's body. This can be done with:

Your child's heart function may be tested. This can be done with:

Treatment

Surgery is needed to correct the defect. The goal of surgery is to reconnect the pulmonary veins to the left atrium.

Lifelong heart monitoring will also be needed.

Prevention

There are no known guidelines to prevent this heart problem.

RESOURCES:

American Heart Association
http://www.heart.org

Family Doctor—American Academy of Family Physicians
http://familydoctor.org

CANADIAN RESOURCES:

Canadian Cardiovascular Society
http://www.ccs.ca

Heart and Stroke Foundation of Canada
http://www.heartandstroke.ca

References:

Stein P. Total anomalous pulmonary venous connection. AORN J. 2007 Mar;85(3):509-520.

Total anomalous pulmonary venous connection. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/total-anomalous-pulmonary-venous-connection. Accessed March 10, 2021.

Total anomalous pulmonary venous connection (TAPVC). American Heart Association website. Available at:
...(Click grey area to select URL)
Accessed March 10, 2021.

Total anomalous pulmonary venous return. Johns Hopkins University, Cove Point Foundation website. Available at:
...(Click grey area to select URL)
Accessed March 10, 2021.

Last reviewed December 2020 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 3/10/2021

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