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.

Heart Chambers and Valves

Blood Flow Through the Heart

With TAPVC, the pulmonary veins that return oxygenated 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 receive 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 be present, as well.

Causes

TAPVC is a congenital defect. This means that the heart forms incorrectly when the baby is in the womb. It is not known exactly why the heart develops this way in some babies.

Risk Factors

More research is needed to confirm causes and risk factors for TAPVC. Risk factors thought to be related to this condition include:

Family history of heart defects
Having other heart defects
Environmental exposures

Symptoms

Symptoms may include:

Blue or pale grayish skin color
Difficulty breathing
Poor growth

Diagnosis

You will be asked about your child’s symptoms and medical history. A physical exam will be done. An irregular heart rate may be detected during the exam.

Your child's bodily fluids may be tested. This can be done with blood tests.

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

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

Treatment

Talk with the doctor about the best treatment plan for your child. Treatment options include:

Surgery

Surgery is needed to correct the defect. It can range from a simple repair to a complex repair. If blood flow is obstructed, emergency surgery will need to be done. The goal of surgery is to reconnect the pulmonary veins to the left atrium.

Lifelong Monitoring

Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent an infection in the heart.

Prevention

Preventing fetal heart defects may not always be possible, but you can reduce your risk by:

Visiting the doctor regularly to monitor your health and the health of the baby. Prenatal tests may detect a heart defect in a growing fetus.
Having a healthy lifestyle
Eating nutritious food and take prenatal vitamins
Not drinking alcohol, smoking, or using drugs during pregnancy
Practicing good hygiene and staying away from people who are sick
Avoiding exposure to environmental toxins

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:

Total anomalous pulmonary venous connection. EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated June 7, 2017. Accessed December 27, 2017.

Total anomalous pulmonary venous connection (TAPVC). American Heart Association website. Available at:
...(Click grey area to select URL)
Updated October 13, 2016. Accessed December 27, 2017.

Total anomalous pulmonary venous return. Johns Hopkins University, Cove Point Foundation website. Available at:
...(Click grey area to select URL)
Updated January 24, 2017. Accessed December 27, 2017.

Last reviewed November 2018 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 12/20/2014

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