Total anomalous pulmonary venous return
TAPVR; Total veins; Congenital heart defect - TAPVR; Cyanotic heart disease - TAPVRTotal anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease).
Congenital heart disease
Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth.
Read Article Now Book Mark ArticleCauses
The cause of total anomalous pulmonary venous return is unknown.
In normal circulation, blood is sent from the right ventricle to pick up oxygen in the lungs. It then returns through the pulmonary (lung) veins to the left side of the heart, which sends blood out through the aorta and around the body.
In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body.
For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body.
Atrial septal defect
Atrial septal defect (ASD) is a heart defect that is present at birth (congenital). As a baby develops in the womb, a wall (septum) forms that divide...
Read Article Now Book Mark ArticlePatent foramen ovale
Patent foramen ovale (PFO) is a hole between the left and right atria (upper chambers) of the heart. This hole exists in everyone before birth, but ...
Read Article Now Book Mark ArticleHow severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.
Symptoms
The infant may appear very sick and may have the following symptoms:
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Bluish color to the skin (cyanosis) due to low oxygen level in the blood
Bluish color to the skin (cyanosis)
A bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.
Read Article Now Book Mark Article - Frequent respiratory infections
- Lethargy
- Poor feeding
- Poor growth
- Rapid breathing
Note: Sometimes, no symptoms may be present in infancy or early childhood.
Exams and Tests
Tests may include:
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Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached
Cardiac catheterization
Cardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...
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ECG shows enlargement of the ventricles (ventricular hypertrophy)
ECG
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
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Echocardiogram may show that the pulmonary vessels are attached
Echocardiogram
An echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...
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MRI or CT scan of the heart can show the connections between the pulmonary vessels
MRI
Heart magnetic resonance imaging is an imaging method that uses powerful magnets and radio waves to create pictures of the heart. It does not use ra...
Read Article Now Book Mark ArticleCT scan
A computed tomography (CT) scan of the heart is an imaging method that uses x-rays to create detailed pictures of the heart and its blood vessels. Th...
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X-ray of the chest shows a normal to small heart with fluid in the lungs
X-ray of the chest
A chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.
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Treatment
Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed.
Outlook (Prognosis)
If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. Infants who have obstructed veins have worsened survival.
Possible Complications
Complications may include:
- Breathing difficulties
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Heart failure
Heart failure
Heart failure is a condition in which the heart is no longer able to pump oxygen-rich blood to the rest of the body efficiently. This causes symptom...
Read Article Now Book Mark Article - Irregular, fast heart rhythms (arrhythmias)
- Lung infections
- Pulmonary hypertension
When to Contact a Medical Professional
This condition may be apparent at the time of birth. However, symptoms may not be present until later.
Contact your health care provider if you notice symptoms of TAPVR. Prompt attention is required.
Prevention
Women who plan to become pregnant should be immunized against rubella if they are not already immune. Rubella infection in a pregnant woman can cause congenital heart disease.
Women who are pregnant should get good prenatal care:
- Avoid alcohol and illegal drugs during pregnancy.
- Tell your provider that you are pregnant before taking any new medicines.
- Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible exposure to rubella and get vaccinated right after delivery.
- Pregnant women who have diabetes should try to get good control over their blood sugar level.
Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.
References
Valente AM, Dorfman AL, Babu-Narayan SV, Kreiger EV. Congenital heart disease in the adolescent and adult. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 82.
Well A, Fraser CD. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 21st ed. St Louis, MO: Elsevier; 2022:chap 59.
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Heart - section through the middle - illustration
The interior of the heart is composed of valves, chambers, and associated vessels.
Heart - section through the middle
illustration
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Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease.
Totally anomalous pulmonary venous return - X-ray
illustration
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Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This X-ray shows an enlarged heart, a large liver, and increased size of the lung blood vessels (pulmonary vascularity).
Totally anomalous pulmonary venous return - X-ray
illustration
-
Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This X-ray shows an enlarged heart, a large liver, and increased pulmonary vascularity.
Totally anomalous pulmonary venous return - X-ray
illustration
-
Heart - section through the middle - illustration
The interior of the heart is composed of valves, chambers, and associated vessels.
Heart - section through the middle
illustration
-
Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease.
Totally anomalous pulmonary venous return - X-ray
illustration
-
Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This X-ray shows an enlarged heart, a large liver, and increased size of the lung blood vessels (pulmonary vascularity).
Totally anomalous pulmonary venous return - X-ray
illustration
-
Totally anomalous pulmonary venous return - X-ray - illustration
Totally anomalous pulmonary venous return (TAPVR) is a form of congenital heart disease. This X-ray shows an enlarged heart, a large liver, and increased pulmonary vascularity.
Totally anomalous pulmonary venous return - X-ray
illustration
Review Date: 10/23/2023
Reviewed By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.