Total anomalous pulmonary venous return is a heart disease that is present at birth (
TAPVR; Total veins
The cause of total anomalous pulmonary venous return (TAPVR) 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
How 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.
The infant may appear very sick and may have the following symptoms:
- Bluish color of the skin (cyanosis)
- Frequent respiratory infections
Note: Sometimes, no symptoms may be present in infancy or early childhood.
Exams and Tests
- Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached
- ECG shows enlargement of the ventricles (ventricular hypertrophy)
- Echocardiogram may show that the pulmonary vessels are attached
- MRI of the heart can show the connections between the pulmonary vessels
- X-ray of the chest shows a normal to small heart with fluid in the lungs
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.
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.
- Breathing difficulties
- Heart failure
- 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.
Call your health care provider if you notice symptoms of TAPVR — prompt attention is required.
There is no known way to prevent TAPVR.