Total anomalous pulmonary venous connection

Drenatge venós anòmal pulmonar total a Vall d'Hebron

Total anomalous pulmonary venous connection (TAPVC) means that there is no connection from the pulmonary veins to the left atrium. Instead, they are redirected to the right atrium through an unusual connection. This defect affects new-borns and may need urgent surgery depending on its severity. Other, less severe cases may be detected when the baby is a few weeks or months old. In these cases, surgery is also necessary.

Description

What is it?

In a heart that functions normally, veins take oxygenated blood from the lungs to the left side, and from here to all parts of the body.

In people with TAPVC, oxygenated blood reaches the right side, where it mixes with deoxygenated blood and passes through a hole in the interatrial wall to the left atrium. This means that oxygen levels are lower in these children.

Symptoms

This anomaly works in different ways depending on the level of severity:

TAPVC without obstruction: babies present few symptoms and are often not diagnosed until a few weeks or months after birth.

TAPVC with obstruction: babies have a blockage in the vessels which take blood from the pulmonary veins back to the right atrium. This makes it difficult for blood to leave the lungs and produces breathing difficulties.

Diagnosis

An echocardiogram can detect if a child has TAPVC. This is carried out within a few hours of birth for babies with TAPVC with obstruction. In other children, it is customary to carry out the test after listening to the heart and detecting a murmur and observing low blood oxygen levels.

In addition, an additional imaging test is usually done, such as computed tomography (CT scan) or an MRI to study the anomaly or defect.

Typical treatment

Patients with TAPVC have to undergo surgery. In this procedure, the pulmonary vein flow is redirected to the left atrium and the anomalous communication with the right atrium corrected.

New-borns with TAPVC with obstruction are in a life-threatening situation. It is therefore necessary to perform surgery as soon as they are born to correct the anomaly.

For patients with TAPVC without obstruction, elective surgery can be performed once diagnosis is made.

Children who have had surgery for TAPVC can live a normal life and do not require further procedures. However, cardiological monitoring is necessary.