What are the four types of TAPVR?
Generally, there are four types of TAPVR:
- Supracardiac TAPVR. The pulmonary veins drain into the right atrium through the superior vena cava.
- Infracardiac TAPVR. The pulmonary veins drain into the right atrium through the liver (hepatic) veins and the inferior vena cava.
- Cardiac TAPVR. There are two types.
- Mixed TAPVR.
Is TAPVC normal?
Total anomalous pulmonary venous return (TAPVR) is a rare congenital malformation in which all four pulmonary veins do not connect normally to the left atrium. Instead the four pulmonary veins drain abnormally to the right atrium (right upper chamber) by way of an abnormal (anomalous) connection.
What causes total anomalous pulmonary venous return?
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.
How common is partial anomalous pulmonary venous return?
This is a very rare finding and mostly involves the anomalous drainage of the right upper pulmonary vein into the superior vena cava. Only 3% of patients had PAPVC from the left lung to the innominate vein.
What is TAPVC in cardiology?
Total anomalous pulmonary venous return (TAPVR) (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN), or connection (TAPVC) is a birth defect of the heart in which the veins bringing blood back from the lungs (pulmonary veins) don’t connect to the left atrium like usual.
Is total anomalous pulmonary venous return hereditary?
Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart defect that, without surgical correction, has a high mortality rate in the first year of life. It usually occurs without a family history and has a low recurrence risk.
Is TAPVC curable?
To repair this defect, doctors usually connect the pulmonary veins to the left atrium, close off any abnormal connections between blood vessels, and close the atrial septal defect. Infants whose defects are surgically repaired are not cured; they may have lifelong complications.
Can you live with TAPVR?
Key points about TAPVR Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body. All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart. Many children with TAPVR will grow and develop normally after they have surgery.
How do you treat TAPVR?
How is TAPVR treated?
- Supplemental oxygen or a machine that helps your baby breathe (ventilator).
- Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart.
- Different medicine to support the function of the heart.
- ECMO (extracorporeal membrane oxygenation).
What are the symptoms of partial anomalous pulmonary venous return?
In partial anomalous venous return (PAPVR), if only one vein is affected, there are usually no symptoms. If two veins from the same side are affected, symptoms of shortness of breath with exertion and low stamina may occur during childhood. The symptoms are usually mild and may not ever occur.
How is TAPVC diagnosed?
TAPVR might be diagnosed during pregnancy with an ultrasound (which creates pictures of the body). Some findings from the ultrasound may make the health care provider suspect a baby could have TAPVR. If so, the health care provider can request a fetal echocardiogram to confirm the diagnosis.
What is anomalous pulmonary venous connection?
Total anomalous pulmonary venous connection (TAPVC) consists of an abnormality of blood flow in which all four pulmonary veins drain into systemic veins or the right atrium with or without pulmonary venous obstruction. Systemic and pulmonary venous blood mix in the right atrium.