Total anomalous pulmonary venous return (TAPVR) is a rare, life-threatening congenital heart defect that affects newborns. It happens when veins from your baby's lungs connect to the wrong place in their heart, leading to problems with blood flow. Difficulty breathing and blueish skin usually appear right after birth. Healthcare providers treat TAPVR with surgery.
Total anomalous pulmonary venous return (TAPVR) is a life-threatening heart defect. It’s congenital, which means it’s present at birth. Most of the time, babies show signs of this condition soon after they’re born. But some babies aren’t diagnosed until they’re several weeks or months old. Healthcare providers also call this condition total anomalous pulmonary venous connection (TAPVC).
TAPVR happens when veins (blood vessels) from your baby's lungs connect to the wrong place in their heart. As a result, their heart can’t pump fully oxygenated blood to the rest of their body. Babies with TAPVR have trouble breathing and their skin may appear blue. These symptoms will be present at birth if there's any narrowing in the connection of your baby's lung veins to their heart. Doctors call this type of narrowing obstructed TAPVR.
This condition requires surgical repair. With early surgery, most children with this heart defect survive into adulthood, but some will need repeat surgery or procedures to treat narrowing in their veins later in life. Because of this, people with TAPVR need to see a cardiologist (a provider who specializes in the heart) regularly to monitor their health following surgery.
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TAPVR is a rare condition. It affects about 1 of every 10,000 live births. Nearly every baby with TAPVR needs surgery to survive. Though extremely rare, there are some adults with TAPVR who receive this diagnosis later in life and didn't have surgery as a baby. However, these patients usually have high pressures within their lungs, called pulmonary hypertension, that can make surgical repair very challenging.
All children with TAPVR also have an atrial septal defect (ASD). An ASD is a hole between the heart’s right and left atriums.
TAPVR is a type of congenital heart condition. It happens when veins that run from your baby's lungs to their heart connect in the wrong place. This abnormality causes problems with how blood flows through their body.
In a normally functioning cardiovascular system, the lungs send oxygen-rich blood to the heart’s left atrium (the chamber in the heart’s upper left side). In babies with TAPVR, the oxygen-rich blood flows through pulmonary veins to the heart’s right atrium instead, most commonly through another vein called a vertical vein. This vein is often the site of narrowing that leads to a diagnosis of obstructed TAPVR. In the right-sided atrium, the oxygen-rich blood mixes with blood that doesn’t have as much oxygen. This low-oxygen blood travels out of the heart to the rest of your baby's body.
The types of TAPVR are:
Healthcare providers aren’t sure what causes TAPVR. It happens when your baby's heart and blood vessels are forming in the uterus during fetal development. Cardiologists can sometimes diagnose this condition in the prenatal period with a prenatal echocardiogram, which is very helpful to planning.
The condition doesn't seem to be inherited (passed down through families). But there have been cases of siblings who are born with the same heart defect.
Symptoms of TAPVR usually appear very soon after birth. But some babies don’t have symptoms for several weeks. Symptoms may include:
Your baby’s pediatrician will do an exam and listen to your baby’s heart. To see pictures of your child’s heart, they may recommend:
These tests allow your baby's healthcare provider to see images of your baby’s heart and veins. They also help your baby's provider evaluate blood flow and look for abnormalities.
Healthcare providers treat TAPVR with open-heart surgery. Most often, providers perform this surgery as soon as possible after diagnosing the condition.
While your baby is asleep under general anesthesia, a surgeon makes cuts (incisions) in your baby’s chest and heart. They connect the pulmonary veins to the correct place in your baby's heart. If your baby also has a hole between the left and right atria (atrial septal defect), the surgeon will close the hole.
It isn’t possible to prevent this congenital heart condition. If you have a family history of heart defects or you have TAPVR, talk to your healthcare provider before starting a family.
With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%. Talk to your child’s healthcare provider about their ability to participate in sports and physical activities. Your child may need to limit vigorous exercise.
Your child will need regular visits with their cardiologist as they grow into adulthood. Life-long follow-up visits can help cardiologists detect problems such as an irregular heart beat or blocks (obstructions) in the pulmonary arteries.
Kids with this condition have a higher risk of developing endocarditis, which happens when bacteria enter their bloodstream and affect their heart’s valves. To prevent endocarditis, your child may need to take antibiotics before getting some dental work. Ask your child’s healthcare provider if this is necessary.
Get medical help right away if your baby:
A note from Cleveland Clinic
Total anomalous pulmonary venous return (TAPVR) is a serious congenital heart defect. This rare condition is fatal if untreated. But surgical treatments are extremely effective, and the outlook for people with this condition is very good. If your baby has signs of TAPVR, get help right away. Early diagnosis and treatment are critical to a good prognosis. Following surgery, be sure to visit your child’s healthcare provider for regular follow-up visits. These visits help your child’s provider treat problems and monitor their health.
Last reviewed by a Cleveland Clinic medical professional on 05/18/2022.
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