Total anomalous pulmonary venous connection (TAPVC) is a rare heart defect.
In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle through the tricuspid valve. The blood travels to the lungs through the pulmonary valve to pick up fresh oxygen. Next, the blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body.
Heart Chambers and Valves
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Blood Flow Through the Heart
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With TAPVC, the pulmonary veins that return oxygenated blood from the lungs connect to the right side of the heart, instead of the left atrium. This leads to the mixing of oxygenated and de-oxygenated blood. The body tissue does not receive as much oxygen as it is supposed to. TAPVC can be mild to severe. There can be a range of connection problems. Other heart problems may be present, as well.
TAPVC is a congenital defect. This means that the heart forms incorrectly when the baby is in the womb. It is not known exactly why the heart develops this way in some babies.
More research is needed to confirm causes and risk factors for TAPVC. Risk factors thought to be related to this condition include: Family history of heart defectsHaving other heart defectsEnvironmental exposures
Symptoms may include: Blue or pale grayish skin colorDifficulty breathingPoor growth
You will be asked about your child’s symptoms and medical history. A physical exam will be done. An irregular heart rate may be detected during the exam.
Your child's bodily fluids may be tested. This can be done with blood tests.
Images may be taken of your child's bodily structures. This can be done with: EchocardiogramChest x-ray
Your child's heart function may be tested. This can be done with: ElectrocardiogramCardiac catheterization
Talk with the doctor about the best treatment plan for your child. Treatment options include:
Surgery is needed to correct the defect. It can range from a simple repair to a complex repair. If blood flow is obstructed, emergency surgery will need to be done. The goal of surgery is to reconnect the pulmonary veins to the left atrium.
Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent an infection in the heart.
Preventing fetal heart defects may not always be possible, but you can reduce your risk by: Visiting the doctor regularly to monitor your health and the health of the baby. Prenatal tests may detect a heart defect in a growing fetus.Having a healthy lifestyleEating nutritious food and take prenatal vitamins
Not drinking alcohol,
, or using drugs during pregnancy
Practicing good hygiene and staying away from people who are sickAvoiding exposure to environmental toxins
Total anomalous pulmonary venous connection. EBSCO DynaMed website. Available at:
http://www.ebscohost.com/dynamed. Updated August 9, 2013. Accessed November 10, 2014.
Total anomalous pulmonary venous connection (TAPVC). American Heart Association website. Available at:
http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Total-Anomalous-Pulmonary-Venous-Connection-TAPVC_UCM_307039_Article.jsp. Updated January 27, 2014. Accessed November 10, 2014.
Total anomalous pulmonary venous return. Johns Hopkins University, Cove Point Foundation website. Available at:
http://www.pted.org/?id=tapvr1. Updated May 16, 2011. Accessed November 10, 2014.
Last reviewed November 2014 by Michael Woods, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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