Total anomalous pulmonary venous connection is a rare condition in which the pulmonary veins are connected to the heart's upper-right chamber (the right atrium) instead of its upper-left chamber (the left atrium).

Pulmonary veins carry oxygen-rich blood from the lungs to the heart. Normally, they carry it to the left side of the heart, which pumps this oxygen-rich blood into the body. But this defect causes blood to flow from the lungs to the right atrium instead.

In the right atrium, the oxygen-rich blood mixes with the oxygen-poor blood entering the heart from the body. From the right atrium, this mixture of oxygen-rich and oxygen-poor blood flows through a hole called an atrial septal defect (ASD) and into the left atrium. The left atrium sends blood to the left ventricle, which pumps blood into the body.

When oxygen-poor blood from the right atrium is pumped into the body along with oxygen-rich blood from the lungs, the fingers, toes, and lips may appear blue. This condition is called cyanosis.

How is it treated?

Surgery is used to connect the pulmonary veins to the left atrium and to close the atrial septal defect. When surgery is done early enough, the long-term outlook is very good. Still, the child will need lifelong follow-up to make sure that a blockage does not develop in the pulmonary veins. There is also an increased risk for an abnormal heart rhythm (called an arrhythmia).

See also on this site: Congenital Heart Disease

See on other sites:

MedlinePlus
www.nlm.nih.gov/medlineplus/ency/article/001115.htm
Total anomalous pulmonary venous return

American Heart Association 
www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/
AboutCongenitalHeartDefects/Total-Anomalous-Pulmonary-Venous-Connection-TAPVC_UCM_307039_Article.jsp 
Total Anomalous Pulmonary Venous Connection (TAPVC)

Texas Adult Congenital Heart Center (TACH) program 
www.texaschildrens.org/carecenters/heart/adult_congenital_heart_program
/Default.aspx enables patients with congenital heart disease to receive a seamless continuation of care from birth to old age. 
  

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Updated September 2011