Total anomalous pulmonary venous return

In total anomalous pulmonary venous return, sometimes called total anomalous pulmonary venous connection, the blood vessels that lead from the lungs (pulmonary veins) attach to the heart in incorrect places, instead of attaching correctly to the upper left heart chamber (left atrium). This is a heart defect present at birth (congenital).

Instead of flowing into the left atrium as it normally does, oxygen-rich blood returning to the heart from the pulmonary veins returns to the upper right heart chamber (right atrium) and mixes with oxygen-poor blood either before entering the heart or in the right atrium.

Some blood may then move through a hole between the atria (atrial septal defect) to the left atrium, and then to the lower left heart chamber (left ventricle) and on to the main blood vessel that delivers blood to the body (aorta). But less oxygen is in the blood that flows to the body than is needed. If the hole between the atrium is small, not enough blood may lead to the body, leading to heart failure.

Because not enough oxygen is in the blood that goes out to the body, babies can appear blue (cyanotic). If the blood flow through the pulmonary veins is reduced or blocked, babies can also look blue.

Some blood also moves from the right atrium to the lower right heart chamber (right ventricle), and the blood is then delivered to the pulmonary artery and out to the lungs.

Diagnosis

To diagnose the condition, your child's doctor may review your child's signs and symptoms, conduct a physical exam, and listen to your child's heart with a stethoscope to check for a heart murmur.

An echocardiogram is generally used to diagnose the condition. This test uses sound waves to produce video images of your child's heart in motion. An echocardiogram can show the pulmonary veins leading to the right atrium, holes in the heart, and the size of the heart chambers. It can also measure blood flow rates.

Other tests, such as electrocardiograms or chest X-rays, may be conducted if more information is needed.

Treatment

Surgery is generally needed when a child is young. To repair this congenital heart defect, surgeons connect the pulmonary veins to the left atrium and close the hole between the atria.

Children with this congenital heart defect will need regular follow-up appointments and continuing care with pediatric cardiologists. Adults who have this congenital heart defect require lifelong care and regular follow-up appointments with cardiologists trained in congenital heart disease (adult congenital cardiologists) to monitor for any changes in their condition.

March 06, 2018
References
  1. Total anomalous pulmonary venous connection (TAPVC). American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Total-Anomalous-Pulmonary-Venous-Connection-TAPVC_UCM_307039_Article.jsp#.Wc1Wh9jrvcs. Accessed Sept. 19, 2017.
  2. Bonow RO, et al., eds. Congenital heart disease. In: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2015. https://www.clinicalkey.com. Accessed Sept. 19, 2017.
  3. Soriano B, et al. Total anomalous pulmonary venous connection. https://www.uptodate.com/contents/search. Accessed Sept. 19, 2017.
  4. Facts about total anomalous pulmonary venous return or TAPVR. Centers for Disease Control and Prevention. https://www.cdc.gov/ncbddd/heartdefects/tapvr.html. Accessed Sept. 28, 2017.
  5. What are congenital heart defects? National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/health-topics/topics/chd. Accessed Sept. 28, 2017.