Partial anomalous pulmonary venous return

Partial anomalous pulmonary venous return, sometimes called partial anomalous pulmonary venous connection, is a heart defect present at birth (congenital) in which some of the pulmonary veins carrying blood from the lungs to the heart flow into other blood vessels or into the heart's upper right chamber (right atrium), instead of correctly entering the heart's upper left chamber (left atrium). This causes some oxygen-rich blood from the lungs to mix with oxygen-poor blood before entering the right atrium.

This defect is due to some of the pulmonary veins incorrectly attaching to the heart's right atrium or other blood vessels, instead of connecting to the left atrium as they normally do.

Some people with this defect also have a hole between the upper heart chambers (atrial septal defect), which allows blood to flow between the upper heart chambers (atria).

People with this condition may also have other congenital heart defects.

Diagnosis

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

This condition is usually diagnosed with an echocardiogram. This test uses sound waves to produce video images of your heart in motion. Doctors may use this test to determine whether the right atrium or ventricle is enlarged, and see how many pulmonary veins are attaching to the heart's left atrium.

Doctors may also use other imaging tests, such as an MRI or CT scan, to diagnose your condition or gather more information about your condition.

Treatment

If you aren't experiencing symptoms or if there isn't much mixing of oxygen-rich and oxygen-poor blood, you may not need surgery to treat this condition.

Surgery may be needed if there is a large amount of oxygen-rich and oxygen-poor blood mixing in the heart, or if you have had many pulmonary infections. If you're having surgery for another heart condition, surgeons may repair this heart defect at the same time.

To repair this defect, surgeons generally reconnect the pulmonary veins to the left atrium. Surgeons will also close the hole between the atria, if there is one.

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

March 09, 2018
References
  1. Fulton DR, et al. Partial anomalous pulmonary venous connection. https://www.uptodate.com/contents/search. Accessed Sept. 29, 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. Kliegman RM, et al. Acyanotic congenital heart disease: Left-to-right shunt lesions. In: Nelson Textbook of Pediatrics. 20th ed. Philadelphia, Pa.: Elsevier; 2016. https://www.clinicalkey.com. Accessed Sept. 19, 2017.
  4. What are congenital heart defects? National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health/health-topics/topics/chd. Accessed Oct. 2, 2017.