Pulmonary atresia (uh-TREE-zhuh) is a heart defect present at birth (congenital) that's normally diagnosed within the first few hours or days of life. In pulmonary atresia, the valve that lets blood out of the heart to go to your baby's lungs (pulmonary valve) doesn't form correctly.
Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So blood can't travel by its normal route to pick up oxygen from the lungs. Instead, some blood travels to the lungs through other natural passages within the heart and its arteries.
These passages are necessary when your baby is developing in the womb and they normally close soon after birth. Babies with pulmonary atresia typically have a bluish cast to their skin because they aren't getting enough oxygen.
Pulmonary atresia is a life-threatening situation. Procedures to correct your baby's heart condition and medications to help your baby's heart work more effectively are the first steps to treat pulmonary atresia.
Pulmonary atresia care at Mayo Clinic
Aug. 04, 2017
- Facts about pulmonary atresia. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/pulmonaryatresia.html. Accessed Oct. 28, 2015.
- Single ventricle defects. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp#.VjE3ztiFOic. Accessed Oct. 28, 2015.
- Axelrod DM, et al. Pulmonary atresia with intact ventricular septum (PA/IVS). http://www.uptodate.com/home. Accessed Oct. 28, 2015.
- Congenital heart defects. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/chd#. Accessed Oct. 29, 2015.
- Congenital heart defects: Tracking and research. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/research.html. Accessed Nov. 20, 2015.
- Congenital heart defects and CCHD. March of Dimes. http://www.marchofdimes.org/baby/congenital-heart-defects.aspx. Accessed Nov. 20, 2015.
- Isotretinoin and other retinoids during pregnancy. March of Dimes. http://www.marchofdimes.org/pregnancy/isotretinoin-and-other-retinoids-during-pregnancy.aspx. Accessed Nov. 20, 2015.
- Perloff JK, et al. Pulmonary atresia with intact ventricular septum. In: Perloff's Clinical Recognition of Congenital Heart Disease. 6th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.clinicalkey.com. Accessed Nov. 23, 2015.
- Kouchoukos NT, et al. Pulmonary atresia and intact ventricular septum. In: Kirklin/Barratt-Boyles Cardiac Surgery. 4th ed. Philadelphia, Pa.: Saunders Elsevier; 2013. http://www.clinicalkey.com. Accessed Nov. 23, 2015.
- Geggel RL. Diagnosis and initial management of cyanotic heart disease in the newborn. http://www.uptodate.com/home. Accessed Nov. 23, 2015.
- Park MK. Cyanotic congenital heart defects. In: Pediatric Cardiology for Practitioners. 6th ed. Philadelphia, Pa.: Mosby Elsevier; 2014. http://www.clinicalkey.com. Accessed Nov. 23, 2015.
- Hay WW, et al. Cardiovascular diseases. In: Current Diagnosis & Treatment: Pediatrics. 22nd ed. New York, N.Y.: McGraw-Hill Education; 2014. http://www.accessmedicine.com. Accessed Nov. 25, 2015.
- Your child's special needs. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/CongenitalHeartDefectsToolsResources/Web-Booklet-If-Your-Child-Has-a-Congenital-Heart-Defect_UCM_316608_Article.jsp#.VlYG3diFOic. Accessed Nov. 25, 2015.
- Riggen EA. Allscripts EPSi. Mayo Clinic, Rochester, Minn. Oct. 20, 2015.
- Taggart NW (expert opinion). Mayo Clinic, Rochester, Minn. Jan. 6, 2016.