Pulmonary atresia - intact ventricular septum; PA/IVS; Congenital heart disease - pulmonary atresia; Cyanotic heart disease - pulmonary atresia; Valve - disorder pulmonary atresia
Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs.
In pulmonary atresia, the fused valve leaflets form a solid sheet of tissue where the valve opening should be. This blocks normal blood flow to the lung. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.
As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is linked with another type of congenital heart defect called a patent ductus arteriosus (PDA).
Pulmonary atresia may occur with or without a ventricular septal defect (VSD).
- If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS).
- If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot.
Although both conditions are called pulmonary atresia, they are actually different defects. This article discusses pulmonary atresia without a VSD.
People with PA/IVS may also have a poorly developed tricuspid valve. They may also have an underdeveloped or very thick right ventricle, and abnormal blood vessels feeding the heart. Less commonly, structures in the left ventricle, aortic valve, and right atrium are involved.
Symptoms most often occur in the first few hours of life, although it may take up to a few days.
Symptoms may include:
- Bluish colored skin (cyanosis)
- Fast breathing
- Poor eating habits (babies may get tired while nursing or sweat during feedings)
- Shortness of breath
Exams and Tests
The health care provider will use a stethoscope to listen to the heart and lungs. People with a PDA have a heart murmur that can be heard with a stethoscope.
The following tests may be ordered:
A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a PDA.
Multiple treatments are possible, but depend on the extent of the heart abnormalities that accompany the pulmonary valve defect. Potential invasive treatments include:
- Biventricular repair -- This surgery separates the blood flow to the lungs from the circulation to the rest of the body by creating two pumping ventricles.
- Univentricular palliation -- This surgery separates the blood flow to the lungs from the circulation to the rest of the body by constructing one pumping ventricle.
- Heart transplant.
Most cases can be helped with surgery. How well a baby does depends on:
- Size and connections of the pulmonary artery (the artery that takes blood to the lungs)
- How well the heart is beating
- How well the other heart valves are formed or how much they are leaking
Outcome varies because of the different forms of this defect. A baby may need only a single procedure or could need three or more surgeries and have only a single working ventricle.
Complications may include:
- Delayed growth and development
- Infectious endocarditis
- Heart failure
When to Contact a Medical Professional
Call your provider if the baby has:
- Problems breathing
- Skin, nails or lips that appear blue (cyanosis)
There is no known way to prevent this condition.
All pregnant women should get routine prenatal care. Many congenital defects can be found on routine ultrasound exams.
If the defect is found before birth, medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) can be present at the birth, and ready to help as needed. This preparation can mean the difference between life and death for some babies.
Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. Philadelphia, PA: Elsevier Saunders; 2017:chap 58.
Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.
Last reviewed on: 12/8/2017
Reviewed by: Steven Kang, MD, Director, Cardiac Electrophysiology, Alta Bates Summit Medical Center, Stanford Healthcare, Oakland, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.