Learn About Pulmonary Atresia

What is the definition of 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 valve leaflets are fused. This causes a solid sheet of tissue to form where the valve opening should be. Normal blood flow to the lung is blocked as a result. Because of this defect, blood from the right side of the heart is restricted from reaching the lungs to pick up oxygen.

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What are the alternative names for Pulmonary Atresia?

Pulmonary atresia - intact ventricular septum; PA/IVS; Congenital heart disease - pulmonary atresia; Cyanotic heart disease - pulmonary atresia; Valve - disorder pulmonary atresia

What are the causes of Pulmonary Atresia?

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, in fact, 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.

What are the symptoms of Pulmonary Atresia?

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
  • Fatigue
  • Poor eating habits (babies may get tired while nursing or sweat during feedings)
  • Shortness of breath
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What are the current treatments for Pulmonary Atresia?

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 which one is selected depends 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.
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What is the outlook (prognosis) for Pulmonary Atresia?

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.

What are the possible complications of Pulmonary Atresia?

Complications may include:

  • Delayed growth and development
  • Seizures
  • Stroke
  • Infectious endocarditis
  • Heart failure
  • Death
When should I contact a medical professional for Pulmonary Atresia?

Call your provider if the baby has:

  • Problems breathing
  • Skin, nails, or lips that appear blue (cyanosis)
How do I prevent Pulmonary Atresia?

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.

Heart - section through the middle
Heart - front view
What are the latest Pulmonary Atresia Clinical Trials?
Targeting Normoxia in Neonates With Cyanotic Congenital Heart Disease in the Intra-operative and Immediate Post-operative Period (T-NOX)

Summary: This clinical trial is studying the use of different levels of oxygen exposure during and after cardiopulmonary bypass in eligible infants to learn about its safety during heart surgery. In addition to having the various doses of oxygen, patients that participate will also have blood samples, ultrasounds of the head, and brain wave patterns monitored. The hypotheses of this trial are: that there w...

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Follow up of Congenital Lung Anomalies (CLA) With Antenatal Diagnosis - a Swiss Multicentric Database

Summary: Congenital lung anomalies include different pathologies such as congenital cystic adenomatoid malformation, pulmonary sequestration, bronchial atresia, emphysema, bronchogenic cyst. They concern less than 1/10000 births and their physiopathological origin is still poorly understood. The main goal of this project is to pool the cases from different swiss centers on a prospective cohort study, first...

What are the Latest Advances for Pulmonary Atresia?
Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.
Individualized right ventricular outflow tract reconstruction using autologous pulmonary tissue in situ for the treatment of pulmonary atresia with ventricular septum defect.
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A Novel Modified Hybrid Therapy for Pulmonary Atresia With Intact Ventricular Septum.
Who are the sources who wrote this article ?

Published Date: October 10, 2021
Published By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Valente AM, Dorfman AL, Babu-Narayan SV, Kreiger EV. Congenital heart disease in the adolescent and adult. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 82.

Well A, Fraser CD. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice. 21st ed. Philadelphia, PA: Elsevier; 2022:chap 59.