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Pulmonary atresia

Definition

Pulmonary atresia is a form of heart disease that occurs from birth (congenital heart disease), in which the pulmonary valve does not form properly. 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, a solid sheet of tissue forms where the valve opening should be, and the valve stays closed. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.

Alternative Names

Pulmonary atresia - intact ventricular septum; PA/IVS

Causes

As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is associated 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.

Persons with pulmonary atresia with intact ventricular septum may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart.

Symptoms

Symptoms usually 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

Exams and Tests

The health care provider will use a stethoscope to listen to the heart and lungs. Persons with a PDA have a heart murmur that can be heard with a stethoscope.

The following tests may be ordered:

  • Chest x-ray
  • Echocardiogram
  • Electrocardiogram (ECG)
  • Heart catheterization
  • Pulse oximetry - shows the amount of oxygen in the blood

Treatment

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 patent ductus arteriosus (PDA).

Multiple treatments are possible, but depend on the extent of the heart abnormalities that accompany the pulmonary valve defect. Potential treatments include:

  • A thin, flexible tube (heart catheterization) to repair the problem
  • Open heart surgery to repair or replace the valve, or to place a tube between the right ventricle and the pulmonary (lung) arteries
  • Reconstructing the heart as a single ventricle (one pumping chamber instead of two)
  • Heart transplant

Outlook (Prognosis)

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.

Possible Complications

  • Delayed growth and development
  • Seizures
  • Stroke
  • Infectious endocarditis
  • Heart failure
  • Death

When to Contact a Medical Professional

Call your health care provider if the baby has:

  • Problems breathing
  • Skin or nails that appear blue (cyanosis)

Prevention

There is no known way to prevent this condition.

All pregnant women should receive routine prenatal care. Many congenital defects can be discovered on routine ultrasound examinations.

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.

References

Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 65.


Review Date: 11/5/2013
Reviewed By: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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