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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.

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Alternative Names

Pulmonary atresia - intact ventricular septum; PA/IVS

Causes, incidence, and risk factors

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).

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

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.

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

Signs 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).

Other 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 (for some patients)
Heart transplant

Expectations (prognosis)

Most cases can be helped with surgery. However, how well a baby does depends on:

The size and connections of the pulmonary artery (the artery that takes blood to the lungs)
How well the heart is beating
How much the other heart valves are leaking

Outcomes can vary 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

Delayed growth and development
Seizures
Stroke
Infectious endocarditis
Heart failure
Death

Calling your health care provider

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: Saunders Elsevier; 2011:chap 65.

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