Pulmonary atresia

Pulmonary atresia. It is a form of congenital heart disease in which the pulmonary valve is not properly formed. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (pumping chamber on the right side) to the lungs.
In pulmonary atresia, a solid layer of tissue forms where the valve opening should be, and the valve remains closed. Due to this defect, the blood from the right side of the heart cannot travel to the lungs to be oxygenated.


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  • 1 Causes, incidence and risk factors
  • 2 Symptoms
  • 3 Signs and tests
  • 4 Treatment
  • 5 Expectations
  • 6 Complications
  • 7 Situations requiring medical assistance
  • 8 Prevention
  • 9 Alternative names
  • 10 Source

Causes, incidence and risk factors

As with most congenital heart defects, there is no known cause for pulmonary atresia. The condition is associated with another type of congenital heart disease called patent ductus arteriosus (CAP).
People with pulmonary atresia may also have a tricuspid valve with poor development. Similarly, they may have an underdeveloped right ventricle and abnormal blood vessels supplying the heart.
Pulmonary atresia can occur with or without the presence of a ventricular septal defect (IVC). If the person does not have this communication, the condition is called intact septum pulmonary atresia (APTI). If the person has both problems, the condition is called pulmonary atresia with ventricular septal defect and is an extreme form of tetralogy of Fallot. Although the two conditions are called pulmonary atresia, they really are different abnormalities.


Symptoms usually appear in the first hours of life, although they can take up to several days.
Such symptoms may include:

  • Bluish discoloration of the skin ( cyanosis )
  • Fast breathing
  • Fatigue
  • Poor eating habits (babies may get tired while breastfeeding or sweat during feedings)
  • Breathing difficulty

Signs and tests

The doctor will use a stethoscope to listen to the heart and lungs. People with a patent ductus arteriosus have a characteristic heart murmur that can be heard with this instrument.
The following exams can be ordered:

  • Chest x-ray
  • Echocardiography
  • Electrocardiogram (ECG)
  • Cardiac catheterization
  • Pulse oximetry that shows the amount of oxygen in the blood


Generally, a medicine called prostaglandin E1 is used to help blood move (circulate) to the lungs. This medicine maintains an open blood vessel, called a patent ductus arteriosus (CAP), between the pulmonary artery and the aorta.
Other treatments include:

  • Cardiac 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
  • Reconstruct the heart as a single ventricle (for some patients)
  • Heart transplant


Surgery can help in most cases. However, the baby’s prognosis depends on:

  • The quality of the blood vessels supplying the heart
  • How well your heart is beating
  • The amount of leakage from the other heart valves

Forecasts may vary due to different forms of this anomaly. The baby may only have a single catheter procedure, or he may need three or more surgeries and have a single functional ventricle. The prognosis is difficult to predict without knowing all the details about the patient’s condition.


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

Situations requiring medical assistance

Call the doctor if the baby has:

  • Respiratory problems
  • Bluish-looking nails or skin (cyanosis)


There is no known form of prevention.
All pregnant women should receive routine prenatal care. Many birth defects can be discovered on routine ultrasounds. If the defect is detected before birth, some medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) may be present and ready to assist as needed. This preparation can mean the difference between life and death for some babies.

Alternative names

Pulmonary atresia with full septum; APT


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