Pulmonary atresia is an extremely rare form of congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is a flap-like opening on the right side of the heart that allows blood to move to the lungs.

In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.

Atresia - pulmonary; PA/IVS

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.

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)
• Shortness of breath
• Fast breathing
• Poor eating habits (babies may get tired while nursing or sweat during feedings)
• Fatigue

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

The following tests may be ordered:

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