Pulmonary atresia is a serious birth defect in the heart which causes congenital heart disease.
The heart has four chambers. One of these chambers is called the right ventricle. The job of the right ventricle is to pump blood into the main lung arteries so the lungs can give oxygen to the blood. In pulmonary atresia, the opening of the right ventricle that leads to the main lung arteries is abnormally formed. The opening is either severely narrowed or absent. Blood cannot be effectively pumped into the lungs, which results in symptoms due to low oxygen in the blood. Affected babies usually have other heart and blood vessel defects as well.
This condition affects the infant within the first few days after birth and generally causes: a bluish color to the skin, known as cyanosisrapid breathinglow oxygen levels in the bloodheart murmurs, or abnormal heart sounds
As with most birth defects, the cause is usually unknown. Some cases may be caused by: infections passed from the mother to the unborn child during pregnancycertain medications taken by the mother during pregnancy, such as medications to control seizuresalcohol use by a woman during pregnancygenetic disorders, such as Down syndrome
All women of childbearing age should make sure they have received all their recommended vaccines before getting pregnant. A woman who is taking any medications should talk to the healthcare professional about the risk of birth defects should she get pregnant. Alcohol should not be used at all during pregnancy. Genetic counseling may be helpful for couples with a personal or family history of birth defects.
The healthcare professional may suspect pulmonary atresia when he or she examines the child. An echocardiogram, which is an imaging test that uses ultrasound, can show the heart defect. Additional special x-ray tests may be needed to define the exact type of heart defect.
Most children with this condition cannot survive without aggressive, immediate treatment. Even with the best treatment available, an infant may die from the condition.
There generally are no risks to others. Parents with an affected child may have a higher risk of having a second child with heart defects.
Emergency treatment is usually needed. The infant may need to be put on a ventilator (an artificial breathing machine). Special medications are usually needed to keep the infant alive while waiting for surgery. Open heart surgery is generally the only hope for a cure. Without it, infants will usually die.
Death can occur even with excellent treatment, as these children are very sick and frail. Surgery can be complicated by bleeding, infection, or reactions to anesthesia.
If the surgery is successful, the child may recover completely. Affected children usually need to visit healthcare professionals regularly for several years to monitor for further heart problems.
A person who has had pulmonary atresia repair during infancy should always inform his or her dentist of the fact, so that he or she can receive antibiotics for routine dental procedures. This precaution helps prevent the heart valves from becoming infected during the unavoidable showers of bacteria through the bloodstream that result from dental work.
Before and after surgery, intensive monitoring is required. This may include blood tests, x-ray tests, urine tests, and other monitoring. The child will need close monitoring by a heart specialist for several years after surgery.
Heart Disease, 1997, Braunwald et al.
