Pulmonary atresia natural history


 * Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [mailto:psingh@perfuse.org], ; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Prognosis
The outcome varies for every child. If the condition is left uncorrected it is fatal, but the prognosis has greatly improved over the years for babies with pulmonary atresia. Some factors that affect how well the child does include how well the heart is beating, the condition of the blood vessels that supply the heart, and how leaky the other heart valves are. Most cases of pulmonary atresia can be helped with surgery. If the patient's right ventricle is exceptionally small, many surgeries will be needed in order to help stimulate normal circulation of blood to the heart.

If uncorrected, babies with this type of congenital heart disease will only survive for the first few days of life while the fetal shunts between left and right circulations remain patent. Many children with pulmonary atresia will go on to lead relatively normal, uncomplicated healthy lives.

Doctors are unsure of the cause of congenital heart defects, but there are some medical conditions that have been found to increase the risk of having a baby with a heart defect such as congenital heart disease in the mother, father, brother, or sister of the baby, a diabetic mother, use of drugs or alcohol or over the counter prescriptions during pregnancy. These conditions do not mean that the baby will definitely be born with a heart defect, but that it runs a higher chance. Sometimes babies are born without any of these conditions; the cause is unknown. All pregnant women should do all that they can to ensure and maintain the health of their unborn child.

Children with pulmonary atresia will need to be seen regularly throughout their lifetime by a cardiologist to be sure that their heart is functioning properly. Due to their increased risk of endocarditis, children diagnosed with pulmonary atresia should have amoxicillin before any type of dental work or other types of surgeries to help prevent this infection from occurring.