Pulmonary atresia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]
Overview
Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. Atresia means "no opening". The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs.
Oxygen-poor blood travels directly from the right atrium to the left side of the heart through a shunt (patent foramen ovale or any other type of atrial septal defect). This oxygen-poor blood is then pumped through the aorta to the rest of the body, making fingers, toes, and lips appear blue or cyanotic.
Babies with this type of cyanotic congenital heart disease survive only for the first few days of life while the normal fetal shunts (patent ductus arteriousus, patent foramen ovale) between left and right circulations remain patent. Without an operation in that period to open the pulmonary valve or to make a shunt between the aorta and the pulmonary arteries, the condition is fatal.
The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, open heart surgery can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform an operation called the Fontan procedure. In this procedure, the right atrium is connected directly to the pulmonary artery. Many children with Pulmonary Atresia will go on to lead 'normal' lives.