Pulmonary Atresia

Overview

Babies who have pulmonary atresia with intact ventricular septum (PA/IVS) need immediate medical attention. Mayo Clinic pediatric specialists have extensive experience in treating children and adults who have PA/IVS. Mayo Clinic is an established center for the diagnosis and surgical treatment of congenital heart problems. A team of medical experts coordinates patients' care, and ensures that all of their needs are met. This integrated approach is essential to achieve the optimal outcome.

Diagnosis of Pulmonary Atresia with Intact Ventricular Septum

The diagnosis is usually made in childhood. A physician may suspect pulmonary atresia when examining the patient, but generally more testing is necessary to confirm a diagnosis. An echocardiogram (ultrasound of the heart) can show the heart defect, and additional tests may be needed, including cardiac catheterization, electrocardiogram (ECG) and X-rays. Read more about pulmonary atresia diagnosis.

Treatment Options

There are four surgical treatment options: heart catheterization, balloon atrial septostomy, open-heart surgery and other additional heart surgeries, depending on the patient's condition. Read more about pulmonary atresia treatment options.

About Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)

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Pulmonary atresia (atresia means "no opening") with intact ventricular septum (PA/IVS) is a rare congenital heart disease. Pulmonary atresia is when the valve that lets blood flow from the lower-right chamber (right ventricle) of the heart to the lungs has not formed properly or is closed. This abnormality is present from the very early stages of heart development (around the first eight weeks of pregnancy), resulting in an underdeveloped right ventricle. Babies who have PA/IVS need immediate attention in a medical center with experienced pediatric cardiologists and pediatric cardiovascular surgeons. Read more about pulmonary atresia.