Pulmonary Atresia with Intact Septum

  • This defect is associated with complete obstruction of the Pulmonary Artery. However, as there is no associated VSD, blood is diverted from the right atrium to the left atrium, via the 'Foramen Ovale' or a defect in the Atrial Septum (ASD). The right ventricle (RV) is usually small (Hypoplastic), though its wall may be thickened (Hypertrophied).

    Survival depends on the ductus remaining open in the early days of life (in order for blood to reach the lungs). Affected infants usually become blue soon after birth (as the ductus closes). Early surgery often involves opening the Pulmonary Valve and a 'Shunt' operation.  Corrective surgery is carried out later in childhood if the right ventricle is adequate. In some infants the RV is too small to allow complete repair and other forms of surgery may be required (e.g. Fontan operation)


    Surgery is often needed urgently, in the first week or two of life and involves a 'shunt' operation (often combined with opening the obstructed valve at the same time). Complete repair may be possible later in childhood, if the right ventricle is large enough.