Pulmonary Atresia with Intact Septum

Abstract

Pulmonary atresia with intact ventricular septum (PAIVS) is a congenital malformation that consists of an atretic pulmonary valve and in which there is no associated ventricular septal defect (VSD). There are variable degrees of right ventricular (RV) cavity hypoplasia and estimating RV size is critically important as it guides management. PAIVS is also associated with coronary artery abnormalities and in some cases coronary flow is dependent on blood supply from the RV (a ‘RV dependent coronary circulation’). A wide spectrum of management options that often combine surgical and catheter intervention exist, and there is no single defined strategy that caters to all patients. Specific operations or a combination of procedures are individualised according to the patients’ morphology, potential changes in RV size with time and centre preference. During the neonatal period, management aims include: securing pulmonary blood flow, relieving RV outflow tract obstruction (if an RV dependent coronary circulation is excluded) and establishing antegrade flow through the RV. The ultimate goal of surgery is to separate the pulmonary and systemic circulation by either biventricular, univentricular or one and a half ventricle pathways. Survival estimates are variable and often epoch related. Five- and ten-year survival of up to 80% have been reported. The univentricular management route is the preferred route for patients with an RV dependent coronary circulation.