Pulmonary atresia with intact ventricular septum (PAIVS) is a congenital heart defect where the pulmonary valve is blocked, preventing blood flow from the right ventricle to the lungs. It occurs in 1-3% of congenital heart diseases. Surgical interventions for PAIVS have improved, with 5-year survival rates now around 80%. Treatment depends on factors like the size of the tricuspid valve and whether the coronary arteries depend on blood flow from the right ventricle. Options include biventricular repair, univentricular repair, or transplantation.