Pulmonary atresia with intact ventricular septum is defined as atresia of one or more of the three – the main pulmonary artery, the pulmonary valve, and the right and left bifurcations of the pulmonary artery – and is often associated with varying degrees of right ventricular and tricuspid valve dysplasia. The ventricular septum is intact and the aortic relationships are normal. Pulmonary atresia with intact ventricular septum is a rare congenital heart disease with a prevalence of 1% to 1.5% of congenital heart disease, with no gender difference. 90% of children have cyanosis at birth or within a short time after birth, and it is progressively worse. The degree of cyanosis depends mainly on the blood flow to the lungs through the ductus arteriosus and other body-pulmonary artery traffic. If the ductus arteriosus is small and cyanosis is severe, survivors have pestle-like fingers (toes). Growth and development disorders, often with palpitations and shortness of breath after activity, but squatting is rare. If the body-lung traffic is large, cyanosis is mild, susceptible to respiratory infections, and heart failure can often occur early. If the arterial duct tends to close, the cyanosis is progressively worse and requires urgent surgical treatment. In severe cases, the partial pressure of arterial oxygen may drop to 20 mmHg and the oxygen saturation is only about 40%. Right heart failure is most often seen in patients with tricuspid valve insufficiency and small atrial septal defects, with hepatomegaly, swelling and apical rhythm. If the birth is normal, the child can generally develop better, with cyanosis, dyspnea and metabolic acidosis after birth. Diagnosis can be made by physical signs, chest radiograph, electrocardiogram, cardiac color ultrasound, CT, MRI, right heart catheterization and cardiography. Among them, CT and MRI can show the development of the pulmonary arteries and the establishment of collateral circulation. The natural prognosis of pulmonary atresia with an intact septum is poor, with 50% of children dying two weeks after birth and 85% of children dying six months after birth. The main causes of death are hypoxia and metabolic acidosis, which are particularly likely to occur after ductus arteriosus closure. The few patients who survive into childhood do so primarily through a large atrial septal defect and patent ductus arteriosus, and very rarely through a large extracardiac collateral circulation into adulthood. Surgical treatment is indicated once the diagnosis is made. Most surgical procedures are staged. Postnatal intravenous prostaglandin E1 is administered to delay the time to ductus arteriosus closure. Body-pulmonary artery shunts are performed first or simultaneous pulmonary valvotomy under extracorporeal circulation; stage 2 is performed at 3 to 5 years of age to establish a right ventricular to pulmonary artery flow pathway and to close the atrial traffic and extracardiac shunts. The survival rate for children undergoing palliative surgery in the neonatal period was 27% for pulmonary valvotomy alone, 30% for body-pulmonary shunt alone, and 79% for both procedures. The early mortality rate for radical surgery is 25%, and the main cause of death is left ventricular insufficiency. The main complication of applying an extracardiac tube with a valve is stenosis, which requires reoperation for replacement. In children with long-term survival, cardiac function can be restored to class I-II.