The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with or without conventional surgical intervention. Therefore, a comprehensive program of medical and surgical treatment is necessary to improve long-term outlook for these infants. Such a program consists of management of the neonate at initial presentation with prompt administration of prostaglandins and institution of a combination of surgical procedures (isolated pulmonary valvotomy, valvotomy plus modified Blalock-Taussig shunt, Blalock-Taussig shunt plus balloon atrial septostomy, or Blalock-Taussig shunt alone) depending on the results of morphological analysis of the right ventricle; this treatment regimen is designed to relieve hypoxemia, encourage right ventricular growth, and provide adequate egress of blood from the right atrium. Another important element of management is to perform follow-up hemodynamic and angiographic studies when the patient is between 6 and 12 months old to ensure that the objectives of the comprehensive program are being met. Finally, a definitive repair should be offered. This can be done by using or bypassing the right ventricle, depending on whether it can support the pulmonary circuit.