Pulmonary atresia with intact ventricular septum

Pulmonary atresia with intact ventricular septum is an apparently simple cyanotic congenital heart disease; however it presents with diverse morphology and associated lesions that imply a more complex diagnosis and treatment. Its high mortality before six months of age makes the surgical treatment mandatory. The most important aspects of morphology, pathophysiology, diagnosis and surgical treatment are discussed. The key objectives of surgical treatment are the separation of the systemic from the pulmonary circulation to avoid cyanosis without a decrease of cardiac output and increase of central venous pressure and to recruit the right ventricle for the pulmonary circulation whenever the coronary circulation does not depend from it. Furthermore, the role of echocardiography and interventional cardiology techniques are discussed. A review of all surgical possibilities in this disease is also performed.