Management of pulmonary atresia with ventricular septal defect

Opinion statement It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patient’s pericardium, and this patient continues to do well. In the subsequent four decades, great advances have been made in the diagnosis, with regard to the often very complex anatomy of pulmonary blood supply that this extremely heterogeneous group of patients manifest, and their subsequent surgical management. Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. Although the ideal extracardiac conduit has yet to be developed, necessitating periodic conduit replacement in corrected patients, the late results in this group of patients are generally excellent, with many now achieving survival well into the adulthood years with good quality of life. Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.