Staged Repair of Pulmonic Stenosis and Hypoplastic Right Ventricle

ISOLATED pulmonic valvular stenosis can be successfully treated by either closed or open techniques. Good results have been obtained with a transventricular valvulotome,1under direct vision with hypothermic circulatory arrest,2or with extracorporeal circulation.3 When severe pulmonic stenosis is associated with a diminutive right ventricular cavity, the prognosis is poor and most of these patients die in infancy.4 This report concerns two infants with this combination of anomalies and who were successfully treated by a two-stage surgical procedure. Initial closed transventricular valvotomy was performed under emergency conditions in each patient. Subsequently a definitive correction involving a more precise valvotomy and prosthetic enlargement of the right ventricular outflow tract was performed with cardiopulmonary bypass. Analysis of previous and present results suggests that prognosis is improved by a two-stage repair, consisting of an initial rapid palliative valvotomy and a later definitive procedure. Case Histories Case 1. —A 1-year-old