Surgical outcomes for patients with congenital heart disease have improved dramatically over the past few decades due, in part, to improvements in preoperative and postoperative management, cardiopulmonary bypass and intraoperative technique, and the development of pediatric cardiology as a subspecialty. Patients with pulmonary hypertension, however, remain a particularly challenging subgroup worldwide. Determination of operability and, when reasonable, timing of surgery, remains at times difficult. An overview of the evaluation process for this patient population is presented along with a review of evidence for risk stratification and a generalized account of the current state of medical therapy.