Diagnosis and treatment of pulmonary artery hypertension.

It is intellectually unsatisfying for physicians to admit that a group of patients remain for whom no cure is available in modern medicine. Despite our inability to cure pulmonary artery hypertension, therapeutic medical and surgical advances over the past decade have resulted in significant improvements in the outcome for children with various forms of pulmonary hypertension. This review discusses the work-up and evaluation of a child who presents with pulmonary artery hypertension and discusses recent therapeutic advances. The roles of anticoagulation, vasodilator therapy (including inhaled nitric oxide and chronic intravenous prostacyclin), and transplantation are discussed. These therapeutic modalities have improved quality of life, exercise capacity, and pulmonary hemodynamics as well as long-term survival for children with pulmonary hypertension.