Pediatric Primary Pulmonary Hypertension.

Primary pulmonary hypertension is a disease for which there is no single best therapy. Rather, it is a process that progresses inexorably to disability and death, for which there are a variety of palliative therapies, all with significant side effects, and none curative. Nevertheless, it is clear that the available therapies improve the quality of life and prolong life; failure to offer therapy for patients with this disease in the current era is indefensible. As primary pulmonary hypertension progresses, one must chose from among the available therapies the regimen that provides the most benefit for the patient with the least associated morbidity. Organ replacement is appropriate only after all other available therapies have been exhausted. The recommended hierarchy of therapy is 1) anticongestive therapy, anticoagulation, and supplemental oxygen, 2) calcium channel blockade, 3) continuous intravenous prostacyclin, 4) beta-receptor agonists for cardiac support, and 5) lung transplantation. Newer therapies, described in this review, soon will be incorporated into this hierarchy.