I thank Dr. Raja for an excellent appraisal of the role of sildenafil (Viagra; Pfizer Laboratories, New York, NY) in the management of rebound pulmonary hypertension after withdrawal of inhaled prostacyclin, as highlighted in our recent case report.1Dr. Raja has correctly highlighted that sildenafil is an alternative to iloprost in this setting.2–7Our discussion of iloprost in the case report focused on its advantages over inhaled prostacyclin in the withdrawal of inhaled pulmonary vasodilator therapy. The pharmacokinetics of iloprost highlight a limitation of inhaled prostacyclin, namely its short half-life, that may facilitate serious rebound pulmonary hypertension.
However, this discussion was by no means intended to minimize the role of alternative approaches to the management of rebound pulmonary hypertension. As emphasized, a tiered multimodal therapeutic approach to pulmonary hypertension is essential for successful management.1,8,9Indeed, this multimodal therapeutic approach to this clinical scenario not only includes sildenafil but also extends beyond this agent. The withdrawal of inhaled pulmonary vasodilators with a short half-life (nitric oxide, prostacyclin) should be managed in the setting of optimized ventilation, and where required, sufficient supplemental pulmonary vasodilator, whether inhaled, intravenous, or oral. There is a wide selection of possible agents that may be administered alone or in synergistic combination.8,10The choice of regimen should also take into account drug availability, drug familiarity, and patient idiosyncrasies.
In summary, rebound pulmonary hypertension with withdrawal of nitric oxide or prostacyclin should be approached in a tiered multimodal fashion. Although sildenafil is eminently suitable, it is but one of a possible menu of pharmacologic choices.
Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. firstname.lastname@example.org