Inhaled prostanoid therapy for pulmonary arterial hypertension - a promise unfulfilled?

摘要

Pulmonary arterial hypertension (PAH) is an incurable, progressive disease resulting from narrowing of the precapillary vasculature, ultimately leading to right ventricular failure and death. PAH-specific vasodilators have drastically altered the trajectory of this once fatal disease, improving walk distance and hemodynamics, reducing clinical worsening and enhancing quality of life. Although current treatments are aimed at restoring the balance of endogenous vasoactive agents by enhancing the beneficial effects of nitric oxide (NO) and prostacyclin and blunting the deleterious effects of endothelin, only continuous infusion prostacyclin therapy has demonstrated improved survival in randomized clinical trials [1]. Although highly efficacious, continuous infusion therapy remains cumbersome, requiring a portable pump, and either a central venous catheter for intravenous delivery or a smaller catheter for subcutaneous delivery.