| | Prostacyclin analogues | Potent pulmonary and systemic vasodilators
Antagonist of platelet aggregation
Epoprostenol is the most commonly used drug in this class, is efficacious, but has several adverse effects related to safety, tolerability, and route of administration (short half-life, requiring the insertion of central venous access)
Nebulized iloprost, 6–9 times a day, has shown improved symptoms, exercise tolerance, and functional capacity
Treprostinil, a stable and long-acting prostacyclin analogue, can be administered subcutaneously and intravenous |
| | Endothelin antagonist | Bosentan, a nonselective ET-1 receptor antagonist, improves exercise tolerance, functional capacity, and pulmonary hemodynamics, but also can worsen hepatic dysfunction and deteriorate renal failure especially in patients with type 2 HRS
Ambrisentan: a selective ETA receptor antagonist, oral and once a day administration, with minimal hepatotoxicity risk |
| | Phosphodiesterase-5-inhibitors | Sildenafil and tadalafil, their use results in an increased NO-mediated vasodilatation in the pulmonary vasculature |
| | Vasopressin analogues | Terlipressin: several reports have shown that the administration of terlipressin decreases pulmonary artery pressure in cirrhotic patients with mild pulmonary hypertension, and reduces portal pressure, improves hyperdynamic circulation and functional renal failure via stimulation of mesenteric V1 receptors [12] |
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