Portopulmonary hypertension and hepatopulmonary syndrome

Table 4 Portopulmonary hypertension treatments[69]

Molecule	Mechanism of action	Half-life	Administration	Adverse effects	Advantages	Drawbacks	Main results
Nitric Oxide	Selective dilation of the pulmonary vasculature	Seconds	Inhaled	Methemoglobinemia	Selective on pulmonary circulation	Endotracheal intubation	Improved hemodynamics
Epoprostenol	Vasodilation of all vascular beds/platelet inhibition	Minutes	Intravenous	Flushing, headache, nausea	Best studied drug	Long term central venous access/cost	Improved hemodynamics allowing LT[70,71]
Iloprost	Vasodilation of pulmonary vascular bed/platelet inhibition	Minutes	Inhaled	Flushing, headache, nausea	Selective on pulmonary circulation	Frequent administration/cost	Increased exercise tolerance and survival[26,27,30]
Bosentan	Endothelin receptor antagonist	5 h	Oral	Hepatotoxicity, peripheral edema	Oral administration, twice daily	Cost	Improved survival[29]
Sildenafil	Phosphodiesterase type 5 inhibitor	4 h	Oral	Flushing, headache, priapism	Oral administration, three times daily	Cost	Increased exercise tolerance[31]

LT: Liver transplantation.