Table 2.
Non-surgical Therapies for Hepatopulmonary Syndrome and Portopulmonary Hypertension
| Hepatopulmonary syndrome | Portopulmonary hypertension | ||
|---|---|---|---|
| Supplemental oxygen | Maintain SpO2 >88%, extrapolated from studies of hypoxemia from other etiologies(18) | Supplemental oxygen | Maintain SpO2 >88%, extrapolated from studies of hypoxemia from other etiologies(18) |
| TIPS | Transient improvement in hypoxemia for 1–3 months, unclear long-term benefit. Possible increased benefit using left branch of PV(42–44) | TIPS | Contraindicated in moderate to severe disease(1,83,84) |
| Coil embolization | Most useful in cases of severe hypoxemia and discrete AV connections, possible case of PAH following treatment(45,46,105) | Anti-coagulation | No clear benefit in PAH patients(78,79) |
| Congenital portosystemic shunts | Correction leads to improvement in oxygenation(5) | Calcium channel blockers | No clear benefit in PAH patients, possible harm(80,81) |
| Inhaled L-NAME | Reduced hypoxemia in experimental models, no clear benefit in human studies(106,107) | Beta-blockers | Consider stopping in moderate to severe disease, banding for variceal bleeding prevention(82) |
| Inhaled iloprost | No clear benefit in small study(108) | Prostacyclin analogues | IV Epoprostenol, treprostinil with improved hemodynamics in small studies. Ongoing studies of inhaled iloprost and oral berprost, treprostinil, and selexipag(87–90) |
| Garlic extract | Improved oxygenation in 3 small studies, though reports of DILI(50,51) | Endothelin receptor antagonists | Ambrisentan and bosentan with improved symptoms and hemodynamics in small studies. Currently enrolling RCT of macitentan vs placebo(92–94) |
| Norfloxacin | Reduced hypoxemia in experimental models, no clear benefit in human studies(109) | Phospho- diesterase-5 inhibitors | Sildenafil with increased functional class, exercise tolerance, and hemodynamics in small studies. Ongoing investigation of oral tadalafil(95–97) |
| Pentoxyfylline | Reduced hypoxemia in experimental models, no clear benefit in human studies(47–49) | Riociguat | Improved exercise capacity in RCT of PAH patients, including 13 patients with PoPH(85) |
| Sorafenib | No clear benefit(110) | Combination therapy | Sildenafil or bosentan with prostacyclin showed improved hemodynamics in small studies(91,98) |
SpO2: peripheral oxygen saturation; TIPS: transjugular intrahepatic portosystemic shunt; PV: portal vein; AV: arteriovenous; PAH: pulmonary arterial hypertension; L-NAME: L-N-nitro arginine methyl ester; DILI: drug-induced liver injury; RCT: randomized controlled trial; PoPH portopulmonary hypertension