3 The presence of hypoxemia in a patient with portal hypertension should raise the suspicion of HPS. Usually the PaO2 is less than 80 mmHg and alveolar- arterial gradient Y-27632 datasheet is greater than 15 mmHg on room air.7 Contrast echocardiography

is a vital diagnostic tool for HPS. The appearance of agitated saline in the left atrium after three cardiac cycles is diagnostic of intrapulmonary shunting.7 POPH and HPS also differ in terms of their treatment options. Pulmonary vasodilatation is the mainstay of treatment in POPH. Intravenous epoprostenol has been shown to cause improvement in hemodynamics and symptoms in POPH but requires constant intravenous access for drug infusion and a highly compliant patient.4 Also, both oral and nebulized forms of prostacyclin have been used in POPH and have demonstrated comparable results to intravenous prostacyclin.5 The oral dual endothelin receptor antagonist bosentan has the beneficial effects

of improvement in exercise capacity and hemodynamics in POPH. Bosentan blocks endothelin receptors thereby decreasing the targets for endothelin-1 levels of which are increased in POPH.4 PAH of any severity in a cirrhotic patient with portal hypertension carries a poor prognosis and severe PAH carries a high mortality after liver transplantation.3 In many transplant centers, a mean Navitoclax order PAP >50 mmHg is considered as an absolute contraindication for liver transplantation.3 Therefore PAH is generally treated with vasodilators with the aim of reducing mean PAP below 35 mmHg before liver transplantation.6 Austin et al. described a case of POPH where the pulmonary arterial pressure was reduced successfully with triple therapy including iloprost, sildenafil and bosentan before liver transplantation.5 The treatment of HPS includes correction of hypoxemia by oxygen and liver transplantation. Liver transplantation is the only treatment that has been shown to alter the natural course

of the disease with improvement in hypoxemia.7 Patients with refractory Resminostat hypoxemia carry a higher mortality and morbidity when undergoing liver transplantation in both the preoperative and postoperative periods.7 The 5 year survival rate of POPH is 14% without any treatment as compared to 45% for those who receive medical vasodilator therapy.6 The 5 year survival for HPS without liver transplant is 23%.7 The treatment strategy in a patient who presents with both HPS and POPH is challenging. Liver transplantation is required for HPS but the presence of POPH carries a poor prognosis before and after liver transplantation. The aim of the treatment is to lower the PAH with vasodilators before liver transplantation and use oxygen for hypoxemia. Further studies are needed to understand more about the patho-physiology of the coexistence of these two syndromes.