Inhaled iloprost for hepatopulmonary syndrome: improvement of hypoxemia

Hepatopulmonary syndrome is characterized by advanced liver disease, hypoxemia, and intrapulmonary shunting. The only reported curative option is orthotopic liver transplantation. We describe here a beneficial effect of inhaled prostacyclin including a decrease in respiratory symptoms and improved oxygenation in this clinical situation, with no approved pharmacological long-term therapy. The prostanoid iloprost, approved for pulmonary and portopulmonary hypertension, caused an increase in oxygenation, relief of dyspnea, and increased exercise tolerance in a patient suffering from liver-cirrhosis-associated hepatopulmonary syndrome. After liver transplantation, restitution of hepatopulmonary syndrome did not occur immediately. Inhaling iloprost resulted in improved physical condition and better clinical rehabilitation potential until hypoxemia finally resolved 3 months after transplantation. Therefore, iloprost could improve quality of life in patients with hepatopulmonary syndrome waiting for liver transplantation and post surgery until the resolution of the hypoxemia.