iloprost and Hepatopulmonary-Syndrome

Patients with portal hypertension may develop pulmonary complications such as hepatopulmonary syndrome (HPS) or portopulmonary hypertension (PPHT). PPHT is defined as elevated pulmonary pressure, elevated pulmonary vascular resistance, a normal pulmonary capillary wedge pressure, and portal hypertension in the absence of other known causes pulmonary hypertension. Various factors such as hyperdynamic circulation, volume overload, and circulating vasoactive mediators are suspected to be involved in the pathogenesis of PPHT. The prognosis of patients with severe PPHT is significantly reduced due to the risk of right heart failure. In patients with moderate to severe PPHT liver transplantation is associated with a significantly increased mortality. The chief symptom of PPHT may be dyspnoe in the presence of typical histomorphological alterations comparable with idiopathic pulmonary hypertension. Continuous intravenous application of prostacyclin is currently regarded as the treatment of choice for patients with severe PPHT. Inhaled prostacyclin or its analogue iloprost or oral treatment with the endothelin-receptor antagonist bosentan may be promising alternatives which should be further investigated in randomized controlled trials.

Topics: Administration, Inhalation; Administration, Oral; Antihypertensive Agents; Bosentan; Epoprostenol; Hepatopulmonary Syndrome; Humans; Hypertension, Portal; Hypertension, Pulmonary; Iloprost; Infusions, Intravenous; Lung; Pulmonary Wedge Pressure; Sulfonamides; Survival Rate; Treatment Outcome; Vascular Resistance; Vasodilator Agents

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.

Topics: Administration, Inhalation; Female; Hepatopulmonary Syndrome; Humans; Hypoxia; Iloprost; Liver Transplantation; Middle Aged; Oxygen; Partial Pressure; Postoperative Care; Vasodilator Agents