[Epoprostenol in the treatment of portopulmonary hypertension]

Abstract

Primary or non-hemodynamic pulmonary hypertension is characterized by the increased pulmonary arterial pressure, higher than 3.32 kPa, and normal pulmonary capillary pressure less than 1.99 kPa. Primary pulmonary hypertension is a rare complication of portal hypertension, which significantly increases surgical morbidity and mortality, and it is not reversible after liver transplantation. As a rule, clinical manifestations of pulmonary hypertension are disguised by complications of portal hypertension. The symptoms of chronic pulmonary heart, such as peripheral edema and ascites, are usually ascribed to liver insufficiency and/or portal hypertension. Clinical significance of portopulmonary hypertension follows from the fact that the failure of the right heart is most commonly the direct cause of death in these patients. The majority of authors concurs that the right heart catheterization is the most valid method for diagnosis of portopulmonary hypertension. Doppler echocardiography represents quite valuable non-invasive diagnostic method, especially in patients with extensive spontaneous and surgical porto-systemic collaterals, rendering the high risk group of cases. Epoprostenol (prostacyclin), administered via continuous infusion, diminishes the platelet aggregation and causes the intense pulmonary and systemic vasodilatation. Good hemodynamic effect of this substance was verified in patients with primary pulmonary hypertension. Several articles have reported that long-term, continual epoprostenol administration in dose of 10 to 28 ng/kg/min. Has significantly reduced pulmonary vascular pressure and pulmonary vascular resistance. Portopulmonary hypertension represented the absolute contraindication for liver transplantation. Huge clinical significance of epoprostenol reflects in the fact that, along with the improvement of pulmonary hemodynamics, it provides the prerequisite for liver transplantation in patients with portopulmonary hypertension.