Bosentan for the treatment of pulmonary arterial hypertension (II)

Evaluation of: Hoeper MM, Halank M, Marx C et al. Bosentan therapy for portopulmonary hypertension. Eur. Respir. J. 25(3), 502–508 (2005). Portopulmonary hypertension is defined as pulmonary arterial hypertension occurring in the presence of portal hypertension. It is classified as a subset of pulmonary arterial hypertension and accordingly it is defined hemodynamically. Portopulmonary hypertension shares the main pathologic features as well as diagnostic approach with other forms of pulmonary arterial hypertension. Several nonpharmacologic and pharmacologic approaches are currently available. Among the pharmacologic approaches prostacycline and its derivatives, phosphodiesterase-5 inhibitors such as sildenafil and endothelin receptor antagonists such as bosentan, have been used in portopulmonary hypertension treatment. This is a case series report on the long-term efficacy of bosentan treatment for severe (New York Heart Association functional Class III and IV) portopulmonary hypertension.