How should we treat portopulmonary hypertension?

Abstract

In summary, portopulmonary hypertension is a vaso-obstructive process whose pathophysiology (in genetically susceptible patients) most likely involves a progression from a high-flow state to endothelial/smooth muscle proliferation, in situ thrombosis and plexogenic arteriopathy within the pulmonary arterial circulation [1]. Hence portopulmonary hypertension is more than simply vasoconstriction. The data of REICHENBERGER et al. [4], showing initial improvement at 3 months (yet worsening at 12 months) suggest that the vasodilation approach provided by sildenafil alone may not be the optimal treatment in moderate-to-severe portopulmonary hypertension. The role of sildenafil (or anti-proliferative agents) in the earliest presentation of portopulmonary hypertension is unknown. Questioning the effectiveness of anti-platelet aggregation and anti-proliferative approaches in combination with a pulmonary vasodilator therapy would be reasonable hypotheses. Inclusion of portopulmonary hypertension patients in randomised controlled trials with portopulmonary hypertension subgroup analysis would make additional sense. In appropriately selected patients, early medical treatment of the vaso-obstructive pulmonary vascular process, followed by timely liver transplantation to correct the hepatic/portal pathophysiology that facilitates the evolution of pulmonary arterial hypertension, may provide not only control, but even a cure for portopulmonary hypertension. Optimal treatment for portopulmonary hypertension remains an unanswered, but important question. Copyright ©ERS Journals Ltd 2006.