Treatment of Portopulmonary Hypertension in WHO Functional Class II or III
This protocol applies to patients with portopulmonary hypertension presenting in WHO functional class II or III — a population in whom targeted pharmacological intervention is indicated to reduce pulmonary vascular burden and improve functional capacity.
Endothelin receptor antagonists are targeted for patients with a functional class of II and III, whether as monotherapy or in conjunction with other agents. The functional class directly informs agent selection in this setting.
Management centres on endothelin receptor antagonist monotherapy as the primary approach. Supportive measures addressing oxygen saturation and volume overload are given alongside the primary treatment. The complete agent selection, dosing sequence, and stepwise algorithm are available through the full regimen.
Reduction of mean pulmonary arterial pressure and pulmonary vascular resistance, with improved exercise tolerance measured by the 6-minute walking test. Response is evaluated at 3-month follow-up right heart catheterisation.