This protocol is for patients with WHO functional class II or III portopulmonary hypertension in whom a first-line single-agent regimen has been assessed at 3 months and found to fall short of the required hemodynamic targets on right heart catheterization.
The target population is WHO functional class II or III portopulmonary hypertension. Endothelin receptor antagonists are indicated for patients in these functional classes, whether initially as monotherapy or, when the single-agent approach proves insufficient, as part of a combined strategy.
The prior protocol used an endothelin receptor antagonist as monotherapy, or alternatively a guanylate cyclase stimulant, together with supportive measures. At the 3-month follow-up right heart catheterization, the targets for reduction in mean pulmonary arterial pressure and pulmonary vascular resistance, and for improved exercise tolerance on the 6-minute walking test, were not reached. This protocol addresses that documented escalation.
When monotherapy is insufficient, the approach shifts to a combined strategy pairing two complementary drug classes. The complete agent selection, decision points, and clinical considerations are available in the full protocol below.
Adequate hemodynamic response is defined as a mean pulmonary arterial pressure below 35 mm Hg and a pulmonary vascular resistance below 3 WU, confirmed on 3-month follow-up heart catheterization.
DOI: 10.1097/TXD.0000000000001517
The endothelin receptor antagonists are targeted for patients with a functional class of II and III, whether as monotherapy or in conjunction with phosphodiesterase inhibitors as a combined therapy.
The AASLD guidelines suggest that OLT can be offered to patients with mild PoPH and those with an excellent response to medical treatment (MPAP after treatment <35 mm Hg and peripheral vascular resistance <3 WU).
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