Portopulmonary hypertension
ICD-10 K76.9 · ICD-11 DB99.3

WHO Functional Class II–III Portopulmonary Hypertension When Combined ERA and PDE-5 Inhibitor Therapy Has Not Met Hemodynamic Targets

This protocol applies to patients with WHO functional class II or III portopulmonary hypertension (PoPH) in whom a combined pharmacological regimen has not achieved the required hemodynamic thresholds, and a next-step intervention is under evaluation.

Clinical Scenario

Portopulmonary hypertension at WHO functional class II or III warrants targeted pulmonary arterial hypertension pharmacotherapy. Endothelin receptor antagonists are indicated for this functional class, whether as monotherapy or combined with phosphodiesterase inhibitors.

Prior Therapy — Failure Condition

The preceding treatment used a combined regimen of an endothelin receptor antagonist plus a phosphodiesterase-5 inhibitor (Sildenafil). That line aimed for a mean pulmonary arterial pressure below 35 mmHg and a pulmonary vascular resistance below 3 Wood units on 3-month follow-up heart catheterization. When those targets are not reached, this protocol defines the next step.

Next-Step Approach (Partial)

For patients who have not responded to combined pharmacological therapy, liver transplantation is among the interventions evaluated in this protocol, depending on candidacy criteria defined in the full regimen.

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References

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.

On the other hand, a liver transplantation should be considered for patients with mild PoPH or those with an MPAP between 35 and 45 mm Hg that can successfully lower the MPAP <35 mm Hg post–vasodilation therapy.

OLT can resolve portal hypertension and is an effective treatment for PoPH.

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