Treatment of Chronic Liver Failure with Portal Hypertension and Portopulmonary Hypertension (mPAP ≥25 mmHg)

This protocol addresses patients with chronic liver failure in the context of portal hypertension who have developed portopulmonary hypertension (PPHT) — defined by a mean pulmonary arterial pressure (mPAP) of ≥25 mmHg. A diagnosis of PPHT should be considered in any patient with established portal hypertension in the absence of other causes of pulmonary arterial or venous hypertension. PPHT is graded by severity: mild (mPAP ≥25 and <35 mmHg), moderate (mPAP ≥35 and <45 mmHg), and severe (mPAP ≥45 mmHg).

Management requires discontinuation of beta-blockers, with variceal risk addressed through endoscopic therapy. For patients with mPAP ≥35 mmHg, targeted therapy aimed at lowering mean pulmonary arterial pressure and improving right ventricular function is initiated. A specific procedural intervention is contraindicated in this setting. The complete decision pathway — including which therapy to select and the clinical thresholds that guide each step — is available in the full structured protocol.

Reduction of mean pulmonary arterial pressure to below 35 mmHg and normalisation of pulmonary vascular resistance (below 400 dynes/s per cm⁵) with preserved right ventricular function.