This protocol addresses patients with liver cirrhosis who have developed portopulmonary hypertension — specifically a mean pulmonary arterial pressure (mPAP) of at least 35 mmHg in the setting of established portal hypertension.
The preceding treatment step managed portopulmonary hypertension with beta-blocker withdrawal, endoscopic management of varices, and targeted pulmonary vasodilator therapy — comprising endothelin receptor antagonists, phosphodiesterase subtype-5 inhibitors, or prostacyclin analogues.
Escalation to this protocol is indicated when that approach has not achieved its goals: mPAP reduced to below 35 mmHg and pulmonary vascular resistance normalised to below 400 dynes/s cm⁵ with preserved right ventricular function.
When prior targeted therapy has not met the haemodynamic thresholds above, a surgical transplant approach may become an option — but only when specific pulmonary pressure and right ventricular function criteria are fulfilled. The full eligibility criteria, decision thresholds, and contraindications are detailed in the structured protocol.
Therapy to lower mPAP and improve right ventricular function should be commenced in patients with mPAP ≥35 mmHg. Right ventricular function should be periodically evaluated (II-2,1).
If mPAP <35 mmHg and right ventricular function is preserved, LT should be considered (II-2,1). A mPAP of ≥45 mmHg should be considered an absolute contraindication to LT irrespective of therapy applied (III,1).
DOI: 10.1016/j.jhep.2018.03.024
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