Treatment of Chronic Liver Failure with Portopulmonary Hypertension When PAH-Targeted Therapy Has Not Achieved Pressure Targets
This protocol covers chronic liver failure in the setting of portal hypertension complicated by portopulmonary hypertension (PPHT) — defined as a mean pulmonary arterial pressure (mPAP) of ≥25 mmHg — specifically in patients whose prior pulmonary arterial hypertension-targeted treatment did not reach the required haemodynamic goals.
Clinical scenario
Portal hypertension with confirmed portopulmonary hypertension (mPAP ≥25 mmHg). PPHT should be considered in any patient with established portal hypertension in the absence of other causes of pulmonary arterial or venous hypertension. Severity is graded as mild (mPAP ≥25 and <35 mmHg), moderate (mPAP ≥35 and <45 mmHg), or severe (mPAP ≥45 mmHg).
Previous treatment & escalation trigger
The preceding line of management for portopulmonary hypertension included stopping beta-blockers, controlling varices by endoscopic therapy, and commencing a pulmonary arterial hypertension-targeted drug — such as bosentan, sildenafil, epoprostenol, or iloprost. The goals of that line were reduction of mPAP to below 35 mmHg and normalisation of pulmonary vascular resistance (to below 400 dynes/s per cm⁵), with preserved right ventricular function. Failure to achieve those targets is the condition that brings this protocol into consideration.
Next-line approach — partial overview
For carefully selected patients, a definitive intervention targeting the underlying liver disease may be appropriate — but eligibility depends strictly on the current pulmonary haemodynamic values and the status of right ventricular function. Whether and when a patient qualifies involves specific pressure thresholds that also determine contraindications. The complete eligibility criteria, decision pathway, and full management algorithm are in the structured protocol below.
References
DOI: 10.1016/j.jhep.2018.03.024
A diagnosis of PPHT should be considered in a patient with established portal hypertension in the absence of other causes of pulmonary artery or venous hypertension.
PPHT is graded as mild (mPAP ≥25 and <35 mmHg); moderate (mPAP ≥35 and <45 mmHg), and severe (mPAP ≥45 mmHg).
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).
View source ↗