This protocol addresses liver cirrhosis in patients who have developed portopulmonary hypertension — a mean pulmonary arterial pressure of at least 35 mmHg in the setting of established portal hypertension.
Portopulmonary hypertension (PPHT) is a complication of portal hypertension characterised by elevated mean pulmonary arterial pressure. When mPAP reaches or exceeds 35 mmHg, targeted therapy is indicated and right ventricular function requires ongoing periodic evaluation.
In proven PPHT at this severity, management involves stopping beta-blockers, transitioning variceal care to endoscopic therapy, and initiating targeted pulmonary vasodilator therapy — including endothelin receptor antagonists (used with caution given the risk of hepatic impairment) — to lower mPAP and preserve right ventricular function; the full agent selection, sequencing, and decision algorithm are available in the complete protocol.
Lower mPAP to below 35 mmHg and normalise pulmonary vascular resistance to below 400 dynes/s cm5, with preserved right ventricular function.
DOI: 10.1016/j.jhep.2018.03.024
Beta-blockers should be stopped and varices managed by endoscopic therapy in cases of proven PPHT (II-3;1).
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).
Therapies that have been approved for primary pulmonary arterial hypertension may have benefit in PPHT to improve exercise tolerance and haemodynamics. However, endothelin antagonists should be used with caution because of concerns over hepatic impairment (II-2;1).
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