When severe hepatopulmonary syndrome (PaO₂ <50 mmHg) coexists with borderline portopulmonary hemodynamics on therapy (e.g., mPAP high-30s with PVR ~3 WU), how do you sequence optimization and listing strategy, and what physiologic thresholds make you proceed to transplant versus defer for further pulmonary vascular optimization?
This clinical scenario is more common than previously thought. Other than oxygen supplementation for hepatopulmonary syndrome (HPS), there is no other specific therapy. Portopulmonary hypertension (PoPH) needs to be treated to reach the threshold of desired mean pulmonary arterial pressure (mPAP) of less than 35 mmHg and pulmonary vascular resistance (PVR) ideally less than 5 Wood units (WU), or less than 3 WU if mPAP is between 35 to 45 mmHg. In a rarer scenario, vasodilator therapy may worsen oxygen levels. These patients should be transplanted with a Model for End-Stage Liver Disease (MELD) exception, but their postoperative course may be very complex, and the pulmonary consultants should be actively involved in management. Normalization...
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