Delayed-onset portopulmonary hypertension following liver transplantation with near hemodynamic normalization on triple therapy.

Portopulmonary hypertension (PoPH) is a distinct subtype of pulmonary arterial hypertension (PAH) occurring in the setting of portal hypertension. Although liver transplantation (LT) may relieve portal pressure, pulmonary vascular remodeling can persist or, rarely, develop de novo after transplantation, posing diagnostic and therapeutic challenges. Delayed-onset PoPH after LT remains poorly characterized, with limited data to guide recognition and management. We describe a case of a 67-year-old male who developed severe PoPH three years after orthotopic LT for end-stage liver disease secondary to alcohol use and metabolic dysfunction- associated steatotic liver disease (MASLD). He presented with hypoxic respiratory failure, dyspnea, and volume overload. Echocardiography and right-heart catheterization (RHC) confirmed severe precapillary pulmonary hypertension. Combination therapy with intravenous epoprostenol, macitentan, and sildenafil resulted in marked hemodynamic and clinical improvement. He was subsequently transitioned from intravenous epoprostenol to oral selexipag and has maintained near-normal pulmonary pressures for two years. This case highlights delayed post-LT PoPH and demonstrates that even late-presenting disease may achieve substantial hemodynamic improvement with aggressive, guideline-directed PAH therapy.