Abstract
BACKGROUND: Liver transplantation (LTx) is vital in patients with end-stage liver disease, with metabolic dysfunction-associated steatotic liver disease being the most common indication. Primary sclerosing cholangitis (PSC) is an important indication. Portopulmonary hypertension, associated with portal hypertension, poses a significant perioperative risk, making pretransplant screening essential. CASE SUMMARY: We report the case of a 41-year-old woman with PSC who developed severe pulmonary hypertension years after a successful LTx. She presented with worsening dyspnea on exertion and presyncope. Diagnostic evaluation confirmed severe precapillary pulmonary hypertension without evidence of recurrent portal hypertension. Initial management with Sildenafil and Macitentan led to a significant improvement in her symptoms, exercise capacity, and biomarkers. This case highlights the rare development of de novo pulmonary hypertension in a liver transplant recipient without recurrent portal hypertension, possibly linked to autoimmune processes or primary liver disease itself. The patient's positive response to the combination therapy underscores the importance of prompt diagnosis and aggressive management. CONCLUSION: In conclusion, pulmonary arterial hypertension post-LTx is a rare but serious complication with a poor prognosis, necessitating further research to better understand its mechanisms and to develop effective strategies for prevention and treatment.