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)...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 pul-monary 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 hy-pertension.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 re-sponse to the combination therapy underscores the importance of prompt diag-nosis 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.展开更多
文摘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 pul-monary 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 hy-pertension.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 re-sponse to the combination therapy underscores the importance of prompt diag-nosis 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.
