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作 者:Sami Alharbi Noura Alturaif Yehia Mostafa Abdullah Alfhaid Ali Albenmousa Saad Alghamdi
机构地区:[1]Department of Lung Health,King Faisal Specialist Hospital and Research Centre,Riyadh 11211,Saudi Arabia [2]Department of Liver and Small Bowel Health,King Faisal Specialist Hospital and Research Centre,Riyadh 11211,Saudi Arabia
出 处:《World Journal of Gastrointestinal Surgery》2024年第12期3875-3880,共6页世界胃肠外科杂志(英文)
摘 要: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.
关 键 词:Pulmonary hypertension Liver transplant AUTOIMMUNITY Primary sclerosing cholangitis Case report
分 类 号:R543.2[医药卫生—心血管疾病]
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