Alharbi Sami, Alturaif Noura, Mostafa Yehia, Alfhaid Abdullah, Albenmousa Ali, Alghamdi Saad
Department of Lung Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia.
Department of Liver and Small Bowel Health, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Saudi Arabia.
World J Gastrointest Surg. 2024 Dec 27;16(12):3875-3880. doi: 10.4240/wjgs.v16.i12.3875.
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.
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.
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.
肝移植（LTx）对于终末期肝病患者至关重要，代谢功能障碍相关脂肪性肝病是最常见的适应证。原发性硬化性胆管炎（PSC）是一个重要的适应证。与门静脉高压相关的门肺高压带来显著的围手术期风险，因此移植前筛查至关重要。
我们报告一例41岁患有PSC的女性病例，该患者在成功进行肝移植数年之后出现严重肺动脉高压。她表现为活动时呼吸困难加重和前驱晕厥。诊断评估证实存在严重的毛细血管前肺动脉高压，且无复发性门静脉高压的证据。最初使用西地那非和马昔腾坦治疗使她的症状、运动能力和生物标志物有显著改善。该病例凸显了在无复发性门静脉高压的肝移植受者中罕见的新发肺动脉高压的发生，这可能与自身免疫过程或原发性肝病本身有关。患者对联合治疗的积极反应强调了及时诊断和积极管理的重要性。
总之，肝移植后肺动脉高压是一种罕见但严重的并发症，预后较差，需要进一步研究以更好地了解其机制并制定有效的预防和治疗策略。
