Department of Geriatric Medicine, Shenzhen Third People's Hospital, Shenzhen, China.
Washington University in St. Louis, St. Louis, USA.
ESC Heart Fail. 2023 Aug;10(4):2718-2721. doi: 10.1002/ehf2.14431. Epub 2023 Jun 14.
Pulmonary arterial hypertension-targeted therapies in portopulmonary hypertension (PoPH) are scarce, let alone for patients with chronic liver failure (CLF) and hepatopulmonary syndrome (HPS). A 48-year male was admitted to the hospital because of cirrhosis for 18 years, systemic oedema, and chest distress after exercise for 1 week. He was diagnosed with CLF, PoPH, and HPS. After 7 weeks of macitentan treatment, the patient's activity tolerance, pulmonary artery systolic pressure, arterial partial pressure of oxygen (PaO ), cTNI, and NT-proBNP changes indicated gradual recovery, without hepatic safety concerns. This case indicated that administering macitentan in patients diagnosed as PoPH (with CLF and HPS) may be efficient and safe enough in a clinical setting.
在门脉高压性肺高压（PoPH）中，靶向肺动脉高压的治疗方法很少，更不用说用于慢性肝功能衰竭（CLF）和肝肺综合征（HPS）的患者了。一名 48 岁男性因 18 年的肝硬化、全身性水肿和运动后 1 周出现胸部不适而入院。他被诊断为 CLF、PoPH 和 HPS。在接受麦角类内皮素受体拮抗剂治疗 7 周后，患者的活动耐量、肺动脉收缩压、动脉血氧分压（PaO2）、cTNI 和 NT-proBNP 变化表明逐渐恢复，且无肝安全性问题。该病例表明，在临床环境中，给予麦角类内皮素受体拮抗剂治疗诊断为 PoPH（伴 CLF 和 HPS）的患者可能是有效且安全的。
