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目的:通过回顾无锡市人民医院5例双肺移植治疗特发性肺动脉高压(idiopathic pulmonary artery hypertension,IPAH)的临床资料,并结合国内外相关文献,探讨肺移植治疗IPAH的适应证、术式选择、手术并发症以及围术期处理。方法:IPAH受者5例,男2例,女3例,年龄14~30岁,术前均为重度肺动脉高压,WHO心功能评级Ⅲ~Ⅳ级,在全麻体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)支持下行序贯式双肺移植术。结果:5例患者中4例术后需延长ECMO使用,时间12~20 h不等;所有患者术后早期均出现不同程度的急性左心功能不全,机械通气延迟撤离,住重症监护病房时间延长。最终5例患者均康复出院,随访至今,心肺功能良好,长期生活质量佳。结论:肺移植目前仍然是IPAH唯一的治疗选择,双肺移植是IPAH当前首选的手术方法。围术期常规应用ECMO支持技术,可大大提高手术的安全性。
OBJECTIVE: To review the clinical data of 5 cases of idiopathic pulmonary artery hypertension (IPAH) treated by double-lung transplantation in Wuxi People’s Hospital and to discuss the indications of lung transplantation in the treatment of IPAH combined with relevant literature at home and abroad. , Surgical complications and perioperative management. Methods: There were 5 IPAH recipients, 2 males and 3 females, aged 14 to 30 years old, with severe pulmonary hypertension before operation. The cardiac function of WHO was classified as grade Ⅲ ~ Ⅳ, and in extracorporeal membrane oxygenation (extracorporeal membrane) Oxyation, ECMO) Supports Downstream Sequential Dual Lung Transplantation. Results: In 5 patients, 4 patients were required to prolong the use of ECMO after 12-20 h. All patients had different degrees of acute left ventricular dysfunction, delayed withdrawal of mechanical ventilation and prolonged ICU stay in the early postoperative period . The final 5 patients were discharged from hospital, up to now, cardiopulmonary function is good, long-term quality of life is good. CONCLUSIONS: Lung transplantation is still the only treatment option for IPAH and dual lung transplantation is currently the preferred surgical procedure for IPAH. Perioperative routine use of ECMO support technology, can greatly improve the safety of surgery.