Applied Immunobiology and Transplantation Group, Institute of Cellular Medicine, Medical School, University of Newcastle upon Tyne, Freeman Hospital, High Heaton, NE7 7DN, UK.
Expert Rev Respir Med. 2011 Jun;5(3):441-54. doi: 10.1586/ers.11.21.
Transplantation remains the only therapeutic option for selected patients with advanced pulmonary arterial hypertension (PAH) who continue to deteriorate despite optimal pulmonary vasodilator therapy - including intravenous prostanoids and combination therapy. Identification of poor prognostic markers in PAH, including persistence in the New York Heart Association functional class III and IV, and adverse pulmonary hemodynamics at right heart catheterization should prompt early referral for transplantation. There is a need for inclusion of more discriminatory markers of PAH prognosis in donor-lung allocation scores to identify patients at risk and optimize survival to transplantation, given the current shortage of donor organ availability worldwide. Double-lung transplantation is the recommended operation for idiopathic PAH. Heart-lung transplantation is reserved for selected patients with idiopathic PAH with severe right ventricular dysfunction, or congenital heart disease with complex or ventricular septal defect-associated PAH. Novel surgical strategies, including atrial septostomy or the pumpless Novalung® lung assist device with conduits, from the pulmonary artery to the left atrium, can be considered as a bridge to transplant for patients with rapid clinical decline, despite maximal medical therapy. Recent transplant outcomes for PAH are encouraging, albeit with early postoperative risks, a requirement for long-term surveillance, immunosuppression and transplant immunosuppression-specific morbidity.
移植仍然是选定的晚期肺动脉高压 (PAH) 患者的唯一治疗选择，这些患者尽管接受了最佳的肺血管扩张剂治疗 - 包括静脉内前列腺素和联合治疗 - 仍在继续恶化。PAH 的预后不良标志物的识别，包括纽约心脏协会功能分类 III 和 IV 的持续存在，以及右心导管检查时的不良肺血流动力学，应促使尽早转介进行移植。鉴于全球供体器官短缺，需要在供肺分配评分中纳入更具鉴别力的 PAH 预后标志物，以识别风险患者并优化移植的生存机会。双肺移植是特发性 PAH 的推荐手术。心肺移植保留给特发性 PAH 伴有严重右心室功能障碍或伴有复杂或室间隔缺损相关 PAH 的先天性心脏病的选定患者。新型手术策略，包括房间隔造口术或无泵 Novalung®肺辅助装置与导管，从肺动脉到左心房，可以作为快速临床恶化患者的移植桥接，尽管接受了最大的药物治疗。最近的 PAH 移植结果令人鼓舞，尽管存在术后早期风险、长期监测、免疫抑制和移植免疫抑制特异性发病率的要求。
