Sundaresan S
Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA.
Semin Thorac Cardiovasc Surg. 1998 Apr;10(2):152-9. doi: 10.1016/s1043-0679(98)70010-3.
Primary pulmonary hypertension (PPH) is a rare cardiovascular disease with a variable course; however, in general, its prognosis is poor. Among the various treatment options available, transplantation (initially heart-lung transplantation, and later isolated single or bilateral lung transplantation) has become an accepted modality. Heart-lung transplantation is necessary only in a minority of patients because right ventricular recovery has been gratifying after isolated lung transplantation. Furthermore, the scarcity of suitable donor organs mandates the achievement of the maximal number of heart and lung transplants from the limited donor pool. Available published data show that both single and bilateral lung transplantation are suitable alternatives for the majority of patients with pulmonary hypertension. Bronchiolitis obliterans syndrome (BOS), the main cause of late mortality and morbidity in lung transplant recipients, affects pulmonary hypertensive patients as it does other recipient subgroups. The available data regarding the impact of BOS on single versus bilateral lung recipients with pulmonary hypertension are somewhat scanty. Although some have suggested that BOS is more prevalent among PPH recipients, this is not uniformly supported through the literature. Other reports have documented severe ventilation-perfusion imbalance associated with graft dysfunction secondary to BOS in single lung transplant recipients with PPH. Despite this, there are no available data to document a significant survival benefit for PPH patients receiving bilateral versus single lung transplantation. Our own transplantation experience at Washington University in St. Louis with pulmonary hypertension shows a trend toward better survival in bilateral lung recipients, although this difference is not significant. Ultimately, both single and bilateral lung replacement seem to be satisfactory transplant options in PPH. Both recipient groups are affected by BOS, and longer follow-up of larger numbers of patients may document superior survival and functional outcome with bilateral lung replacement.
原发性肺动脉高压（PPH）是一种病程多变的罕见心血管疾病；然而，总体而言，其预后较差。在现有的各种治疗选择中，移植（最初是心肺移植，后来是单纯的单侧或双侧肺移植）已成为一种被认可的治疗方式。仅在少数患者中需要进行心肺移植，因为在单纯肺移植后右心室功能恢复情况良好。此外，合适供体器官的稀缺使得必须从有限的供体库中实现最大数量的心肺移植。已发表的现有数据表明，单侧和双侧肺移植都是大多数肺动脉高压患者的合适替代方案。闭塞性细支气管炎综合征（BOS）是肺移植受者晚期死亡和发病的主要原因，它对肺动脉高压患者的影响与对其他受者亚组的影响相同。关于BOS对肺动脉高压单侧与双侧肺移植受者影响的现有数据有些稀少。尽管有人认为BOS在PPH受者中更为普遍，但文献中对此并不一致支持。其他报告记录了PPH单侧肺移植受者中与BOS继发的移植物功能障碍相关的严重通气-灌注失衡。尽管如此，尚无可用数据证明接受双侧肺移植而非单侧肺移植的PPH患者有显著的生存获益。我们在圣路易斯华盛顿大学对肺动脉高压患者的移植经验表明，双侧肺移植受者的生存情况有改善趋势，尽管这种差异并不显著。最终，单侧和双侧肺置换似乎都是PPH患者令人满意的移植选择。两个受者组都受到BOS的影响，对更多患者进行更长时间的随访可能会证明双侧肺置换在生存和功能结局方面更优。
