Department of Surgery and Division of Cardiovascular Surgery, University of Pennsylvania, Philadelphia, PA.
Department of Medicine and Division of Cardiology, University of Pennsylvania, Philadelphia, PA.
Chest. 2020 Nov;158(5):2097-2106. doi: 10.1016/j.chest.2020.05.597. Epub 2020 Jun 18.
Thoracic transplantation is considered for patients with Eisenmenger syndrome (ES) who have refractory right ventricular failure despite optimal therapy for pulmonary arterial hypertension. This study compared the outcomes of bilateral lung transplantation (BLT) with cardiac defect repair vs combined heart-lung transplantation (HLT).
This study presents an updated analysis using a US national registry to evaluate the outcomes of patients diagnosed with ES who underwent HLT or BLT with repair of cardiac defects.
This study identified patients with ES who underwent thoracic transplantation from 1987 to 2018 from the United Network for Organ Sharing database. Survival curves were estimated by using the Kaplan-Meier method and were compared by using the log-rank test.
During the study period, 442 adults with ES underwent thoracic transplantation (316 HLTs and 126 BLTs). Following BLT, overall survival 1, 5, and 10 years' posttransplant was 63.1%, 38.5%, and 30.2%, respectively. Following HLT, overall survival 1, 5, and 10 years' posttransplant was 68.0%, 47.3%, and 30.5% (P = .6). When survival analysis was stratified according to type of defect, patients with an atrial septal defect had better survival following BLT than following HLT (88.3% vs 63.2% 1 year posttransplant, P < .01; 71.1% vs 49.8% 3 years' posttransplant, P < .01; and 37.4% vs 29.9% 10 years' posttransplant, P = .08). Patients with a ventricular septal defect (VSD) exhibited better survival following HLT than following BLT (78.2% vs 49.6% 1 year posttransplant, P < .01; 55.6% vs 34.3% 5 years' posttransplant, P < .01; and 35.7% vs 26.5% 10 years' posttransplant, P = .03). The most common cause of mortality in patients with VSD undergoing BLT was cardiac ventricular failure.
This study suggests that the best transplant option for patients with VSD remains HLT, which prevents subsequent development of ventricular failure. BLT with cardiac defect repair should be considered as the first-line treatment option in patients with ES due to an uncorrected atrial septal defect. These patients can be considered to have isolated and reversible right ventricular failure akin to patients with advanced pulmonary arterial hypertension.
对于 Eisenmenger 综合征（ES）患者，即使接受了肺动脉高压的最佳治疗，仍出现难治性右心衰竭，可考虑进行胸廓移植。本研究比较了心脏缺陷修复的双侧肺移植（BLT）与心肺联合移植（HLT）的结果。
本研究通过美国国家登记处的一项更新分析，评估了诊断为 ES 并接受 HLT 或 BLT 联合心脏缺陷修复的患者的结局。
本研究从 1987 年至 2018 年，从器官共享联合网络数据库中确定了接受胸廓移植的 ES 患者。使用 Kaplan-Meier 方法估计生存曲线，并使用对数秩检验比较生存曲线。
在研究期间，442 名 ES 成人接受了胸廓移植（316 例 HLT 和 126 例 BLT）。BLT 后，移植后 1、5 和 10 年的总体生存率分别为 63.1%、38.5%和 30.2%。HLT 后，移植后 1、5 和 10 年的总体生存率分别为 68.0%、47.3%和 30.5%（P=0.6）。当根据缺陷类型对生存分析进行分层时，BLT 治疗后的房间隔缺损患者生存率优于 HLT（移植后 1 年，88.3% vs 63.2%，P<.01；移植后 3 年，71.1% vs 49.8%，P<.01；移植后 10 年，37.4% vs 29.9%，P=0.08）。BLT 治疗后的室间隔缺损（VSD）患者生存率优于 HLT（移植后 1 年，78.2% vs 49.6%，P<.01；移植后 5 年，55.6% vs 34.3%，P<.01；移植后 10 年，35.7% vs 26.5%，P=0.03）。VSD 行 BLT 患者死亡的最常见原因是心脏心室衰竭。
本研究表明，VSD 患者的最佳移植选择仍然是 HLT，这可预防随后发生的心室衰竭。由于存在未矫正的房间隔缺损，BLT 联合心脏缺陷修复应被视为 ES 患者的一线治疗选择。这些患者可被视为患有类似晚期肺动脉高压的孤立且可逆转的右心衰竭。
