胸膜后胸膜纤维细胞增生症肺移植的疗效：一项法国多中心回顾性研究。

IF 6.4 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Journal of Heart and Lung Transplantation Pub Date : 2024-06-21 DOI:10.1016/j.healun.2024.06.009

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引用次数: 0

摘要

背景：胸膜后胸膜纤维细胞增生症（PPFE）目前尚无特效治疗方法。肺移植（LT）治疗胸膜前胸膜纤维母细胞增生症（PPFE）的益处鲜有文献记载：我们对2012年至2022年间法国因PPFE继发慢性终末期肺病而接受肺移植或心肺移植的患者进行了一项全国性多中心回顾性研究：结果：共纳入31名患者。移植时的中位年龄为 48 岁 [IQR 35 - 55]。64.5%为女性。21例（67.7%）为特发性PFFE。16例（52%）进行了双侧LT，10例（32%）进行了单侧LT，4例（13%）进行了肺叶移植，1例（3%）进行了心肺移植。手术死亡率为 3.2%。早期死亡率（<90天或首次住院期间）为32%。11名患者（35.5%）因止血而再次手术。8名患者（30.8%）出现支气管并发症。机械通气时间为 10 天 [IQR 2-55]。重症监护室和住院时间分别为 34 [IQR 18-73] 天和 64 [IQR 36-103] 天。中位生存期为 21 个月。移植后1年、2年和5年的存活率分别为57.9%、42.6%和38.3%。低白蛋白血症（p=0.046）、FVC（p=0.021）、FEV1（p=0.009）和高急诊肺移植（p=0.04）与早期死亡率增加有关。超大移植物往往与较高的死亡率相关（p=0.07）：结论：LT 治疗 PPFE 与术后高死亡率有关。结论：PPFE的LT与高术后发病率和死亡率有关，需要进行高度紧急肺移植的晚期疾病、营养不良或临床状况危急的患者的预后更差：Gov identifier：NCT05044390。

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Outcomes of lung transplantation for pleuroparenchymal fibroelastosis: A French multicentric retrospective study

Background

Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented.

Methods

We conducted a nation-wide multicentric retrospective study in patients who underwent lung or heart-lung transplantation for chronic end-stage lung disease secondary to PPFE between 2012 and 2022 in France.

Results

Thirty-one patients were included. At transplantation, median age was 48 years [IQR 35–55]. About 64.5% were women. Twenty-one (67.7%) had idiopathic PFFE. Sixteen (52%) had bilateral LT, 10 (32%) had single LT, 4 (13%) had lobar transplantation and one (3%) had heart-lung transplantation. Operative mortality was 3.2%. Early mortality (<90 days or during the first hospitalization) was 32%. Eleven patients (35.5%) underwent reoperation for hemostasis. Eight (30.8%) experienced bronchial complications. Mechanical ventilation time was 10 days [IQR 2–55]. Length of stay in intensive care unit and hospital were 34 [IQR 18–73] and 64 [IQR 36–103] days, respectively. Median survival was 21 months. Post-transplant survival rates after 1, 2, and 5 years were 57.9%, 42.6% and 38.3% respectively. Low albuminemia (p = 0.046), FVC (p = 0.021), FEV1 (p = 0.009) and high emergency lung transplantation (p = 0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (p = 0.07).

Conclusion

LT for PPFE is associated with high post-operative morbi-mortality rates. Patients requiring high emergency lung transplantation with advanced disease, malnutrition, or critical clinical status experienced worse outcomes.

ClinicalTrials.gov Identifier

NCT05044390.

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来源期刊

Journal of Heart and Lung Transplantation 医学-呼吸系统

CiteScore

10.10

自引率

6.70%

发文量

1667

审稿时长

69 days

期刊介绍： The Journal of Heart and Lung Transplantation, the official publication of the International Society for Heart and Lung Transplantation, brings readers essential scholarly and timely information in the field of cardio-pulmonary transplantation, mechanical and biological support of the failing heart, advanced lung disease (including pulmonary vascular disease) and cell replacement therapy. Importantly, the journal also serves as a medium of communication of pre-clinical sciences in all these rapidly expanding areas.