特发性肺纤维化合并胸膜肺弹力纤维增生症患者肺移植的预后：一项法国多中心回顾性研究。

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

机构信息

Department of Thoracic Surgery and Lung Transplantation, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.

Department of Thoracic Surgery and Heart-Lung transplantation, Université Paris-Saclay, Marie Lannelongue Hospital, Le Plessis Robinson, France.

出版信息

J Heart Lung Transplant. 2024 Oct;43(10):1727-1736. doi: 10.1016/j.healun.2024.06.009. Epub 2024 Jun 21.

DOI:10.1016/j.healun.2024.06.009

PMID:38909712

Abstract

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.

GOV IDENTIFIER

NCT05044390.

摘要

背景

特发性肺纤维化合并寻常型间质性肺炎（PPFE）目前尚无特效治疗方法。肺移植（LT）治疗 PPFE 的疗效尚缺乏充分证据。

方法

我们对 2012 年至 2022 年期间在法国因 PPFE 导致慢性终末期肺病而接受 LT 或心肺联合 LT 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 例患者，LT 时中位年龄为 48 岁（IQR 35-55），约 64.5%为女性。21 例（67.7%）为特发性 PFFE。16 例（52%）接受了双侧 LT，10 例（32%）接受了单侧 LT，4 例（13%）接受了肺叶移植，1 例（3%）接受了心肺联合移植。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 例（35.5%）因出血行再次手术，8 例（30.8%）发生支气管并发症。机械通气时间为 10 天（IQR 2-55）。重症监护病房和住院时间分别为 34 天（IQR 18-73）和 64 天（IQR 36-103）。中位生存期为 21 个月。LT 后 1、2、5 年的生存率分别为 57.9%、42.6%和 38.3%。低白蛋白血症（p=0.046）、FVC（p=0.021）、FEV1（p=0.009）和急诊 LT（p=0.04）与早期死亡率增加相关。肺移植供体过大与死亡率增加相关（p=0.07）。