{"title":"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。","authors":"","doi":"10.1016/j.healun.2024.06.009","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><p>Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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 (<em>p</em> = 0.046), FVC (<em>p</em> = 0.021), FEV1 (<em>p</em> = 0.009) and high emergency lung transplantation (<em>p</em> = 0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (<em>p</em> = 0.07).</p></div><div><h3>Conclusion</h3><p>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.</p></div><div><h3><span><span>ClinicalTrials.gov</span><svg><path></path></svg></span> Identifier</h3><p>NCT05044390.</p></div>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":null,"pages":null},"PeriodicalIF":6.4000,"publicationDate":"2024-06-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1053249824017017/pdfft?md5=5edd65f389543b10b043c68e7785325b&pid=1-s2.0-S1053249824017017-main.pdf","citationCount":"0","resultStr":"{\"title\":\"Outcomes of lung transplantation for pleuroparenchymal fibroelastosis: A French multicentric retrospective study\",\"authors\":\"\",\"doi\":\"10.1016/j.healun.2024.06.009\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><p>Pleuroparenchymal fibroelastosis (PPFE) has no currently available specific treatment. Benefits of lung transplantation (LT) for PPFE are poorly documented.</p></div><div><h3>Methods</h3><p>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.</p></div><div><h3>Results</h3><p>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 (<em>p</em> = 0.046), FVC (<em>p</em> = 0.021), FEV1 (<em>p</em> = 0.009) and high emergency lung transplantation (<em>p</em> = 0.04) were associated with increased early mortality. Oversized graft tended to be correlated to a higher mortality (<em>p</em> = 0.07).</p></div><div><h3>Conclusion</h3><p>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.</p></div><div><h3><span><span>ClinicalTrials.gov</span><svg><path></path></svg></span> Identifier</h3><p>NCT05044390.</p></div>\",\"PeriodicalId\":15900,\"journal\":{\"name\":\"Journal of Heart and Lung Transplantation\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":6.4000,\"publicationDate\":\"2024-06-21\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.sciencedirect.com/science/article/pii/S1053249824017017/pdfft?md5=5edd65f389543b10b043c68e7785325b&pid=1-s2.0-S1053249824017017-main.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Heart and Lung Transplantation\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1053249824017017\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Heart and Lung Transplantation","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1053249824017017","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
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.
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.