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Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease

Olsson, Karl Wilhelm ; Mani, Kevin ; Burdess, Anne ; Patterson, Suzannah ; Scali, Salvatore T. ; Kölbel, Tilo LU ; Panuccio, Giuseppe ; Eleshra, Ahmed ; Bertoglio, Luca and Ardita, Vincenzo , et al. (2023) In JAMA Surgery 158(8). p.832-839
Abstract

Importance: Endovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma. Objective: To assess the midterm outcomes of endovascular aortic repair in patients with CTD. Design, Setting, and Participants: For this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December... (More)

Importance: Endovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma. Objective: To assess the midterm outcomes of endovascular aortic repair in patients with CTD. Design, Setting, and Participants: For this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December 2021 to November 2022. Exposure: All principal endovascular aortic repairs, including redo surgery and complex repairs of the aortic arch and visceral aorta. Main Outcomes and Measures: Short-term and midterm survival, rates of secondary procedures, and conversion to open repair. Results: In total, 171 patients were included: 142 with Marfan syndrome, 17 with Loeys-Dietz syndrome, and 12 with vascular Ehlers-Danlos syndrome (vEDS). Median (IQR) age was 49.9 years (37.9-59.0), and 107 patients (62.6%) were male. One hundred fifty-two (88.9%) were treated for aortic dissections and 19 (11.1%) for degenerative aneurysms. One hundred thirty-six patients (79.5%) had undergone open aortic surgery before the index endovascular repair. In 74 patients (43.3%), arch and/or visceral branches were included in the repair. Primary technical success was achieved in 168 patients (98.2%), and 30-day mortality was 2.9% (5 patients). Survival at 1 and 5 years was 96.2% and 80.6% for Marfan syndrome, 93.8% and 85.2% for Loeys-Dietz syndrome, and 75.0% and 43.8% for vEDS, respectively. After a median (IQR) follow-up of 4.7 years (1.9-9.2), 91 patients (53.2%) had undergone secondary procedures, of which 14 (8.2%) were open conversions. Conclusions and Relevance: This study found that endovascular aortic interventions, including redo procedures and complex repairs of the aortic arch and visceral aorta, in patients with CTD had a high rate of early technical success, low perioperative mortality, and a midterm survival rate comparable with reports of open aortic surgery in patients with CTD. The rate of secondary procedures was high, but few patients required conversion to open repair. Improvements in devices and techniques, as well as ongoing follow-up, may result in endovascular treatment for patients with CTD being included in guideline recommendations.

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Contribution to journal
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published
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JAMA Surgery
volume
158
issue
8
pages
8 pages
publisher
American Medical Association
external identifiers
  • pmid:37314760
  • scopus:85167482285
ISSN
2168-6254
DOI
10.1001/jamasurg.2023.2128
language
English
LU publication?
yes
id
83690b70-4676-4445-979c-fe6bc8a6dcc0
date added to LUP
2023-11-01 11:17:25
date last changed
2024-04-19 03:23:36
@article{83690b70-4676-4445-979c-fe6bc8a6dcc0,
  abstract     = {{<p>Importance: Endovascular treatment is not recommended for aortic pathologies in patients with connective tissue diseases (CTDs) other than in redo operations and as bridging procedures in emergencies. However, recent developments in endovascular technology may challenge this dogma. Objective: To assess the midterm outcomes of endovascular aortic repair in patients with CTD. Design, Setting, and Participants: For this descriptive retrospective study, data on demographics, interventions, and short-term and midterm outcomes were collected from 18 aortic centers in Europe, Asia, North America, and New Zealand. Patients with CTD who had undergone endovascular aortic repair from 2005 to 2020 were included. Data were analyzed from December 2021 to November 2022. Exposure: All principal endovascular aortic repairs, including redo surgery and complex repairs of the aortic arch and visceral aorta. Main Outcomes and Measures: Short-term and midterm survival, rates of secondary procedures, and conversion to open repair. Results: In total, 171 patients were included: 142 with Marfan syndrome, 17 with Loeys-Dietz syndrome, and 12 with vascular Ehlers-Danlos syndrome (vEDS). Median (IQR) age was 49.9 years (37.9-59.0), and 107 patients (62.6%) were male. One hundred fifty-two (88.9%) were treated for aortic dissections and 19 (11.1%) for degenerative aneurysms. One hundred thirty-six patients (79.5%) had undergone open aortic surgery before the index endovascular repair. In 74 patients (43.3%), arch and/or visceral branches were included in the repair. Primary technical success was achieved in 168 patients (98.2%), and 30-day mortality was 2.9% (5 patients). Survival at 1 and 5 years was 96.2% and 80.6% for Marfan syndrome, 93.8% and 85.2% for Loeys-Dietz syndrome, and 75.0% and 43.8% for vEDS, respectively. After a median (IQR) follow-up of 4.7 years (1.9-9.2), 91 patients (53.2%) had undergone secondary procedures, of which 14 (8.2%) were open conversions. Conclusions and Relevance: This study found that endovascular aortic interventions, including redo procedures and complex repairs of the aortic arch and visceral aorta, in patients with CTD had a high rate of early technical success, low perioperative mortality, and a midterm survival rate comparable with reports of open aortic surgery in patients with CTD. The rate of secondary procedures was high, but few patients required conversion to open repair. Improvements in devices and techniques, as well as ongoing follow-up, may result in endovascular treatment for patients with CTD being included in guideline recommendations.</p>}},
  author       = {{Olsson, Karl Wilhelm and Mani, Kevin and Burdess, Anne and Patterson, Suzannah and Scali, Salvatore T. and Kölbel, Tilo and Panuccio, Giuseppe and Eleshra, Ahmed and Bertoglio, Luca and Ardita, Vincenzo and Melissano, Germano and Acharya, Amish and Bicknell, Colin and Riga, Celia and Gibbs, Richard and Jenkins, Michael and Bakthavatsalam, Arvind and Sweet, Matthew P. and Kasprzak, Piotr M. and Pfister, Karin and Oikonomou, Kyriakos and Heloise, Tessely and Sobocinski, Jonathan and Butt, Talha and Dias, Nuno and Tang, Ching and Cheng, Stephen W.K. and Vandenhaute, Sarah and Van Herzeele, Isabelle and Sorber, Rebecca A. and Black, James H. and Tenorio, Emanuel R. and Oderich, Gustavo S. and Vincent, Zoë and Khashram, Manar and Eagleton, Matthew J. and Pedersen, Steen Fjord and Budtz-Lilly, Jacob and Lomazzi, Chiara and Bissacco, Daniele and Trimarchi, Santi and Huerta, Abigail and Riambau, Vincent and Wanhainen, Anders}},
  issn         = {{2168-6254}},
  language     = {{eng}},
  number       = {{8}},
  pages        = {{832--839}},
  publisher    = {{American Medical Association}},
  series       = {{JAMA Surgery}},
  title        = {{Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease}},
  url          = {{http://dx.doi.org/10.1001/jamasurg.2023.2128}},
  doi          = {{10.1001/jamasurg.2023.2128}},
  volume       = {{158}},
  year         = {{2023}},
}