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Editor's Choice – Management of Descending Thoracic Aorta Diseases

Clinical Practice Guidelines of the European Society for Vascular Surgery (ESVS)
  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    V. Riambau
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    D. Böckler
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    J. Brunkwall
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    P. Cao
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    R. Chiesa
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    G. Coppi
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    M. Czerny
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    G. Fraedrich
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    S. Haulon
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    M.J. Jacobs
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    M.L. Lachat
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    F.L. Moll
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    C. Setacci
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    P.R. Taylor
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    M. Thompson
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    S. Trimarchi
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    H.J. Verhagen
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    E.L. Verhoeven
    Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
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  • Author Footnotes
    b ESVS Guidelines Committee: Philippe Kolh (Chair) (Belgium), Gert Jan de Borst (Co-Chair) (Netherlands), Nabil Chakfé (France), Eike Sebastian Debus (Germany), Robert J. Hinchliffe (United Kingdom), Stavros Kakkos (Greece and United Kingdom), Igor Koncar (Serbia), Jes S. Lindholt (Denmark), Melina Vega de Ceniga (Spain), Frank Vermassen (Belgium), Fabio Verzini (Italy).
    ESVS Guidelines Committee
    Footnotes
    b ESVS Guidelines Committee: Philippe Kolh (Chair) (Belgium), Gert Jan de Borst (Co-Chair) (Netherlands), Nabil Chakfé (France), Eike Sebastian Debus (Germany), Robert J. Hinchliffe (United Kingdom), Stavros Kakkos (Greece and United Kingdom), Igor Koncar (Serbia), Jes S. Lindholt (Denmark), Melina Vega de Ceniga (Spain), Frank Vermassen (Belgium), Fabio Verzini (Italy).
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  • P. Kolh
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  • G.J. de Borst
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  • N. Chakfé
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  • E.S. Debus
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  • R.J. Hinchliffe
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  • S. Kakkos
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  • I. Koncar
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  • J.S. Lindholt
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  • M. Vega de Ceniga
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  • F. Vermassen
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  • F. Verzini
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  • Author Footnotes
    c Document Reviewers: Philippe Kolh (Review Coordinator) (Belgium), James H. Black, III (United States), Rolf Busund (Norway), Martin Björck (Sweden), Michael Dake (United States), Florian Dick (Switzerland), Holger Eggebrecht (Germany), Arturo Evangelista (Spain), Martin Grabenwöger (Austria), Ross Milner (United States), A. Ross Naylor (United Kingdom), Jean-Baptiste Ricco (France), Hervé Rousseau (France), Jürg Schmidli (Switzerland).
    Document Reviewers
    Footnotes
    c Document Reviewers: Philippe Kolh (Review Coordinator) (Belgium), James H. Black, III (United States), Rolf Busund (Norway), Martin Björck (Sweden), Michael Dake (United States), Florian Dick (Switzerland), Holger Eggebrecht (Germany), Arturo Evangelista (Spain), Martin Grabenwöger (Austria), Ross Milner (United States), A. Ross Naylor (United Kingdom), Jean-Baptiste Ricco (France), Hervé Rousseau (France), Jürg Schmidli (Switzerland).
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  • P. Kolh
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  • J.H. Black III
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  • R. Busund
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  • M. Björck
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  • M. Dake
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  • F. Dick
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  • H. Eggebrecht
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  • A. Evangelista
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  • M. Grabenwöger
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  • R. Milner
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  • A.R. Naylor
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  • J.-B. Ricco
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  • H. Rousseau
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  • J. Schmidli
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  • Author Footnotes
    a Writing Committee: Vincent Riambau (Chair) (Spain), Dittmar Böckler (Germany), Jan Brunkwall (Germany), Piergiorgio Cao (Italy), Roberto Chiesa (Italy), Gioachino Coppi (Italy), Martin Czerny (Germany), Gustav Fraedrich (Austria), Stephan Haulon (France), Michael J. Jacobs (Netherlands), Mario L. Lachat (Switzerland), Frans L. Moll (Netherlands), Carlo Setacci (Italy), Peter R. Taylor (United Kingdom), Matt Thompson (United Kingdom), Santi Trimarchi (Italy), Hence J. Verhagen (Netherland) and Eric L. Verhoeven (Germany).
    b ESVS Guidelines Committee: Philippe Kolh (Chair) (Belgium), Gert Jan de Borst (Co-Chair) (Netherlands), Nabil Chakfé (France), Eike Sebastian Debus (Germany), Robert J. Hinchliffe (United Kingdom), Stavros Kakkos (Greece and United Kingdom), Igor Koncar (Serbia), Jes S. Lindholt (Denmark), Melina Vega de Ceniga (Spain), Frank Vermassen (Belgium), Fabio Verzini (Italy).
    c Document Reviewers: Philippe Kolh (Review Coordinator) (Belgium), James H. Black, III (United States), Rolf Busund (Norway), Martin Björck (Sweden), Michael Dake (United States), Florian Dick (Switzerland), Holger Eggebrecht (Germany), Arturo Evangelista (Spain), Martin Grabenwöger (Austria), Ross Milner (United States), A. Ross Naylor (United Kingdom), Jean-Baptiste Ricco (France), Hervé Rousseau (France), Jürg Schmidli (Switzerland).
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      Keywords

      Abbreviations and acronyms

      AAA
      Abdominal Aortic Aneurysm
      AAS
      Acute Aortic Syndromes
      AAST
      American Association for Surgery of Trauma
      AD
      Aortic Dissection
      ATBAD
      Acute Type B Aortic Dissection
      CA
      Coeliac Artery
      CAD
      Coronary Artery Disease
      CCA
      Common Carotid Artery
      COPD
      Chronic Obstructive Pulmonary Disease
      CSF
      Cerebrospinal Fluid
      CT
      Computed Tomography
      CTA
      Computed Tomographic Angiography
      CTBAD
      Chronic Type B Aortic Dissection
      DSA
      Digital Subtraction Angiography
      DTA
      Descending Thoracic Aorta
      DTAA
      Descending Thoracic Aortic Aneurysm
      ECG
      Electrocardiogram
      EDS
      Ehlers-Danlos Syndrome
      EJVES
      European Journal of Vascular and Endovascular Surgery
      ESR
      Erythrocyte Sedimentation Rate
      ESVS
      European Society for Vascular Surgery
      FL
      False Lumen
      GCA
      Giant Cells Arteritis
      IMH
      Intramural Haematoma
      IRAD
      International Registry of Aortic Dissection
      IVUS
      Intravascular Ultrasonography
      LDS
      Loeys-Dietz Syndrome
      LHB
      Left Heart Bypass
      LSA
      Left Subclavian Artery
      MAP
      Mean Arterial Pressure
      MEP
      Motor Evoked Potentials
      MFS
      Marfan Syndrome
      MRA
      Magnetic Resonance Angiography
      MRI
      Magnetic Resonance Imaging
      NSF
      Nephrogenic Systemic Fibrosis
      OR
      Open Repair
      PAU
      Penetrating Aortic Ulcer
      PET
      Positron Emission Tomography
      PMR
      Polymyalgia Rheumatica
      RCT
      Randomized Clinical Trial
      SCI
      Spinal Cord Ischaemia
      SMA
      Superior Mesenteric Artery
      SSEP
      Somatosensory Evoked Potentials
      TA
      Takayasu Arteritis
      TAAA
      Thoraco-Abdominal Aortic Aneurysm
      TAI
      Thoracic Aortic Injury
      TBAD
      Type B Aortic Dissection
      TEVAR
      Thoracic Endovascular Repair
      TL
      True Lumen
      TOE
      Transoesophageal Echocardiography
      TS
      Turner Syndrome
      TTE
      Transthoracic Echocardiography
      WC
      Writing Committee

      1. Introduction

      1.1 Purpose

      The European Society for Vascular Surgery (ESVS) appointed the Descending Thoracic Aorta (DTA) Writing Committee (WC) to produce the current clinical practice guidelines document for surgeons and other physicians who are involved in the overall care of patients with DTA disorders. The goal of these guidelines is to summarize and evaluate all current available evidence to assist physicians in selecting the best management strategies for all DTA pathologies. However, each respective physician must make the ultimate decision regarding the particular care of an individual patient.
      The present guidelines document aims to improve decision making and decrease variability in the vascular surgical care of patients presenting with pathology of the DTA. Unfortunately, robust evidence from prospective and randomized studies is not available for management of most DTA diseases. Consequently, the recommendations in these guidelines are entirely based on level B and C evidence. Nevertheless, when managing DTA pathology, it is clinically helpful to have access to the most recent and best available clinical and experimental knowledge to determine the current standard of care.
      The DTA WC intentionally agreed to exclude pathology of the ascending aorta and aortic arch from the current document to avoid potential inter-specialty conflict. The cost analysis of different treatments was also excluded because of differences in financial management and differing health systems across Europe. Primarily infectious or mycotic disease processes were also considered outside of the scope of this document because of their low incidence and poor outcomes.
      All disorders originating in the DTA from the left subclavian artery (LSA) origin to the diaphragm were considered for these guidelines. Pathology involving the thoraco-abdominal segment of the aorta was also included.

      1.2 Methodology

      The DTA WC was formed by members of the ESVS from different European countries, various academic and private hospitals, and by both vascular surgeons and endovascular specialists, to maximize the applicability of the final guidelines document. The DTA Guidelines Committee met in November 2011 for the first time to discuss the purpose, contents, methods, and timeline of the following recommendations.
      The DTA WC performed a systematic English literature search in the MEDLINE, EMBASE, and COCHRANE Library databases for each of the different topics that are discussed and reviewed. The latest literature search was performed in December 2015. With regard to evidence gathered, the following eligibility criteria were applied:
      • Only peer reviewed published literature was considered
      • Published abstracts or congress proceedings were excluded
      • Randomized clinical trials (RCT) as well as meta-analyses and systematic reviews were assessed with priority
      • Non-randomized clinical trials and non-controlled studies were included
      • Well conducted observational studies (cohort and case control studies) were included
      • Previous guidelines, position papers, and published consensus documents were included as part of the review process when new evidence was absent
      • We minimized the use of reports from a single medical device or from pharmaceutical companies in order to reduce the risk of bias across studies.
      A grading system was adopted based on the European Society of Cardiology (ESC) guidelines methodology. The level of evidence classification provides information about the study characteristics supporting the recommendation and expert consensus, according to the categories shown in Table 1.
      Table 1Levels of evidence.
      Table thumbnail fx1
      The recommendation grade indicates the strength of a recommendation. Definitions of the classes of recommendation are shown in Table 2.
      Table 2Grades of strength of recommendations according to the ESC grading system.
      Table thumbnail fx2
      For each recommendation, two members of the WC assessed the strength of a recommendation and the quality of supporting evidence independently. A full master copy of the manuscript with all recommendations was electronically circulated and approved by all WC members. Recommendations that required consensus were discussed and voted on by email among all members of the WC.
      This system permits strong recommendations, supported by low or very low quality evidence from downgraded RCTs or observational studies, only when a general consensus is achieved among the WC members and reviewers.
      Two members of the WC prepared each part of the guidelines document. An internal review process was performed before the manuscript was sent to independent external reviewers. External reviewers made critical suggestions, comments, and corrections on all preliminary versions of this guideline. In addition, each member participated in the consensus process concerning conflicting recommendations. The final document was reviewed and approved by the ESVS Guidelines Committee and submitted to the European Journal of Vascular and Endovascular Surgery (EJVES). Further updated guidelines documents on DTA management will be provided periodically by the ESVS when new evidence and/or new clinical practice arise in this field.
      To optimize the implementation of the current document, the length of the guideline has been kept as short as possible to facilitate access to guideline information. Following this decision, the “References” list has been limited to the most relevant references related to these guidelines. Nevertheless, an Appendix of recommended additional references, also reviewed by the WC, has been added for further information for readers. This clinical guidelines document was constructed as a guide, not a document of rules, allowing for flexibility with various patient presentations. The resulting clinical practice guidelines provide recommendations for the clinical care of patients with thoracic aortic diseases including pre-, peri-, and post-operative care.
      Conflicts of interest of each WC member were collected prior to the writing process. These conflicts were assessed and accepted by each member of the WC and are reported on the ESVS website. In addition, the WC agreed that all intellectual work should be expressed without any interference beyond the honesty and professionalism of all members and assistants during the writing process.

      2. General Aspects

      2.1 The normal descending thoracic aorta

      The DTA originates from the isthmus, the region of the thoracic aorta between the origin of the LSA and the ductus arteriosus. The descending thoracic aorta runs in a left para-spinal location until its distal segment, where it passes anteriorly through the diaphragmatic aortic hiatus and inferiorly into the abdomen. Important aortic side branches originating from the descending thoracic aorta include the intercostal arteries, spinal arteries, and bronchial arteries. The normal diameter of the mid-descending aorta ranges from 24 to 29 mm in men and 24 to 26 mm in women, whereas the normal diameter at the level of the diaphragm is 24 to 27 mm in men and 23 to 24 mm in women. Aortic diameter is influenced by age and body mass index.
      • Johnston K.W.
      • Rutherford R.B.
      • Tilson M.D.
      • Shah D.M.
      • Hollier L.
      • Stanley J.C.
      Suggested standards for reporting on arterial aneurysms. Subcommittee on Reporting Standards for Arterial Aneurysms, Ad Hoc Committee on Reporting Standards, Society for Vascular Surgery and North American Chapter, International Society for Cardiovascular Surgery.
      • Kälsch H.
      • Lehmann N.
      • Möhlenkamp S.
      • Becker A.
      • Moebus S.
      • Schmermund A.
      • et al.
      Body-surface adjusted aortic reference diameters for improved identification of patients with thoracic aortic aneurysms: results from the population-based Heinz Nixdorf Recall study.
      The aortic wall is composed of three layers: the intima, media, and adventitia. The intima, the innermost layer, consists of an endothelial monolayer and an internal elastic lamina. Because it is in direct contact with blood, the function of the intima is to prevent thrombosis and atherosclerosis. Its anti-thrombotic and anti-atheroscle-rotic function can be reduced by risk factors, such as smoking, hypertension, hyperlipidaemia, diabetes, and direct trauma, each making patients more prone to aortic disease.
      The media consists of concentric layers of elastin, collagen, and smooth muscle cells. These components are responsible for aortic wall elasticity, which accommodates the changes in stroke volume during the cardiac cycle, converts pulsatile inflow into a smoother outflow (Windkessel function), and maintains the integrity of the aortic wall. Congenital or hereditary disorders (e.g. bicuspid aortic valve, Marfan syndrome [MFS], Ehlers-Danlos syndrome [EDS]), risk factors (hypertension, atherosclerosis, and trauma) all influence aortic wall function. These conditions can stiffen the aortic wall, decreasing its ability to accommodate the stroke volume, resulting in systemic hypertension, or weakening of the aortic wall, leading to dilatation or dissection.
      The adventitia is the outermost layer of the aortic wall and is composed mainly of collagen fibres, external elastic lamina, and small vessels (the vasa vasorum), which provide the blood supply to the aortic wall and surrounding nerves.

      2.2 Epidemiology of descending thoracic aortic disease

      DTA diseases consist of a broad spectrum of degenerative, structural, acquired, genetic, and traumatic disorders. The true incidence of descending thoracic aortic pathology remains unclear. Epidemiological studies are sparse and it is likely that many DTA related deaths are attributed to other cardiovascular diseases, such as cardiac arrest, myocardial infarction, cerebrovascular accidents, or abdominal aneurysm rupture. Therefore, the overall incidence of DTA disease is likely to be underestimated.
      The pathophysiology of thoracic aortic diseases is believed to be multifactorial, resulting both from genetic susceptibility and environmental exposure. Therefore, the incidence of the different thoracic aortic diseases can vary significantly among different population groups.
      Acute aortic syndromes (AAS) consist of three interrelated diseases: aortic dissection, penetrating aortic ulcer (PAU), and intramural haematoma (IMH). Type B aortic dissection (TBAD) most commonly affects male patients and has an incidence between 2.9 and 4.0 per 100,000 person-years.
      • Clouse W.D.
      • Hallet Jr., J.W.
      • Shaff H.V.
      • Spittell P.C.
      • Rowland C.M.
      • Ilstrup C.M.
      • et al.
      Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture.
      The incidence of TBAD seems to be increasing. A recent prospective analysis of 30,412 middle aged men and women from Malmö, Sweden with a 20 year follow up reported an incidence of acute aortic dissection of 15 per 100,000 patient years.
      • Landenhed M.
      • Engstrom G.
      • Gottsater A.
      • Caulfield M.P.
      • Hedblad B.
      • Newton-Cheh C.
      • et al.
      Risk profiles for aortic dissection and ruptured or surgically treated aneurysms: a prospective cohort study.
      This increase is probably caused by the increasing age of the population and improving diagnostic modalities. The exact incidence remains unknown, but PAU has been diagnosed with increasing frequency because of the widespread use of advanced cross sectional imaging techniques. In symptomatic patients suspected of AAS, the prevalence of PAU is 2.3–7.6% and the lesion is localised in the DTA in 90% of patients.
      • Eggebrecht H.
      • Nienaber C.A.
      • Neuhauser M.
      • Baumgart D.
      • Kische S.
      • Schmermund A.
      • et al.
      Endovascular stent graft placement in aortic dissection: a meta-analysis.
      IMH may be related to PAU, accounting for 5–20% of patients with AAS and more commonly involving the DTA (60%) than the ascending aorta.
      • Evangelista A.
      • Mukherjee D.
      • Mehta R.H.
      • O'Gara P.T.
      • Fattori R.
      • Cooper J.V.
      • et al.
      Acute intramural hematoma of the aorta: a mystery in evolution.
      Trauma is the leading cause of death during the first four decades of life, accounting for more than 250,000 deaths every year in the European Union alone. Blunt aortic injury is the second leading cause of death in these patients, and although it occurs in less than 1% of all motor vehicle accidents, it accounts for 16% of all traumatic deaths.

      European Commission. Mobility and transport. Road safety. http://ec.europa.eu/transport/road_safety/specialist/statistics/.

      Concerning ruptured descending thoracic aortic aneurysm (DTAA), a Swedish population study reported an incidence of 5 per 100,000 person-years. The mean age of patients in this cohort was 70 years for men and 72 years for women.
      • Johansson G.
      • Markström U.
      • Swedenborg J.
      Ruptured thoracic aortic aneurysms: a study of incidence and mortality rates.
      Aneurysmal dilatation of the DTA is a degenerative disease with an estimated incidence of 6–10.4 per 100,000 person-years. The incidence seems to be increasing with ageing of the general population and continually improving diagnostic modalities.
      • Fowkes F.G.
      • Macintyre C.C.
      • Ruckley C.V.
      Increasing incidence of aortic aneurysms in England and Wales.
      Aortitis is a relatively uncommon disorder with a broad spectrum of clinical features. The most common autoimmune disorders affecting the DTA are Takayasu's arteritis (TA), giant cell arteritis (GCA), and Behçet's disease. The best estimates of the incidence of TA suggest that two or three cases occur each year per million people.
      • Karageorgaki Z.T.
      • Bertsias G.K.
      • Mavragani C.P.
      • Kritikos H.D.
      • Spyropoulou-Vlachou M.
      • Drosos A.A.
      • et al.
      Takayasu arteritis: epidemiological, clinical, and immunogenetic features in Greece.
      There is a 9:1 female to male predominance. Although the disease has a worldwide distribution, it appears to occur more frequently in Asian women.
      GCA is the most common type of vasculitis observed in patients older than 50 years. Predominantly observed in populations of Scandinavian descent, it has a reported prevalence that varies between 1 and 30 per 100,000 people.
      • Nordborg E.
      • Nordborg C.
      Giant cell arteritis: epidemiological clues to its pathogenesis and an update on its treatment.
      The male to female ratio is around 2.5 to 1 and is highly dependent on geographic and genetic parameters.
      • Lee J.L.
      • Naguwa S.M.
      • Cheema G.S.
      • Gershwin M.E.
      The geo-epidemiology of temporal (giant cell) arteritis.
      Behçet's disease has been observed most commonly along the classic Silk Route, with a peak prevalence in Turkey of 80–370 per 100,000 people, compared with 1–3 per million people in the Western world. Presentation is typically in the third to fifth decade of life, and both genders are affected equally.
      • Idil A.
      • Gurler A.
      • Boyvat A.
      • Caliskan D.
      • Ozdemir O.
      • Isik A.
      • et al.
      The prevalence of Behcet's disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey.
      Coarctation of the aorta is a congenital cardiovascular defect, most commonly occurring at the level of the isthmus and accounts for 5–8% of all congenital heart defects. The overall incidence ranges between 20 and 60 per 100,000 person-years, with a slight male predominance. Patients with Turner syndrome (TS) are more commonly affected.
      • Teo L.L.
      • Cannell T.
      • Babu-Narayan S.V.
      • Hughes M.
      • Mohiaddin R.H.
      Prevalence of associated cardiovascular abnormalities in 500 patients with aortic coarctation referred for cardiovascular magnetic resonance imaging to a tertiary center.

      2.3 Diagnostic methods in descending thoracic aortic disease

      2.3.1 Medical history and physical examination

      A comprehensive medical and family history, assessment of symptoms, and careful physical examination including blood pressure measurement and electrocardiography (ECG), are required in all patients suspected of thoracic aortic disease. Patients should be examined for suspicious clinical signs such as aortic regurgitation, cardiac murmur, pericardial rub, signs of tamponade, and an expansile abdominal aorta. The diagnosis of thoracic aortic disease is based on imaging and the choice of imaging modality should be based on the patient's condition and the availability of different imaging modalities.
      • Goldstein S.A.
      • Evangelista A.
      • Abbara S.
      • Arai A.
      • Asch F.M.
      • Badano L.P.
      • et al.
      Multimodality imaging of diseases of the thoracic aorta in adults: from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.

      2.3.3 Transthoracic echocardiography

      The use of TTE to assess the DTA is limited by structures in the thorax that weaken or distort the ultrasound signal and compromise image quality. Via a suprasternal, subcostal, or parasternal view, small parts of the DTA can be visualized, while in the case of a pleural effusion, the back of the patient can be used for transthoracic imaging.
      • Evangelista A.
      • Flachskampf F.A.
      • Erbel R.
      • Antonini-Canterin F.
      • Vlachopoulos C.
      • Rocchi G.
      • et al.
      Echocardiography in aortic diseases: EAE recommendations for clinical practice.
      The major advantage of TTE is that it is non-invasive and can be used to visualize the ascending aorta, aortic arch, and supra-aortic vessels. In addition, the abdominal aorta can be visualized to check for abdominal extension of aortic dissection (AD). During TTE, all planes should be used to assess the extent of aortic disease and to exclude additional aortic or cardiac involvement. Because of its non-invasive nature and wide availability, TTE is increasingly used in the emergency department of community hospitals to screen patients suspected of having one or other acute aortic syndrome, such as type A dissection. However, the value of TTE in the diagnosis of DTA pathology remains limited. In cases of examination limitations or inconclusive diagnosis, the use of additional imaging modalities is recommended.
      • Evangelista A.
      • Flachskampf F.A.
      • Erbel R.
      • Antonini-Canterin F.
      • Vlachopoulos C.
      • Rocchi G.
      • et al.
      Echocardiography in aortic diseases: EAE recommendations for clinical practice.
      There are currently no specific studies to validate the usefulness of TTE for diagnosis of DTA pathology.

      2.3.4 Transoesophageal echocardiography

      TOE can visualize the DTA from the LSA to the coeliac artery (CA). This diagnostic test is generally used as a second line imaging modality and is useful to differentiate between AD, IMH, and PAU. With a reported sensitivity of 98% and a specificity of 95%, TOE is an accurate diagnostic tool for aortic disease, providing functional information in both the pre- and intra-operative settings.
      • Evangelista A.
      • Flachskampf F.A.
      • Erbel R.
      • Antonini-Canterin F.
      • Vlachopoulos C.
      • Rocchi G.
      • et al.
      Echocardiography in aortic diseases: EAE recommendations for clinical practice.
      The semi-invasive nature of TOE has rare procedure related risks, but it can cause patient discomfort, requires sedation, and is contraindicated in the presence of oesophageal pathologies. In the majority of cases, computed tomographic angiography (CTA) scanning is performed as the first imaging modality, providing all required information. TOE may be used in specific circumstances as a second line option.

      2.3.5 Computed tomographic angiography

      CTA offers a detailed visualisation of the entire aorta and its surrounding structures. It can distinguish different aortic pathologies and is quick and widely available. Over the last two decades, CTA has become more sophisticated and is more readily available, with an increase in the number of scanners, the use of retrospective and prospective ECG-gated techniques, and through advances in post-processing software. These advances have resulted in motion free images with better resolution, reduced scanning times, and better visualisation.
      • Parodi J.
      • Berguer R.
      • Carrascosa P.
      • Khanafer K.
      • Capunay C.
      • Wizauer E.
      Sources of error in the measurement of aortic diameter in computed tomography scans.
      Figure thumbnail fx4

      2.3.6 Magnetic resonance imaging

      MRI has an excellent diagnostic capability that is comparable with CTA and TOE and can be used for both pre-operative planning and follow up.
      • François C.J.
      • Markl M.
      • Schiebler M.L.
      • Niespodzany E.
      • Landgraf B.R.
      • Schlensak C.
      Four-dimensional, flow-sensitive magnetic resonance imaging of blood flow patterns in thoracic aortic dissections.
      A major advantage of MRI is that it offers multiplanar evaluation with good differentiation between different soft tissues. Moreover, MRI can provide additional dynamic imaging regarding entry tear flow or arterial vessel involvement.
      • François C.J.
      • Markl M.
      • Schiebler M.L.
      • Niespodzany E.
      • Landgraf B.R.
      • Schlensak C.
      Four-dimensional, flow-sensitive magnetic resonance imaging of blood flow patterns in thoracic aortic dissections.
      Although contrast is not usually required, the use of gadolinium enhances the quality of MRI. MRI can be used to visualize the aortic wall in detail and is, therefore, more commonly used in patients with aortic wall pathologies such as IMH or aortitis. Furthermore, MRI does not require ionizing radiation and offers an alternative for patients with renal insufficiency in some circumstances.
      Important disadvantages of MRI include its limited availability and longer scanning times, which makes it less suitable for critically ill or unstable patients. In addition, the ability of MRI to detect calcification is decreased (compared with CTA), and artefacts from respiration or metallic implants reduce image quality. Post-gadolinium nephrogenic systemic fibrosis (NSF) is a rare, but devastating, side-effect in patients with impaired renal function. Using alternative imaging modalities or using the lowest possible amount of gadolinium may prevent NSF.
      • Wang Y.
      • Alkasab T.K.
      • Narin O.
      • Nazarian R.M.
      • Kaewlai R.
      • Kay J.
      • et al.
      Incidence of nephrogenic systemic fibrosis after adoption of restrictive gadolinium-based contrast agent guidelines.

      2.3.7 Positron emission tomography

      PET is a nuclear imaging modality that is based on the detection of increased metabolic activity in inflammatory cells, resulting in increased uptake of a gammagraphic tracer, most commonly fluorodeoxyglucose ([18F] FDG). PET can be used for the diagnosis of aortitis and in the assessment of the extent and activity of any inflammatory disease. The diagnostic value of FDG-PET differs among the various inflammatory aortic diseases with a reported sensitivity ranging between 83% and 100% and specificity between 77% and 100%.
      • Walter M.A.
      • Melzer R.A.
      • Schindler C.
      • Muller-Brand J.
      • Tyndall A.
      • Nitzsche E.U.
      The value of [18F]FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease.
      Development of PET in combination with computed tomography (CT) scanners has made it possible to combine functional and anatomic imaging, thereby making it possible for PET findings to be correlated with adjacent anatomical features. Although the availability of PET and PET/CT is limited, this modality may be used for diagnosis and follow up of aortitis.
      • Litmanovich D.E.
      • Yıldırım A.
      • Bankier A.A.
      Insights into imaging of aortitis.
      Increased patient radiation exposure is a major disadvantage of PET/CT (when compared with PET or CT examinations alone), as the effective radiation dose is a combination of the dose from both scans.
      • Huang B.
      • Law M.W.
      • Khong P.L.
      Whole-body PET/CT scanning: estimation of radiation dose and cancer risk.

      2.3.8 Intra-vascular ultrasonography

      IVUS permits 360° visualisation of the aortic wall. It can be very helpful in confirming intimal defects when CTA and digital subtraction angiography (DSA) are inconclusive in the diagnosis of aortic injuries. IVUS is an operator and experience-dependent invasive procedure, and a complete evaluation of the aorta using IVUS can be time consuming. In some centres, IVUS is routinely used as an adjuvant imaging technique during endovascular repair (see also Section 3.1.4.1). Table 3 compares different imaging diagnostic tests for DTA according to their features and performance.
      Table 3Comparison of different imaging modalities for DTA diagnosis.
      • Goldstein S.A.
      • Evangelista A.
      • Abbara S.
      • Arai A.
      • Asch F.M.
      • Badano L.P.
      • et al.
      Multimodality imaging of diseases of the thoracic aorta in adults: from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.
      CharacteristicsTTETOECTAMRIPETIVUS
      Non-invasiveness++++++
      Ease of use+++++++++++++
      Availability++++++++++++
      Portability/bedside use+++++++/−
      Interventional guidance use+++++++
      Safe for serial examinations++++++++++++
      Wall contour assessment+++++++++++++
      Supra-aortic vessel status evaluation+++++++++++++
      Aortic valve status evaluation++++++
      Pericardial effusion detection++++++++++++++
      Inflammation/Infection detection+++++++++
      Number of signs (+ or −) represents the estimated potential value.
      TTE = transthoracic echocardiography; TOE = transoesophageal echocardiography; CTA = computed tomographic angiography; MRI = magnetic resonance imaging; PET = positron emission tomography; IVUS = intra-vascular ultrasonography.

      2.4 Neurological complications: prevention and management

      The most feared and devastating complications associated with management of DTA disease are spinal cord ischaemia (SCI), resulting in paraparesis or paraplegia, and cerebral embolism, resulting in stroke.
      • Svensson L.G.
      • Crawford E.S.
      • Hess K.R.
      • Coselli J.S.
      • Safi H.J.
      Experience with 1509 patients undergoing thoracoabdominal aortic operations.
      SCI can develop immediately after surgery or with a delayed presentation. Although SCI rates as high as 20% have been reported, the incidence probably ranges between 2% and 6%.
      • Coselli J.S.
      • LeMaire S.A.
      • Koksoy C.
      • Schmittling Z.C.
      • Curling P.E.
      Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial.
      The incidence of paraplegia is related to the duration and degree of spinal ischaemia resulting from an abrupt cessation of blood flow to the spinal cord and from a reperfusion injury mediated by biochemical mediators.
      • Jacobs M.J.
      • Mess W.
      • Mochtar B.
      • Nijenhuis R.J.
      • Statius van Eps R.G.
      • Schurink G.W.
      The value of motor evoked potentials in reducing paraplegia during thoracoabdominal aneurysm repair.
      The presence of stroke after DTA surgery seems to be similar to aortic arch repair and often has an embolic aetiology, with an incidence up to 8% in elective cases or higher in the emergency setting.
      • Goldstein L.J.
      • Davies R.R.
      • Rizzo J.A.
      • Davila J.J.
      • Cooperberg M.R.
      • Shaw R.K.
      Stroke in surgery of the thoracic aorta: incidence, impact, etiology, and prevention.
      Classic predictors for these complications are diverse and include increasing patient age, previous carotid artery disease, aortic cross-clamp time, the extent of the aortic resection, aortic rupture, concurrent aneurysm disease of ascending aorta and/or aortic arch, emergency surgery, the use and duration of hypothermic arrest, peri-operative hypotension, and pre-operative renal dysfunction.
      • Svensson L.G.
      • Crawford E.S.
      • Hess K.R.
      • Coselli J.S.
      • Safi H.J.
      Experience with 1509 patients undergoing thoracoabdominal aortic operations.
      • Goldstein L.J.
      • Davies R.R.
      • Rizzo J.A.
      • Davila J.J.
      • Cooperberg M.R.
      • Shaw R.K.
      Stroke in surgery of the thoracic aorta: incidence, impact, etiology, and prevention.
      Several methods for spinal cord and brain protection have been used to reduce the incidence of neurological complications. These methods are described in the following sections.

      2.4.2 Prevention of spinal cord ischaemia in open repair

      Surgical techniques have been developed over the years to prevent SCI. Techniques like the Crawford inlay, single clamp repair, sequential aortic clamping, and others have been introduced with varying results. As revascularisation of all of the intercostal arteries is not feasible, the identification of important vessels is relevant for success.
      • Estrera A.L.
      • Miller 3rd, C.C.
      • Huynh T.T.
      • Porat E.
      • Safi H.J.
      Neurologic outcome after thoracic and thoracoabdominal aortic aneurysm repair.
      Revascularisation of the artery of Adamkiewicz and the intercostal arteries of T11/T12 has been reported, although not widely implemented. Pre-operative angiography has been used to identify the most important vessels, but as angiography itself can induce SCI (resulting from embolisation), other modalities such as magnetic resonance angiography (MRA) and CTA have become more popular.
      • Nijenhuis R.J.
      • Jacobs M.J.
      • Schurink G.W.
      • Kessels A.G.H.
      • Van Engelshoven J.M.A.
      • Backes W.H.
      Comparison of magnetic resonance with computed tomography angiography for preoperative localization of the Adamkiewicz artery in thoracoabdominal aortic aneurysm patients.
      Cerebrospinal fluid (CSF) pressure increases with aortic cross-clamping, and will eventually exceed the venous pressure, compromising venous outflow, leading to spinal cord malperfusion and secondary SCI. Randomized controlled trials and meta-analyses have shown that CSF drainage has a role in the prevention of paraplegia and paraparesis, with a risk reduction up to 75% (OR 0.48, 95% CI 0.25–0.92), but additional studies are recommended.
      • Coselli J.S.
      • LeMaire S.A.
      • Koksoy C.
      • Schmittling Z.C.
      • Curling P.E.
      Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial.
      • Khan S.N.
      • Stansby G.
      Cerebrospinal fluid drainage for thoracic and thoracoabdominal aortic aneurysm surgery.
      During aortic cross-clamping, prophylactic CSF drainage aims to maintain CSF pressure at 10 mm Hg intra-operatively and for 48–72 hours after completion of the aneurysm repair. In fact, delayed and late onset of neurological deficit are also described and potentially worsened by haemodynamic instability.
      • Estrera A.L.
      • Miller 3rd, C.C.
      • Huynh T.T.
      • Porat E.
      • Safi H.J.
      Neurologic outcome after thoracic and thoracoabdominal aortic aneurysm repair.
      Recent studies have shown that CSF drainage combined with intrathecal papaverine solution can be beneficial.
      • Lima B.
      • Nowicki E.R.
      • Blackstone E.H.
      • Williams S.J.
      • Roselli E.E.
      • Sabik J.F.
      • et al.
      Spinal cord protective strategies during descending and thoracoabdominal aortic aneurysm repair in the modern era: the role of intrathecal papaverine.
      Potential complications related to CSF drainage occur in fewer than 5% of cases, and include meningitis, epidural haematoma, subdural haematoma, and CSF leakage syndrome.
      • Khan S.N.
      • Stansby G.
      Cerebrospinal fluid drainage for thoracic and thoracoabdominal aortic aneurysm surgery.
      • Lima B.
      • Nowicki E.R.
      • Blackstone E.H.
      • Williams S.J.
      • Roselli E.E.
      • Sabik J.F.
      • et al.
      Spinal cord protective strategies during descending and thoracoabdominal aortic aneurysm repair in the modern era: the role of intrathecal papaverine.
      • Wynn M.M.
      • Mell M.W.
      • Tefera G.
      • Hoch J.R.
      • Acher C.W.
      Complications of spinal fluid drainage in thoracoabdominal aortic aneurysm repair: a report of 486 patients treated from 1987 to 2008.
      The use of left heart bypass (LHB) prevents heart failure and maintains distal aortic perfusion and CSF pressures as close to baseline as possible, and thereby reduces the risk of post-operative paraplegia and paraparesis in patients undergoing repair of type I and type II thoraco-abdominal aortic aneurysm (TAAA)
      • Coselli J.S.
      The use of left heart bypass in the repair of thoracoabdominal aortic aneurysms: current techniques and results.
      (see also Section 3.4.). Extra-corporeal circulation allows sequential aortic clamping, maintaining retrograde perfusion of all of the vital organs and the spinal cord. These techniques have been shown to be beneficial in patients with TAAA, reducing the incidence of SCI by perfusion of the distal aorta from 11.2% to 4.5%.
      • Coselli J.S.
      The use of left heart bypass in the repair of thoracoabdominal aortic aneurysms: current techniques and results.
      Another important advantage of circulatory bypass is that it permits the induction of systemic hypothermia for additional neurological protection. Distal perfusion is often combined with CSF drainage or hypothermia, and has proven beneficial in reducing the incidence of SCI.
      • Estrera A.L.
      • Miller 3rd, C.C.
      • Huynh T.T.
      • Porat E.
      • Safi H.J.
      Neurologic outcome after thoracic and thoracoabdominal aortic aneurysm repair.
      • Lima B.
      • Nowicki E.R.
      • Blackstone E.H.
      • Williams S.J.
      • Roselli E.E.
      • Sabik J.F.
      • et al.
      Spinal cord protective strategies during descending and thoracoabdominal aortic aneurysm repair in the modern era: the role of intrathecal papaverine.
      • Kulik A.
      • Castner C.F.
      • Kouchoukos N.T.
      Outcomes after thoracoabdominal aortic aneurysm repair with hypothermic circulatory arrest.
      Induced systemic hypothermia by cardiopulmonary bypass and intermittent cardiac arrest might be an effective method to reduce the risk of SCI.
      • Kulik A.
      • Castner C.F.
      • Kouchoukos N.T.
      Outcomes after thoracoabdominal aortic aneurysm repair with hypothermic circulatory arrest.
      Moderate systemic hypothermia to temperatures of 32 °C improves outcome without significant risks for the patient.
      • Kulik A.
      • Castner C.F.
      • Kouchoukos N.T.
      Outcomes after thoracoabdominal aortic aneurysm repair with hypothermic circulatory arrest.
      • Svensson L.G.
      • Khitin L.
      • Nadolny E.M.
      • Kimmel W.A.
      Systemic temperature and paralysis after thoracoabdominal and descending aortic operations.
      However, the systemic anticoagulation required in these patients increases the risk of bleeding, and, in such conditions, CSF drainage and intrathecal manoeuvres can be dangerous.
      • Wynn M.M.
      • Mell M.W.
      • Tefera G.
      • Hoch J.R.
      • Acher C.W.
      Complications of spinal fluid drainage in thoracoabdominal aortic aneurysm repair: a report of 486 patients treated from 1987 to 2008.
      • Kulik A.
      • Castner C.F.
      • Kouchoukos N.T.
      Outcomes after thoracoabdominal aortic aneurysm repair with hypothermic circulatory arrest.
      • Svensson L.G.
      • Khitin L.
      • Nadolny E.M.
      • Kimmel W.A.
      Systemic temperature and paralysis after thoracoabdominal and descending aortic operations.
      During the post-operative period, the maintenance of mean arterial pressure (MAP) between 80 and 100 mm Hg has been suggested by high volume centers.
      • Estrera A.L.
      • Miller 3rd, C.C.
      • Huynh T.T.
      • Porat E.
      • Safi H.J.
      Neurologic outcome after thoracic and thoracoabdominal aortic aneurysm repair.
      After a period of ischaemia caused by cross-clamping, reperfusion injury can occur. Biochemical mediators, especially iron-related free radicals, induce cellular damage. Free radical scavengers have been studied in the prevention of ischaemia, which suggests that numerous agents may protect the spinal cord from transient ischaemia.
      • de Haan P.
      • Kalkman C.J.
      • Jacobs M.J.
      Pharmacologic neuroprotection in experimental spinal cord ischaemia: a systematic review.
      Figure thumbnail fx6

      2.4.3 Prevention of spinal cord ischaemia in thoracic endovascular repair

      Figure thumbnail fx7

      2.4.4 Prevention of stroke

      Intra-operative embolism is the main cause of stroke in patients undergoing surgical repair of the DTA, emphasizing the importance of precautions during the operative period.
      Debris from aortic atheroma/thrombus can result in an embolic ischaemic stroke and the release of debris should be prevented during surgery. Reducing manipulation of the DTA to a strict minimum
      • Coselli J.S.
      The use of left heart bypass in the repair of thoracoabdominal aortic aneurysms: current techniques and results.
      and TOE mapping of the sites of cannulation are techniques that can minimize the risk of embolisation.
      • Evangelista A.
      • Flachskampf F.A.
      • Erbel R.
      • Antonini-Canterin F.
      • Vlachopoulos C.
      • Rocchi G.
      • et al.
      Echocardiography in aortic diseases: EAE recommendations for clinical practice.
      When clamping is neither possible nor recommended, deep hypothermic circulatory arrest must be instituted.
      • Svensson L.G.
      • Khitin L.
      • Nadolny E.M.
      • Kimmel W.A.
      Systemic temperature and paralysis after thoracoabdominal and descending aortic operations.
      In patients with atrial fibrillation, the use of arteriovenous femoro-femoral bypass or deep hypothermic arrest and the avoidance of cannulation of the left atrium with possible manipulation of thrombus may be a useful alternative.
      • Coselli J.S.
      The use of left heart bypass in the repair of thoracoabdominal aortic aneurysms: current techniques and results.
      • Svensson L.G.
      • Khitin L.
      • Nadolny E.M.
      • Kimmel W.A.
      Systemic temperature and paralysis after thoracoabdominal and descending aortic operations.
      Figure thumbnail fx8

      3. Specific Thoracic Aortic Disorders

      3.1 Acute thoracic aortic syndrome

      AAS covers a heterogeneous group of patients with similar anatomic and clinical characteristics. This group includes AD, IMH, and PAU.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      Traumatic aortic transection and ruptured DTAA also may be included. The most common symptom is chest pain. Histopathologically, all of these entities involve disruption of the media of the aorta, with bleeding between the layers of the aorta or transmurally in the case of rupture. Each condition can progress to another one of the group and each pathology may coexist in the same patient.

      3.1.1 Acute type B aortic dissection

      3.1.1.1 Definition, risk factors, and clinical presentation

      Acute type B aortic dissection (ATBAD) is the result of a tear in the intimal arterial layer, which allows blood to propagate within the medial layer. This creates a flap, which divides the aorta into a true lumen (TL), and a false lumen (FL). The most common site for the proximal intimal tear in ATBAD is located just distal to the origin of the left subclavian artery. In 90% of cases, ATBAD has a secondary tear that allows blood to re-enter the TL at what is known as the re-entry site.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      • Mackenzie K.S.
      • LeGuillan M.P.
      • Steinmetz O.K.
      • Montreuil B.
      Management trends and early mortality rates for acute type B aortic dissection: a 10 year single-institution experience.
      The Stanford and DeBakey classifications are most commonly used to describe ATBAD (Fig. 1). DeBakey classified AD, based on the origin of the intimal tear and the extent of the dissection. The Stanford classification is based on the involvement of the ascending aorta.
      • Daily P.O.
      • Trueblood H.W.
      • Stinson E.B.
      • Wuerflein R.D.
      • Shumway N.E.
      Management of acute aortic dissections.
      A type A dissection is defined by involvement of the ascending aorta and type B by the absence of ascending aortic involvement. There is no consensus about the classification of arch AD without involvement of the ascending aorta.
      • Hiratzka L.F.
      • Bakris G.L.
      • Beckman J.A.
      • Bersin R.M.
      • Carr V.F.
      • Casey Jr., D.E.
      • et al.
      American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.
      Type B dissections originate distal to the ostium of the left subclavian artery. This classification is comparable with a DeBakey type III (including type IIIb), in which the dissection extends into the abdominal aorta. ATBAD accounts for 30–40% of all dissections.
      • Mackenzie K.S.
      • LeGuillan M.P.
      • Steinmetz O.K.
      • Montreuil B.
      Management trends and early mortality rates for acute type B aortic dissection: a 10 year single-institution experience.
      • Hiratzka L.F.
      • Bakris G.L.
      • Beckman J.A.
      • Bersin R.M.
      • Carr V.F.
      • Casey Jr., D.E.
      • et al.
      American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Tsai T.T.
      • Trimarchi S.
      • Nienaber C.A.
      Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).
      AD is defined as “acute dissection” within 14 days of the onset of symptoms. Thereafter, it is defined as sub-acute between 2 weeks and 3 months and chronic after 3 months (see also Section 3.2).
      Figure thumbnail gr1
      Figure 1Schematic drawings of De Bakey (I, II, IIIa and IIIb) and Stanford (A and B) combined classifications for aortic dissection.
      Any condition that increases intimal shear stress or decreases arterial wall strength is considered to be a risk factor. Systemic hypertension is present in almost 80% of ATBAD patients.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      Increasing age and atherosclerosis are other important risk factors,
      • Collins J.S.
      • Evangelista A.
      • Nienaber C.A.
      • Bossone E.
      • Fang J.
      • Cooper J.V.
      • et al.
      Differences in clinical presentation, management, and outcomes of acute type a aortic dissection in patients with and without previous cardiac surgery.
      as are congenital bicuspid or uni-commissural aortic valves,
      • Bonderman D.
      • Gharehbaghi-Schnell E.
      • Wollenek G.
      • Maurer G.
      • Baumgartner H.
      Mechanisms underlying aortic dilatation in congenital aortic valve malformation.
      cocaine abuse,
      • Eagle K.A.
      • Isselbacher E.M.
      • DeSanctis R.W.
      Cocaine-related aortic dissection in perspective.
      pregnancy,
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      strenuous activities, and severe emotional stress.
      • Hatzaras I.S.
      • Bible J.E.
      • Koullias G.J.
      • Tranquilli M.
      • Singh M.
      • Elefteriades J.A.
      • et al.
      Role of exertion or emotion as inciting events for acute aortic dissection.
      Another important risk factor is a positive family history of thoracic aortic diseases. ATBAD has a prevalence of 13–22% in patients who have a first degree relative with a history of DTAA or AD.
      • Albornoz G.
      • Coady M.A.
      • Roberts M.
      • Davies R.R.
      • Tranquilli M.
      • Rizzo J.A.
      • et al.
      Familial thoracic aortic aneurysms and dissections – incidence, modes of inheritance, and phenotypic patterns.
      The “familial thoracic aorta and dissection syndrome” is related to several identified gene mutations, including fibrillin-1 (FBN1), transforming growth factor-β1 (TGFBR1), transforming growth factor-β2 (TGFRB2), α-actin 2 (ACTA 2), and myosin heavy chain 11 (MYH11), increasing insight into the genetic pathology of the disease.
      • Hasham S.N.
      • Willing M.C.
      • Guo D.C.
      • Muilenburg A.
      • He R.
      • Tran V.T.
      • et al.
      Mapping a locus for familial thoracic aneurysms and dissections (TAAD2) to 3p24–25.
      • Renard M.
      • Callewaert B.
      • Baetens M.
      • Campens L.
      • Macdermot K.
      • Fryns J.P.
      • et al.
      Novel MYH11 and ACTA2 mutations reveal a role for enhanced TGFβ signaling in FTAAD.
      Structural weakness of the aortic wall is associated with multiple connective tissue disorders such as MFS, EDS, and Loeys-Dietz syndrome (LDS) (see also Section 3.7). In patients younger than 40 years of age, who present with aortic dissection, about 50% will have MFS or a related genetic disorder. This group of patients, as well as those with a family history of thoracic aortic diseases, should receive genetic counselling.
      • Albornoz G.
      • Coady M.A.
      • Roberts M.
      • Davies R.R.
      • Tranquilli M.
      • Rizzo J.A.
      • et al.
      Familial thoracic aortic aneurysms and dissections – incidence, modes of inheritance, and phenotypic patterns.
      The aortic diameter is not closely related to ATBAD, although an increased risk of ATBAD has been observed in patients with a dilated DTA.
      • Elefteriades J.A.
      Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks.
      The mean diameter in ATBAD has been reported as 41 mm.
      • Trimarchi S.
      • Jonker F.H.
      • Froehlich J.B.
      • Upchurch G.R.
      • Moll F.L.
      • Muhs B.E.
      • et al.
      Descending aortic diameter of 5.5 cm or greater is not an accurate predictor of acute type B aortic dissection.
      Indeed, many cases of ATBAD can occur in patients with normal diameter aortas.
      • Trimarchi S.
      • Jonker F.H.
      • Froehlich J.B.
      • Upchurch G.R.
      • Moll F.L.
      • Muhs B.E.
      • et al.
      Descending aortic diameter of 5.5 cm or greater is not an accurate predictor of acute type B aortic dissection.
      • Trimarchi S.
      • Jonker F.H.
      • Froehlich J.B.
      • Upchurch G.R.
      • Moll F.L.
      • Muhs B.E.
      • et al.
      International Registry of Acute Aortic Dissection (IRAD) Investigators
      Acute type B aortic dissection in the absence of aortic dilatation.
      The clinical presentation of patients presenting with ATBAD can be diverse and may mimic a wide range of other disorders. The classical presentation is acute onset excruciating chest or interscapular pain, which is present in about 80% of ATBAD patients.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      The main clinical signs and symptoms are reported in Table 4. The IRAD showed that hypotension (OR 23.8, 95% CI 10.31–54.94, p < .0001), absence of chest/back pain (OR 3.5, 95% CI 1.3–9.52, p = 0.01), and branch vessel involvement (OR 2.9, 95% CI 1.21–6.99, p = 0.02) are predictors of in hospital mortality.
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      Although most patients present with these symptoms, some may have a non-specific presentation or even no symptoms, which can delay the diagnosis.
      • Nallamothu B.K.
      • Mehta R.H.
      • Saint S.
      • Llovet A.
      • Bossone E.
      • Cooper J.V.
      • et al.
      Syncope in acute aortic dissection: diagnostic, prognostic, and clinical implications.
      • Park S.W.
      • Hutchison S.
      • Mehta R.H.
      • Isselbacher E.M.
      • Cooper J.V.
      • Fang J.
      • et al.
      Association of painless acute aortic dissection with increased mortality.
      For that reason, physicians must be familiar with an atypical presentation and a low threshold for diagnostic imaging should be maintained.
      Table 4Main clinical presentations of ATBAD.
      Signs and symptomsIncidence, %
      Acute excruciating chest or interscapular pain80
      Chest pain79
      Back pain64
      Abdominal pain43
      Syncope4
      Pulse deficits9
      Hypotension/shock4
      Visceral ischaemia7
      Renal ischaemia15
      Limb ischaemia9
      Recurrent pain, refractory pain, or refractory hypertension18
      Spinal cord ischaemia3
      The most severe ATBAD complications include aortic rupture and occlusion of arterial branches, with consequent loss of arterial perfusion. Aortic rupture is associated with a high mortality, irrespective of the type of treatment,
      • Mackenzie K.S.
      • LeGuillan M.P.
      • Steinmetz O.K.
      • Montreuil B.
      Management trends and early mortality rates for acute type B aortic dissection: a 10 year single-institution experience.
      • Estrera A.L.
      • Miller C.C.
      • Goodrick J.
      • Porat E.E.
      • Achouh P.E.
      • Dhareshwar J.
      • et al.
      Update on outcomes of acute type B aortic dissection.
      • Trimarchi S.
      • Nienaber C.A.
      • Rampoldi V.
      • Myrmel T.
      • Suzuki T.
      • Bossone E.
      • et al.
      Role and results of surgery in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
      • Sakakura K.
      • Kubo N.
      • Ako J.
      • Ikeda N.
      • Funayama H.
      • Hirahara T.
      • et al.
      Determinants of in hospital death and rupture in patients with a Stanford B aortic dissection.
      • Williams D.M.
      • Lee D.Y.
      • Hamilton B.H.
      • Marx M.V.
      • Narasimham D.L.
      • Kazanjian S.N.
      • et al.
      The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise.
      whereas patients with impending rupture who have no haemodynamic complications have better outcomes.
      • Tsai T.T.
      • Trimarchi S.
      • Nienaber C.A.
      Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).
      • Estrera A.L.
      • Miller C.C.
      • Goodrick J.
      • Porat E.E.
      • Achouh P.E.
      • Dhareshwar J.
      • et al.
      Update on outcomes of acute type B aortic dissection.
      Renal and/or visceral ischaemia may be difficult to detect. Renal ischaemia can increase creatinine levels and potentially induce refractory hypertension. Treatment of renal ischaemia is important to prevent permanent renal insufficiency and refractory hypertension which is likely to result in a more rapid expansion of the affected aorta. Visceral ischaemia was the third most common cause of death (after aortic rupture and tamponade) in AD patients in the IRAD study,
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      and can result from malperfusion or systemic hypotension.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Tsai T.T.
      • Trimarchi S.
      • Nienaber C.A.
      Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      Serum lactate levels are elevated when the ischaemic injury progresses or becomes irreversible; therefore early diagnosis is essential. ATBAD patients who present with or who develop recurrent abdominal pain should undergo repeat cross sectional imaging. In this situation, there should be a low threshold for undertaking laparoscopic inspection of the peritoneal cavity.
      • Tshomba Y.
      • Coppi G.
      • Marone E.M.
      • Bertoglio L.
      • Kahlberg A.
      • Carlucci M.
      • et al.
      Diagnostic laparoscopy for early detection of acute mesenteric ischaemia in patients with aortic dissection.
      Gastrointestinal haemorrhage is a rare complication and every patient presenting with bleeding and abdominal pain should be suspected of having mesenteric ischaemia.
      • Born C.
      • Forster A.
      • Rock C.
      • Pfeifer K.J.
      • Rieger J.
      • Reiser M.A.
      • et al.
      A case of an upper gastrointestinal bleeding due to a ruptured dissection of a right aortic arch.
      Acute limb ischaemia may present with paralysis of one or both lower limbs. Because of the dynamic nature of the dissection membrane, the presence of palpable peripheral pulses may be misleading.
      • Vedantham S.
      • Picus D.
      • Sanchez L.A.
      • Braverman A.
      • Moon M.R.
      • Sundt III, T.
      • et al.
      Percutaneous management of ischaemic complications in patients with type-B aortic dissection.
      Paraplegia/paraparesis represents a catastrophic, but rare, complication of ATBAD secondary to SCI. Other complications include refractory pain and refractory hypertension, which are both indirect signs of impending rupture and are associated with increased in hospital mortality.
      • Trimarchi S.
      • Eagle K.A.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Jonker F.H.
      • Suzuki T.
      • et al.
      Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
      Complicated ATBAD is defined as the presence of rapid aortic expansion, aortic rupture and/or hypotension/shock, visceral, renal, or limb ischaemia, paraplegia/paraparesis, peri-aortic haematoma, recurrent or refractory pain, and refractory hypertension despite adequate medical therapy.
      Figure thumbnail fx9
      Complicated ATBAD patients with severe comorbidities (e.g. ischaemic heart disease, chronic pulmonary disease, or malignancy) may not benefit from invasive management and should be evaluated individually.
      • Elefteriades J.A.
      Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks.
      • Estrera A.L.
      • Miller C.C.
      • Goodrick J.
      • Porat E.E.
      • Achouh P.E.
      • Dhareshwar J.
      • et al.
      Update on outcomes of acute type B aortic dissection.
      • Trimarchi S.
      • Tolenaar J.L.
      • Tsai T.T.
      • Froehlich J.
      • Pegorer M.
      • Upchurch G.R.
      • et al.
      Influence of clinical presentation on the outcome of acute B aortic dissection: evidences from IRAD.
      ATBAD may also present without complications in almost 50% of cases. This cohort is defined as uncomplicated ATBAD.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Trimarchi S.
      • Tolenaar J.L.
      • Tsai T.T.
      • Froehlich J.
      • Pegorer M.
      • Upchurch G.R.
      • et al.
      Influence of clinical presentation on the outcome of acute B aortic dissection: evidences from IRAD.
      Despite the absence of complications at the time of presentation, these patients have an in hospital mortality of 3–10%.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Trimarchi S.
      • Tolenaar J.L.
      • Tsai T.T.
      • Froehlich J.
      • Pegorer M.
      • Upchurch G.R.
      • et al.
      Influence of clinical presentation on the outcome of acute B aortic dissection: evidences from IRAD.
      In the presence of complications (such as visceral, renal or limb ischaemia, and/or aortic rupture), mortality rises to 20% by day 2 and 25% by day 30. Like type A dissection, advanced age, rupture, shock, and malperfusion are important predictors of increased early mortality.
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      • Suzuki T.
      • Isselbacher E.M.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Eagle K.A.
      • Tsai T.T.
      • et al.
      Type-selective benefits of medications in treatment of acute aortic dissection (from the International Registry of Acute Aortic Dissection [IRAD]).

      3.1.1.2 Management

      The aims of treating ATBAD are to maintain or restore perfusion of the vital organs and to prevent both progression of the dissection and aortic rupture. Therefore, it is important to make a risk assessment at an early stage to determine the merits of medical, endovascular, or surgical intervention.

      3.1.1.2.1 Medical management

      Medical therapy with antihypertensive agents is widely accepted to be the first line treatment in uncomplicated ATBAD patients.
      • Mackenzie K.S.
      • LeGuillan M.P.
      • Steinmetz O.K.
      • Montreuil B.
      Management trends and early mortality rates for acute type B aortic dissection: a 10 year single-institution experience.
      • Hiratzka L.F.
      • Bakris G.L.
      • Beckman J.A.
      • Bersin R.M.
      • Carr V.F.
      • Casey Jr., D.E.
      • et al.
      American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Tsai T.T.
      • Trimarchi S.
      • Nienaber C.A.
      Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      • Elefteriades J.A.
      Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks.
      • Estrera A.L.
      • Miller C.C.
      • Goodrick J.
      • Porat E.E.
      • Achouh P.E.
      • Dhareshwar J.
      • et al.
      Update on outcomes of acute type B aortic dissection.
      • Trimarchi S.
      • Eagle K.A.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Jonker F.H.
      • Suzuki T.
      • et al.
      Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
      • Trimarchi S.
      • Tolenaar J.L.
      • Tsai T.T.
      • Froehlich J.
      • Pegorer M.
      • Upchurch G.R.
      • et al.
      Influence of clinical presentation on the outcome of acute B aortic dissection: evidences from IRAD.
      • Suzuki T.
      • Isselbacher E.M.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Eagle K.A.
      • Tsai T.T.
      • et al.
      Type-selective benefits of medications in treatment of acute aortic dissection (from the International Registry of Acute Aortic Dissection [IRAD]).
      Medical management is based on blood pressure reduction to limit aortic wall stress and to reduce the force of left ventricular ejection.
      • Sakakura K.
      • Kubo N.
      • Ako J.
      • Ikeda N.
      • Funayama H.
      • Hirahara T.
      • et al.
      Determinants of in hospital death and rupture in patients with a Stanford B aortic dissection.
      The goal is to reduce systolic blood pressure between 100 and 120 mm Hg and, when attainable, the heart rate below 60 beats/min.
      • Hiratzka L.F.
      • Bakris G.L.
      • Beckman J.A.
      • Bersin R.M.
      • Carr V.F.
      • Casey Jr., D.E.
      • et al.
      American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American College of Radiology; American Stroke Association; Society of Cardiovascular Anesthesiologists; Society for Cardiovascular Angiography and Interventions; Society of Interventional Radiology; Society of Thoracic Surgeons; Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.
      In ATBAD, initial medical therapy consists of β-blockers.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Suzuki T.
      • Isselbacher E.M.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Eagle K.A.
      • Tsai T.T.
      • et al.
      Type-selective benefits of medications in treatment of acute aortic dissection (from the International Registry of Acute Aortic Dissection [IRAD]).
      • Genoni M.
      • Paul M.
      • Jenni R.
      • Graves K.
      • Seifert B.
      • Turina M.
      Chronic beta blocker therapy improves outcome and reduces treatment costs in chronic type B aortic dissection.
      In patients who do not respond to β-blockers or who do not tolerate the drug, calcium channel antagonists and/or renin-angiotensin inhibitors can be used as alternatives.
      • Genoni M.
      • Paul M.
      • Jenni R.
      • Graves K.
      • Seifert B.
      • Turina M.
      Chronic beta blocker therapy improves outcome and reduces treatment costs in chronic type B aortic dissection.
      • Lacro R.V.
      • Dietz H.C.
      • Sleeper L.A.
      • Yetman A.T.
      • Bradley T.J.
      • Colan S.D.
      • et al.
      Pediatric Heart Network Investigators
      • Forteza A.
      • Evangelista A.
      • Sánchez V.
      • Teixidó-Turà G.
      • Sanz P.
      • Gutiérrez L.
      • et al.
      Efficacy of losartan vs. atenolol for the prevention of aortic dilation in Marfan syndrome: a randomized clinical trial.
      • Neal B.
      • MacMahon S.
      • Chapman N.
      Effects of ACE inhibitors, calcium antagonists, and other blood-pressure-lowering drugs: results of prospectively designed overviews of randomised trials. Blood Pressure Lowering Treatment Trialists' Collaboration.
      β-blockers and calcium channel blockers have been associated with improved long-term survival in ATBAD patients.
      • Neal B.
      • MacMahon S.
      • Chapman N.
      Effects of ACE inhibitors, calcium antagonists, and other blood-pressure-lowering drugs: results of prospectively designed overviews of randomised trials. Blood Pressure Lowering Treatment Trialists' Collaboration.
      Recent data have shown that angiotensin II type 1 receptor blockers have similar positive effects to atenolol in terms of growth reduction of the aortic root in children and young adults with MFS.
      • Lacro R.V.
      • Dietz H.C.
      • Sleeper L.A.
      • Yetman A.T.
      • Bradley T.J.
      • Colan S.D.
      • et al.
      Pediatric Heart Network Investigators
      • Forteza A.
      • Evangelista A.
      • Sánchez V.
      • Teixidó-Turà G.
      • Sanz P.
      • Gutiérrez L.
      • et al.
      Efficacy of losartan vs. atenolol for the prevention of aortic dilation in Marfan syndrome: a randomized clinical trial.
      Other alternative therapies include sodium nitroprusside, A1-adrenergic, and non-selective β blockers.
      • Eggebrecht H.
      • Schmermund A.
      • von Birgelen C.
      • Naber C.K.
      • Bartel T.
      • Wenzel R.R.
      Resistant hypertension in patients with chronic aortic dissection.
      Pain relief is also an important component of optimal medical therapy, as persisting pain may indicate progression of the dissection or impending rupture, requiring additional therapy.
      • Trimarchi S.
      • Eagle K.A.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Jonker F.H.
      • Suzuki T.
      • et al.
      Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
      Figure thumbnail fx10

      3.1.1.2.2 Endovascular repair

      TEVAR has developed as the first line therapeutic option in patients with complicated ATBAD.
      • Tsai T.T.
      • Fattori R.
      • Trimarchi S.
      • Isselbacher E.
      • Myrmel T.
      • Evangelista A.
      • et al.
      Long-term survival in patients presenting with type B acute aortic dissection: insights from the International Registry of Acute Aortic Dissection.
      • Cambria R.P.
      • Clouse W.D.
      • Davison J.K.
      • Dunn P.F.
      • Corey M.
      • Dorer D.
      • et al.
      A multicenter clinical trial of endovascular stent graft repair of acute catastrophes of the descending thoracic aorta.
      • Zeeshan A.
      • Woo E.Y.
      • Bavaria J.E.
      • Fairman R.M.
      • Desai N.D.
      • Pochettino A.
      • et al.
      Thoracic endovascular aortic repair for acute complicated type B aortic dissection: superiority relative to conventional OS and medical therapy.
      • Svensson L.G.
      • Kouchoukos N.T.
      • Miller D.C.
      • Bavaria J.E.
      • Coselli J.S.
      • Curi M.A.
      • et al.
      Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent grafts.
      • Umana J.P.
      • Lai D.T.
      • Mitchell R.S.
      • Moore K.A.
      • Rodriguez F.
      • Robbins R.C.
      • et al.
      What is the best treatment for patients with acute type B aortic dissections – medical, surgical, or endovascular stent grafting?.
      • Nienaber C.A.
      • Fattori R.
      • Lund G.
      • Dieckmann C.
      • Wolf W.
      • von Kodolitsch Y.
      • et al.
      Nonsurgical reconstruction of thoracic aortic dissection by stent graft placement.
      • Sayer D.
      • Bratby M.
      • Brooks M.
      • Loftus I.
      • Morgan R.
      • Thompson M.
      Aortic morphology following endovascular repair of acute and chronic type B aortic dissection: implications for management.
      • Verhoye J.P.
      • Miller D.C.
      • Sze D.
      • Dake M.D.
      • Mitchell R.S.
      Complicated acute type B aortic dissection: midterm results of emergency endovascular stent grafting.
      • Steuer J.
      • Eriksson M.O.
      • Nyman R.
      • Bjorck M.
      • Wanhainen A.
      Early and long-term outcome after thoracic endovascular aortic repair (TEVAR) for acute complicated type B aortic dissection.
      • Fattori R.
      • Tsai T.T.
      • Myrmel T.
      • Evangelista A.
      • Cooper J.V.
      • Trimarchi S.
      • et al.
      Complicated acute type B dissection: is surgery still the best option?: a report from the International Registry of Acute Aortic Dissection.
      • Dake M.D.
      • Kato N.
      • Mitchell R.S.
      • Semba C.P.
      • Razavi M.K.
      • Shimono T.
      Endovascular stent graft placement for the treatment of acute aortic dissection.
      The aim of endovascular repair for treating impending aortic rupture or malperfusion is to cover the primary entry tear and to reduce blood pressure within the FL.
      • Nienaber C.A.
      • Fattori R.
      • Lund G.
      • Dieckmann C.
      • Wolf W.
      • von Kodolitsch Y.
      • et al.
      Nonsurgical reconstruction of thoracic aortic dissection by stent graft placement.
      • Dake M.D.
      • Kato N.
      • Mitchell R.S.
      • Semba C.P.
      • Razavi M.K.
      • Shimono T.
      Endovascular stent graft placement for the treatment of acute aortic dissection.
      Reduction in FL perfusion can prevent extension of the dissection, which may lead to FL thrombosis, aortic remodelling, and aortic wall stabilisation.
      Although there are no randomized controlled trials, there is increasing evidence that in complicated ATBAD, emergency and urgent interventions have been beneficial.
      • Hagan P.G.
      • Nienaber C.A.
      • Isselbacher E.M.
      • Bruckman D.
      • Karavite D.J.
      • Russman P.L.
      • et al.
      The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
      • Suzuki T.
      • Mehta R.H.
      • Ince H.
      • Nagai R.
      • Sakomura Y.
      • Weber F.
      • et al.
      Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
      • Trimarchi S.
      • Eagle K.A.
      • Nienaber C.A.
      • Pyeritz R.E.
      • Jonker F.H.
      • Suzuki T.
      • et al.
      Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
      • Cambria R.P.
      • Clouse W.D.
      • Davison J.K.
      • Dunn P.F.
      • Corey M.
      • Dorer D.
      • et al.
      A multicenter clinical trial of endovascular stent graft repair of acute catastrophes of the descending thoracic aorta.
      • Zeeshan A.
      • Woo E.Y.
      • Bavaria J.E.
      • Fairman R.M.
      • Desai N.D.
      • Pochettino A.
      • et al.
      Thoracic endovascular aortic repair for acute complicated type B aortic dissection: superiority relative to conventional OS and medical therapy.
      In these settings, TEVAR has shown a substantial advantage over OR in terms of early mortality.
      • Zeeshan A.
      • Woo E.Y.
      • Bavaria J.E.
      • Fairman R.M.
      • Desai N.D.
      • Pochettino A.
      • et al.
      Thoracic endovascular aortic repair for acute complicated type B aortic dissection: superiority relative to conventional OS and medical therapy.
      • Svensson L.G.
      • Kouchoukos N.T.
      • Miller D.C.
      • Bavaria J.E.
      • Coselli J.S.
      • Curi M.A.
      • et al.
      Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent grafts.
      • Umana J.P.
      • Lai D.T.
      • Mitchell R.S.
      • Moore K.A.
      • Rodriguez F.
      • Robbins R.C.
      • et al.
      What is the best treatment for patients with acute type B aortic dissections – medical, surgical, or endovascular stent grafting?.
      • Verhoye J.P.
      • Miller D.C.
      • Sze D.
      • Dake M.D.
      • Mitchell R.S.
      Complicated acute type B aortic dissection: midterm results of emergency endovascular stent grafting.
      • Steuer J.
      • Eriksson M.O.
      • Nyman R.
      • Bjorck M.
      • Wanhainen A.
      Early and long-term outcome after thoracic endovascular aortic repair (TEVAR) for acute complicated type B aortic dissection.
      • Fattori R.
      • Tsai T.T.
      • Myrmel T.
      • Evangelista A.
      • Cooper J.V.
      • Trimarchi S.
      • et al.
      Complicated acute type B dissection: is surgery still the best option?: a report from the International Registry of Acute Aortic Dissection.
      Currently, there are three meta-analyses available that report short- and mid-term results in complicated ATBAD patients treated with TEVAR. Technical success ranged from 95% to 99%, hospital mortality ranged from 2.6% to 9.8%, and neurological complications ranged from 0.6% to 3.1%.
      • Eggebrecht H.
      • Nienaber C.A.
      • Neuhauser M.
      • Baumgart D.
      • Kische S.
      • Schmermund A.
      • et al.
      Endovascular stent graft placement in aortic dissection: a meta-analysis.
      • Parker J.D.
      • Golledge J.
      Outcome of endovascular treatment of acute type B aortic dissection.
      • Xiong J.
      • Jiang B.
      • Guo W.
      • Wang S.M.
      • Tong X.Y.
      Endovascular stent graft placement in patients with type B aortic dissection: a meta-analysis in China.
      A prospective, multicentre European clinical registry showed a 30 day mortality of 8%, a stroke rate of 8%, and a SCI rate of 2% in 50 ATBAD patients.
      • Heijmen R.
      • Fattori R.
      • Thompson M.
      • Eggebrecht H.
      • Degriecke I.
      • Nienaber C.
      • et al.
      Virtue Registry Investigators
      The VIRTUE Registry of type B thoracic dissections – study design and early results.
      The initial results of a single arm multicentre study for endovascular repair of complicated AD, using a composite device design (PETTICOAT technique), which includes an uncovered infra-diaphragmatic aortic stent in addition to a standard TEVAR, showed a 1 year mortality of 10%. Stroke, transient ischaemic attack, or progression of dissection occurred in 7.5%, 2.5%, and 5% of patients, respectively.
      • Lombardi J.V.
      • Cambria R.P.
      • Nienaber C.A.
      • Chiesa R.
      • Toebken O.
      • Lee A.
      • et al.
      Prospective multicenter clinical trial (STABLE) on the endovascular treatment of complicated type B aortic dissection using a composite device design.
      Another study confirmed these findings with an in hospital mortality of 4%, 40%, and 33% in TEVAR, OR, and medically treated patients, respectively.
      • Zeeshan A.
      • Woo E.Y.
      • Bavaria J.E.
      • Fairman R.M.
      • Desai N.D.
      • Pochettino A.
      • et al.
      Thoracic endovascular aortic repair for acute complicated type B aortic dissection: superiority relative to conventional OS and medical therapy.
      However, there is no evidence that extended coverage of the DTA is needed to restore distal perfusion.
      Among complicated ATBAD patients, those presenting with visceral malperfusion experience the poorest outcomes. Although visceral vessel patency following TEVAR is maintained in up to 97% of patients, the 30 day mortality is high (17–34%), as are aortic related complications during the chronic stage.
      • Trimarchi S.
      • Jonker F.H.
      • Froehlich J.B.
      • Upchurch G.R.
      • Moll F.L.
      • Muhs B.E.
      • et al.
      International Registry of Acute Aortic Dissection (IRAD) Investigators
      Acute type B aortic dissection in the absence of aortic dilatation.
      • Umana J.P.
      • Lai D.T.
      • Mitchell R.S.
      • Moore K.A.
      • Rodriguez F.
      • Robbins R.C.
      • et al.
      What is the best treatment for patients with acute type B aortic dissections – medical, surgical, or endovascular stent grafting?.
      These patients seem to benefit from aortic balloon fenestration and branch stenting.
      • Barnes D.M.
      • Williams D.M.
      • Dasika N.L.
      • Patel H.J.
      • Weder A.B.
      • Stanley J.C.
      • et al.
      A single center experience treating renal malperfusion after aortic dissection with central aortic fenestration and renal artery stenting.
      • Deeb G.M.
      • Patel H.J.
      • Williams D.M.
      Treatment for malperfusion syndrome in acute type A and B aortic dissection: a long-term analysis.
      • Midulla M.
      • Renaud A.
      • Martinelli T.
      • Koussa M.
      • Mounier-Vehier C.
      • Prat A.
      • et al.
      Endovascular fenestration in aortic dissection with acute malperfusion syndrome: immediate and late follow up.
      A dynamic obstruction can be managed by increasing FL outflow with fenestration of the intimal flap, while a static obstruction or ostial disruption should be treated by stenting of the malperfused branch vessel.
      • Williams D.M.
      • Lee D.Y.
      • Hamilton B.H.
      • Marx M.V.
      • Narasimham D.L.
      • Kazanjian S.N.
      • et al.
      The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise.
      • Barnes D.M.
      • Williams D.M.
      • Dasika N.L.
      • Patel H.J.
      • Weder A.B.
      • Stanley J.C.
      • et al.
      A single center experience treating renal malperfusion after aortic dissection with central aortic fenestration and renal artery stenting.
      • Deeb G.M.
      • Patel H.J.
      • Williams D.M.
      Treatment for malperfusion syndrome in acute type A and B aortic dissection: a long-term analysis.
      • Midulla M.
      • Renaud A.
      • Martinelli T.
      • Koussa M.
      • Mounier-Vehier C.
      • Prat A.
      • et al.
      Endovascular fenestration in aortic dissection with acute malperfusion syndrome: immediate and late follow up.
      TEVAR has also been advocated in the treatment of uncomplicated ATBAD, to prevent long-term aortic dilatation and rupture. IRAD reported reduced mortality at 5 years in ATBAD patients treated by TEVAR, compared with those managed medically.
      • Fattori R.
      • Montgomery D.
      • Lovato L.
      • Kische S.
      • Di Eusanio M.
      • Ince H.
      • et al.
      Survival after endovascular therapy in patients with type B aortic dissection: a report from the International Registry of Acute Aortic Dissection (IRAD).
      ADSORB, the only randomized control trial in patients with uncomplicated ATBAD, was not sufficiently powered for mortality at 1 year follow up. This trial did, however, show higher rates of FL thrombosis in patients randomized to TEVAR, and FL thrombosis is associated with fewer late complications and increased aortic remodelling following repair of ATBAD.
      • Brunkwall J.
      • Kasprzak P.
      • Verhoeven E.
      • Heijmen R.
      • Taylor P.
      the ADSORB Trialists
      Endovascular repair of acute uncomplicated aortic type B dissection promotes aortic remodelling: 1 year results of the ADSORB trial.
      Although TEVAR results in this setting are favourable, endovascular related complications can be devastating and may require revision with OR.
      • Bockler D.
      • Schumacher H.
      • Ganten M.
      • von Tengg-Kobligk H.
      • Schwarzbach M.
      • Fink C.
      • et al.
      Complications after endovascular repair of acute symptomatic and chronic expanding Stanford type B aortic dissections.
      Stroke is reported to occur in 3–10% of patients undergoing TEVAR because of manipulation of catheters in the arch and ascending aorta, and occurs more commonly in patients with severe arch atherosclerosis.
      • Buth J.
      • Harris P.L.
      • Hobo R.
      • van Eps R.
      • Cuypers P.
      • Duijm L.
      • et al.
      Neurologic complications associated with endovascular repair of thoracic aortic pathology: incidence and risk factors. a study from the European Collaborators on Stent/Graft Techniques for Aortic Aneurysm Repair (EUROSTAR) registry.
      Although rare in ATBAD patients, SCI is related to the extent of aortic coverage, history of previous aortic surgery and the presence of hypotension at initial presentation.
      • Schlösser F.J.
      • Verhagen H.J.
      • Lin P.H.
      • Verhoeven E.L.
      • van Herwaarden J.A.
      • Moll F.L.
      • et al.
      TEVAR following prior abdominal aortic aneurysm surgery: increased risk of neurological deficit.
      Arm ischaemia, paraparesis, and paraplegia may occur from LSA or intercostal artery occlusion, which may require revascularisation.
      • Matsumura J.S.
      • Lee W.A.
      • Mitchell R.S.
      • Farber M.A.
      • Murad M.H.
      • Lumsden A.B.
      • et al.
      The Society for Vascular Surgery Practice Guidelines: management of the left subclavian artery with thoracic endovascular aortic repair.
      • Rizvi A.Z.
      • Murad M.H.
      • Fairman R.M.
      • Erwin P.J.
      • Montori V.M.
      The effect of left subclavian artery coverage on morbidity and mortality in patients undergoing endovascular thoracic aortic interventions: a systematic review and meta-analysis.
      (see Section 2.4.4). Other complications (device or procedure related) can include aortic rupture during deployment, angulation, migration, or collapse of the stent graft, false aneurysm formation at the proximal or distal end of the stent graft, graft erosion, or stent frame fracture.
      • Kasirajan K.
      • Dake M.D.
      • Lumsden A.
      • Bavaria J.
      • Makaroun M.S.
      Incidence and outcomes after infolding or collapse of thoracic stent grafts.
      A retrograde type A dissection is associated with devastating outcomes. TEVAR for aortic dissection is particularly prone to retrograde type A aortic dissection. The risk of retrograde type A dissection seems to be increased with the use of proximal balloon dilatation, proximal bare metal stents, and with rigid non-compliant devices.
      • Eggebrecht H.
      • Thompson M.
      • Rousseau H.
      • Czerny M.
      • Lonn L.
      • Mehta R.H.
      • et al.
      Retrograde ascending aortic dissection during or after thoracic aortic stent graft placement: insight from the European registry on endovascular aortic repair complications.
      To facilitate the patient selection process, important anatomical and clinical features that characterize the aortic dissection pathology were recently summarized in a new categorisation scheme (DISSECT), which may be helpful in making the decision of whether or not to intervene.
      • Dake M.D.
      • Thompson M.
      • van Sambeek M.
      • Vermassen F.
      • Morales J.P.
      DEFINE Investigators
      DISSECT: a new mnemonic-based approach to the categorisation of aortic dissection.
      Figure thumbnail fx11

      3.1.1.2.3 Open repair

      The aim of OR in the treatment of ATBAD is to replace the DTA with a graft and excise the intimal tear, to restore peripheral perfusion and treat or prevent aortic rupture. Partial cardiopulmonary bypass has been widely used and hypothermic circulatory arrest has been adopted for cerebral protection in a subset of patients who were managed by OR approaches.
      • Coselli J.S.
      • LeMaire S.A.
      • de Figueiredo L.P.
      • Kirby R.P.
      Paraplegia after thoracoabdominal aortic aneurysm repair: is dissection a risk factor?.
      • Miller D.C.
      Surgical management of acute aortic dissection: new data.
      • Svensson L.G.
      • Crawford E.S.
      • Hess K.R.
      • Coselli J.S.
      • Safi H.J.
      Dissection of the aorta and dissecting aortic aneurysms. Improving early and long-term surgical results.
      There are no randomized controlled trials available to compare the different OR techniques and the level of evidence supporting various techniques is low. In patients presenting with complications such as imminent rupture, rapid expansion, or malperfusion syndromes, classic OR carries a significant risk of morbidity, including irreversible spinal injury and post-operative death.
      • Coselli J.S.
      • LeMaire S.A.
      • de Figueiredo L.P.
      • Kirby R.P.
      Paraplegia after thoracoabdominal aortic aneurysm repair: is dissection a risk factor?.
      • Miller D.C.
      Surgical management of acute aortic dissection: new data.
      • Svensson L.G.
      • Crawford E.S.
      • Hess K.R.
      • Coselli J.S.
      • Safi H.J.
      Dissection of the aorta and dissecting aortic aneurysms. Improving early and long-term surgical results.
      Surgical aortic fenestration or extra-anatomical bypass has been used for treating complicated ATBAD, but with the introduction of minimally invasive techniques, this procedure is only used as an alternative when endovascular repair fails or is contraindicated.
      • Trimarchi S.
      • Jonker F.H.
      • Muhs B.E.
      • Grassi V.
      • Righini P.
      • Upchurch G.R.
      • et al.
      Long-term outcomes of surgical aortic fenestration for complicated acute type B aortic dissections.
      Figure thumbnail fx12

      3.1.2 Intramural haematoma and penetrating aortic ulcer

      3.1.2.1 Definition and natural history

      Intramural haematoma (IMH) is defined as the presence of blood within the aortic wall without intimal disruption or an identifiable entry point on imaging. IMH may be a precursor to both classic AD and penetrating aortic ulcer (PAU). It is distinguished from AD by the absence of an intimal flap and from PAU by the absence of any connection with the aortic lumen.
      • Nienaber C.A.
      • Eagle K.A.
      Aortic dissection: new frontiers in diagnosis and management: part I: from etiology to diagnostic strategies.
      Most cases of IMH (50–85%) are located in the DTA. At least 5–15% of patients admitted with IMH have an AD. The exact pathophysiology underlying IMH remains controversial. One theory suggests that IMH is a consequence of rupture of vasa vasorum in the medial layer of the aortic wall, which then causes a secondary tear into the aortic lumen. This process is typically associated with hypertension and is initiated by aortic wall infarction. Another theory suggests that IMH results following an intimal entry tear, allowing blood from the aortic lumen to enter the aortic wall. The blood then thromboses within the intimal layer so that no entry tears can be detected.
      • Nienaber C.A.
      • Eagle K.A.
      Aortic dissection: new frontiers in diagnosis and management: part I: from etiology to diagnostic strategies.
      • Nienaber C.A.
      • Sievers H.H.
      Intramural hematoma in acute aortic syndrome; more than one variant of dissection?.
      Modern imaging suggests that IMH, PAU, and AD can develop from each other and, therefore, they are likely to be variants of the same pathological process. IMH evolves longitudinally between the medial layers and may progress, regress, or remain unchanged.
      • Nienaber C.A.
      • Eagle K.A.
      Aortic dissection: new frontiers in diagnosis and management: part I: from etiology to diagnostic strategies.
      IMH has identical clinical manifestations and treatment principles to those of AD. The classification of IMH follows that of the Stanford classification of AD. Type A IMH involves the ascending aorta. Type B IMH is localised in the aortic arch and in the DTA.
      • Nienaber C.A.
      • Sievers H.H.
      Intramural hematoma in acute aortic syndrome; more than one variant of dissection?.
      Cross sectional imaging techniques (CTA, MRA) are used to differentiate between IMH, PAU, and AD. The characteristic finding of IMH on axial imaging is a thickening of the aortic wall greater than 5 mm in an eccentric or concentric pattern. Mural thrombus has an irregular luminal surface, narrows the lumen, and does not extend longitudinally as much as IMH. Discrimination between IMH and acute dissection with a thrombosed FL may be difficult. Unenhanced CT acquisition is crucial for the diagnosis of IMH. A high attenuation crescentic thickening of the aortic wall, extending in a longitudinal, non-spiral fashion, is pathognomonic. The aortic lumen is preserved and the luminal wall is curvilinear and smooth, as opposed to a rough, irregular border seen with aortic atherosclerosis and PAU. IMH is generally a more localised process than classic AD, which typically propagates along the entire aorta to the iliac arteries.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      The natural history of type B IMH is similar to that of ATBAD.
      • Evangelista A.
      • Mukherjee D.
      • Mehta R.H.
      • O'Gara P.T.
      • Fattori R.
      • Cooper J.V.
      • et al.
      Acute intramural hematoma of the aorta: a mystery in evolution.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      Conservative medical treatment is used for isolated uncomplicated type B IMH.
      • Ince H.
      • Nienaber C.A.
      Diagnosis and management of patients with aortic dissection.
      Treatment with β-blockers has a survival rate of 95%, compared with 67% for those treated without β-blockade.
      • Ince H.
      • Nienaber C.A.
      Diagnosis and management of patients with aortic dissection.
      IMH, if associated with PAU, has a significantly worse prognosis with a higher risk of expansion and rupture.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      Regression of acute IMH occurs in one third, progression in 20%, and up to 40% evolve into AD.
      • Evangelista A.
      • Mukherjee D.
      • Mehta R.H.
      • O'Gara P.T.
      • Fattori R.
      • Cooper J.V.
      • et al.
      Acute intramural hematoma of the aorta: a mystery in evolution.
      Indications for treatment in type B IMH are refractory chest pain, evidence of increasing size of the expanding haematoma, aortic rupture, and a progressive pleural effusion.
      • Evangelista A.
      • Mukherjee D.
      • Mehta R.H.
      • O'Gara P.T.
      • Fattori R.
      • Cooper J.V.
      • et al.
      Acute intramural hematoma of the aorta: a mystery in evolution.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      • Ince H.
      • Nienaber C.A.
      Diagnosis and management of patients with aortic dissection.
      Similarly, there is also considerable controversy regarding the aetiology of PAU.
      • Vilacosta I.
      • Aragoncillo P.
      • Cañadas V.
      • San Román J.A.
      • Ferreirós J.
      • Rodríguez E.
      Acute aortic syndrome: a new look at an old conundrum.
      PAU may result from progressive erosion of atheromatous mural plaque with penetration of the elastic lamina. PAU may also develop in younger patients with intimal tears which remain localised and fail to progress to AD or IMH. PAU is more often present in the DTA and occurs more often in older patients with arterial hypertension, hyperlipoproteinaemia, and aortic sclerosis. Complicated PAU involves degeneration towards pseudoaneurysm formation, dissection, or rupture. Careful imaging is needed to evaluate both the diameter and depth of PAU.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      Although the specific growth rate is unknown, 20–30% of asymptomatic PAUs show evidence of progression over time.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      Symptomatic PAUs have an ominous natural history of progression and rupture. Urgent repair is commonly recommended in this setting.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      • Nathan D.P.
      • Boonn W.
      • Lai E.
      • Wang G.J.
      • Desai N.
      • Woo E.Y.
      • et al.
      Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease.
      Currently, there is a lack of data concerning the natural history of patients with asymptomatic PAU. Progression with pseudoaneurysm formation may occur in 15–50% of cases. The association between aortic diameter and rupture risk remains unclear. However, patients with a PAU that initially measures >20 mm in diameter or >10 mm in depth have a high risk of disease progression and should be considered candidates for early endovascular repair.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      • Nathan D.P.
      • Boonn W.
      • Lai E.
      • Wang G.J.
      • Desai N.
      • Woo E.Y.
      • et al.
      Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease.

      3.1.2.2 Management

      Patients presenting with uncomplicated type B IMH are primarily treated by medical therapy and intensive care monitoring, in line with the management of AD (see Section 3.1.1.2.1).
      • von Kodolitsch Y.
      • Csösz S.K.
      • Koschyk D.H.
      • Schalwat I.
      • Loose R.
      • Karck M.
      • et al.
      Intramural hematoma of the aorta: predictors of progression to dissection and rupture.
      Endovascular repair is currently indicated in symptomatic or complicated patients or in those with evolution towards AD because of a higher risk of peri-operative morbidity and the risk of rupture.
      • Nathan D.P.
      • Boonn W.
      • Lai E.
      • Wang G.J.
      • Desai N.
      • Woo E.Y.
      • et al.
      Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease.
      Endovascular repair is associated with lower peri-operative morbidity and mortality than OR.
      • Geisbüsch P.
      • Kotelis D.
      • Weber T.F.
      • Hyhlik-Dürr A.
      • Kauczor H.U.
      • Böckler D.
      Early and midterm results after endovascular stent graft repair of penetrating aortic ulcers.
      • Eggebrecht H.
      • Plicht B.
      • Kahlert P.
      • Erbel R.
      Intramural hematoma and penetrating ulcers: indications to endovascular treatment.
      • Clough R.E.
      • Mani K.
      • Lyons O.T.
      • Bell R.E.
      • Zayed H.A.
      • Waltham M.
      • et al.
      Endovascular treatment of acute aortic syndrome.
      Nevertheless, the role of endovascular repair in patients with type B IMH is debatable and identifying appropriate indications for treatment is critical. Although the literature provides no compelling guidelines for treatment, the WC recommends that treatment of IMH should follow similar guidelines as for the treatment of AD in the corresponding segment of the aorta, especially if it is associated with an evolving PAU, expansion of IMH, intimal tear disruption, or peri-aortic haematoma or progression to AD.
      • Evangelista A.
      • Czerny M.
      • Nienaber C.
      • Schepens M.
      • Rousseau H.
      • Cao P.
      • et al.
      Interdisciplinary expert consensus on management of type B intramural haematoma and penetrating aortic ulcer.
      Figure thumbnail fx13

      3.1.3 Ruptured aneurysm of the descending thoracic aorta

      3.1.3.1 Definition

      Most thoracic aortic aneurysms are either located in the ascending aorta or the DTA, but either type can extend into the aortic arch. Rupture risk correlates with aneurysm diameter. Aortic rupture is defined as disruption of all the layers of the aortic wall (intima, media, and adventitia). In the acute phase, active extravasation of blood (as detected by contrast enhanced CTA, MRA, or echocardiography), is pathognomonic for rupture. Generally, DTAA rupture is contained by periaortic structures (pleura, pericardium) or intrathoracic organs (oesophagus, lungs, heart).

      3.1.3.2 Management

      Rupture of the DTAA is an acute condition resulting in a high mortality. Most patients die before receiving treatment or do not survive treatment. Survivors are at risk of multisystem organ failure and/or cerebral/spinal insult. Traditionally, DTAA rupture has been treated by OR but, in the last few decades, endovascular repair has emerged as an alternative option in selected patients. Symptomatic and ruptured DTAAs should be treated urgently because of the risk of imminent exsanguination and death.

      3.1.3.2.1 Open repair

      Traditionally, ruptured DTAA has been managed by open repair. A recent meta-analysis of 224 patients with ruptured DTAA demonstrated a 30 day mortality of 33% for patients treated with OR.
      • Jonker F.H.
      • Trimarchi S.
      • Verhagen H.J.
      • Moll F.L.
      • Sumpio B.E.
      • Muhs B.E.
      Meta-analysis of open versus endovascular repair for ruptured descending thoracic aortic aneurysm.
      Major complications of myocardial infarction, stroke, and paraplegia have been reported to occur in 11.1%, 10.2%, and 5.5%, respectively. Another recent multicenter, retrospective review of 69 patients with ruptured DTAA published by the same authors demonstrated a composite outcome of death, stroke, or permanent paraplegia in 36.2%.
      • Jonker F.H.
      • Verhagen H.J.
      • Lin P.H.
      • Heijmen R.H.
      • Trimarchi S.
      • Lee W.A.
      • et al.
      OR versus endovascular repair of ruptured thoracic aortic aneurysms.
      These results were confirmed by a larger study, including data from the US Nationwide Inpatient Sample data on 559 patients with ruptured DTAA, with a reported mortality rate of 28.6%.
      • Gopaldas R.R.
      • Dao T.K.
      • LeMaire S.A.
      • Huh J.
      • Coselli J.S.
      Endovascular versus open repair of ruptured descending thoracic aortic aneurysms: a nationwide risk-adjusted study of 923 patients.
      These data confirm that OR for ruptured DTAA is associated with high mortality and morbidity.

      3.1.3.2.2 Endovascular repair

      TEVAR has emerged as a less invasive therapeutic option for the management of ruptured DTAA. No prospective, randomized study has compared stent grafting versus OR in the treatment of ruptured DTAA. However, the results of meta-analyses and multicentre studies suggest lower mortality and complication rates following TEVAR.
      • Jonker F.H.
      • Trimarchi S.
      • Verhagen H.J.
      • Moll F.L.
      • Sumpio B.E.
      • Muhs B.E.
      Meta-analysis of open versus endovascular repair for ruptured descending thoracic aortic aneurysm.
      • Jonker F.H.
      • Verhagen H.J.
      • Lin P.H.
      • Heijmen R.H.
      • Trimarchi S.
      • Lee W.A.
      • et al.
      OR versus endovascular repair of ruptured thoracic aortic aneurysms.
      • Gopaldas R.R.
      • Dao T.K.
      • LeMaire S.A.
      • Huh J.
      • Coselli J.S.
      Endovascular versus open repair of ruptured descending thoracic aortic aneurysms: a nationwide risk-adjusted study of 923 patients.
      In a recent meta-analysis comparing endovascular repair and OR for ruptured DTAA, the 30 day mortality was 19% and 33%, respectively.
      • Jonker F.H.
      • Trimarchi S.
      • Verhagen H.J.
      • Moll F.L.
      • Sumpio B.E.
      • Muhs B.E.
      Meta-analysis of open versus endovascular repair for ruptured descending thoracic aortic aneurysm.
      Lower rates of myocardial infarction (3.5%), stroke (4.1%), and paraplegia (3.1%) were noted for endovascular repair. The composite outcome of death, stroke, and myocardial infarction was 21.7% in the TEVAR group compared with 36.2% in the OR group (odds ratio 0.49, 95% CI 0.24–0.97, p = .044). By contrast, in the study using the US Nationwide Inpatient Sample data, TEVAR was not associated with a significantly lower mortality rate compared with OR (23.4% vs. 28.6%, respectively, p > .1).
      • Gopaldas R.R.
      • Dao T.K.
      • LeMaire S.A.
      • Huh J.
      • Coselli J.S.
      Endovascular versus open repair of ruptured descending thoracic aortic aneurysms: a nationwide risk-adjusted study of 923 patients.
      The need for LSA revascularisation in challenging proximal aortic neck anatomy is controversial, especially in acute cases. LSA coverage, to achieve a satisfactory proximal seal during TEVAR for ruptured DTAA, is reported in up to 38% of cases. LSA revascularisation was not performed in most of these cases.
      • Jonker F.H.
      • Verhagen H.J.
      • Lin P.H.
      • Heijmen R.H.
      • Trimarchi S.
      • Lee W.A.
      • et al.
      OR versus endovascular repair of ruptured thoracic aortic aneurysms.
      • Gopaldas R.R.
      • Dao T.K.
      • LeMaire S.A.
      • Huh J.
      • Coselli J.S.
      Endovascular versus open repair of ruptured descending thoracic aortic aneurysms: a nationwide risk-adjusted study of 923 patients.
      Comprehensive data regarding the rationale for LSA coverage without revascularisation are unavailable. In one study, subclavian artery bypass was performed in half of the cases (10/19 covered) before TEVAR.
      • Patel H.J.
      • Williams D.M.
      • Upchurch Jr., G.R.
      • Dasika N.L.
      • Deeb G.M.
      A 15 year comparative analysis of open and endovascular repair for the ruptured descending thoracic aorta.
      In cases in which the LSA is to be covered, prior revascularisation of the LSA in the emergency setting is recommended in patients with a left internal mammary artery to coronary artery bypass, or in those with a clearly dominant left vertebral artery. In all other emergency patients, LSA coverage without revascularisation can be performed
      • Matsumura J.S.
      • Lee W.A.
      • Mitchell R.S.
      • Farber M.A.
      • Murad M.H.
      • Lumsden A.B.
      • et al.
      The Society for Vascular Surgery Practice Guidelines: management of the left subclavian artery with thoracic endovascular aortic repair.
      • Patel H.J.
      • Williams D.M.
      • Upchurch Jr., G.R.
      • Dasika N.L.
      • Deeb G.M.
      A 15 year comparative analysis of open and endovascular repair for the ruptured descending thoracic aorta.
      (see also Section 2.4.4.).
      Figure thumbnail fx14

      3.1.4 Blunt traumatic thoracic aortic injury

      3.1.4.1 Definition and diagnostic testing

      Blunt traumatic thoracic aortic injury (TAI) most often occurs after sudden deceleration as a result of head on or side impact collisions, usually in high speed motor vehicle accidents or falls from great heights.
      The classic site of TAI is at the aortic isthmus in about 55–90% of patients admitted to hospital alive. Other regions of the thoracic aorta are less often affected.
      • Burkhart H.M.
      • Gomez G.A.
      • Jacobson L.E.
      • Pless J.E.
      • Broadie T.A.
      Fatal blunt aortic injuries: a review of 242 autopsy cases.
      • Neschis D.G.
      • Scalea T.M.
      • Flinn W.R.
      • Griffith B.G.
      Blunt aortic injury.
      Trauma to the distal segment of the thoracic aorta can be associated with injury to the diaphragm and adjacent compression fractures of the thoracic spine.
      • Burkhart H.M.
      • Gomez G.A.
      • Jacobson L.E.
      • Pless J.E.
      • Broadie T.A.
      Fatal blunt aortic injuries: a review of 242 autopsy cases.
      After traumatic brain injury, TAI is the second most common cause of death in blunt trauma patients. The morbidity and mortality of this injury are high, causing sudden death in 80–90% of cases.
      • Nagy K.
      • Fabian T.
      • Rodman G.
      • Fulda G.
      • Rodriguez A.
      • Mirvis S.
      Guidelines for the diagnosis and management of blunt aortic injury: an EAST Practice Management Guidelines Work Group.
      With improved rescue and rapid detection of TAI, patients who initially survive are more likely to undergo successful repair.
      The damage incurred in TAI can be partial or circumferential. With more accurate diagnostic tools, the term “minimal aortic injury”, which implies the presence of a small intimal flap with minimal to no peri-aortic haematoma, has been introduced to describe a lesion that carries a low risk of rupture.
      • Malhorta A.K.
      • Fabian T.C.
      • Croce M.A.
      • Weiman D.S.
      • Gavant M.L.
      • Pate J.W.
      Minimal aortic injury: a lesion associated with advancing diagnostic techniques.
      A classification scheme for TAI has been proposed: type I (intimal tear), type II (intramural haematoma), type III (pseudoaneurysm), and type IV (rupture).
      • Azizzadeh A.
      • Keyhani K.
      • Miller III, C.C.
      • Coogan S.M.
      • Safi H.J.
      • Estrera A.L.
      • et al.
      Blunt traumatic aortic injury: initial experience with endovascular repair.
      About 2–8% of patients with an initially unrecognized TAI may develop a chronic post-traumatic pseudoaneurysm.
      • Heystraten F.M.
      • Rosenbusch G.
      • Kingma L.M.
      • Lacquet L.K.
      Chronic posttraumatic aneurysm of the thoracic aorta: surgically correctable occult threat.
      There are only a few reports on the natural history of this type of pseudoaneurysm. The largest series reporting 413 patients was published in 1982.
      • Finkelmeier B.A.
      • Mentzner R.M.
      • Kaiser D.L.
      • Tegtmeyer C.J.
      • Nolan S.P.
      Chronic traumatic thoracic aneurysm – influence of operative treatment on natural history: an analysis of reported case 1950–1980.
      Up to 85% underwent surgical repair, and one third of the remaining patients died from their untreated lesions within 20 years of the initial trauma. Other authors report the development of late symptoms in about 50%, with aneurysmal expansion in about 20%, and death secondary to aortic rupture in 20% within 15 years of the injury.
      • Bennett D.E.
      • Cherry J.K.
      The natural history of traumatic aneurysms of the aorta.
      Clinical presentation ranges from non-specific symptoms to thoracic or interscapular pain. Signs of chest wall injury, pseudo-coarctation syndrome, a systolic murmur, or paraplegia can be present. The risk assessment for TAI begins with a high index of suspicion based on the mechanism of injury. Abdominal injury, thoracic injury, hypotension, and lack of restraint in motor vehicle accidents have been identified as clinical predictors of TAI. The greater the blunt trauma force the higher the index of suspicion for TAI should be.
      • Kirkham J.R.
      • Blackmore C.C.
      Screening for aortic injury with chest radiography and clinical factors.
      Initial plain chest X-rays have a significant false negative rate in patients with TAI. Consequently, once aortic transection is suspected, computed tomography evaluation is recommended.
      • Ekeh A.P.
      • Peterson W.
      • Woods R.J.
      • Walusimbi M.
      • Nwuneli N.
      • Saxe J.M.
      • et al.
      Is chest x-ray an adequate screening tool for the diagnosis of blunt thoracic aortic injury?.