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When Should Surgery Be Performed in Marfan Syndrome and Other Connective Tissue Disorders to Protect Against Type A Dissection?

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Controversies in Aortic Dissection and Aneurysmal Disease

Abstract

In western countries, thoracic aortic aneurysms and dissections (TAAD) are a common cause of death. Among patients with TAAD, 9 % have Marfan syndrome, and another 19 % exhibit a family history of TAAD which is unrelated to Marfan syndrome. Patients with heritable TAAD usually develop aortic rupture or dissection at an age under 40 years. Before the evolution of open-heart surgery, affected persons died from aortic dissection or rupture at young age. Currently, Marfan patients and most other individuals with heritable TAAD face a close to normal life-expectancy because elective replacement of the proximal aorta (type A dissection) is performed before aortic dissection or rupture develop.

We discuss all major medical rationales for performing surgery in Marfan syndrome and other connective tissue disorders to protect against type A dissection. These rationales comprise consideration of guidelines (1), of aortic biomechanics (2), of expected normal aortic diameters (3), of the speed of aortic growth (4), of aortic geometry and shape (5), and of etiology of aortic disease (6). The discussion of each of these six approaches follows the same pattern, which is first, explanation of the basic rationale of each approach with presentation of supporting data, second discussion of the limits and presentation of conflicting data, and third a final conclusion with statement of our personal view on the respective issue. Finally, we introduce the concept of our so-called “strategic decision making paradigm” that introduces the patient as a person into the surgical decision making process.

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Acknowledgement

 We wish to express our gratitude to Professor Christian Detter and Professor Tilo Kölbel for their thoughtful comments on the paper. We also want to thank Sabine Wuttke for her valuable help with the graphical artwork.

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Authors and Affiliations

  1. Department of Cardiology/Angiology, Centre of Cardiology and Cardiovascular Surgery, University Hospital Hamburg—Eppendorf, Hamburg, Germany

    Yskert von Kodolitsch MD, MBA

  2. Klinik für conservative und interventionelle Kardiologie, Universitares Herzzentrum Hamburg, Martinistrasse 52, Hamburg, 20146, Germany

    Yskert von Kodolitsch MD, MBA

  3. Wissenschaftlicher Mitarbeiter, Institut für Angewandte Genetik, Charité – Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin, Germany

    Peter N. Robinson MD, MSc

  4. Institut für Statistik und Datenverabeitung in der Medizinr Statistik und Datenverabeitung in der Medizin, Universitatsklinik Hamburg-Eppendorf, Martinistrasse 52, Hamburg, 20146, Germany

    Jürgen Berger PhD

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  1. Yskert von Kodolitsch MD, MBA
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  1. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Robert S. Bonser

  2. University Hospitals Birmingham, Queen Elizabeth Hospital, Birmingham, United Kingdom

    Domenico Pagano

  3. Thoracic and Cardiovascular Surgery Depts., Hannover Medical School, Hannover, Germany

    Axel Haverich

  4. University Hospitals Birmingham, Queen Elizabeth Hospital & Centre, Birmingham, United Kingdom

    Jorge Mascaro

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© 2014 Springer-Verlag London

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von Kodolitsch, Y., Robinson, P.N., Berger, J. (2014). When Should Surgery Be Performed in Marfan Syndrome and Other Connective Tissue Disorders to Protect Against Type A Dissection?. In: Bonser, R., Pagano, D., Haverich, A., Mascaro, J. (eds) Controversies in Aortic Dissection and Aneurysmal Disease. Springer, London. https://doi.org/10.1007/978-1-4471-5622-2_2

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  • DOI: https://doi.org/10.1007/978-1-4471-5622-2_2

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  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-5621-5

  • Online ISBN: 978-1-4471-5622-2

  • eBook Packages: MedicineMedicine (R0)

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