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Optimising Aortic Endovascular Repair in Patients with Marfan Syndrome

Quentin Pellenc1, Antoine Girault2, Arnaud Roussel2

  • 1Vascular and Thoracic Surgery Department, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France; Paris-Diderot University, Paris, France; Centre de Référence pour le Syndrome de Marfan et apparentés, Bichat Hospital, Assistance Publique-Hopitaux de Paris (AP-HP), 75018 Paris, France; INSERM U 1148, LVTS, Bichat Hospital, 75018 Paris, France.

European Journal of Vascular and Endovascular Surgery : the Official Journal of the European Society for Vascular Surgery
|December 23, 2019
PubMed
Abstract

Insights

This study presents a modified protocol for endovascular aortic repair in Marfan syndrome (MFS) patients, significantly reducing complications and improving aortic diameter. The protocol optimizes outcomes for MFS patients undergoing aortic repair.

Area of Science:

  • Cardiovascular Surgery
  • Vascular Surgery
  • Genetics and Hereditary Diseases

Background:

  • Marfan syndrome (MFS) patients undergoing endovascular aortic repair (EVAR) face risks of aortic wall injury, dissection, dilatation, and false aneurysms at landing zones.
  • Modified practices may mitigate these EVAR-related complications in MFS patients.

Purpose of the Study:

  • To describe the experience and outcomes of a specific protocol for EVAR in patients with MFS.
  • To evaluate the safety and efficacy of modified EVAR techniques in Marfan syndrome.

Main Methods:

  • Prospective inclusion of MFS patients treated with EVAR between February 2015 and August 2018.
  • Application of a protocol excluding stent grafts with bare stents/barbs, utilizing proximal landing in pre-existing grafts or minimizing oversizing (<10%), and distal undersizing for chronic dissections.

Main Results:

  • Technical success rate was 100% in 18 MFS patients.
  • No mortality, spinal cord ischemia, stroke, or retrograde aortic dissection occurred post-operatively.
  • Significant reduction in mean maximum aortic diameter from 59 mm to 45 mm (p=0.0005) with a mean follow-up of 21.4 months.

Conclusions:

  • A specific EVAR protocol appears to optimize results in Marfan syndrome patients.
  • The described protocol facilitates significant aortic remodeling and reduces complication risks.
  • This approach enhances the safety and effectiveness of EVAR for MFS patients.
Keywords:
Aortic dissectionEndovascular aortic repairMarfan syndromeThoracic endovascular aortic repair

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