Friable but treatable: coronary artery dissections in Ehlers-Danlos syndrome

Alexandre C Zago1, Bruno S Matte

  • 1Division of Cardiology, Hospital de Clínicas de Porto Alegre/Federal University of Rio Grande do Sul, Porto Alegre, RS, Brazil. zagoac@uol.com.br

Insights

Vascular Ehlers-Danlos syndrome poses challenges for coronary revascularization. This case report details successful percutaneous coronary intervention (PCI) and coronary artery bypass graft (CABG) in a patient with this condition.

Area of Science:

  • Cardiology
  • Genetics
  • Vascular Surgery

Background:

  • Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic connective tissue disorder.
  • vEDS is characterized by a high risk of arterial dissection and rupture.
  • Coronary revascularization in vEDS patients presents significant challenges due to arterial friability.

Observation:

  • Percutaneous coronary intervention (PCI) carries an increased risk of dissection at stent borders due to vessel fragility and deployment pressures.
  • Coronary artery bypass graft (CABG) surgery is also complicated by the friability of vessels, including the left internal mammary artery.
  • Optimal treatment strategies for coronary artery disease in vEDS patients remain unclear, with limited published case studies.

Findings:

  • This report describes a successful PCI procedure in a 54-year-old woman with vEDS during an acute coronary syndrome episode.
  • The patient also underwent a successful CABG procedure during a separate acute coronary syndrome event.
  • Specific technical considerations during both PCI and CABG were crucial for successful outcomes in this vEDS patient.

Implications:

  • Successful PCI and CABG in vEDS patients are achievable with careful technical execution.
  • This case highlights the importance of tailored approaches to revascularization in patients with vascular Ehlers-Danlos syndrome.
  • Further case reports and studies are needed to refine optimal management strategies for coronary artery disease in vEDS.

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