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Spontaneous coronary artery dissection in Ehlers-Danlos syndrome

Chetan V Hampole1, Femi Philip, Alexis Shafii

  • 1Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio 44106, USA. hamploc@ccf.org

The Annals of Thoracic Surgery
|November 5, 2011
PubMed

Insights

Ehlers-Danlos syndrome type IV, a severe vascular condition, can cause arterial dissection. This case report details a successful coronary artery bypass graft for a spontaneous left anterior descending artery dissection in such a patient.

Area of Science:

  • Cardiology
  • Genetics
  • Vascular Surgery

Background:

  • Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
  • Vascular EDS (vEDS, type IV) is the most severe subtype, characterized by fragile blood vessels.
  • Spontaneous arterial dissections and aneurysms are common, life-threatening complications of vEDS.

Observation:

  • This report describes a patient diagnosed with Ehlers-Danlos syndrome type IV.
  • The patient presented with a spontaneous dissection of the left anterior descending coronary artery.
  • This is a rare and critical manifestation of vascular EDS.

Findings:

  • The patient underwent successful surgical intervention.
  • Coronary artery bypass grafting (CABG) was performed to treat the left anterior descending artery dissection.
  • This demonstrates a viable treatment option for coronary complications in vEDS.

Implications:

  • Coronary artery dissection is a potential complication of vascular Ehlers-Danlos syndrome.
  • Coronary artery bypass grafting can be a successful treatment modality for these dissections.
  • Early recognition and surgical management are crucial for improving outcomes in patients with vEDS and cardiac events.

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