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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.
Abstract:
Vascular Ehlers-Danlos syndrome is a rare connective tissue disorder associated with arterial dissection or rupture. Percutaneous coronary intervention (PCI) is often critical in patients with this syndrome because their coronary arteries are prone to dissection, enhancing the risk of stent borders dissection when conventional stent deployment pressures are used. Coronary artery bypass graft (CABG) treatment for these patients may also raise concerns because the left internal mammary artery is probably friable. Therefore, coronary artery revascularization in vascular Ehlers-Danlos syndrome either using PCI or CABG is challenging due to the arteries friability. A small number of cases have been published describing the friability of the vessels and associated complications; nevertheless, the optimum treatment remains unclear. We report the case of a 54-year-old woman treated successfully with PCI and CABG in two different acute coronary syndrome episodes, in which specific technical issues related to both procedures were decisive.
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