title: Friable but treatable: coronary artery dissections in Ehlers-Danlos syndrome.
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.
authors.affiliation: Division of Cardiology, Hospital de Clínicas de Porto Alegre/Federal University of Rio Grande do Sul, Porto Alegre, RS, Brazil. zagoac@uol.com.br
authors.forename: Alexandre C
authors.initials: AC
authors.lastname: Zago
authors.forename: Bruno S
authors.initials: BS
authors.lastname: Matte
mesh_terms: D000784:Aortic Dissection / Q000000981:diagnostic imaging / Q000601:surgery*; D015906:Angioplasty, Balloon, Coronary / Q000009:adverse effects / Q000379:methods*; D002637:Chest Pain / Q000175:diagnosis / Q000209:etiology; D003323:Coronary Aneurysm / Q000000981:diagnostic imaging / Q000601:surgery*; D017023:Coronary Angiography / Q000379:methods; D001026:Coronary Artery Bypass / Q000379:methods; D023921:Coronary Stenosis / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007387:Internal Mammary-Coronary Artery Anastomosis / Q000379:methods*; D008875:Middle Aged; D018570:Risk Assessment; D012720:Severity of Illness Index; D015607:Stents; D016896:Treatment Outcome