Endovascular procedures in patients with Ehlers-Danlos syndrome: a review of clinical outcomes and iatrogenic complications.

BACKGROUND: Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder caused by mutations in genes involved with collagen matrix formation that results in weakened blood vessels. Endovascular therapy on patients with EDS is fraught with concerns of vessel dissection and access site complications. We describe the technical and clinical outcomes of patients with EDS who have undergone a range of endovascular procedures.

METHODS: Patients with EDS undergoing endovascular procedures at a single-institution academic center between 1994 and 2010 were retrospectively reviewed. Perioperative data, including details of the procedure, hospital course, complications, and in-hospital mortality, were evaluated using nonparametric tests.

RESULTS: In all, 26 patients (8 with classic EDS, 15 with hypermobile EDS, and 3 with vascular EDS) who underwent 48 endovascular procedures (5 diagnostic, 43 interventional; 13 arterial, 35 venous) were identified. The indications for endovascular therapy included pelvic venous varices, visceral aneurysms/pseudoaneurysms, visceral/peripheral occlusive disease, coronary artery disease, and others. Median length of hospital stay was 2 days (range: 0-21 days). The rate of perioperative vascular injury and access site complications was low (2%), and it was not found to be associated with the type of vascular access technique, arterial versus venous procedures, target vessel site, sheath size, or method of closure (all: p > 0.1). Median follow-up period was 7.5 years. There were no late complications from the initial endovascular procedure.

CONCLUSIONS: Certain endovascular procedures for patients with EDS can be safely performed with a low rate of dissections and access site complications. However, some indications (particularly aortic interventions) still remain to be determined.