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Outcomes of Endovascular Interventions in Patients with Connective Tissue Diseases
J Westley Ohman, MD, Chandu Vemuri, MD, Patrick J. Geraghty, MD, Luis A. Sanchez, MD, Alan Braverman, MD, Jeffrey Jim, MD.
WashU STL, Saint Louis, MO, USA.

OBJECTIVES:Patients with connective tissue disorders (CTD) requiring aortic repair are traditionally treated with open surgery as endovascular repair has been contraindicated due to concern about tissue integrity and outcomes with TEVAR. Our objective is to review a single center experience in endovascular treatment in CTD patients.
METHODS: A retrospective chart review was performed to identify patients with a diagnosis of a CTD who underwent an endovascular intervention between 12/1/2004 and 12/1/2013. Preoperative demographic information and postoperative outcomes were collected.
RESULTS: During the study period, a total of 6 patients met selection criteria, and were followed for 42.5 +/- 13.9 months. 4 carried a diagnosis of Marfan syndrome, 1 had Loeys-Dietz syndrome, and 1 was unspecified. 4 were treated for aneurysmal disease: 1 thoracic aorta, 2 abdominal aortoiliac, and 1 popliteal; and 2 were treated for acute complications from Type B aortic dissection. 1 patient had a recent thoracotomy with proximal suture line anastomosis dehiscence whom was not felt to be able to tolerate an open procedure following a prolonged code and was treated with proximal thoracic endovascular aortic aneurysm repair (TEVAR) on an emergent basis, the remainder were considered to be at prohibitive risk due to comorbidities and prior procedures. There was 1 periprocedural complications, the emergent TEVAR patient developed asymptomatic retrograde dissection
In follow-up, secondary interventions were required in two patients including embolization of a type II endoleak at 7 months and stent graft placement for a type IB endoleak at 1 month. The patient treated for popliteal aneurysm required open popliteal aneurysmorrhaphy at 15 months. 5 patients are alive with 1 death at 9.2 years from a gastrointestinal bleed unrelated to the endovascular repair.
CONCLUSIONS:Endovascular treatment for patients with CTD are associated with high rates of complication and reintervention. The use of this treatment modality should be reserved for patients where traditional open reconstruction is at prohibitive risk.


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