Results of Aortic Surgery in Patients with Marfan Syndrome

Iguchi, Atsushi; Saiki, Yoshikatsu; Oda, Kazuaki; Tabayashi, Koichi

doi:10.1007/s00595-004-2909-z

Cited by 4 publications

(1 citation statement)

References 25 publications

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“…The trend towards early elective replacement of the aortic root in Marfan patients has allowed to significantly increase the overall survival. 5,6 However a subset of patients with CTD will necessitate multiple operations for progression of the disease on other aortic segments. Treatment of such patients is challenging and is often linked to a poor outcome.…”

Section: Discussionmentioning

confidence: 99%

Thoracic Aortic Stent Grafting in Patients with Connective Tissue Disorders: A Word of Caution

Mohammadi¹,

Normand²,

Voisine³

et al. 2007

Innovations�(Phila)

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Objective Use of thoracic Stent-graft in patients with connective tissue disorders (CTD) remains limited. We herein report 3 patients with CTD who underwent stent grafting. Methods and Results Case 1; A male Marfan patient was operated for thoraco-abdominal aneurysm. On computed tomography (CT), large false aneurysm at the proximal anastomosis was documented which was excluded with a 30 mm Talent stent-graft with 10–15% oversize. Case 2; A female with Ehlers-Danlos syndrome had undergone resection of descending aortic thoracic aneurysm presented with an enlarging aneurysm distal to the graft. Three Talent stent-grafts (15% oversize) were deployed with balloon dilatation to exclude the aneurysm. The immediate postoperative period was complicated by an extensive intramural hematoma of the descending aorta with hemothorax, managed conservatively. Case 3; A female Marfan patient had undergone Bentall procedure and mitral repair followed with resection of the proximal descending aorta. Three months later a false aneurysm at the distal anastomosis was treated with a 24 mm Valiant stent-graft (30% oversize). Aortic dissection distal to stent was documented on the early postoperative CT. The dissected aneurysm enlarged significantly with a type I distal endoleak during follow-up. Concomitantly, the patient presented a class III dyspnea owing to a severe mitral regurgitation. The patient underwent a successful MVR and stent-graft explantation with replacement of the descending aorta. Conclusion Significant complications supervened when stent-grafts were deployed in native aorta. We thus recommend that deploying a stent-graft in a CTD diseased aorta should be considered a relative contraindication. In cases with prohibitive or high risk surgery, use of a stent-graft with minimal radial force and minimal oversizing without balloon dilatation should be considered.

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Section: Discussionmentioning

confidence: 99%