Osler's disease is an autosomal dominant disorder characterized by epistaxis, telangiectases, and arteriovenous malformations of the internal organs. Herein, we report the first published case of dissecting thoracoabdominal aortic aneurysm repair in a 66-year-old woman with hereditary hemorrhagic telangiectasia associated with ACVRL1 gene mutations. We maintained the activated clotting time around 400 seconds during cardiopulmonary bypass to avoid lethal hemorrhage from telangiectases or arteriovenous malformations. The Adamkiewicz artery could not be identified on imaging studies preoperatively. Therefore, we used the snake technique for intercostal revascularization, which was effective in preventing paraplegia.
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| http://dx.doi.org/10.1016/j.athoracsur.2021.12.010 | DOI Listing |
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