Aortic Valve Replacement in a Patient With Osler-Rendu-Weber Disease

doi:10.1016/j.athoracsur.2009.02.014

The Annals of Thoracic Surgery

Volume 88, Issue 1, July 2009, Pages e3-e4

https://doi.org/10.1016/j.athoracsur.2009.02.014 Get rights and content

Osler-Rendu-Weber (hereditary hemorrhagic telangiectasia) disease is an uncommon disease characterized by the presence of abnormal telangiectasias and arteriovenous malformations that cause recurrent episodes of bleeding. We present a patient with Osler-Rendu-Weber disease, with a history of multiple major bleeding events and severe aortic valve stenosis, who underwent aortic valve replacement. Unexpectedly, the postoperative course was uneventful, and there was no untoward bleeding in the early or in the late postoperative follow-up.

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O-R-W (hereditary hemorrhagic telangiectasia) is inherited as an autosomal dominant trait, with a prevalence of 1 in 8000. It can be caused by mutation of the endoglin gene (O-R-W 1) and the activin receptor gene (O-R-W 2), which are essential products for angiogenesis and normal vascular maturation [2]. These mutations produce the formation of telangiectasias and AVMs, which tend to rupture easily and bleed. They are usually localized in skin and mucosa, causing epistaxis and gastrointestinal

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Snake Technique in Osler's Disease–Associated Thoracoabdominal Aortic Aneurysm
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Citation Excerpt :
During surgery for patients with HHT, prevention of life-threatening hemorrhage from telangiectases or AVMs is of great importance. We managed the ACT around 400 seconds during CPB,7 which should usually be more than 480 seconds in usual cases. We also simply cross-clamped the aorta instead of using hypothermic circulatory arrest, which could increase the risk of lethal hemorrhage.
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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Aortic valve replacement for aortic insufficiency induced by infectious endocarditis in a patient with Osler-Rendu-Weber Syndrome: A case report and literature review
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Case reportAortic Valve Replacement in a Patient With Osler-Rendu-Weber Disease

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J Thorac Cariovasc Surg

J Thorac Cardiovasc Surg

Am J Surg

Case report
Aortic Valve Replacement in a Patient With Osler-Rendu-Weber Disease