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Amyloid Deposits in a Functionally Unicuspid Stenotic Aortic Valve

Amyloidosis concomitant to aortic stenosis usually occurs with myocardial infiltration by the transthyretin protein. To our knowledge, this is the first report of localized amyloidosis of indeterminate type in a severely calcified and functionally unicuspid aortic valve. Isolated dystrophic valvular...

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Detalles Bibliográficos
Autores principales: Zenses, Anne-Sophie, Leduc, Charles, Béchard, Stéphanie, Forcillo, Jessica, El Haffaf, Zaki, Do, Quoc-Bao, Pibarot, Philippe, Tournoux, François
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9764129/
https://www.ncbi.nlm.nih.gov/pubmed/36562019
http://dx.doi.org/10.1016/j.cjco.2022.09.002
Descripción
Sumario:Amyloidosis concomitant to aortic stenosis usually occurs with myocardial infiltration by the transthyretin protein. To our knowledge, this is the first report of localized amyloidosis of indeterminate type in a severely calcified and functionally unicuspid aortic valve. Isolated dystrophic valvular amyloidosis is believed to be related to fibrocalcific valve disease. In light of the literature on this topic, the present case raises new hypotheses on pathophysiology and further supports the contributory role of unusual non-tricuspid valve morphology in the development of dystrophic amyloid, likely secondary to altered hemodynamic stress.