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Amyloid Deposits in a Functionally Unicuspid Stenotic Aortic Valve.

ABSTRACT: 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.

SUBMITTER: Zenses AS

PROVIDER: S-EPMC9764129 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

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Amyloid Deposits in a Functionally Unicuspid Stenotic Aortic Valve.

Zenses Anne-Sophie AS Leduc Charles C Béchard Stéphanie S Forcillo Jessica J El Haffaf Zaki Z Do Quoc-Bao QB Pibarot Philippe P Tournoux François F

CJC open 20220922 12

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 cont ...[more]

PMID: 36562019

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