Amyloid Deposits in a Functionally Unicuspid Stenotic Aortic ValveNovel Teaching Points
Anne-Sophie Zenses, Eng, PhD,
Charles Leduc, MD, MSc,
Stéphanie Béchard, NP,
Jessica Forcillo, MD, PhD,
Zaki El Haffaf, MD,
Quoc-Bao Do, MD, MSc,
Philippe Pibarot, DVM, PhD,
François Tournoux, MD, PhD
Affiliations
Anne-Sophie Zenses, Eng, PhD
Research Centre of the University of Montreal Hospital (CRCHUM), Montreal, Quebec, Canada; Corresponding author: Dr Anne-Sophie Zenses, Centre de recherche du CHUM (CRCHUM) - Pavillon R, 11e étage – 900, rue Saint-Denis, Montréal, Québec H2X 0A9, Canada. Tel.: +1-514-890-8000 ext. 13517.
Charles Leduc, MD, MSc
Department of Pathology and Cell Biology, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
Stéphanie Béchard, NP
Department of Cardiology, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
Jessica Forcillo, MD, PhD
Department of Surgery, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
Zaki El Haffaf, MD
Department of Genetic Medicine, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
Quoc-Bao Do, MD, MSc
Department of Surgery, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
Philippe Pibarot, DVM, PhD
Research Center of the Quebec Heart and Lung Institute, Laval University, Quebec, Quebec, Canada
François Tournoux, MD, PhD
Department of Cardiology, University of Montreal Hospital (CHUM), Montreal, Quebec, Canada
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. Résumé: Une amyloïdose associée à une sténose aortique survient généralement avec infiltration du myocarde par la protéine transthyrétine. Le cas que nous décrivons est, à notre connaissance, le premier cas rapporté d’amyloïdose localisée de type indéterminé dans une valve aortique sévèrement calcifiée et fonctionnellement unicuspide. L’amyloïdose valvulaire dystrophique isolée serait liée à l'atteinte fibrocalcique de la valve. À la lumière de la littérature à ce sujet, le cas décrit ici permet de soulever de nouvelles hypothèses physiopathologiques et appuie le lien entre une morphologie valvulaire inhabituelle (non tricuspide) et l’apparition de substances amyloïdes dystrophiques, probablement secondaire à une altération des contraintes hémodynamiques.
