title: The pathology and pathobiology of bicuspid aortic valve: State of the art and novel research perspectives.
abstract: Bicuspid aortic valve is the most prevalent cardiac valvular malformation. It is associated with a high rate of long-term morbidity including development of calcific aortic valve disease, aortic regurgitation and concomitant thoracic aortic aneurysm and dissection. Recently, basic and translational studies have identified some key processes involved in the development of bicuspid aortic valve and its morbidity. The development of aortic valve disease and thoracic aortic aneurysm and dissection is the result of complex interactions between genotypes, environmental risk factors and specific haemodynamic conditions created by bicuspid aortic valve anatomy. Herein, we review the pathobiology of bicuspid aortic valve with a special emphasis on translational aspects of these basic findings. Important but unresolved problems in the pathology of bicuspid aortic valve and thoracic aortic aneurysm and dissection are discussed, along with the molecular processes involved.
authors: Laboratoire d'Études Moléculaires des Valvulopathies (LEMV), Groupe de Recherche en Valvulopathies (GRV), Department of Surgery Quebec Heart and Lung Institute/Research Center, Laval University Quebec Canada.
authors: Patrick
authors: P
authors: Mathieu
authors: Department of Molecular Medicine, Quebec Heart and Lung Institute/Research Center Laval University Québec Canada.
authors: Yohan
authors: Y
authors: Bossé
authors: Molecular Cardiology Research Institute Center for Translational Genomics, Tufts Medical Center Boston Massachussetts USA.
authors: Gordon S
authors: GS
authors: Huggins
authors: Department of Cardiothoracic Sciences, Cardiac Surgery Second University of Naples 80131 Naples Italy.
authors: Alessandro
authors: A
authors: Della Corte
authors: Department of Molecular Medicine, Quebec Heart and Lung Institute/Research Center Laval University Québec Canada.
authors: Philippe
authors: P
authors: Pibarot
authors: Division of Cardiovascular Diseases, Mayo Clinic Rochester Minnesota USA.
authors: Hector I
authors: HI
authors: Michelena
authors: Department of Cardiology and Cardiothoracic and Respiratory Sciences, Cardiologia SUN, Monaldi Hospital, AO Colli Naples Italy.
authors: Giuseppe
authors: G
authors: Limongelli
authors: Laboratoire d'Études Moléculaires des Valvulopathies (LEMV), Groupe de Recherche en Valvulopathies (GRV), Department of Surgery Quebec Heart and Lung Institute/Research Center, Laval University Quebec Canada.
authors: Marie-Chloé
authors: MC
authors: Boulanger
authors: Department of Cardiology Hospital Universitary Vall d'Hebron Barcelona Spain.
authors: Arturo
authors: A
authors: Evangelista
authors: Department of Molecular Medicine, Quebec Heart and Lung Institute/Research Center Laval University Québec Canada.
authors: Elisabeth
authors: E
authors: Bédard
authors: Heart Department University Hospital "San Giovanni di Dio e Ruggi d'Aragona" Salerno Italy.
authors: Rodolfo
authors: R
authors: Citro
authors: Department of Anesthesiology, Perioperative and Pain Medicine Center for Perioperative Genomics, Brigham and Women's Hospital Boston Massachusetts USA.
authors: Simon C
authors: SC
authors: Body
authors: Laboratory for Cardiac Development and Differentiation University of Ottawa Ontario Canada.
authors: Mona
authors: M
authors: Nemer
authors: Department of Pathology Brigham and Women's Hospital, Harvard Medical School USA.
authors: Frederick J
authors: FJ
authors: Schoen
keywords: aorta dilation; bicuspid aortic valve; calcific aortic valve disease; pathophysiology; thoracic aortic aneurysm