The pathology and pathobiology of bicuspid aortic valve: State of the art and novel research perspectives

The Journal of Pathology: Clinical Research

Patrick Mathieu³  Yohan Bossé¹⁰  Gordon S Huggins⁹  Alessandro Della Corte²  Philippe Pibarot¹⁰  Hector I. Michelena⁵  Giuseppe Limongelli⁶  Marie-Chloé Boulanger³  Arturo Evangelista⁷  Elisabeth Bédard¹⁰  Rodolfo Citro⁸  Simon C Body⁴  Mona Nemer^1,3 

[1] Laboratory for Cardiac Development and Differentiation, University of Ottawa, Ontario, Canada;Department of Cardiothoracic Sciences, Cardiac Surgery, Second University of Naples, Naples, Italy;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;Department of Anesthesiology, Perioperative and Pain Medicine, Center for Perioperative Genomics, Brigham and Women's Hospital, Boston, Massachusetts, USA;Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA;Department of Cardiology and Cardiothoracic and Respiratory Sciences, Cardiologia SUN, Monaldi Hospital, AO Colli, Naples, Italy;Department of Cardiology, Hospital Universitary Vall d'Hebron, Barcelona, Spain;Heart Department, University Hospital “San Giovanni di Dio e Ruggi d'Aragona”, Salerno, Italy;Molecular Cardiology Research Institute Center for Translational Genomics, Tufts Medical Center, Boston, Massachussetts, USA;Department of Molecular Medicine, Quebec Heart and Lung Institute/Research Center, Laval University, Québec, Canada

关键词: bicuspid aortic valve;    pathophysiology;    calcific aortic valve disease;    aorta dilation;    thoracic aortic aneurysm;   

DOI  :  10.1002/cjp2.21

来源: Wiley

【 摘 要 】

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.

【 授权许可】

CC BY-NC-ND   
© 2015 John Wiley and Sons Ltd and The Pathological Society of Great Britain and Ireland

Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.