TY - JOUR
T1 - Normal and abnormal development of the aortic valve and ascending aortic wall
T2 - a comprehensive overview of the embryology and pathology of the bicuspid aortic valve
AU - Grewal, Nimrat
AU - Lindeman, Jan H.
AU - Klautz, Arthur
AU - Driessen, Antoine
AU - Klautz, Robert J. M.
AU - Poelmann, Robert E.
N1 - Publisher Copyright: © Annals of Cardiothoracic Surgery. All rights reserved.
PY - 2022/7/1
Y1 - 2022/7/1
N2 - A bicuspid aortic valve (BAV) is the most prevalent congenital cardiac anomaly, in which the valve has only two leaflets, instead of the normal three. Patients with a BAV have an increased risk of aneurysm formation and the development of an aortic dissection. Vascular smooth muscle cells in both the non and dilated aortic wall are characterized by a maturation defect in all BAV patients, as compared to patients with a tricuspid aortic valve, which can contribute to inherent developmental susceptibility. Besides structural abnormalities of the vascular wall, a turbulent blood flow, caused by bicuspid valve geometry, could expedite the pathological process in the aortic wall, leading to aortopathy. Although the risk for aortopathy is significant, not all BAV patients experience (acute) aortic complications in their lifespan, highlighting the complexity of the pathogenetic process. Recent studies have focused on the embryonic development of semilunar valves and the ascending aortic wall. Their findings highlight that a defect in the embryogenesis could not only explain the development of a malformed aortic valve but also the increased risk for ascending aorta and arch pathology. This review presents an overview of the normal and abnormal development of the aortic valve and the aortic wall: a common defect in early embryogenesis causes the development of a BAV and associated aortopathy.
AB - A bicuspid aortic valve (BAV) is the most prevalent congenital cardiac anomaly, in which the valve has only two leaflets, instead of the normal three. Patients with a BAV have an increased risk of aneurysm formation and the development of an aortic dissection. Vascular smooth muscle cells in both the non and dilated aortic wall are characterized by a maturation defect in all BAV patients, as compared to patients with a tricuspid aortic valve, which can contribute to inherent developmental susceptibility. Besides structural abnormalities of the vascular wall, a turbulent blood flow, caused by bicuspid valve geometry, could expedite the pathological process in the aortic wall, leading to aortopathy. Although the risk for aortopathy is significant, not all BAV patients experience (acute) aortic complications in their lifespan, highlighting the complexity of the pathogenetic process. Recent studies have focused on the embryonic development of semilunar valves and the ascending aortic wall. Their findings highlight that a defect in the embryogenesis could not only explain the development of a malformed aortic valve but also the increased risk for ascending aorta and arch pathology. This review presents an overview of the normal and abnormal development of the aortic valve and the aortic wall: a common defect in early embryogenesis causes the development of a BAV and associated aortopathy.
KW - Aortopathy
KW - Bicuspid aortic valve (bav)
KW - Embryology
KW - Neural crest cells
KW - Pathology
UR - http://www.scopus.com/inward/record.url?scp=85135418516&partnerID=8YFLogxK
U2 - https://doi.org/10.21037/ACS-2021-BAV-14
DO - https://doi.org/10.21037/ACS-2021-BAV-14
M3 - Article
C2 - 35958528
SN - 2225-319X
VL - 11
SP - 380
EP - 388
JO - Annals of Cardiothoracic Surgery
JF - Annals of Cardiothoracic Surgery
IS - 4
ER -