Niwa Koichiro
Department of Cardiology,Cardiovascular Center,St Luke's International Hospital,Tokyo,Japan.
Cardiol Young. 2017 Dec;27(10):1959-1965. doi: 10.1017/S1047951117002116.
Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatation and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, a new clinical entity can be used to call this association of aortic pathophysiological abnormality, aortic dilation, and aorto-left ventricular interaction - "aortopathy".
马凡综合征、二叶式主动脉瓣和/或主动脉缩窄与升主动脉或缩窄旁主动脉的中层异常有关。升主动脉中层异常在其他类型的先天性心脏病（CHD）患者中很常见，如单心室、永存动脉干、大动脉转位、左心发育不全综合征和法洛四联症，涵盖广泛的年龄范围，可能易导致扩张、动脉瘤和破裂，需要进行主动脉瓣和主动脉根部手术。这些先天性心脏病表现为主动脉根部持续扩张、主动脉弹性降低和主动脉僵硬度增加，这可能与主动脉根部的内在特性有关。这些主动脉扩张和僵硬度增加可导致主动脉瘤、主动脉破裂和主动脉瓣关闭不全，还可引起左心室肥厚、冠状动脉血流减少和左心室衰竭。因此，一种新的临床实体可用于称呼这种主动脉病理生理异常、主动脉扩张以及主动脉-左心室相互作用的关联——“主动脉病变”。
