Jux C, Kaulitz R, von Wasielewski R, Peuster M, Fink C, Paul T, Hausdorf G
Abteilung für Pädiatrische Kardiologie und Pädiatrische Intensivmedizin, Medizinische Hochschule Hannover.
Z Kardiol. 2000 Jun;89(6):502-7. doi: 10.1007/s003920070221.
In aortic atresia, coronary perfusion normally occurs through retrograde blood flow in the ascending aorta. We report on two patients with antegrade flow in the ascending aorta despite aortic atresia. In one patient with hypoplastic left heart syndrome (aortic atresia, severe mitral stenosis), an intact interatrial septum/premature closure of the foramen ovale was found. While no other way of left atrial or ventricular decompression was found, echocardiography, angiography and the post-mortem examination showed left ventricular to coronary sinusoids as the sole pathway for systemic oxygenation. In a second patient with complex congenital heart disease, including aortic atresia, antegrade flow in the ascending aorta was through a left coronary fistula with shunt flow originating from the pulmonary trunk. This report describes systemic perfusion depending on retrograde coronary flow due to coronary-cameral (sinusoids) and coronary arterio-venous fistulas leading to the phenomenon of antegrade blood flow in the ascending aorta despite aortic atresia.
在主动脉闭锁时,冠状动脉灌注通常通过升主动脉内的逆行血流发生。我们报告了两名尽管存在主动脉闭锁但升主动脉有顺行血流的患者。在一名患有左心发育不全综合征(主动脉闭锁、严重二尖瓣狭窄)的患者中,发现房间隔完整/卵圆孔过早闭合。虽然未发现左心房或心室减压的其他途径,但超声心动图、血管造影和尸检显示左心室至冠状窦状隙是全身氧合的唯一途径。在第二名患有包括主动脉闭锁在内的复杂先天性心脏病的患者中,升主动脉的顺行血流是通过左冠状动脉瘘,分流血流源自肺动脉干。本报告描述了由于冠状-心腔(窦状隙)和冠状动静脉瘘导致的依赖逆行冠状动脉血流的全身灌注,尽管存在主动脉闭锁,但仍导致升主动脉出现顺行血流现象。