Muñoz Castellanos L, de la Cueva R, Zavaleta D, Kuri Nivón M
Instituto Nacional de Cardiología Ignacio Chávez.
Arch Inst Cardiol Mex. 1989 Jul-Aug;59(4):375-82.
Left juxtaposition of atrial appendages is an anomaly of their origin and position which is associated with a high incidence of complex congenital heart defects. We studied six cases of juxtaposed right atrial appendages. They emerged from the anterior wall adjacent to the atrial septum and were situated to the left of the origin of the great arteries. Associated congenital heart defects were: single outlet, right ventricular hypoplasia, absent right atrioventricular connection, ventricular and atrial septal defects and pulmonary outflow obstruction. Other abnormal morphological features present were anatomically corrected malposition of the great arteries, bilateral infundibulum and right aortic arch. Although the association of these malformations with juxtaposed atrial appendages does not per se constitute a syndrome, its presence is an ominous sign of severe cyanotic congenital heart disease which should alert both clinicians and surgeons. There are various possible embryological explanations for the genesis of this malformation. We believe that juxtaposition is the consequence of the ectopic origin of the atrial appendage rudiment, which in the presence of truncal alterations grows behind and to the left of the great arteries.
左心耳并置是一种起源和位置异常,与复杂先天性心脏缺陷的高发生率相关。我们研究了6例右心耳并置病例。它们起源于与房间隔相邻的前壁,位于大动脉起源的左侧。相关的先天性心脏缺陷包括:单心室出口、右心室发育不全、右房室连接缺如、室间隔和房间隔缺损以及肺流出道梗阻。其他存在的异常形态学特征包括大动脉解剖矫正转位、双侧漏斗部和右位主动脉弓。虽然这些畸形与心耳并置的关联本身并不构成一种综合征,但其存在是严重紫绀型先天性心脏病的不祥征兆,应引起临床医生和外科医生的警惕。对于这种畸形的发生有多种可能的胚胎学解释。我们认为并置是心耳原基异位起源的结果,在存在干异常的情况下,心耳原基在大动脉后方和左侧生长。