Szkutnik M, Religa Z, Bialkowski J, Wojtalik M
Department of Pediatric Cardiology, Silesian Academy of Medicine, Zabrze, Poland.
Tex Heart Inst J. 1998;25(3):212-5.
We report the case of a 13-year-old girl with a diffusely hypoplastic right aortic arch, anomalous origin of the left subclavian artery, and a small, insignificant ventricular septal defect. The patient's pulse was forceful at the carotid arteries, but it was markedly weaker at all extremities. Catheterization revealed that both common carotid arteries arose from the dilated ascending aorta; the right subclavian and vertebral arteries arose from the hypoplastic posterior segment of the aortic arch, and the left subclavian artery arose from the normally developed descending aorta. The pressure gradient between the ascending and descending aorta was 80 mmHg. A bypass grafting procedure was performed to connect the ascending and the upper abdominal aorta. No pressure gradient remained after the operation, nor was a gradient detected during 2 years of follow-up. The origin of both subclavian arteries distal to the area of coarctation resulted in an atypical clinical picture and delayed diagnosis. Ten previously reported cases of coarctation of the aorta with right aortic arch are reviewed.
我们报告了一名13岁女孩的病例，她患有弥漫性右主动脉弓发育不全、左锁骨下动脉异常起源以及一个小的、无明显意义的室间隔缺损。患者颈动脉搏动有力，但四肢搏动明显较弱。心导管检查显示，双侧颈总动脉均发自扩张的升主动脉；右锁骨下动脉和椎动脉发自发育不全的主动脉弓后段，左锁骨下动脉发自正常发育的降主动脉。升主动脉和降主动脉之间的压力梯度为80 mmHg。进行了旁路移植手术，以连接升主动脉和上腹部主动脉。术后不再存在压力梯度，随访2年期间也未检测到压力梯度。缩窄区域远端的双侧锁骨下动脉起源导致了非典型的临床表现和诊断延迟。本文回顾了之前报道的10例伴有右主动脉弓的主动脉缩窄病例。
