McElhinney D B, Thompson L D, Weinberg P M, Jue K L, Hanley F L
Division of Cardiothoracic Surgery, University of California, San Francisco, USA.
Cardiol Young. 2000 May;10(3):212-9. doi: 10.1017/s1047951100009136.
Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases.
Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1.
Repair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others).
Structural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.
头臂动脉分支异常和主动脉弓偏侧性在颈主动脉弓患者中很常见。此外，在相当多的病例中还发现主动脉弓的结构异常，如梗阻、动脉瘤和迂曲。
1990年至1998年间，6例患者接受了右颈主动脉弓梗阻的手术治疗。所有患者的横行或远端主动脉弓均存在明显梗阻，2例患者在先前修复后复发。1例患者，与梗阻相邻的主动脉段还存在多叶状动脉瘤，2例患者主动脉弓有明显迂曲。所有病例中，头颈部血管的起源顺序均异常，3例患者发现1条或多条头臂血管梗阻。所有患者均存在血管环，4例患者为右主动脉弓和迷走左锁骨下动脉，2例患者为双主动脉弓且右侧颈主动脉弓占优势。3例患者降主动脉呈迂曲状（左侧）。3例患者通过标准右后外侧开胸手术修复，3例通过正中胸骨切开术修复。2例患者采用补片扩大主动脉成形术，2例患者采用从升主动脉到降主动脉的管状移植物，1例患者采用降主动脉与近端主动脉弓的端侧吻合，1例患者采用直接吻合重建双弓闭锁的左侧部分。
所有病例修复均成功，无围手术期并发症。在1至9年的随访中，所有患者均存活且状况良好，无主动脉弓梗阻复发或其他重大并发症。荧光原位杂交显示1例患者存在22q11染色体微缺失（其他患者未进行此项检查）。
颈主动脉弓患者中主动脉弓结构异常相对常见。此类异常可能是血流动力学状况和/或与主动脉弓的颈部位置或其胚胎学前身相关的异常血管组织所致。鉴于复杂颈主动脉弓患者的解剖结构高度可变，手术考虑也会有所不同。
