Background Abnormalities of brachiocephalic arterial branching and arch lat
erality are common in patients with a cervical aortic arch. In addition, st
ructural anomalies of the arch such as obstruction, aneurysms, and tortuosi
ty are found in a significant number of cases. Methods. Between 1990 and 19
98, 6 patients underwent surgery for an obstructed right cervical arch. A s
ignificant 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 ob
struction, 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 obstruct
ion of 1 or more brachiocephalic vessels was found in 3. A vascular ring wa
s present in all patients, with a right aortic arch and aberrant left subcl
avian artery in 4 patients and a double aortic arch with a dominant right c
ervical arch in 2. The descending aorta was circumflex (left-sided) in 3 pa
tients. Three patients were repaired through a standard right posterolatera
l thoracotomy, and 3 through a median sternotomy. Patch augmentation aortop
lasty 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 proxima
l arch in I, and direct anastomosis to reconstruct an atretic left-sided co
mponent of a double arch in 1. Results: Repair was successful in all cases,
with no perioperative complications. At follow-up ranging from 1 to 9 year
s, all patients were alive and well, with no recurrence of arch obstruction
or other significant complications. Fluorescent in situ hybridization reve
aled microdeletion of chromosome 22q11 in 1 patient (not performed in the o
thers). Conclusions: Structural anomalies of the arch are relatively common
in patients with a cervical aortic arch. Such abnormalities may be the res
ult of hemodynamic conditions and/or abnormal vascular tissue related eithe
r 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.