Two patients with left cervical aortic arch are presented and are analyzed with the 13 previously reported cases. This anomaly is thought to represent a persistent third embryonic aortic arch with regression of the normal fourth arch.
The patient is usually asymptomatic but occasionally has wheezing suggesting a vascular ring. Clues to the diagnosis are a pulsatile mass above either clavicle and blood pressure differences between the arms, with strong femoral pulses. The normal precordial examination contrasts to the loud murmur and thrill over the cervical mass. A chest roentgenogram usually shows an abnormal aorta or high mediastinal shadow, which may be confirmed as a right or left cervical aorta by aortography. No surgical therapy is indicated.