. 2022 Aug 26;30(4):231–262. doi: 10.4250/jcvi.2022.0058

Table 1. Summary of aortic arch anomalies and variants.

Vascular anomalies		Possible embryology	Arch and ductus	Clinical features
Lt-sided arch
	Lt aortic arch with ARSA	Regression of the Rt 4^th arch between the RCCA and RSA	Lt arch, Lt ductus	Most common congenital anomaly of the aorta (0.5–2%)
	Lt aortic arch with ARSA	Regression of the Rt 4^th arch between the RCCA and RSA	Lt arch, Lt ductus	Usually isolated
	Lt aortic arch with ARSA with Kommerell diverticulum	Regression of the Rt 4^th arch between the RCCA and RSA and persistence of the Rt 6^th arch component	Lt arch, Rt ductus, vascular ring (+)	Dysphagia lusoria (if tight vascular ring)
	Lt aortic arch with ARSA with Kommerell diverticulum		Lt arch, Rt ductus, vascular ring (+)	15–30% of ARSA
	Lt aortic arch with isolated Rt subclavian artery	Regression of the Rt 4^th arch between the RCCA and RSA and regression of the segment distal to the RSA and ductus	Lt arch, no ductus	Congenital subclavian steal syndrome, vertebrobasilar insufficiency, syncope
	Lt circumflex aorta	Regression of the Rt 4^th arch between the RCCA and RSA and persistence of the Rt 6^th arch and Rt dorsal aorta	Lt arch, Rt ductus, Rt descending aorta, vascular ring (+)	Extremely rare
				Arch itself courses behind the esophagus
				Severe tracheobronchial compression
Rt-sided arch
	Rt aortic arch with mirror image branching	Regression of the Lt 4^th arch between the Lt ductus and dorsal aorta and persistence of the Lt 6^th arch	Rt arch, Lt ductus	Usually asymptomatic
	Rt aortic arch with mirror image branching		Rt arch, Lt ductus	Strong association with CHD (> 75%)
	Rt aortic arch with ALSA with Kommerell diverticulum	Regression of the Lt 4^th arch between the LCCA and LSA and persistence of the Lt 6^th arch component	Rt arch, Lt ductus, vascular ring (+)	Most common form of Rt-sided aortic arch
				Usually isolated (CHD in 5–10%)
				Dysphagia lusoria (if tight vascular ring)
				60% of ALSA
	Rt aortic arch with ALSA without Kommerell diverticulum	Regression of the Lt 4^th arch between the LCCA and LSA and regression of the Lt 6^th arch	Rt arch, Rt ductus, or absent ductus	Usually combined with CHD
	Right aortic arch with isolated RSA	Regression of the Lt 4^th arch between the LCCA and LSA origin and regression distal to the LSA and ductus	Rt arch, no ductus	Associated with CHD (> 50%), vertebrobasilar insufficiency, or subclavian steal syndrome
	Right circumflex aorta	Regression of the Lt 4^th arch between the LCCA and LSA and persistence of the Lt 6^th arch and Lt dorsal aorta	Rt arch, Lt ductus, Lt descending aorta, vascular ring (+)	More common than Lt circumflex aorta
Miscellaneous
	Double aortic arch	Abnormal persistence of both aortic arches	Both aortic arches, vascular ring (+)	Most common cause of symptomatic vascular ring (50%)
	Cervical aortic arch	i) Persistence of the 2^nd or 3^rd branchial arches with regression of the 4^th arch	Variable branching pattern	More common on the Rt-sided arch
	Cervical aortic arch	or ii) failure of caudal migration of the 4^th arch	Variable branching pattern	Usually asymptomatic (occasionally pulsatile neck mass)
	Persistent fifth aortic arch	Persistence of the primitive 5^th aortic arch	Variable branching pattern, vascular ring (+/−)	Usually associated with CHD
	Persistent fifth aortic arch	Persistence of the primitive 5^th aortic arch	Variable branching pattern, vascular ring (+/−)	Often misdiagnosed as ductus arteriosus or aortopulmonary window
	Interrupted aortic arch	i) Regression distal to the LSA,	Complete discontinuity between the ascending and descending aorta	Extremely rare
		or ii) regression between the LCCA and LSA,		Shock or severe heart failure occurs a few weeks after birth
		or iii) regression between the RBCA and LCCA
	Hypoplastic aortic arch	May be associated with restriction of aortic flow	Relatively small diameter of the aortic arch	With or without other CHD (m/c with aortic coarctation)
	Coarctation of the aorta	i) Abnormal ductal tissue extension to the aortic isthmus or	Focal stenosis of the aortic arch adjacent to the aortic isthmus	Relatively common anomaly (0.04%)
	Coarctation of the aorta	ii) altered fetal hemodynamics due to an abnormal angle between the ductus and aorta or abnormal pre-ductal flow		Commonly accompanied by CHD or an arch anomaly (44–84%, m/c with bicuspid aortic valve)
	Pseudocoarctation	Failure of compression and fusion of the dorsal roots and 4^th arch segments	Segmental kinking or buckling at the aortic isthmus	Incidentally found without clinical symptoms

ALSA: aberrant left subclavian artery, CHD: congenital heart disease, LCCA: left common carotid artery, LSA: left subclavian artery, Lt: left, m/c: most common, RBCA: right brachiocephalic artery, RCCA: right common carotid artery, RSA: right subclavian artery, Rt: right.