Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification

Cardiovasc Pathol. 2019 Mar-Apr;39:38-50. doi: 10.1016/j.carpath.2018.11.004. Epub 2018 Dec 6.


The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta.

Keywords: Aberrant right innominate artery; Anomalous origin of pulmonary artery; Aortic arch; Aortic arch abnormality classification; Circumflex retro-esophageal aortic arch; Congenital aortic arch abnormalities; Congenital heart diseases; Double aortic arch; Left aortic arch; Persistent V aortic arch; Right aortic arch; Vascular ring.

Publication types

  • Review

MeSH terms

  • Aorta, Thoracic / abnormalities*
  • Aorta, Thoracic / diagnostic imaging
  • Clinical Decision-Making
  • Heart Defects, Congenital / classification*
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / therapy
  • Humans
  • Predictive Value of Tests
  • Prognosis
  • Terminology as Topic*
  • Vascular Malformations / classification*
  • Vascular Malformations / diagnostic imaging
  • Vascular Malformations / therapy