Hostname: page-component-cd9895bd7-hc48f Total loading time: 0 Render date: 2024-12-23T12:19:13.448Z Has data issue: false hasContentIssue false
  • English
  • Français

Persistent fifth aortic arch: the “great pretender” in clinical practice

Published online by Cambridge University Press:  30 August 2017

David F. A. Lloyd ,
S. Y. Ho ,
Kuberan Pushparajah ,
Subhasis Chakraborty ,
Mohamed Nasser ,
Hideki Uemura ,
Rodney Franklin  and
Alan G. Magee
David F. A. Lloyd*
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
S. Y. Ho
Affiliation:
Cardiac Morphology Unit, Royal Brompton Hospital, London, United Kingdom
Kuberan Pushparajah
Affiliation:
Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
Subhasis Chakraborty
Affiliation:
Paediatric Radiology Department, John Radcliffe Hospital, Oxford, United Kingdom
Mohamed Nasser
Affiliation:
Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
Hideki Uemura
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
Rodney Franklin
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
Alan G. Magee
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
*
Correspondence to: Dr D. Lloyd, Department of Congenital Heart Disease, Evelina Children’s Hospital, 6th Floor, London, SE1 7TH, United Kingdom. Tel: 02071888552; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Persistence of the embryonic “fifth aortic arch” in postnatal life is a rare, enigmatic – and at times controversial – condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the “great pretender” by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of “persistent fifth aortic arch” while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

Keywords

CHDcoarctation of the aortaarterial ductpersistent fifth aortic arch
Type
Review Article
Information
Cardiology in the Young , Volume 28 , Issue 2 , February 2018 , pp. 175 - 181
Check for updates
Copyright
© Cambridge University Press 2017 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Bamforth, SD, Chaudhry, B, Bennett, M, et al. Clarification of the identity of the mammalian fifth pharyngeal arch artery. Clin Anat 2013; 26: 173–182.Google Scholar
2. Van Praagh, R, Van Praagh, S. Persistent fifth arterial arch in man. Congenital double-lumen aortic arch. Am J Cardiol 1969; 24: 279–282.CrossRefGoogle ScholarPubMed
3. Izukawa, T, Scott, ME, Durrani, F, Moes, CA. Persistent left fifth aortic arch in man. Report of two cases. Br Heart J 1973; 35: 1190–1195.CrossRefGoogle ScholarPubMed
4. Moes, CA, Izukawa, T. Persistent fifth arterial arch. Diagnosis during life, with postmortem confirmation. Radiology 1974; 111: 175–176.CrossRefGoogle ScholarPubMed
5. Lawrence, TY, Stiles, QR. Persistent fifth aortic arch in man. Am J Dis Child 1975; 129: 1229–1231.Google ScholarPubMed
6. Anzai, T, Konishi, T, Kawabe, M. Persistent fifth aortic arch left subclavian aneurysm in an adult. Jpn J Surg 1982; 12: 414–418.CrossRefGoogle ScholarPubMed
7. Cabrera, A, Galdeano, J, Lekuona, I. Persistent left sided fifth aortic arch in a neonate. Br Heart J 1985; 54: 105–106.CrossRefGoogle ScholarPubMed
8. Culham, JA, Reed, MH. Persistent fifth aortic arch with coarctation of the aorta. Cardiovasc Intervent Radiol 1985; 8: 137–139.CrossRefGoogle ScholarPubMed
9. Herrera, MA, D’Souza, VJ, Link, KM, Weesner, KM, Formanek, AG. A persistent fifth aortic arch in man: a double-lumen aortic arch (presentation of a new case and review of the literature). Pediatr Cardiol 1987; 8: 265–269.CrossRefGoogle Scholar
10. Gerlis, LM, Dickinson, DF, Wilson, N, Gibbs, JL. Persistent fifth aortic arch. A report of two new cases and a review of the literature. Int J Cardiol 1987; 16: 185–192.CrossRefGoogle Scholar
11. Gerlis, LM, Ho, SY, Anderson, RH, Da Costa, P. Persistent 5th aortic arch–a great pretender: three new covert cases. Int J Cardiol 1989; 23: 239–247.CrossRefGoogle ScholarPubMed
12. Freedom, RM, Yoo, S-J, Mikailian, H, Williams, WG. The Natural and Modified History of Congenital Heart Disease. Wiley-Blackwell, New York, NY, 2008.Google Scholar
13. Gupta, SK, Gulati, GS, Anderson, RH. Clarifying the anatomy of the fifth arch artery. Ann Pediatr Cardiol 2016; 9: 62–67.CrossRefGoogle ScholarPubMed
14. Geva, T, Ray, RA, Santini, F, Van Praagh, S, Van Praagh, R. Asymptomatic persistent fifth aortic arch (congenital double-lumen aortic arch) in an adult. Am J Cardiol 1990; 65: 1406–1407.CrossRefGoogle ScholarPubMed
15. Kirsch, J, Julsrud, PR. Magnetic resonance angiography of an ipsilateral double aortic arch due to persistent left fourth and fifth aortic arches. Pediatr Radiol 2007; 37: 501–502.CrossRefGoogle ScholarPubMed
16. Atsumi, N, Moriki, N, Sakakibara, Y, Mitsui, T, Horigome, H, Kamma, H. Persistent fifth aortic arch associated with type A aortic arch interruption. Histological study and morphogenesis. Jpn J Thorac Cardiovasc Surg 2001; 49: 509–512.CrossRefGoogle ScholarPubMed
17. Isomatsu, Y, Takanashi, Y, Terada, M, Kasama, K. Persistent fifth aortic arch and fourth arch interruption in a 28-year-old woman. Pediatr Cardiol 2004; 25: 696–698.CrossRefGoogle ScholarPubMed
18. Zhao, YH, Su, ZK, Liu, JF, Cao, DF, Ding, WX. Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch. Ann Thorac Surg 2007; 84: 1016–1019.CrossRefGoogle ScholarPubMed
19. Carroll, SJ, Ferris, A, Chen, J, Liberman, L. Efficacy of prostaglandin E1 in relieving obstruction in coarctation of a persistent fifth aortic arch without opening the ductus arteriosus. Pediatr Cardiol 2006; 27: 766–768.CrossRefGoogle ScholarPubMed
20. Uysal, F, Bostan, OM, Cil, E. Coarctation of persistent 5th aortic arch: first report of catheter-based intervention. Tex Heart Inst J 2014; 41: 411–413.CrossRefGoogle ScholarPubMed
21. Santoro, G, Caianiello, G, Palladino, MT, Iacono, C, Russo, MG, Calabro, R. Aortic coarctation with persistent fifth left aortic arch. Int J Cardiol 2009; 136: e33–e34.CrossRefGoogle ScholarPubMed
22. Yoshii, S, Matsukawa, T, Hosaka, S, Ueno, A, Tsuji, A. Repair of coarctation with persistent fifth arterial arch and atresia of the fourth aortic arch. J Cardiovasc Surg (Torino) 1990; 31: 812–814.Google ScholarPubMed
23. Lim, C, Kim, WH, Kim, SC, Lee, JY, Kim, SJ, Kim, YM. Truncus arteriosus with coarctation of persistent fifth aortic arch. Ann Thorac Surg 2002; 74: 1702–1704.CrossRefGoogle ScholarPubMed
24. Parmar, RC, Pillai, S, Kulkarni, S, Sivaraman, A. Type I persistent left fifth aortic arch with truncus arteriosus type A3: an unreported association. Pediatr Cardiol 2004; 25: 432–433.CrossRefGoogle ScholarPubMed
25. Zhong, Y, Jaffe, RB, Zhu, M, Sun, A, Li, Y, Gao, W. Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children. Pediatr Radiol 2007; 37: 256–263.CrossRefGoogle ScholarPubMed
26. Tomita, H, Fuse, S, Chiba, S. Coarctation of persistent right fifth aortic arch and pulmonary sequestration. Cardiol Young 1998; 8: 509–511.CrossRefGoogle ScholarPubMed
27. Suda, K, Matsumura, M, Matsumoto, M. Balloon dilation of the stenotic fifth aortic arch in a newborn with double lumen aortic arch. Heart 2004; 90: 245.CrossRefGoogle Scholar
28. Chiu, CC, Wu, JR, Chen, HM, Lin, YT. Persistent fifth aortic arch: an ignored and underestimated disease. Jpn Heart J 2000; 41: 665–671.CrossRefGoogle ScholarPubMed
29. Hwang, MS, Chang, YS, Chu, JJ, Su, WJ. Isolated persistent fifth aortic arch with systemic-to-pulmonary arterial connection. J Thorac Cardiovasc Surg 2003; 126: 1643–1644.CrossRefGoogle ScholarPubMed
30. Jowett, V, Rubens, M, Ho, SY, Uemura, H, Gardiner, HM. Prenatal visualization of persistent 5th aortic arch artery. JACC Cardiovasc Imaging 2012; 5: 1288–1289.CrossRefGoogle ScholarPubMed
31. Boshoff, D, Gewillig, M. A review of the options for treatment of major aortopulmonary collateral arteries in the setting of tetralogy of Fallot with pulmonary atresia. Cardiol Young 2006; 16: 212–220.CrossRefGoogle ScholarPubMed
32. Yoo, SJ, Moes, CA, Burrows, PE, Molossi, S, Freedom, RM. Pulmonary blood supply by a branch from the distal ascending aorta in pulmonary atresia with ventricular septal defect: differential diagnosis of fifth aortic arch. Pediatr Cardiol 1993; 14: 230–233.CrossRefGoogle ScholarPubMed
33. Serra, AJ, Chamie, F, Freedom, RM. Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? A 5th aortic arch with a systemic-to-pulmonary arterial connection. Cardiol Young 2000; 10: 419–422.CrossRefGoogle Scholar
34. Lee, ML, Tsao, LY, Wang, BT, Lee, MH, Chiu, IS. Maternally inherited unbalanced translocation of chromosome 22 in a 5-day-old neonate with persistent fifth aortic arch and tetralogy of Fallot. Int J Cardiol 2003; 90: 337–339.CrossRefGoogle Scholar
35. Donti, A, Soavi, N, Sabbatani, P, Picchio, FM. Persistent left fifth aortic arch associated with tetralogy of Fallot. Pediatr Cardiol 1997; 18: 229–231.CrossRefGoogle ScholarPubMed
36. Koch, A, Ludwig, J, Zink, S, Singer, H. Isolated left pulmonary artery: interventional stenting of a persistent fifth aortic arch. Catheter Cardiovasc Interv 2007; 70: 105–109.CrossRefGoogle ScholarPubMed
37. Lee, ML, Chen, HN, Chen, M, et al. Persistent fifth aortic arch associated with 22q11.2 deletion syndrome. J Formos Med Assoc 2006; 105: 284–289.CrossRefGoogle ScholarPubMed
38. Zartner, P, Schneider, MB, Bein, G. Prostaglandin E1 sensitive persistent fifth aortic arch type 2. Heart 2000; 84: 142.CrossRefGoogle ScholarPubMed
39. Wang, JN, Wu, JM, Yang, YJ. Double-lumen aortic arch with anomalous left pulmonary artery origin from the main pulmonary artery – bilateral persistent fifth aortic arch – a case report. Int J Cardiol 1999; 69: 105–108.CrossRefGoogle ScholarPubMed
40. Bernheimer, J, Friedberg, M, Chan, F, Silverman, N. Echocardiographic diagnosis of persistent fifth aortic arch. Echocardiography 2007; 24: 258–262.CrossRefGoogle ScholarPubMed
41. Tehrai, M, Saidi, B, Goudarzi, M. Multi-detector computed tomography demonstration of double-lumen aortic arch – persistent fifth arch – as an isolated anomaly in an adult. Cardiol Young. 2012; 22: 353–355.CrossRefGoogle ScholarPubMed
42. Anderson, RH, Chaudhry, B, Mohun, TJ, et al. Normal and abnormal development of the intrapericardial arterial trunks in humans and mice. Cardiovasc Res 2012; 95: 108–115.CrossRefGoogle ScholarPubMed
43. Lorandeau, CG, Hakkinen, LA, Moore, CS. Cardiovascular development and survival during gestation in the Ts65Dn mouse model for Down syndrome. Anat Rec (Hoboken) 2011; 294: 93–101.Google Scholar
44. Gupta, SK, Bamforth, SD, Anderson, RH. How frequent is the fifth arch artery? Cardiol Young 2015; 25: 628–646.CrossRefGoogle ScholarPubMed