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Pre-congress course at the 13th World Congress on Endometriosis (WCE2017)
Date: 17 May 2017 
Location:  Vancouver, Canada 
Course Directors: George Condous (Australia) and Sony Singh (Canada) 

A higher incidence of ARSA has been observed in cardiac catheterization studies in Down’s syndrome series, during surgery for congenital cardiac defects (CHD)5 or in fetal echocardiography, underlining its association with chromosomal abnormalities and cardiac defects.^6-7

The first observation of ARSA in fetuses with trisomy 21 was reported by Chaoui et al.⁸ In one of the few prenatal studies on routine population, conduced at first and second trimester screening, there was found a 20% association with other fetal abnormalities, the majority of which were genetic and cardiac anomalies.³

The most frequent genetic abnormalities associated to ARSA are chromosomal aberrations, especially trisomy 21 and trisomy 18. Although in trisomy 21 fetuses ARSA is usually associated to other typical 1st or 2nd trimester ultrasonographic markers of the syndrome^8-12  however it has been also reported as isolated US marker for Down’s syndrome.³.

The combination of ARSA with a cardiac defect, especially conotruncal anomaly, increases the risk for 22q11 microdeletion.^{6, 7, 13}

1.Yoo SJ, Min JY, Lee YH, Roman K, Jaeggi E, Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 2003;22:535–546.

2.Chaoui R, Rake A, Heling KS. Aortic arch with four vessels: aberrant right subclavian artery. Ultrasound Obstet Gynecol 2008;31:115–117

3.Rembouskos G, Passamonti U, De Robertis V, Tempesta A, Volpe G, Gentile M, Volpe P. Aberrant right subclavian artery (ARSA) in unselected population at first and second trimester ultrasonography. Prenat Diagn. 2012 Oct;32(10):968-75.

4.Edwards JE. Vascular rings and slings. In: Moller JH, Neal WA, eds. Fetal, Neonatal, and Infant Cardiac Disease. Norwalk, CT: Appleton &Lange, 1990:745–754.

5.Zapata H, Edwards JE, Titus JL. Aberrant right subclavian artery with left aortic arch: associated cardiac anomalies. Pediatr Cardiol 1993;14:159–61

6.Boudjemline Y, Fermont L, Le Bidois J, Lyonnet S, Sidi D, Bonnet D. Prevalence of 22q11 deletion in fetuses with conotruncal cardiac defects: a 6-year prospective study. J Pediatr 2001; 138: 520–524.

7. Volpe P, Marasini M, Caruso G, Marzullo A, Buonadonna AL, Arciprete P, Di Paolo S, Volpe G, Gentile M. 22q11 deletions in fetuses with malformations of the outflow tracts or interruption of the aortic arch: impact of additional ultrasound signs. Prenat Diagn 2003; 23: 752–757.

8.Chaoui R, Heling KS, Sarioglu N, et al. Aberrant right subclavian artery as a new cardiac sign in second- and third-trimester fetuses with Down syndrome. Am J Obstet Gynecol 2005;192:257-63.

9.Borenstein M, Cavoretto P, Allan L, et al. Aberrant right subclavian artery at 11 + 0 to 13 + 6 weeks of gestation in chromosomally normal and abnormal fetuses. Ultrasound Obstet Gynecol 2008;31:20-4.

10. Borenstein M, Minekawa R, Zidere V, et al. Aberrant right subclavian artery at 16 to 23 + 6 weeks of gestation: a marker for chromosomal abnormality. Ultrasound Obstet Gynecol 2010;36:548-52

11.Chaoui R, Thiel G, Heling KS. Prevalence of an aberrant right subclavian artery (ARSA) in normal fetuses: a new soft marker for trisomy 21 risk assessment. Ultrasound Obstet Gynecol 2005;26:356.

12.Paladini D, Sglavo G, Pastore G, et al. Aberrant Right Subclavian Artery (ARSA). Incidence and correlation with other markers of Down syndrome in 2(nd) trimester fetuses. Ultrasound Obstet Gynecol 2012;39(2):191-5.

13. Zalel Y, Achiron R, Yagel S, Kivilevitch Z. Fetal aberrant right subclavian artery in normal and Down syndrome fetuses. Ultrasound Obstet Gynecol 2008;31:25-9.

14.Tuo G, Volpe P, Bava GL, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiology 103:416-19: 2009

15.Kent PD, Poterucha TH. Images in clinical medicine. Aberrant right subclavian artery and dysphagia lusoria. N Engl J Med 2002; 347: 1636

16.Rauch R, Rauch A, Koch A, et al. Laterality of the aortic arch and anomalies of the subclavian artery-reliable indicators for 22q11.2 deletion syndromes? Eur J Pediatr 2004;163:642-5.

17.McElhinney DB, Clark BJ 3rd, Weinberg PM, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001;37:2114-9.

This article should be cited as: Rembouskos G, Volpe P. Aberrant Right Subclavian Artery VISUOG, July 2014