Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct
A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4–123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6–128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.
KeywordsCongenital heart disease Right aortic arch Aberrant left subclavian artery Vascular ring Prenatal diagnosis Tracheal compression Bronchoscopy
Right aortic arch
Aberrant left subclavian artery
Left arterial duct
We wish to thank Professor Lindsey Allan and the Late Dr Ian Huggon who instigated training of the fetal medicine team at Kings College Hospital to screen for anomalies of the great arteries and for developing understanding of the laterality of the aortic arch in the prenatal period. The foetal and paediatric cardiology team at Evelina London Children’s Hospital who have managed the patients, the cardiac MRI team who performed the cross-sectional imaging, the multidisciplinary airway team who investigated these patients and the cardiothoracic surgeons who performed the surgery.
VZ and JS proposed the study. TV, VZ and JS designed the study. TV, EK, AD and AN collated the data. TV and AD analysed the data and wrote the draft manuscript. AB, JS, KP, AD, AN and VZ critically reviewed and approved the manuscript.
Compliance with Ethical Standards
Conflict of interest
The authors declare they have no conflict of interest.
- 6.International Society of Ultrasound in Obstetrics & Gynecology, Carvalho JS, Allan LD, Chaoui R, Copel JA, DeVore GR, Hecher K, Lee W, Munoz H, Paladini D, Tutschek B, Yagel S (2013) ISUOG practice guidelines (updated): sonographic screening examination of the fetal heart. Ultrasound Obstet Gynecol 41(3):348–359. https://doi.org/10.1002/uog.12403 CrossRefGoogle Scholar
- 8.Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A, Cuneo BF, Huhta JC, Jonas RA, Krishnan A, Lacey S, Lee W, Michelfelder EC Sr, Rempel GR, Silverman NH, Spray TL, Strasburger JF, Tworetzky W, Rychik J (2014) Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 129(21):2183–2242. https://doi.org/10.1161/01.cir.0000437597.44550.5d CrossRefPubMedGoogle Scholar
- 9.Deaprtment of Health (2015) Fetal anomaly screening programme. https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/421650/FASP_Standards_April_2015_final_2_.pdf
- 20.Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW (2009) Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg 44(7):1328–1332. https://doi.org/10.1016/j.jpedsurg.2008.11.062 CrossRefPubMedGoogle Scholar
- 21.Woods RK, Sharp RJ, Holcomb GW 3rd, Snyder CL, Lofland GK, Ashcraft KW, Holder TM (2001) Vascular anomalies and tracheoesophageal compression: a single institution’s 25-year experience. Ann Thorac Surg 72(2):434–438. https://doi.org/10.1016/S0003-4975(01)02806-5 (discussion 438–439)CrossRefPubMedGoogle Scholar
- 25.Lloyd DF, van Amerom JF, Pushparajah K, Simpson JM, Zidere V, Miller O, Sharland G, Allsop J, Fox M, Lohezic M, Murgasova M, Malamateniou C, Hajnal JV, Rutherford M, Razavi R (2016) An exploration of the potential utility of fetal cardiovascular MRI as an adjunct to fetal echocardiography. Prenat Diagn 36(10):916–925. https://doi.org/10.1002/pd.4912 CrossRefPubMedPubMedCentralGoogle Scholar
- 26.Donnelly LF, Fleck RJ, Pacharn P, Ziegler MA, Fricke BL, Cotton RT (2002) Aberrant subclavian arteries: cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression. Am J Roentgenol 178(5):1269–1274. https://doi.org/10.2214/ajr.178.5.1781269 CrossRefGoogle Scholar
- 27.Williamson JP, Armstrong JJ, McLaughlin RA, Noble PB, West AR, Becker S, Curatolo A, Noffsinger WJ, Mitchell HW, Phillips MJ, Sampson DD, Hillman DR, Eastwood PR (2010) Measuring airway dimensions during bronchoscopy using anatomical optical coherence tomography. Eur Respir J 35(1):34–41. https://doi.org/10.1183/09031936.00041809 CrossRefPubMedGoogle Scholar