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.
Bibliographical noteFunding Information:
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. The authors declare they have no conflict of interest.
© 2018, Springer Science+Business Media, LLC, part of Springer Nature.
- Aberrant left subclavian artery
- Congenital heart disease
- Prenatal diagnosis
- Right aortic arch
- Tracheal compression
- Vascular ring