Almeida, T

Janeiro, MC

Martins, D

Santos, N

BMJ Case Reports

English

Repositório do Hospital Prof. Doutor Fernando Fonseca

1Santos N, et al. BMJ Case Rep 2020;13:e233793. doi:10.1136/bcr-2019-233793Pulmonary artery sling: a rare cause of stridor and respiratory distressNádia Santos,1 Teresa Almeida,1 Maria Carlos Janeiro   ,2 Duarte Martins   3 Images in…To cite: Santos N, Almeida T, Janeiro MC, et al. BMJ Case Rep 2020;13:e233793. doi:10.1136/bcr-2019-2337931Department of Pediatrics, Hospital do Espirito Santo EPE, Evora, Portugal2Department of Pediatrics, Hospital Professor Doutor Fernando Fonseca EPE, Amadora, Lisboa, Portugal3Paediatric Cardiology, Centro Hospitalar de Lisboa Ocidental EPE, Lisboa, PortugalCorrespondence toDr Nádia Santos;   nadiasantos15@ hotmail. comAccepted 29 January 2020© BMJ Publishing Group Limited 2020. Re- use permitted under CC BY- NC. No commercial re- use. See rights and permissions. Published by BMJ.Figure 1 Transverse CT image after intravenous administration of iodinated contrast confirms the diagnosis of left pulmonary artery sling (LPAS; yellow arrow) arising from the right pulmonary artery (arrowhead).Figure 2 A 3D- reconstructed CT image (posterior view) shows LPAS arising from the right pulmonary artery (RPA) crossing behind the trachea (Tr) causing stenosis. LPAS, left pulmonary artery sling.DesCripTionA newborn boy, late preterm, with an uneventful pregnancy, presented with stridor and a systolic murmur since birth. A neonatal transthoracic echo-cardiogram revealed a patent ductus arteriosus and ostium secundum atrial septal defect. During the first year of life, he presented recurrent respira-tory distress, cough, wheezing, stridor and multiple respiratory infections. For more than once, he required non- invasive respiratory support. Daily medication included inhaled steroids and montelu-kast but with poor response. He had an adequate development and physical growth, without feeding problems. A bronchoscopy was performed and a right distal tracheobronchial stenosis was identified.At 18 months old, he was referenced to a tertiary paediatric cardiology centre, after the CT angi-ography showed an anomalous origin of the left pulmonary artery (LPA). The LPA originated from the proximal right pulmonary artery, coursing over the right mainstem bronchus, posterior to the distal trachea and anterior to the oesophagus, causing an airway compression (figures 1 and 2).He underwent surgical repair of the pulmonary artery sling (PAS) by median sternotomy. The LPA was repositioned to the left side of the main pulmo-nary artery, in the correct anatomical position, and the ductus arteriosus was ligated, under cardiopul-monary bypass, without tracheoplasty.Postoperative bronchoscopy showed an improve-ment in the distal trachea caliber, without inspiratory collapse and incomplete cartilaginous rings. He was extubated on the first postoperative day showing a regression of the stridor, despite persistent respi-ratory distress. The patient was discharged 8 days after surgery and maintained a regular follow- up with an improvement of respiratory symptoms.This rare form of vascular ring is known as PAS, which has an estimated prevalence of 59 per million school- aged children, but it is an underdi-agnosed condition or misdiagnosed as respiratory pathology.1 Paediatricians must be aware of this cause of respiratory distress and have a high clin-ical suspicion index. It is associated with respiratory symptoms in the first year of life, due to tracheal stenosis, which can be life- threatening.1–3 Patients with tracheomalacia, recurrent or persistent stridor and/or wheezing should be evaluated for vascular rings, to allow an early diagnosis and timely surgical intervention, in order to avoid morbidity and mortality in children.Retrospectively, the chest X- rays showed a narrowing and right indentation of the distal trachea (figure 3), which might have suggested the diagnosis.Although echocardiography may be a helpful imaging tool, it is sometimes insufficient to estab-lish this diagnosis due to its low sensitivity.2 CT angiography is the gold standard imaging technique providing not only an accurate diagnostic eval-uation but also the best guidance for the surgical approach.3Associated cardiovascular malformations are frequent, with a reported prevalence of up to 50%.3 This includes patent ductus arteriosus, which may contribute to encirclement of the trachea.In symptomatic patients, the early reimplantation of the LPA to the anatomic position is effective. The 2 Santos N, et al. BMJ Case Rep 2020;13:e233793. doi:10.1136/bcr-2019-233793images in…Figure 3 Chest X- rays show a narrowing and right indentation of the distal trachea.Copyright 2020 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visithttps://www.bmj.com/company/products-services/rights-and-licensing/permissions/BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.Become a Fellow of BMJ Case Reports today and you can: ► Submit as many cases as you like ► Enjoy fast sympathetic peer review and rapid publication of accepted articles ► Access all the published articles ► Re-use any of the published material for personal use and teaching without further permissionCustomer serviceIf you have any further queries about your subscription, please contact our customer services team on +44 (0) 207111 1105 or via email at support@bmj.com.Visit casereports.bmj.com for more articles like this and to become a Fellowclinical outcome is generally good, depending mostly on associ-ated tracheal or/and cardiac anomalies.Learning points ► Patients with recurrent and/or prolonged stridor and wheeze should be examined for the possible presence of vascular rings and slings, such as pulmonary artery sling. ► There must be a high degree of clinical suspicion regarding this diagnosis because it is a rare cause of respiratory distress. ► CT angiography is the gold standard imaging technique. Early surgical management in symptomatic patients is effective.Contributors NS: substantial contributions to the conception or design of the work, or the acquisition, analysis or interpretation of data. Drafting the work and revising it critically for important intellectual content. Final approval of the version published. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. TA: substantial contributions to the conception or design of the work, or the acquisition, analysis or interpretation of data. Drafting the work. Final approval of the version published. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MCJ, DM: revising it critically for important intellectual content. Final approval of the version published. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not- for- profit sectors.Competing interests None declared.patient consent for publication Parental/guardian consent obtained.provenance and peer review Not commissioned; externally peer reviewed.open access This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY- NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non- commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non- commercial. See: http:// creativecommons. org/ licenses/ by- nc/ 4. 0/.orCiD iDsMaria Carlos Janeiro http:// orcid. org/ 0000- 0003- 2245- 5738Duarte Martins http:// orcid. org/ 0000- 0003- 0662- 5629RefeRences 1 Yu J- M, Liao C- P, Ge S, et al. The prevalence and clinical impact of pulmonary artery sling on school- aged children: a large- scale screening study. Pediatr Pulmonol 2008;43:656–61. 2 Delacour D, Demeyere M, Dubourg B, et al. Left pulmonary artery sling: a rare cause of congenital stridor. Diagn Interv Imaging 2017;98:85–7. 3 Xie J, Juan Y- H, Wang Q, et al. Evaluation of left pulmonary artery sling, associated cardiovascular anomalies, and surgical outcomes using cardiovascular computed tomography angiography. Sci Rep 2017;7:1–10.

Pulmonary artery sling: a rare cause of stridor and respiratory distress

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Pulmonary artery sling: a rare cause of stridor and respiratory distress

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