Hostname: page-component-7bb8b95d7b-dvmhs Total loading time: 0 Render date: 2024-09-23T04:52:48.889Z Has data issue: false hasContentIssue false
  • English
  • Français

Incidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat–Wilson syndrome

Part of: Cardiac Morphology

Published online by Cambridge University Press:  29 June 2018

Juan D. Cano Sierra Open the ORCID record for Juan D. Cano Sierra [Opens in a new window] ,
Camilo F. Mestra  and
Miguel A. Ronderos Dumit
Juan D. Cano Sierra*
Affiliation:
Pediatric Cardiology Hospitalist, Fundación Cardioinfantil, Bogotá, Colombia
Camilo F. Mestra
Affiliation:
Pediatric Cardiology Department, Universidad Del Rosario, Bogotá, Colombia
Miguel A. Ronderos Dumit
Affiliation:
Pediatric Interventional Cardiology Department, Fundación Cardioinfantil, Bogotá, Colombia
*
Author for correspondence: J. D. Cano Sierra, Pediatric Cardiology Hospitalist, Fundación Cardioinfantil, Cl. 163a #13B-60, Bogotá, Colombia. Tel: +057 318 209 8921; Fax: +057 667 2737; E-mail: csdavid0413@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Mowat–Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat–Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat–Wilson syndrome than in the general population.

Keywords

Mowat–Wilson syndrome6th aortic archZEB2pulmonary arterial slinggeneral cardiology
Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 8 , August 2018 , pp. 1074 - 1076
Check for updates
Copyright
© Cambridge University Press 2018 

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.)

Footnotes

Cite this article: Cano Sierra JD, Mestra CF, Ronderos Dumit MA. (2018) Incidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat–Wilson syndrome. Cardiology in the Young28: 1074–1076. doi: 10.1017/S1047951118000689

References

1. Yamada, Y, Nomura, N, Yamada, K, et al. The spectrum of ZEB2 mutations causing the Mowat-Wilson syndrome in Japanese populations. Am J Med Genet A 2014; 164: 1899–1908.CrossRefGoogle Scholar
2. Ivanovski, I, Djuric, O, Giuseppe Caraffi, S, Santodirocco, D. Phenotype and genotype of 87 patients with Mowat–Wilson syndrome and recommendations for care. Genet Med 2018; 2018 Jan 4. doi: 10.1038/gim.2017.221. [Epub ahead of print].Google Scholar
3. Contro, S, Miller, RA, White, H, Potts, WJ. Bronchial obstruction due to pulmonary artery anomalies. II. Pulmonary artery aneurysm. Circulation 1958; 17: 424–427.CrossRefGoogle ScholarPubMed
4. Erickson, L, Cocalis, M, George, L. Partial anomalous left pulmonary artery: new evidence on the development of the pulmonary artery sling. Pediatr Cardiol 1996; 17: 319–321.Google Scholar
5. Kenneth, J, Gunay, R, Kurt, A, et al. Anomalous origin of the left pulmonary artery from the right pulmonary artery. Report of 2 cases and review of the literature. Am J Roentgenol 1965; 95: 598–610.Google Scholar
6. Molin, DGM, Poelmann, RE, DeRuiter, MC, et al. Transforming growth factor β-SMAD2 signaling regulates aortic arch innervation and development. Circ Res 2004; 95: 1109–1117.Google Scholar
7. Yu, JM, Liao, CP, 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: 656–661.Google Scholar
8. Lamouille, S, Xu, J, Derynck, R. Molecular mechanisms of epithelial-mesenchymal transition. Natl Rev Mol Cell Biol 2014; 15: 178–196.Google Scholar