Hostname: page-component-54dcc4c588-tfzs5 Total loading time: 0 Render date: 2025-09-27T23:33:15.511Z Has data issue: false hasContentIssue false

Ductal stenting for hypoplastic left heart syndrome with right aortic arch associated with 20q11.2 microdeletion syndrome

Published online by Cambridge University Press:  23 September 2025

Yoko Kawai*
Affiliation:
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
Yusuke Kita
Affiliation:
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
Shinichiro Oda
Affiliation:
Department of Cardiothoracic Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan
*
Corresponding author: Yoko Kawai; Email: y-kiwi@koto.kpu-m.ac.jp

Abstract

Hypoplastic left heart syndrome with a right aortic arch is rare, and performing the Norwood procedure in these cases is challenging. Here, we describe a 3-month-old patient with hypoplastic left heart syndrome having a right aortic arch accompanied by a 20q11.2 microdeletion. Because of cardiac dysfunction, ductal stenting was performed to avoid an early infantile Norwood procedure.

Information

Type
Brief Report
Copyright
© The Author(s), 2025. Published by Cambridge University Press

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.)

Article purchase

Temporarily unavailable

References

Patel, PM, Bartlett, HL, Scholz, T, Burzynski, J, Behrendt, DM, Burkhart, HM. Norwood modification in a patient with hypoplastic left heart and a right aortic arch. J Thorac Cardiovasc Surg 2007; 134: 10651066.10.1016/j.jtcvs.2007.04.066CrossRefGoogle Scholar
Matsushima, M, Oshima, Y, Matsuhisa, H. Aortic arch reconstruction using a Kommerell diverticulum for hypoplastic left heart syndrome with a right aortic arch. J Thorac Cardiovasc Surg 2016; 152: e35e37.10.1016/j.jtcvs.2016.01.057CrossRefGoogle ScholarPubMed
Rychik, J, Szwast, A, Natarajan, S, et al. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound Obstet Gynecol 2010; 36: 465470.10.1002/uog.7674CrossRefGoogle ScholarPubMed
DiBardino, DJ, McElhinney, DB, Marshall, AC, Bacha, EA. A review of ductal stenting in hypoplastic left heart syndrome: bridge to transplantation and hybrid stage I palliation. Pediatr Cardiol 2008; 29: 251257.10.1007/s00246-007-0012-0CrossRefGoogle ScholarPubMed
Jedraszak, G, Demeer, B, Mathieu-Dramard, M, et al. Clinical and molecular characterization of the 20q11.2 microdeletion syndrome: six new patients. Am J Med Genet Part A 2015; 167A: 504511.10.1002/ajmg.a.36882CrossRefGoogle ScholarPubMed
Pierpont, ME, Brueckner, M, Chung, WK, et al. Genetic basis for congenital heart disease: revisited: a scientific statement from the American heart association. Circulation 2018; 138: e653e711.10.1161/CIR.0000000000000606CrossRefGoogle ScholarPubMed
Zhang, L, Huang, R, Zhou, H, et al. Prenatal diagnosis in fetal right aortic arch using chromosomal microarray analysis and whole exome sequencing: a chinese single-center retrospective study. Mol Cytogenet 2024; 17: 22.10.1186/s13039-024-00691-3CrossRefGoogle ScholarPubMed
McBride, KL, Pignatelli, R, Lewin, M, et al. Inheritance analysis of congenital left ventricular outflow tract obstruction malformations: segregation, multiplex relative risk, and heritability. Am J Med Genet A 2005; 134A: 180186.10.1002/ajmg.a.30602CrossRefGoogle ScholarPubMed
Liu, X, Yagi, H, Saeed, S, et al. The complex genetics of hypoplastic left heart syndrome. Nat Genet 2017; 49: 11521159.10.1038/ng.3870CrossRefGoogle ScholarPubMed