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Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)—Implications for Surgery and Multi-Modal Imaging

Belitsis, Georgios; Schievano, Silvia; Kostolny, Martin; Cook, andrew; (2022) Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)—Implications for Surgery and Multi-Modal Imaging. Journal of Cardiovascular Development and Disease , 9 (8) , Article 262. 10.3390/jcdd9080262. Green open access

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Abstract

Abnormal aortic arches (AAAs) cover a spectrum of malformations, including abnormal laterality, branching patterns, and flow-limiting narrowing, which themselves vary from tubular hypoplasia, through discrete coarctation, to complete interruption of the arch. Neonatal surgery within the first days of life is necessary for most of these morphologies. Patch aortoplasty is widely used as it can offer a good haemodynamic result, being tailored to each combination of presenting pathologies. Our study hypothesis was that arch malformations are frequent in DORV and exhibit a plethora of phenotypes. We reviewed 54 post-mortem heart specimens from the UCL Cardiac Archive, analysing morphological features that would potentially influence the surgical repair, and taking relevant measurements of surgical importance. AAAs were found in half of the specimens, including 22.2% with aortic arch narrowing. In total, 70% and 30% of narrow arches had a subpulmonary and subaortic interventricular defect, respectively. Z-scores were significantly negative for all cases with tubular hypoplasia. We concluded that arch malformations are a common finding among hearts with DORV. Surgery on the neonatal aortic arch in DORV, performed in conjunction with other interventions that aim to balance pulmonary to systemic flow (Qp/Qs), should be anticipated and form an important part of multi-modal imaging.

Type: Article
Title: Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)—Implications for Surgery and Multi-Modal Imaging
Open access status: An open access version is available from UCL Discovery
DOI: 10.3390/jcdd9080262
Publisher version: https://doi.org/10.3390/jcdd9080262
Language: English
Additional information: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Aortic arch; patch aortoplasty; tubular hypoplasia; double outlet right ventricle; DORV; virtual reality; 3D printing
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
URI: https://discovery.ucl.ac.uk/id/eprint/10153737
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