eprintid: 10153737 rev_number: 8 eprint_status: archive userid: 699 dir: disk0/10/15/37/37 datestamp: 2022-08-16 13:44:44 lastmod: 2024-11-06 15:15:58 status_changed: 2022-08-16 13:44:44 type: article metadata_visibility: show sword_depositor: 699 creators_name: Belitsis, Georgios creators_name: Schievano, Silvia creators_name: Kostolny, Martin creators_name: Cook, andrew title: Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)—Implications for Surgery and Multi-Modal Imaging ispublished: pub subjects: GOSH divisions: UCL divisions: D14 divisions: B02 keywords: Aortic arch; patch aortoplasty; tubular hypoplasia; double outlet right ventricle; DORV; virtual reality; 3D printing note: 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. 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. date: 2022-08 date_type: published official_url: https://doi.org/10.3390/jcdd9080262 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1970102 doi: 10.3390/jcdd9080262 lyricists_name: Belitsis, Georgios lyricists_id: GBELI47 actors_name: Belitsis, Georgios actors_id: GBELI47 actors_role: owner funding_acknowledgements: G.B. has been supported by the British Heart Foundation (PG/16/99/32572). [University College London] full_text_status: public publication: Journal of Cardiovascular Development and Disease volume: 9 number: 8 article_number: 262 citation: 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 <https://doi.org/10.3390/jcdd9080262>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10153737/1/jcdd-09-00262.pdf