{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,22]],"date-time":"2025-03-22T04:10:40Z","timestamp":1742616640617,"version":"3.40.2"},"reference-count":21,"publisher":"Cambridge University Press (CUP)","issue":"2","license":[{"start":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T00:00:00Z","timestamp":1732233600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951124026131_as1\"><jats:title>Objectives:<\/jats:title><jats:p>We sought to examine the relative importance of surgical lesion complexity versus the presence of genetic\/syndromic\/extracardiac anomalies (GSAs) in determining survival, morbidity or need for reinterventions following repair for aortic arch hypoplasia.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026131_as2\"><jats:title>Methods:<\/jats:title><jats:p>A single-centre, retrospective cohort study of infants undergoing biventricular aortic arch repair <jats:italic>via<\/jats:italic> sternotomy from 2010 to 2021 was conducted. Survival analysis was performed using Kaplan\u2013Meier methods, with additional Bayesian survival modelling for subgroups. Composite morbidity comprised respiratory, renal, neurologic, or sepsis-related complications.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026131_as3\"><jats:title>Results:<\/jats:title><jats:p>Of 83 included infants, <jats:italic>n<\/jats:italic> = 13\/83 (15.7%) had complex repairs; 27\/83 (32.5%) were GSA+. Operative mortality was significantly higher in GSA+ versus GSA\u2212 patients (18.5% vs. 1.8%; p = 0.01), though not for complex versus non-complex repairs. Overall 10-year Kaplan\u2013Meier survival was 86.7%. Bayesian modelling suggested equivalent post-discharge attrition in non-complex\/GSA+ and complex\/GSA\u2212 patients, with the poorest outcomes in complex\/GSA+ patients; non-complex\/GSA\u2212 patients had 100% survival. GSA+ patients exhibited higher composite morbidity (44.4% vs. 7.1% in GSA\u2212 p &lt; 0.001), with their mode of death seemingly related to a high incidence of respiratory and neurological morbidity, notably in Dandy\u2013Walker syndrome. The 10-year freedom from arch reinterventions was 87.7%; neither complexity, GSA status, nor post-repair peak arch velocity predicted the need for arch reinterventions.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026131_as4\"><jats:title>Conclusions:<\/jats:title><jats:p>Whilst anatomic complexity may have been somewhat neutralised as a risk factor for operative mortality, in contrast to GSA+ status, there is further post-discharge attrition attributable to complexity or GSA+ status, with additive risk effects. Morbidity directly related to certain syndromes underlies some of this risk. Non-anatomic substrates represent a persistent limitation to outcomes of surgical aortic arch repair in infants.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951124026131","type":"journal-article","created":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T09:36:38Z","timestamp":1732268198000},"page":"253-260","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":0,"title":["Anatomic and non-anatomic substrates in infants with two ventricles undergoing aortic arch repair"],"prefix":"10.1017","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7383-2666","authenticated-orcid":false,"given":"Bari","family":"Murtuza","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan","family":"Lehoux","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario 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O.","family":"Morell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2024,11,22]]},"reference":[{"key":"S1047951124026131_ref10","doi-asserted-by":"publisher","DOI":"10.1093\/icvts\/ivac135"},{"key":"S1047951124026131_ref8","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.005864"},{"key":"S1047951124026131_ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2014.04.051"},{"key":"S1047951124026131_ref3","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2018.04.043"},{"key":"S1047951124026131_ref19","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2018.04.025"},{"key":"S1047951124026131_ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2020.05.062"},{"key":"S1047951124026131_ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2016.04.040"},{"key":"S1047951124026131_ref18","doi-asserted-by":"publisher","DOI":"10.1159\/000028968"},{"key":"S1047951124026131_ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2019.01.069"},{"key":"S1047951124026131_ref6","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)34738-5"},{"key":"S1047951124026131_ref9","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951121003747"},{"key":"S1047951124026131_ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2023.02.010"},{"key":"S1047951124026131_ref17","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2017.04.019"},{"key":"S1047951124026131_ref2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2021.11.032"},{"key":"S1047951124026131_ref16","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2022.06.053"},{"key":"S1047951124026131_ref7","doi-asserted-by":"publisher","DOI":"10.1053\/j.semtcvs.2017.11.004"},{"key":"S1047951124026131_ref21","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-021-02749-0"},{"key":"S1047951124026131_ref1","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(99)01144-3"},{"key":"S1047951124026131_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2014.07.029"},{"key":"S1047951124026131_ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2014.05.007"},{"key":"S1047951124026131_ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2020.04.091"}],"container-title":["Cardiology 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