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Neo-aortic valve insufficiency (Neo-AI) represents an emerging complication with implications for long-term survival. This study investigated the clinical impact and patterns of Neo-AI development following staged palliation. Neonates undergoing the Norwood procedure (2001\u20132023) were retrospectively reviewed. Neo-AI was evaluated using serial echocardiograms, with patients classified into two groups: early onset (during the Norwood hospitalization) and late onset (after the Norwood discharge). Neo-aortic root dimensions were measured at four time points during staged palliation, with Z-scores calculated for patient growth. Among 378 patients, significant Neo-AI developed in 30 (7.9%) during median follow-up of 4.3 years. Early onset neo-AI occurred in 13 patients (43.3%) and late onset neo-AI in 17 (56.7%). Cox regression revealed 7.9-fold increased mortality risk in early onset patients (hazard ratios 7.87,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.009), with 5-year survival of 38.5% versus 87.8% in late onset patients. All early onset deaths occurred within the first year. Late onset neo-AI patients demonstrated progressive sinus of Valsalva dilatation (annual progression 0.33 Z-score units). At Fontan completion, late onset neo-AI patients had larger Z-scores than early onset patients [5.14 vs. 2.18,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.006], while surviving early onset patients demonstrated Neo-AI improvement after bidirectional cavopulmonary shunt with subsequent stable root dimensions. Early onset neo-AI associates with acute hemodynamic compromise and higher perioperative mortality. Late onset neo-AI demonstrates progressive root dilatation with superior survival, raising concerns for future valve intervention.\n                  <\/jats:p>","DOI":"10.1007\/s00246-026-04205-3","type":"journal-article","created":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T08:41:13Z","timestamp":1773477673000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Neo-Aortic Valve Insufficiency and Root Dilatation During and After the Staged Palliation for Hypoplastic Left Heart Syndrome and Variants"],"prefix":"10.1007","author":[{"given":"Muneaki","family":"Matsubara","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iulia","family":"Telescu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thibault","family":"Schaeffer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teresa","family":"Lemmen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"R\u00f6hlig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonas","family":"Palm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul Philipp","family":"Heinisch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Piber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfred","family":"Hager","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Ewert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"H\u00f6rer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masamichi","family":"Ono","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,14]]},"reference":[{"key":"4205_CR1","doi-asserted-by":"publisher","first-page":"988","DOI":"10.1016\/j.athoracsur.2005.09.065","volume":"81","author":"BM Cardis","year":"2006","unstructured":"Cardis BM, Fyfe DA, Mahle WT (2006) Elastic properties of the reconstructed aorta in hypoplastic left heart syndrome. 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The study was approved by the Institutional Review Board of the Technical University of Munich (Approval Number 2025-232-S-NP on May 05, 2025).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}},{"value":"The requirement for individual patient consent was waived due to the retrospective design.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to Participate"}}]}}