{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T15:27:25Z","timestamp":1781796445586,"version":"3.54.5"},"reference-count":15,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T00:00:00Z","timestamp":1692230400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>We started with the experience of thrombus formation in the native aorta of a 3-year-old male child with hypoplastic left heart syndrome (HLHS) and severely hypoplastic but patent mitral and aortic valves after Glenn palliation, which occurred soon after left heart decompression by percutaneous stenting of the atrial septum. The diagnosis was incidental, with the child completely asymptomatic, and progressively subsided in a few days with heparin infusion and chronic warfarin therapy. We reviewed the incidence, diagnosis, and management of native aortic thrombosis in HLHS after different stages of Fontan palliation through a systematic literature search. In all 32 cases, native aortic thrombosis in HLHS was found. The HLHS anatomic subtypes included mitral stenosis\/aortic stenosis (fourteen cases or 45.2%), mitral stenosis\/aortic atresia (eleven cases or 35.5%), and mitral atresia\/aortic atresia (four cases or 12.9%). The age at diagnosis ranged from 13 days to 18 years. Clinical presentation varied from incidental findings, chest pain and\/or electrocardiographic abnormalities, cardiac arrest, and transient ischemic attack. Diagnosis was feasible in most of the cases with only transthoracic echocardiography. Mostly (59.4%), patients were treated with anticoagulation, while others underwent surgical (18.7%), direct (12.5%), or systemic (9.3%) thrombolysis. Transplant-free survival was 56.2%, and fatal events occurred in 25%. Major events occurred in 26.3% of those treated with anticoagulation, in 33.3% of patients treated with surgical\/systemic thrombolysis, and in 100% of patients treated with direct thrombolysis. In summary, native aortic thrombosis in HLHS may occur at different ages, with a wide spectrum of presentation from incidental finding to a sudden major event. Diagnosis is feasible with transthoracic echocardiography, and management with anticoagulation is effective despite the incidence of major events remaining high.<\/jats:p>","DOI":"10.3390\/jcm12165357","type":"journal-article","created":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T10:47:02Z","timestamp":1692269222000},"page":"5357","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication\u2014Experience and Literature Review with an Outlook to Diagnosis and Management"],"prefix":"10.3390","volume":"12","author":[{"given":"Massimiliano","family":"Cantinotti","sequence":"first","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4801-608X","authenticated-orcid":false,"given":"Pietro","family":"Marchese","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Assanta","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliana","family":"Franchi","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vitali","family":"Pak","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Barberi","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Pizzuto","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6342-7843","authenticated-orcid":false,"given":"Giuseppe","family":"Santoro","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Giordano","sequence":"additional","affiliation":[{"name":"Adult and Pediatric Cardiac Surgery, Department Advanced Biomedical Sciences, University of Naples \u201cFederico II\u201d, 80138 Napoli, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,8,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1330","DOI":"10.1017\/S1047951121004789","article-title":"Spontaneous thrombus formation in native aortic root in patients with hypoplastic left heart syndrome: Clinical presentation, treatment, and outcomes","volume":"32","author":"Abraham","year":"2022","journal-title":"Cardiol. Young"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1007\/s00246-005-1274-z","article-title":"Thrombus formation in the native aortic root in patients with hypoplastic left heart syndrome","volume":"27","author":"Owens","year":"2006","journal-title":"Pediatr. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1177\/1076029617739702","article-title":"Incidence and Management of Thrombotic and Thromboembolic Complications Following the Superior Cavopulmonary Anastomosis Procedure: A Literature Review","volume":"24","author":"Agarwal","year":"2017","journal-title":"Clin. Appl. Thromb. Hemost."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e737S","DOI":"10.1378\/chest.11-2308","article-title":"Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines","volume":"14","author":"Monagle","year":"2012","journal-title":"Chest"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"72","DOI":"10.4103\/apc.APC_103_20","article-title":"Myocardial infarction due to thrombosis of native aorta late after Fontan procedure for hypoplastic left heart syndrome","volume":"14","author":"Patel","year":"2021","journal-title":"Ann. Pediatr. Cardiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e29","DOI":"10.1016\/j.athoracsur.2006.07.068","article-title":"Neoaortic root modification for late thrombosis after Norwood palliation","volume":"82","author":"Graham","year":"2006","journal-title":"Ann. Thorac. 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Cardiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.case.2021.07.007","article-title":"Native Aortic Root Thrombus in 3-Year-Old Fontan Patient with Hypoplastic Left Heart Syndrome: Presentation and Echocardiographic Findings of This Life-Threatening Complication","volume":"5","author":"Segar","year":"2021","journal-title":"CASE"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1016\/S0003-4975(97)01041-2","article-title":"Causes of death after the modified Norwood procedure: A study of 122 postmortem cases","volume":"64","author":"Bartram","year":"1997","journal-title":"Ann. Thorac. Surg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.jcct.2016.03.003","article-title":"Thrombus in hypoplastic aorta: An uncommon cause of acute myocardial infarction","volume":"10","author":"Keraliya","year":"2016","journal-title":"J. Cardiovasc. Comput. Tomogr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1017\/S1047951107000340","article-title":"Formation of thrombus in a native aortic sinus of Valsalva after palliation of hypoplastic left heart syndrome","volume":"17","author":"Mookerjee","year":"2007","journal-title":"Cardiol. 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