Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management
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 sten...
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| Format: | Article |
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MDPI AG
2023-08-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/12/16/5357 |
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| author | Massimiliano Cantinotti Pietro Marchese Nadia Assanta Eliana Franchi Vitali Pak Elisa Barberi Alessandra Pizzuto Giuseppe Santoro Raffaele Giordano |
| author_facet | Massimiliano Cantinotti Pietro Marchese Nadia Assanta Eliana Franchi Vitali Pak Elisa Barberi Alessandra Pizzuto Giuseppe Santoro Raffaele Giordano |
| author_sort | Massimiliano Cantinotti |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-09T10:55:43Z |
| format | Article |
| id | doaj.art-d1afb0b55ce34656a83fd5c78378e633 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T10:55:43Z |
| publishDate | 2023-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-d1afb0b55ce34656a83fd5c78378e6332023-12-01T01:42:55ZengMDPI AGJournal of Clinical Medicine2077-03832023-08-011216535710.3390/jcm12165357Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and ManagementMassimiliano Cantinotti0Pietro Marchese1Nadia Assanta2Eliana Franchi3Vitali Pak4Elisa Barberi5Alessandra Pizzuto6Giuseppe Santoro7Raffaele Giordano8Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyFondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, ItalyAdult and Pediatric Cardiac Surgery, Department Advanced Biomedical Sciences, University of Naples “Federico II”, 80138 Napoli, ItalyWe 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.https://www.mdpi.com/2077-0383/12/16/5357thrombosishypoplastic left heart syndromepediatric cardiology |
| spellingShingle | Massimiliano Cantinotti Pietro Marchese Nadia Assanta Eliana Franchi Vitali Pak Elisa Barberi Alessandra Pizzuto Giuseppe Santoro Raffaele Giordano Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management Journal of Clinical Medicine thrombosis hypoplastic left heart syndrome pediatric cardiology |
| title | Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management |
| title_full | Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management |
| title_fullStr | Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management |
| title_full_unstemmed | Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management |
| title_short | Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management |
| title_sort | native aortic root thrombosis in hypoplastic left heart syndrome an unusual presentation soon after atrial septal stenting of a relatively unusual complication experience and literature review with an outlook to diagnosis and management |
| topic | thrombosis hypoplastic left heart syndrome pediatric cardiology |
| url | https://www.mdpi.com/2077-0383/12/16/5357 |
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