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Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease

BACKGROUND: Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later i...

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Autores principales: Sinfield, Steven, Ranasinghe, Sachini, Wang, Stephani, Mendoza, Fernando, Khoynezhad, Ali
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864782/
https://www.ncbi.nlm.nih.gov/pubmed/35197098
http://dx.doi.org/10.1186/s13019-022-01768-z
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author Sinfield, Steven
Ranasinghe, Sachini
Wang, Stephani
Mendoza, Fernando
Khoynezhad, Ali
author_facet Sinfield, Steven
Ranasinghe, Sachini
Wang, Stephani
Mendoza, Fernando
Khoynezhad, Ali
author_sort Sinfield, Steven
collection PubMed
description BACKGROUND: Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later into adulthood. This increased survival comes with a new set of medical complications that providers need to be aware of. CASE PRESENTATION: A 27 year old man with a complex cardiac history including an incomplete Shone’s complex and persistent symptomatic atrial flutter presented with sharp chest pain radiating to his back. He was found to have type A aortic dissection on imaging in the setting of severe patient-prosthesis mismatch. He had multiple valvular surgeries in childhood. The patient was being followed-up as an outpatient for an enlarging chronic aortic aneurysm and was non-compliant with his medications. He was taken emergently to the operating room for a skirted Bentall procedure, aortic valve replacement, and right sided MAZE. CONCLUSIONS: Shone’s complex is a rare congenital heart disease associated with significant morbidities including atrial flutter, patient-prosthesis mismatch, and aortic dissection. As patients continue to live longer into adulthood with this disease, it is important to raise awareness of this rare syndrome for providers and highlight its potential complications. Further research is needed to determine appropriate guidelines for when to intervene on aortopathy-associated CHD.
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spelling pubmed-88647822022-02-23 Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease Sinfield, Steven Ranasinghe, Sachini Wang, Stephani Mendoza, Fernando Khoynezhad, Ali J Cardiothorac Surg Case Report BACKGROUND: Shone’s complex is a rare congenital heart disease consisting of a variety of left ventricular inflow and outflow tract lesions. Patients typically present in childhood requiring early surgical intervention; however, with improved surgical techniques, these patients are surviving later into adulthood. This increased survival comes with a new set of medical complications that providers need to be aware of. CASE PRESENTATION: A 27 year old man with a complex cardiac history including an incomplete Shone’s complex and persistent symptomatic atrial flutter presented with sharp chest pain radiating to his back. He was found to have type A aortic dissection on imaging in the setting of severe patient-prosthesis mismatch. He had multiple valvular surgeries in childhood. The patient was being followed-up as an outpatient for an enlarging chronic aortic aneurysm and was non-compliant with his medications. He was taken emergently to the operating room for a skirted Bentall procedure, aortic valve replacement, and right sided MAZE. CONCLUSIONS: Shone’s complex is a rare congenital heart disease associated with significant morbidities including atrial flutter, patient-prosthesis mismatch, and aortic dissection. As patients continue to live longer into adulthood with this disease, it is important to raise awareness of this rare syndrome for providers and highlight its potential complications. Further research is needed to determine appropriate guidelines for when to intervene on aortopathy-associated CHD. BioMed Central 2022-02-23 /pmc/articles/PMC8864782/ /pubmed/35197098 http://dx.doi.org/10.1186/s13019-022-01768-z Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Sinfield, Steven
Ranasinghe, Sachini
Wang, Stephani
Mendoza, Fernando
Khoynezhad, Ali
Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title_full Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title_fullStr Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title_full_unstemmed Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title_short Shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
title_sort shone’s complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864782/
https://www.ncbi.nlm.nih.gov/pubmed/35197098
http://dx.doi.org/10.1186/s13019-022-01768-z
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