Shone's complex and aortic dissection: case report and review of a rare, underdiagnosed congenital heart disease.
Aortic aneurysm
Aortic dissection
Bentall procedure
Congenital heart disease
LVOT obstruction
MAZE procedure
Patient prosthesis mismatch
Shone’s complex
Shone’s syndrome
Journal
Journal of cardiothoracic surgery
ISSN: 1749-8090
Titre abrégé: J Cardiothorac Surg
Pays: England
ID NLM: 101265113
Informations de publication
Date de publication:
23 Feb 2022
23 Feb 2022
Historique:
received:
19
08
2021
accepted:
09
02
2022
entrez:
24
2
2022
pubmed:
25
2
2022
medline:
26
2
2022
Statut:
epublish
Résumé
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. 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. 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.
Sections du résumé
BACKGROUND
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
METHODS
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
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.
Identifiants
pubmed: 35197098
doi: 10.1186/s13019-022-01768-z
pii: 10.1186/s13019-022-01768-z
pmc: PMC8864782
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
21Informations de copyright
© 2022. The Author(s).
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