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Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients

BACKGROUND: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has genera...

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Autores principales: Rijnberg, Friso M., Sojak, Vladimir, Blom, Nico A., Hazekamp, Mark G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6193207/
https://www.ncbi.nlm.nih.gov/pubmed/30134770
http://dx.doi.org/10.1177/2150135118793087
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author Rijnberg, Friso M.
Sojak, Vladimir
Blom, Nico A.
Hazekamp, Mark G.
author_facet Rijnberg, Friso M.
Sojak, Vladimir
Blom, Nico A.
Hazekamp, Mark G.
author_sort Rijnberg, Friso M.
collection PubMed
description BACKGROUND: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients. METHODS: Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016. RESULTS: Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure. CONCLUSIONS: Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis.
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spelling pubmed-61932072018-10-24 Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients Rijnberg, Friso M. Sojak, Vladimir Blom, Nico A. Hazekamp, Mark G. World J Pediatr Congenit Heart Surg Original Articles BACKGROUND: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients. METHODS: Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016. RESULTS: Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure. CONCLUSIONS: Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis. SAGE Publications 2018-08-22 2018-11 /pmc/articles/PMC6193207/ /pubmed/30134770 http://dx.doi.org/10.1177/2150135118793087 Text en © The Author(s) 2018 http://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Articles
Rijnberg, Friso M.
Sojak, Vladimir
Blom, Nico A.
Hazekamp, Mark G.
Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title_full Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title_fullStr Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title_full_unstemmed Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title_short Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients
title_sort long-term outcome of direct relief of subaortic stenosis in single ventricle patients
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6193207/
https://www.ncbi.nlm.nih.gov/pubmed/30134770
http://dx.doi.org/10.1177/2150135118793087
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