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Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms
Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BMJ Publishing Group
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4215278/ https://www.ncbi.nlm.nih.gov/pubmed/24986892 http://dx.doi.org/10.1136/heartjnl-2013-305132 |
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author | Treasure, Tom Takkenberg, J J M Pepper, John |
author_facet | Treasure, Tom Takkenberg, J J M Pepper, John |
author_sort | Treasure, Tom |
collection | PubMed |
description | Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered. |
format | Online Article Text |
id | pubmed-4215278 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BMJ Publishing Group |
record_format | MEDLINE/PubMed |
spelling | pubmed-42152782014-11-13 Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms Treasure, Tom Takkenberg, J J M Pepper, John Heart Review Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered. BMJ Publishing Group 2014-10-15 2014-07-01 /pmc/articles/PMC4215278/ /pubmed/24986892 http://dx.doi.org/10.1136/heartjnl-2013-305132 Text en Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 3.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/3.0/ |
spellingShingle | Review Treasure, Tom Takkenberg, J J M Pepper, John Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title | Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title_full | Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title_fullStr | Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title_full_unstemmed | Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title_short | Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms |
title_sort | surgical management of aortic root disease in marfan syndrome and other congenital disorders associated with aortic root aneurysms |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4215278/ https://www.ncbi.nlm.nih.gov/pubmed/24986892 http://dx.doi.org/10.1136/heartjnl-2013-305132 |
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