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Cardiovascular Manifestation of a Family with Marfan’s Syndrome
Marfan’s syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three son...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Tehran University of Medical Sciences
2011
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3466859/ https://www.ncbi.nlm.nih.gov/pubmed/23074603 |
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author | Davari, Mohammad Hossein Kazemi, Toba Alimirzaei, Hossein Rezvani, Mohamad Reza |
author_facet | Davari, Mohammad Hossein Kazemi, Toba Alimirzaei, Hossein Rezvani, Mohamad Reza |
author_sort | Davari, Mohammad Hossein |
collection | PubMed |
description | Marfan’s syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years’ follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive. |
format | Online Article Text |
id | pubmed-3466859 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Tehran University of Medical Sciences |
record_format | MEDLINE/PubMed |
spelling | pubmed-34668592012-10-16 Cardiovascular Manifestation of a Family with Marfan’s Syndrome Davari, Mohammad Hossein Kazemi, Toba Alimirzaei, Hossein Rezvani, Mohamad Reza J Tehran Heart Cent Case Report Marfan’s syndrome (MFS) is a genetic disorder associated with autosomal dominant inheritance. In MFS, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS (father, two daughters, three sons, and five grandchildren). The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mitral regurgitation. At ten years’ follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive. Tehran University of Medical Sciences 2011 2011-02-28 /pmc/articles/PMC3466859/ /pubmed/23074603 Text en Copyright © Tehran Heart Center, Tehran University of Medical Sciences http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0), which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly. |
spellingShingle | Case Report Davari, Mohammad Hossein Kazemi, Toba Alimirzaei, Hossein Rezvani, Mohamad Reza Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title | Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title_full | Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title_fullStr | Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title_full_unstemmed | Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title_short | Cardiovascular Manifestation of a Family with Marfan’s Syndrome |
title_sort | cardiovascular manifestation of a family with marfan’s syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3466859/ https://www.ncbi.nlm.nih.gov/pubmed/23074603 |
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