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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2011-03-01
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| Series: | Journal of Tehran University Heart Center |
| Subjects: | |
| Online Access: | https://jthc.tums.ac.ir/index.php/jthc/article/view/198 |
| _version_ | 1818110537629696000 |
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| author | Mohammad Hossein Davari Toba Kazemi Hossein Alimirzaei Mohamad Reza Rezvani |
| author_facet | Mohammad Hossein Davari Toba Kazemi Hossein Alimirzaei Mohamad Reza Rezvani |
| author_sort | Mohammad Hossein Davari |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-11T02:48:44Z |
| format | Article |
| id | doaj.art-68ef7ba1982641e4a875979da623e326 |
| institution | Directory Open Access Journal |
| issn | 1735-8620 2008-2371 |
| language | English |
| last_indexed | 2024-12-11T02:48:44Z |
| publishDate | 2011-03-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Journal of Tehran University Heart Center |
| spelling | doaj.art-68ef7ba1982641e4a875979da623e3262022-12-22T01:23:21ZengTehran University of Medical SciencesJournal of Tehran University Heart Center1735-86202008-23712011-03-0161196Cardiovascular Manifestation of a Family with Marfan’s SyndromeMohammad Hossein Davari0Toba Kazemi1Hossein Alimirzaei2Mohamad Reza Rezvani3Birjand Cardiovascular Research Center, Birjand University of Medical Sciences, Birjand, Iran.Birjand Cardiovascular Research Center, Birjand University of Medical Sciences, Birjand, Iran.Vali Asr Hospital, Birjand University of Medical Sciences, Birjand, Iran. Birjand Cardiovascular Research Center, Birjand University of Medical Sciences, Birjand, Iran.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.https://jthc.tums.ac.ir/index.php/jthc/article/view/198Marfan syndromeConnective tissue diseasesFamilyCardiac manifestation |
| spellingShingle | Mohammad Hossein Davari Toba Kazemi Hossein Alimirzaei Mohamad Reza Rezvani Cardiovascular Manifestation of a Family with Marfan’s Syndrome Journal of Tehran University Heart Center Marfan syndrome Connective tissue diseases Family Cardiac manifestation |
| 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 | Marfan syndrome Connective tissue diseases Family Cardiac manifestation |
| url | https://jthc.tums.ac.ir/index.php/jthc/article/view/198 |
| work_keys_str_mv | AT mohammadhosseindavari cardiovascularmanifestationofafamilywithmarfanssyndrome AT tobakazemi cardiovascularmanifestationofafamilywithmarfanssyndrome AT hosseinalimirzaei cardiovascularmanifestationofafamilywithmarfanssyndrome AT mohamadrezarezvani cardiovascularmanifestationofafamilywithmarfanssyndrome |