Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population

Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue. Timely diagnosis and effective therapy are essential for individuals with Marfan syndrome, as they are susceptible to severe cardiovascular consequences, including aortic aneurysms and aortic dissection. The conve...

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Main Author: Patrycja Jędrzejewska-Rzezak
Format: Article
Language:English
Published: Nicolaus Copernicus University in Toruń 2025-01-01
Series:Quality in Sport
Subjects:
Online Access:https://apcz.umk.pl/QS/article/view/57713
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author Patrycja Jędrzejewska-Rzezak
author_facet Patrycja Jędrzejewska-Rzezak
author_sort Patrycja Jędrzejewska-Rzezak
collection DOAJ
description Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue. Timely diagnosis and effective therapy are essential for individuals with Marfan syndrome, as they are susceptible to severe cardiovascular consequences, including aortic aneurysms and aortic dissection. The conventional treatment comprises beta-blockers to mitigate the dilatation of the aorta and aortic surgery. The efficacy of contemporary medicinal and surgical interventions in Marfan syndrome has significantly enhanced mean life expectancy. International guidelines generally discourage physical activity for individuals with Marfan syndrome. Recent recommendations have created exclusions for these patients, indicating benefits from engaging in low-intensity physical activity exclusively. Aim of the study: The primary aim of this research is to elucidate the most recent management ideas pertaining to Marfan syndrome. Significant emphasis is placed on the importance and safety of physical activity in individuals afflicted with this disease. The risks linked to physical activity are addressed, however the advantages of particular activities are highlighted. Materials and methods: A review of the literature available in the PubMed database was performed, using the key words: „Marfan syndrome", „connective tissue disease”,  „physical activity", „aortic aneurysm”, „FBN1”. Conclusion: The prognosis for patients with Marfan syndrome has markedly improved in the last years. Contrary to conventional guidance to refrain from physical activity, emerging research indicates that low-intensity exercise may be advantageous. Research on the safety and health implications of physical activity in patients with Marfan syndrome is exceedingly few. Current information indicates that moderate physical activity at a particular intensity may be safe in this condition. Additional study is necessary to offer targeted recommendations for patient training and enhance their long-term quality of life.
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spelling doaj-art-8afba81a2ee94640950293c5fcad08a82025-01-28T08:25:05ZengNicolaus Copernicus University in ToruńQuality in Sport2450-31182025-01-013710.12775/QS.2024.37.57713Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient PopulationPatrycja Jędrzejewska-Rzezak0https://orcid.org/0000-0003-2144-5810Katolicki Uniwersytet Lubelski Introduction: Marfan syndrome is an autosomal dominant disorder of connective tissue. Timely diagnosis and effective therapy are essential for individuals with Marfan syndrome, as they are susceptible to severe cardiovascular consequences, including aortic aneurysms and aortic dissection. The conventional treatment comprises beta-blockers to mitigate the dilatation of the aorta and aortic surgery. The efficacy of contemporary medicinal and surgical interventions in Marfan syndrome has significantly enhanced mean life expectancy. International guidelines generally discourage physical activity for individuals with Marfan syndrome. Recent recommendations have created exclusions for these patients, indicating benefits from engaging in low-intensity physical activity exclusively. Aim of the study: The primary aim of this research is to elucidate the most recent management ideas pertaining to Marfan syndrome. Significant emphasis is placed on the importance and safety of physical activity in individuals afflicted with this disease. The risks linked to physical activity are addressed, however the advantages of particular activities are highlighted. Materials and methods: A review of the literature available in the PubMed database was performed, using the key words: „Marfan syndrome", „connective tissue disease”,  „physical activity", „aortic aneurysm”, „FBN1”. Conclusion: The prognosis for patients with Marfan syndrome has markedly improved in the last years. Contrary to conventional guidance to refrain from physical activity, emerging research indicates that low-intensity exercise may be advantageous. Research on the safety and health implications of physical activity in patients with Marfan syndrome is exceedingly few. Current information indicates that moderate physical activity at a particular intensity may be safe in this condition. Additional study is necessary to offer targeted recommendations for patient training and enhance their long-term quality of life. https://apcz.umk.pl/QS/article/view/57713Marfan's syndormeConnective Tissue Diseasesphysical activityaortic aneurysmFBN1
spellingShingle Patrycja Jędrzejewska-Rzezak
Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
Quality in Sport
Marfan's syndorme
Connective Tissue Diseases
physical activity
aortic aneurysm
FBN1
title Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
title_full Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
title_fullStr Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
title_full_unstemmed Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
title_short Management of Marfan Syndrome, with a Specific Focus on the Significance of Physical Activity in this Patient Population
title_sort management of marfan syndrome with a specific focus on the significance of physical activity in this patient population
topic Marfan's syndorme
Connective Tissue Diseases
physical activity
aortic aneurysm
FBN1
url https://apcz.umk.pl/QS/article/view/57713
work_keys_str_mv AT patrycjajedrzejewskarzezak managementofmarfansyndromewithaspecificfocusonthesignificanceofphysicalactivityinthispatientpopulation