Marfan syndrome, inherited aortopathies and exercise: What is the right answer?

Cheng, Andrew; Owens, David S.

doi:10.1136/bjsports-2014-306440rep

Cited by 21 publications

(19 citation statements)

References 35 publications

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“…Once the diagnosis of aortic aneurysm has been con rmed in patients with MFS, intense physical activity such as weightlifting should be avoided due to the potential risk inducing rupture or dissection of the aortic aneurysm [12] Meanwhile, regular follow-up is necessary. Concurrently, conservative management is aimed at decreasing the heart rate and lowering the blood pressure to reduce the hemodynamic stress on the proximal aorta [13].…”

Section: Discussionmentioning

confidence: 99%

The deadly “bulging balloon” within the mediastinum: a case of a giant aortic root aneurysm in a young patient with Marfan syndrome

Shen

Gan

Rajaguru

et al. 2020

Preprint

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Introduction: Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age. Case: The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed six-foot-three inches of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. The transthoracic echocardiography (TTE) and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we successfully performed the Bentall procedure based on the intraoperative exploration results. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later. Conclusion It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. The composite replacement of the aortic valve and ascending aorta should be performed if the patient is not suitable for valve-sparing operation.

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Section: Discussionmentioning

confidence: 99%

The deadly “bulging balloon” within the mediastinum: a case of a giant aortic root aneurysm in a young patient with Marfan syndrome

Shen

Gan

Rajaguru

et al. 2020

Preprint

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“…La resistencia vascular periférica y la presión arterial diastólica tienden a disminuir durante el ejercicio dinámico, pero aumentan durante el ejercicio estático. Así, Cheng y Owens 39 concluyen que el ejercicio dinámico de intensidad baja-moderada puede ser beneficioso para pacientes con SM 38 . En el mismo sentido, Dennison y Certo 21 describen que los cambios hemodinámicos causados por el ejercicio, especialmente el ejercicio intenso, pueden aumentar la tensión y el estrés en la pared debilitada de un aneurisma y dar lugar a la ruptura de la misma.…”

Section: Criterios De Exclusiónunclassified

“…El ejercicio es muy importante para los pacientes con SM ya que, además de promover el sentido físico y mental, mejora la capacidad física, controla los niveles de presión arterial, aumenta la densidad ósea y previene enfermedades degenerativas como la obesidad, la diabetes y la aterosclerosis [39][40][41] . Se recomienda que los pacientes con SM no superen el 50% de su capacidad aeróbica y no sobrepasen un mayor ritmo cardíaco de 110 latidos por minuto 22,42 .…”

Section: Criterios De Exclusiónunclassified

Efectos del ejercicio físico en pacientes con síndrome de Marfán (revisión documental 2000-2016)

Flórez¹,

Giraldo²,

Calero³

et al. 2018

Duazary

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El síndrome de Marfán (SM) es un trastorno autosómico dominante del tejido conectivo que rodea a diferentes sistemas. El objetivo principal de este estudio es determinar los efectos del ejercicio físico en pacientes con SM reportados en la literatura durante el periodo 2000-2016. Así, se llevó a cabo una recopilación de artículos científicos provenientes de páginas indexadas en francés, inglés, español y portugués, que se referían al ejercicio físico en pacientes con SM. Los resultados arrojados por los diferentes autores, según se pudo comprobar, dieron a conocer los beneficios y las alteraciones que trae la realización de ejercicio físico en estos pacientes a nivel cardiopulmonar. Teniendo en cuenta los resultados de la revisión, que concluyen de forma unánime que el ejercicio de alta intensidad no debe ser prescrito para pacientes con SM, se concluyó que hacen falta guías de manejo que permitan realizar la prescripción del ejercicio y que expongan de manera específica los parámetros de la misma.

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“…These benefits lead to a greater success in performing activities of daily living (ADL) and thereby enhances quality of life 9,10 . The physical demands and impact on the cardiovascular system vary greatly between different types of sports, and a distinction between two types of sports is therefore emphasized: sports with a high dynamic component, such as long‐distance running, and sports with a high static component, such as weightlifting 11,12 . Whereas dynamic sports and exercises present only a modest increase in BP, more static sports and exercises are characterized by a greater increase in both BP and HR at the same oxygen uptake level.…”

Section: Introductionmentioning

confidence: 99%

“…These surges in blood pressure, which can be exaggerated with Valsalva during exercise, lead to a significantly higher strain and wall stress on the heart and aorta than during a corresponding amount of dynamic exercise. The stress and strain increase with rising BP and aortic diameter 12‐14 …”

Section: Introductionmentioning

confidence: 99%

Marfan syndrome and exercise: A literature review

2020

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Marfan syndrome (MFS) is an autosomal dominant condition caused by a mutation in the fibrillin‐1–producing gene within the connective tissue, which can affect multiple organ systems negatively, including those that play a vital role (eg, heart and vasculature) in physical activity. The most fatal manifestations of MFS are aortic dilation, dissection, and rupture but to what extent regular physical activity will influence this is sparsely described. The current guidelines for MFS and physical training are based on limited research, and this review aims to summarize human and animal studies on exercise and its influence upon MFS presentation. Most studies found positive effects of regular mild to moderate aerobic exercise upon aorta expansion, as well as upon dilation and hypertrophy of the left ventricle together with a positive training response upon aerobic capacity. However, from the present studies, it is not possible to conclude regarding optimal intensity, frequency, duration, and type of exercise. Overall, the guidelines for exercise in MFS patients have so far due to limited research been relative conservative, despite the fact that at least moderate exercise in combination with tight cardiac monitoring does not demonstrate any detrimental effect upon MFS presentation.

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Marfan syndrome, inherited aortopathies and exercise: What is the right answer?

Cited by 21 publications

References 35 publications

The deadly “bulging balloon” within the mediastinum: a case of a giant aortic root aneurysm in a young patient with Marfan syndrome

The deadly “bulging balloon” within the mediastinum: a case of a giant aortic root aneurysm in a young patient with Marfan syndrome

Efectos del ejercicio físico en pacientes con síndrome de Marfán (revisión documental 2000-2016)

Marfan syndrome and exercise: A literature review

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