Safety and cardiovascular behavior during pulmonary function in patients with Marfan syndrome

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dc.contributor.author Cipriano, G. F. B. [UNIFESP]
dc.contributor.author Peres, P. A. T.
dc.contributor.author Cipriano Junior, Gerson [UNIFESP]
dc.contributor.author Arena, R.
dc.contributor.author Carvalho, A. C.
dc.date.accessioned 2016-01-24T14:05:10Z
dc.date.available 2016-01-24T14:05:10Z
dc.date.issued 2010-07-01
dc.identifier http://dx.doi.org/10.1111/j.1399-0004.2010.01367.x
dc.identifier.citation Clinical Genetics. Malden: Wiley-Blackwell, v. 78, n. 1, p. 57-65, 2010.
dc.identifier.issn 0009-9163
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/32702
dc.description.abstract Marfan syndrome (MS) is a dominant autosomal connective tissue disease that impacts multiple systems, such as the cardiovascular system, tissue viscoelastic properties, bone calcification matrix and, most specific to the present investigation, pulmonary parenchyma. the aim of the present study was to evaluate pulmonary function (PF) in patients with MS and relate it to thoracic cage abnormalities (TCA) and the occurrence of cardiac arrhythmias during the spirometric exam (SE). A sample of 75 subjects (46 with MS) underwent clinical, anthropometric, echocardiographic, radiographic and PF evaluation; 51 subjects (33 with MS) had their electrocardiogram (ECG) information evaluated during PF. These individuals were matched and compared with a healthy control group (CG). Forced vital capacity (FVC) and forced expiratory volume (FEV) in the first second (FEV(1)) in the patients with MS were significantly lower in comparison with the CG (p = 0.012 and 0.0006) and predicted values (p = 0.04 and 0.003). Subgroup analysis based on TCA revealed differences between patients with MS with two combined abnormalities (scoliosis + pectus) in comparison with both the CG (p = 0.012 and 0.002) and patients without abnormalities (p = 0.05 and 0.006). There were no differences regarding the occurrence of arrhythmia during exertion on the SE. There was a correlation between clinical history, cardiovascular behavior and PF. PF is reduced in patients with MS, and deformities in the thoracic cage appear to contribute to this reduction. Despite the apparent structural alterations in the cardiovascular system in this population, exertion during the SE appears to be safe. en
dc.description.sponsorship Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)
dc.format.extent 57-65
dc.language.iso eng
dc.publisher Wiley-Blackwell
dc.relation.ispartof Clinical Genetics
dc.rights Acesso restrito
dc.subject anthropometry en
dc.subject cardiovascular system en
dc.subject Marfan syndrome en
dc.subject pulmonary function en
dc.title Safety and cardiovascular behavior during pulmonary function in patients with Marfan syndrome en
dc.type Artigo
dc.rights.license http://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.contributor.institution Virginia Commonwealth Univ
dc.description.affiliation Universidade Federal de São Paulo, Cardiol Sect, Dept Med, Div Cardiol, BR-04023002 São Paulo, Brazil
dc.description.affiliation Universidade Federal de São Paulo, Dept Surg, Div Cardiovasc Surg, BR-04023002 São Paulo, Brazil
dc.description.affiliation Virginia Commonwealth Univ, Dept Phys Therapy, Richmond, VI USA
dc.description.affiliation Virginia Commonwealth Univ, Dept Internal Med, Richmond, VI USA
dc.description.affiliation Virginia Commonwealth Univ, Dept Physiol, Richmond, VI USA
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Cardiol Sect, Dept Med, Div Cardiol, BR-04023002 São Paulo, Brazil
dc.description.affiliationUnifesp Universidade Federal de São Paulo, Dept Surg, Div Cardiovasc Surg, BR-04023002 São Paulo, Brazil
dc.identifier.doi 10.1111/j.1399-0004.2010.01367.x
dc.description.source Web of Science
dc.identifier.wos WOS:000278397300010



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