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

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

Author Cipriano, G. F. B. Autor UNIFESP Google Scholar
Peres, P. A. T. Google Scholar
Cipriano Junior, Gerson Autor UNIFESP Google Scholar
Arena, R. Google Scholar
Carvalho, A. C. Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Virginia Commonwealth Univ
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.
Keywords anthropometry
cardiovascular system
Marfan syndrome
pulmonary function
Language English
Sponsor Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)
Date 2010-07-01
Published in Clinical Genetics. Malden: Wiley-Blackwell, v. 78, n. 1, p. 57-65, 2010.
ISSN 0009-9163 (Sherpa/Romeo, impact factor)
Publisher Wiley-Blackwell
Extent 57-65
Access rights Closed access
Type Article
Web of Science ID WOS:000278397300010

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