Portal de Periódicos da CAPES

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

2010; Wiley; Linguagem: Inglês

10.1111/j.1399-0004.2009.01367.x

1399-0004

Graziella França Bernardelli Cipriano, Paulo Alberto Tayar Peres, Gerson Cipriano, Ross Arena, Antônio Carlos Pires Carvalho,

Cardiovascular Function and Risk Factors

Clinical GeneticsVolume 78, Issue 1 p. 57-65 Safety and cardiovascular behavior during pulmonary function in patients with Marfan syndrome GFB Cipriano, Corresponding Author GFB Cipriano Cardiology Division, Department of MedicineGraziella França Bernardelli Cipriano, Medicine Department, Cardiology Section, Federal University of São Paulo, Napoleão de Barros St., 715, 10° Floor, 04023-002, São Paulo, SP, Brazil.Tel.: +55 11 5576 4127;fax: +55 11 5571 0047;e-mail: grafb@uol.com.brSearch for more papers by this authorPAT Peres, PAT Peres Cardiology Division, Department of MedicineSearch for more papers by this authorG Cipriano Jr, G Cipriano Jr Cardiovascular Surgery Division, Department of Surgery, Federal University of São Paulo, São Paulo, BrazilSearch for more papers by this authorR Arena, R Arena Departments of Physical Therapy, Internal Medicine and Physiology, Virginia Commonwealth University, Richmond, VI, USASearch for more papers by this authorAC Carvalho, AC Carvalho Cardiology Division, Department of MedicineSearch for more papers by this author GFB Cipriano, Corresponding Author GFB Cipriano Cardiology Division, Department of MedicineGraziella França Bernardelli Cipriano, Medicine Department, Cardiology Section, Federal University of São Paulo, Napoleão de Barros St., 715, 10° Floor, 04023-002, São Paulo, SP, Brazil.Tel.: +55 11 5576 4127;fax: +55 11 5571 0047;e-mail: grafb@uol.com.brSearch for more papers by this authorPAT Peres, PAT Peres Cardiology Division, Department of MedicineSearch for more papers by this authorG Cipriano Jr, G Cipriano Jr Cardiovascular Surgery Division, Department of Surgery, Federal University of São Paulo, São Paulo, BrazilSearch for more papers by this authorR Arena, R Arena Departments of Physical Therapy, Internal Medicine and Physiology, Virginia Commonwealth University, Richmond, VI, USASearch for more papers by this authorAC Carvalho, AC Carvalho Cardiology Division, Department of MedicineSearch for more papers by this author First published: 07 June 2010 https://doi.org/10.1111/j.1399-0004.2010.01367.xCitations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Abstract Cipriano GFB, Peres PAT, Cipriano G Jr, Arena R, Carvalho AC. Safety and cardiovascular behavior during pulmonary function in patients with Marfan syndrome. 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 (FEV1) 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. Citing Literature Volume78, Issue1July 2010Pages 57-65 RelatedInformation