Avaliação espirométrica e cardiovascular em pacientes portadores da síndrome de Marfan

• Ano de defesa: 2010
• Autor(a) principal: Cipriano, Graziella França Bernardelli [UNIFESP]
• Tipo de documento: Tese
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Federal de São Paulo (UNIFESP)
• Palavras-chave em Português: Antropometria; Espirometria; Sistema Cardiovascular; Síndrome de Marfan
• Link de acesso: http://repositorio.unifesp.br/handle/11600/9119

Background: Marfan syndrome (MS) is a dominant autosomal connective tissue disease that impacts multiple systems, such as the musculoskeletal, ocular, cardiovascular, pulmonary, tegumentary and neurological. This mutation may produces impairment in the connective tissue which causes modifications in the vascular viscoelastic properties, tegumentary elasticity, bone calcification matrix and pulmonary parenchyma. Purpose: To evaluate pulmonary function test (PFT) in patients with MS and relate it to clinical evaluation aspects, especially possible thoracic cage abnormalities (TCA), and the occurrence of cardiac arrhythmias during the spirometric exam (SE). Method: From a sample of 75 subjects, we evaluate 46 MS patients, 29 female and aged 20±0,51 years, who was underwent clinical, anthropometric, echocardiographic, radiographic and PF evaluation; 51 subjects (33 with MS) had their electrocardiography information evaluated during PFT. These individuals were matched and compared to a healthy control group (CG). Results: Forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1) in the patients with MS were significantly lower in comparison to the CG (p=0.012 and p=0.0006) and predicted values (p=0.04 and p=0.003). Subgroup analysis based on TCA revealed differences between patients with MS with two combined abnormalities (pectus + scoliosis) in comparison to both the CG (p=0.012 and p=0.002) and patients without abnormalities (p=0.05 and p=0.006). Some aspects from clinical evaluation (Arm span to height ratio,m and axillary to xiphoid perimetry ratio,cm), cardiovascular behavior (Left ventricular mass Index-LVMI, g/m2 and Aortic Diameter- Ao,mm) and PF (FEV1/FVC%) has demonstrated a moderate correlation. There were no differences regarding the occurrence of arrhythmia during exertion on the SE. Conclusion: PF is reduced in patients with MS, and deformities in the thoracic cage appear to contribute to this reduction. Some aspects clinical, cardiovascular and PF are associated in MS. Despite the apparent structural alterations in the cardiovascular system in this young population, exertion during the spirometric exam appears to be safe in relation to electrocardiography modifications.