RESUMO

Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.

Assuntos

Aorta/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Dissecção Aórtica/diagnóstico por imagem , Síndrome de Marfan/diagnóstico por imagem , Gêmeos Monozigóticos , Adulto , Dissecção Aórtica/etiologia , Aorta/patologia , Aneurisma Aórtico/etiologia , Atenolol/uso terapêutico , Humanos , Masculino , Síndrome de Marfan/complicações , Ultrassonografia