Cardiovascular manifestation of a family with Marfan's syndrome

ABSTRACT

Marfan's syndrome [MFS] is a genetic disorder associated with autosomal dominant inheritance. In MSF, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS [father, two daughters, three sons, and five grandchildren]. The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mirtal regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive