Marfan syndrome complicated with thoracic aorta dissection - a family case presentation

Abstract

Introduction: Marfan syndrome (MFS) is the most common inherited disorder of connective tissue affecting multiple organs: skeletal, ocular, and cardiovascular systems. The most lifethreatening and life-shortening complication is aortic dissection. Without surgery, life expectancy of MFS patients is reduced to approximately 32 years. Purpose and Objectives: The purpose of this presentation is to reveal the necessity of the early operative treatment in patients diagnosed with Marfan syndrome and the importance of screening tests in this group. Materials and methods: The report is based on the analyses of the medical history of three patients, first degree relatives, diagnosed with Marfan syndrome who were admitted and operated in the department of cardiovascular surgery for the aortic dissection. The diagnosis was based on the echographic, angiographic and computer tomography data. Results: All three patients were discharge in a good physical condition with the proper cardiac function and anticoagulant ion drugs. The follow up of the patients didn’t reveal any further complications. Conclusion: The screening of patients with Marfan syndrome for the aortic aneurysm is a useful and necessary instrument in the prevention of acute aortic dissection. The choice of the surgical procedure is based on the identification of type of the dissection, its extension and the preference of the surgeon.