Marfan’s syndrome with aneurysmal formation of the aortic root and ascending aorta concomitant with aortic valve regurgitation of patients undergoing Bentall operation: the first two cases in southern Thailand

Abstract

The aorta is considered as pathologically dilated when the diameter of the aortic root and ascending aorta exceed the normal for a given age and body size. Such aneurysmal dilatation of the aortic root and ascending aorta frequently leads to significant aortic valve regurgitation. Marfan’s syndrome is a genetic disorder of connective tissue that is marked by abnormalities of the skeletal, ocular and cardiovascular system. The main cardiovascular features of Marfan’s syndrome are progressive dilatation of the aortic root and ascending aorta, which may result in aortic rupture and aortic dissection or in aortic valvular regurgitation. The Bentall operation (one-piece composite valve-graft conduit for aortic valve, aortic root and ascending aorta replacement concomitant with coronary button reimplantation) is considered as a standard procedure to correct and prevent potential complications of this disease. This is a report of the first two cases that had received Bentall operation in southern Thailand. The results of the surgical treatment was satisfactory.

Keywords

• ascending aorta
• aortic root
• aneurysmal formation
• aortic valve regurgitation
• marfan’s syndrome
• aortic rupture
• aortic dissection
• bentall operation
• valve-graft conduit
• coronary button reimplantation.