Differing relationship between hypercholesterolemia and a bicuspid aortic valve according to the presence of aortic valve stenosis or aortic valve regurgitation.

OBJECTIVES: To assess the difference in hyperlipidemia between patients with bicuspid aortic valve (BAV) and those with a normal aortic valve (NAV), and to compare aortic valve stenosis (AS), with aortic valve regurgitation (AR). METHODS: Among 32 patients with BAV and 142 patients with NAV who underwent aortic valve replacement, 81 patients had AR and 91 patients had AS. The preoperative clinical characteristics were compared between the BAV and NAV patients. Patients with replacement of the ascending aorta were included, and those who underwent combined valvular surgery, coronary artery bypass grafting, or statin treatment were excluded. RESULTS: The proportions of females patients (p = 0.42), patients with diabetes (p = 0.26) and patients on dialysis (p = 0.69) were similar in the two groups. Mean age was significantly lower, the mean diameter of the ascending aorta was significantly larger, and the rate of surgical intervention for the ascending aorta was significantly higher in the BAV group than in the NAV group (all p < 0.0001). The mean levels of low-density lipoprotein cholesterol (LDL) (p < 0.0001) and total cholesterol (TC) (p = 0.0003) were significantly higher in the BAV group than in the NAV group, in the analysis of only patients with AS, whereas these levels did not differ significantly between the groups, when only patients with AR were considered. CONCLUSION: BAV with AS is associated with hypercholesterolemia. However, BAV with AR was not associated with hypercholesterolemia.

[Familial Annulo-aortic Ectasia without Marfan Syndrome].

Marfan syndrome is a hereditary disease that presents ocular, skeletal, and cardiovascular abnormalities. In recent years, there have been several reports of patients with familial cardiovascular disease but no physical features of Marfan syndrome. We encountered 3 cases of familial annulo-aortic ectasia (AAE). Their father had also had aortic regurgitation, and died during surgery 10 years before. No case demonstrated any physical characteristics of Marfan syndrome or any other connective tissue disease. All cases were operated successfully. One case showed cystic medial necrosis, and 2 cases showed degenerative change. The present report suggests that familial AAE may be caused by weakness of the aortic wall related to heredity. If AAE is left untreated, it can lead to aortic dissection. Thus, we recommend that patients with familial AAE should undergo screening and follow-up similar to patients with Marfan syndrome.