Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various t...

Whether the dilatation of proximal aorta in patients with bicuspid aortic valve is secondary to hemodynamic effects related to the abnormal aortic valve or a primary manifestation of the genetic disorder remains controversial. We discuss in this paper the recent data on the BAV function and transvalvular flow patterns in relation with the dilatation type of the proximal aorta. Different morphol...

A 27-year-old woman presented with a history of long-standing poor vision in both eyes. Ophthalmologic examination after pupillary dilatation revealed bilateral lens coloboma situated in the inferotemporal quadrant. No associated ocular abnormalities were seen, except amblyopia. A bicuspid aortic valve was observed during echocardiography during systemic evaluation. Lens coloboma usually occurs...

Background Bicuspid aortic valve (BAV) is known to be heritable and often shows a concomitant aortopathy which was thought also to be present in some family members. While one recent study reported normal ascending aortic diameters in family members of the general BAV population, little is known about aortic function in such family members. We used advanced aortic cardiovascular magnetic resona...

I read with great interest the article by Hraska et al. [1], in the May issue of EJCTS, summarizing their 7 years experience with the Ross and Ross–Konno operation in children and adolescents in two centers. Like most other series, their mid-term experience validates the safety and efficacy of Ross operation in treating aortic valve disease in this important subset of patients where growth, act...

Background. The pathogenesis of ascending aortic di-latation in the presence of a bicuspid valve is discussed controversially. Recent experimental evidence suggests that the expression of endothelial nitric oxide synthase (eNOS) may have an influence on aortic valve anatomy and aneurysmal dilatation of the aorta. We investigated the relationship among eNOS expression, valve anatomy, and aortic ...

Congenital aortic stenosis (AS) is caused by abnormal morphological development of the aortic valve. [1, 2] Valvular abnormalities may be accompanied by supraor subvalvular stenosis. The embryogenic process that forms aortic valves begins approxi‐ mately 31–32 days of gestation. Cavity formation in the basal portion of the truncus arteriosus is a key process in the development of the leaflet an...

The Ross procedure is an excellent choice for younger patients in need of aortic valve replacement. While patients have benefited from superior survival rates associated with this procedure, complications related to aortic root dilatation and degeneration of the autograft may be encountered later in life. These challenges may be exacerbated in those with underlying connective tissue abnormaliti...

Background Aortic coarctation (AC) represents 7% of congenital heart disease. It is a reversible secondary cause of systemic hypertension, however up to 35% of patients remain hypertensive and 18% have cardiovascular complications such as aortic aneurysm, dissection or aortic valve disease. AC is associated with bicuspid aortic valve (BAV) in around 60%, both diseases alter aortic wall distensi...

Bicuspid aortic valve disease is the most common congenital heart defect, affecting 1% to 2% of the general population, with a higher prevalence in males (Hoffman & Kaplan, 2002; Movahed et al., 2006). Quite often, the diagnosis of bicuspid aortic valve disease is an incidental finding during an echocardiogram. However, the disease may be associated with significant valvular dysfunction and lea...