BACKGROUND In patients with bicuspid aortic valve and dilated proximal ascending aorta, we sought to assess (1) factors associated with increased longer-term cardiovascular mortality and (2) incremental prognostic use of indexing aortic root to patient height. METHODS AND RESULTS We studied 969 consecutive bicuspid aortic valve patients (50±13 years; 87% men) with proximal aorta ≥4 cm, who al...

AIMS The mesenchymal cushions lining the early embryonic heart undergo complex remodelling to form the membranous ventricular septum as well as the atrioventricular and semilunar valves in later life. Disruption of this process underlies the most common congenital heart defects. Here, we identified a novel role for Slit-Robo signalling in the development of the murine membranous ventricular sep...

BACKGROUND In general, all conduits available for right ventricular outflow tract (RVOT) reconstruction eventually become stenotic or insufficient. Owing to the lack of an ideal conduit and with the hope of reducing the incidence of reoperations, we have developed and utilized a bicuspid valved polytetrafluoroethylene (PTFE) conduit for the reconstruction of the RVOT. The purpose of this study ...

BACKGROUND Bicuspid aortic valve is frequent and is reported to cause numerous complications, but the clinical outcome of patients diagnosed with normal or mildly dysfunctional valve is undefined. METHODS AND RESULTS In 212 asymptomatic community residents from Olmsted County, Minn (age, 32+/-20 years; 65% male), bicuspid aortic valve was diagnosed between 1980 and 1999 with ejection fraction...

BACKGROUND Congenital aortic valve fusion is associated with aortic dilation, aneurysm, and rupture in girls and women with Turner syndrome. Our objective was to characterize aortic valve structure in subjects with Turner syndrome and to determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves. METHODS AND RESULTS The aortic valve and ...

Because the dawning of the era in which interventions for congenital heart disease (CHD) became possible, the primary intellectual framework undergirding pediatric cardiology and cardiothoracic surgery has been based on morphology and hemodynamics (henceforth, the M/H model). This model has been wildly successful, enabling practitioners to devise creative interventions for even the most complex...