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...

A 19-year-old woman with a bicuspid aortic valve was admitted because of active infective endocarditis with a root abscess. Four weeks of antibiotic therapy achieved negative blood cultures and normalized inflammatory signs; however, echocardiography revealed severe aortic regurgitation through an abscess cavity located at the root. The modified Ross procedure, in which the pulmonary autograft ...

Methods In-vivo aortic 3D hemodynamics were evaluated by MRI in 17 patients with unicuspid (n=3), bicuspid (n=9, 3 true bicuspid, 3 right-left (RL) coronary leaflet fusion, and 3 right-non (RN) coronary leaflet fusion), trileaflet (n=3), and quadricuspid aortic valves (n=2). Valve morphology and dynamics were assessed using 2D CINE MRI. Aortic blood flow was measured using ECG and respiration s...

A 53-year-old man was admitted to our center with dyspnea and peripheral edema compatible with cardiac failure. His history was notable for Noonan syndrome, but he had not been seen a physician for 15 years. On examination, pectus excavatum, pectus carinatum, and low implantation of the ears were present (Figure 1). Blood pressure was 145/ 70 mm Hg, and pulse was irregular at 90 to 100 beats pe...

The timing of prophylactic ascending aortic aneurysm surgery in the setting of bicuspid aortic valve disease is complex, with multiple factors influencing the decision. The 2014 ACC/AHA Valve guidelines recommend prophylactic replacement of the aortic root and/or ascending aorta once the aortic diameter exceeds 5.5 cm. This aortic size threshold for surgery is at a larger diameter than had been...

Congenital left ventricular outpouchings (LVOs) are infrequent myocardial malformations, comprising various overlapping abnormalities, whose characterization is often intricate in clinical practice using traditional non-invasive techniques. We describe a rare case of LVO associated with bicuspid aortic valve incidentally found in an asymptomatic adult patient. The LVO was located at basal level...

Echocardiograms were performed in thirty-six patients (aged 4 to 36 years) with proven coarctation of the aorta. Nineteen patients (53%) were found to have marked diastolic eccentricities of their aortic valves (Eccentricity Index greater 1.5), indicating the presence of bicuspid aortic valves. One of these patients also had multilayered aortic root echoes in diastole. Five patients had angiogr...

Among developed countries, valvular aortic stenosis (AS) in pregnant women is primarily due to a congenitally bicuspid aortic valve, which occurs in approximately 1% of the general adult population. Most asymptomatic patients and those with mild to moderate AS can be managed conservatively to full-term pregnancy. However, those with more severe AS with symptoms require more aggressive treatment...