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Concept: Bicuspid aortic valve


Thoracic aortic aneurysm is usually a clinically silent disease; timely detection is largely dependent upon identification of clinical markers of thoracic aortic disease (TAD); (bicuspid aortic valve, intracranial aortic aneurysm, bovine aortic arch, or positive family history). Recently, an association of simple renal cysts (SRC) with abdominal aortic aneurysm and aortic dissection was established. The aim of our study was to evaluate the prevalence of SRC in patients with TAD in order to assess whether the presence of SRC can be used as a predictor of TAD.

Concepts: Aortic aneurysm, Aneurysm, Aortic dissection, Aorta, Abdominal aortic aneurysm, Aortic valve, Bicuspid aortic valve, Thoracic aortic aneurysm


Valve-sparing aortic root reconstruction (VSRR) is an accepted method to treat patients with aortic root dilation. The role of the VSRR is less well defined for patients with bicuspid aortic valve, severe aortic valve insufficiency, congenital heart defects, and type A aortic dissection. We studied the clinical outcome of patients who underwent VSRR for expanded indications.

Concepts: Aortic aneurysm, Aortic dissection, Aorta, Congenital heart defect, Congenital heart disease, Aortic valve, Aortic insufficiency, Bicuspid aortic valve


To evaluate factors associated with aortic enlargement in patients with a bicuspid aortic valve (BAV) and the impact of isolated aortic valve replacement (AVR).

Concepts: Aortic aneurysm, Aortic valve, Aortic valve replacement, Aortic insufficiency, Bicuspid aortic valve


About 1-2% of the babies are born with bicuspid aortic valves instead of the normal aortic valve with three leaflets. A significant portion of the patients with the congenital bicuspid valve morphology suffer from aortic valve stenosis and/or ascending aortic dilatation and dissection thus requiring surgical intervention when they are young adults. Patients with bicuspid aortic valves (BAVs) have also been found to develop valvular stenosis earlier than those with the normal aortic valve. This paper overviews current knowledge of BAVs, where several studies have suggested that the mechanical stresses induced on the valve leaflets and the abnormal flow development in the ascending aorta may be an important factor in the diseases of the valve and the aortic root. The long-term goals of the studies being performed in our laboratory are aimed towards potential stratification of bicuspid valve patients who may be at risk for developing these pathologies based on analyzing the hemodynamic environment of these valves using fluid-structure interaction (FSI) modeling. Patient-specific geometry of the normal tri-cuspid and bicuspid valves are reconstructed from real-time 3D ultrasound images and the dynamic analyses performed in order to determine the potential effects of mechanical stresses on the valve leaflet and aortic root pathology. This paper describes the details of the computational tools and discusses challenges with patient-specific modeling.

Concepts: Aortic dissection, Aorta, Ascending aorta, Left ventricle, Aortic valve, Aortic valve stenosis, Aortic insufficiency, Bicuspid aortic valve


Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However, the size of the aorta and the clinical characteristics among those with Turner syndrome and dissection have received little attention.

Concepts: Aortic aneurysm, Aortic dissection, Aorta, Marfan syndrome, Left ventricle, Aortic valve, Bicuspid aortic valve, Traumatic aortic rupture


The risk factors for aortic and mitral valve diseases that require surgical repair such as congenital bicuspid aortic valve (BAV) and mitral valve prolapse include acquired clinical factors and genetic influences. Whether race affects the prevalence of certain valvular diseases has not been sufficiently investigated. Through the Cleveland Clinic’s Cardiovascular Information Registry, we evaluated the data from 40,419 patients who had undergone aortic valve surgery, mitral valve surgery, and/or coronary artery bypass grafting from 1993 to 2007. Of these patients, 38,366 were white and 2,053 were black. The prospective evaluation of valvular disease was coded, identifying the etiology and morphology by echocardiographic, surgical, and pathologic inspection. At baseline, compared to white patients, the black patients were younger, more often women, had a greater body mass index, and a greater prevalence of hypertension, diabetes, tobacco use, and renal disease. The prevalence of congenital BAV and mitral valve prolapse was considerably lower in blacks than in whites (9% vs 25%, p <0.001, and 27% vs 52%, p <0.001, respectively), as was the presence of calcific aortic stenosis (14% vs 28%; p <0.001), pathologically determined aortic valve calcium (50% vs 67%; p <0.001), and mitral valve chordal rupture (13% vs 31%; p <0.001). In conclusion, in the present large surgical series, the valve etiologies and morphology differed among blacks and whites. Despite an adverse cardiovascular risk profile, blacks had a significantly lower prevalence of valvular calcium and degeneration than did the whites and a lower prevalence of congenital BAV and mitral valve prolapse. Our findings offer insight into the influence of race on the development of mitral valve disease and congenital BAV.

Concepts: Atherosclerosis, Heart, Left ventricle, Mitral valve prolapse, Cardiac anatomy, Aortic valve, Valvular heart disease, Bicuspid aortic valve


Two guidelines from the American College of Cardiology (ACC), the American Heart Association (AHA), and collaborating societies address the risk of aortic dissection in patients with bicuspid aortic valves and severe aortic enlargement: the “2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease” (J Am Coll Cardiol 2010;55:e27-130) and the “2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease” (J Am Coll Cardiol 2014;63:e57-185). However, the 2 guidelines differ with regard to the recommended threshold of aortic root or ascending aortic dilatation that would justify surgical intervention in patients with bicuspid aortic valves. The ACC and AHA therefore convened a subcommittee representing members of the 2 guideline writing committees to review the evidence, reach consensus, and draft a statement of clarification for both guidelines. This statement of clarification uses the ACC/AHA revised structure for delineating the Class of Recommendation and Level of Evidence to provide recommendations that replace those contained in Section of the thoracic aortic disease guideline and Section 5.1.3 of the valvular heart disease guideline.

Concepts: Cardiology, Heart, Surgery, Aortic aneurysm, Aortic dissection, Aorta, Aortic insufficiency, Bicuspid aortic valve


We present a 73 years old woman who presented with pelvic cancer, ongoing sepsis and presumably a new diastolic murmur. Her transthoracic echocardiogram, as part of a sepsis screen, showed an abnormal pulmonary valve with moderate regurgitation. Trans-oesophageal echocardiography using 3D imaging showed a bicuspid pulmonary valve as the cause for the moderate regurgitation, but with no clear source of infection. Bicuspid pulmonary valve as an isolated clinical entity is a rare finding in clinical practice. Bicuspid pulmonary valves tend to be more commonly associated with other congenital cardiac anomalies. Whereas, the incidence of bicuspid aortic valve is estimated to be about 1-2%, the incidence of bicuspid pulmonary valve is thought to be much lower at around 0.1%. This could be an underestimate due to difficulty in visualising the pulmonary valve en-face on standard two-dimensional echocardiography. The true prevalence of the condition may be uncovered by the routine use of 3D echocardiography.

Concepts: Medical statistics, Cardiology, Heart, Echocardiography, Tricuspid valve, Aortic valve, Heart murmur, Bicuspid aortic valve


Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality.

Concepts: The Association, Aortic dissection, Aortic valve, Degree of a polynomial, Valve, Aortic insufficiency, Bicuspid aortic valve, Check valve