Background
Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.
Methods
The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention.
Results
Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8–9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04).
Conclusions
The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.
Background : The factors related to ascending aorta dilation (AAD) in patients with bicuspid aortic valve (BAV) are not completely understood. In addition, the role of cholesterol metabolism in AAD has not been studied. Methods : We analyzed the relationship between different lipid parameters and the ascending aorta diameter/presence of aortic dilatation in 91 consecutive patients with BAV. Results : We observed a positive linear correlation between the total cholesterol, low-density lipoprotein...
Cardiac left ventricular outflow tract (LVOT) defects represent a common but heterogeneous subset of congenital heart disease for which gene identification has been difficult. We describe a 46,XY,t(1;5)(p36.11;q31.2)dn translocation carrier with pervasive
> Everything should be kept as simple as possible, but no simpler. > > —Albert Einstein1 Since its estimated first description >500 years ago by Leonardo da Vinci,2 the bicuspid aortic valve (BAV) has progressively built a reputation; initially, as a curious valvular phenotype with a tendency to develop obstruction and insufficiency. In more contemporary times, however, the BAV is recognized as underlying almost 50% of isolated severe aortic stenosis cases requiring surgery,3 and has been extens...
Bicuspid aortic valve is the most common congenital heart defect in adults. Dilatation of the proximal aorta, or bicuspid aortopathy, is present in approximately half these patients and can lead to complications, including aneurysm formation and aortic dissection.
The clinical and basic research on bicuspid aortic valve (BAV) and associated conditions is an exponentially expanding field. As of 23 October 2013, according to a PubMed article search for the ‘bicuspid aortic valve’ (BAV) keyword in the title, as many as 288 papers have been published on this topic between 2008 and 2012, versus 140 from 2003 through 2007 and only 48 in the preceding 5 years. Nevertheless, very little is known about the genetic background of the most common congenital cardiac m...
Up to 80% of patients with coarctation of the aorta (COA) have a bicuspid aortic valve (BAV). Patients with COA and BAV have elevated risks of aortic complications despite successful surgical repair. The development of such complications involves the interplay between the mechanical forces applied on the artery and the biological processes occurring at the cellular level. The focus of this study is on hemodynamic modifications induced in the aorta in the presence of a COA and a BAV. For this pur...
Bicuspid aortic valve (BAV) is a common congenital malformation with the known sequela of ascending aortic dilation. The morphology of the BAV and the elasticity of the adjacent ascending aorta appear to influence the outcome. We prospectively examined 48 pediatric patients with an isolated, native BAV for the morphology and size of the aortic valve, aortic root, sinotubular junction, and ascending aorta and their elasticity indexes. A cohort of 48 matching subjects with tricuspid aortic valves ...
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 morphological forms of bicuspid aortic valve in relation with the specific transvalvular blood flow patterns a...
: We report the case of an adult patient with the unusual combination of a perimembranous (PM) ventricular septal defect (VSD), asymptomatic ruptured sinus of Valsalva communicating with the right ventricle, and a bicuspid aortic valve. Bulged sinus of Valsalva might conceal the small PM VSD, therefore the patient had no symptom-associated VSD until grown up. However, when aortic valve regurgitation associated with prolapsed right aortic cusp worsened and ruptured sinus of Valsalva was found by ...
#1John S. Ikonomidis(MUSC: Medical University of South Carolina)H-Index: 37
#2Jean Marie Ruddy(MUSC: Medical University of South Carolina)H-Index: 11
Last. Jeffrey A. Jones(MUSC: Medical University of South Carolina)H-Index: 23
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Background Congenital bicuspid aortic valves (BAVs) result from fusion of 2 valve cusps, resulting in left-noncoronary (L-N), right-left (R-L), and right-noncoronary (R-N) morphologic presentations. BAVs predispose to ascending thoracic aortic aneurysms (ATAAs). This study hypothesized that ATAAs with each BAV morphologic group possess unique signatures of matrix metalloproteinases (MMPs) and endogenous tissue inhibitors of metalloproteinases (TIMPs). Methods Ascending thoracic aortic aneurysm t...
Last. Michael Markl(NU: Northwestern University)H-Index: 83
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BACKGROUND Coarctation of the aorta (CoA) typically requires repair, but re-interventions and vascular complications occur, particularly with associated defects like bicuspid aortic valve (BAV). Magnetic resonance imaging (MRI) may identify anatomic and hemodynamic factors contributing to clinical complications. PURPOSE To investigate 4D flow MRI characteristics in pediatric CoA to determine parameters for long-term clinical surveillance. STUDY TYPE Retrospective. POPULATION CoA (n = 21), CoA wi...
Last. Amer Harky(Boston Children's Hospital)H-Index: 1
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Congenital anomalies of the aortic valve frequently necessitate intervention in childhood. The most common aortic valve pathologies present in childhood are aortic stenosis and insufficiency. Presentation of aortic valve disease depends on severity and presence of concomitant syndromes and valvular disorders. Treatment options are largely categorised as medical, percutaneous repair or surgical repair and replacement. Surgical techniques have been refined over the last few years making this the m...
1. Talha Niaz, MBBS* 2. Jonathan N. Johnson, MD*,† 3. Frank Cetta, MD*,† 4. Timothy M. Olson, MD*,† 5. Donald J. Hagler, MD*,† 1. *Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, and 2. †Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN * Abbreviations: AHA : : American Heart Association BAV : : bicuspid aortic valve TTE : : transthoracic echocardiography Bicuspid aortic valve is the most common congenital heart defect in children, adolescents, ...
Abstract The congenital bicuspid aortic valve (BAV) is a heterogeneous condition that is better understood considering 3 types of valvulo-aortopathy presentations: A typical-presentation valvulo-aortopathy, a complex-presentation valvulo-aortopathy and an uncomplicated/undiagnosed-presentation. The burden of BAV-related complications has been elucidated in patients with the typical-presentation valvulo-aortopathy and is discussed in this review. These patients enjoy an overall normal expected lo...
Abstract Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. BAV is a heter...
#1Lajja P Desai(Children's Memorial Hospital)H-Index: 1
#2Haben Berhane(Children's Memorial Hospital)H-Index: 4
Last. Michael Markl(NU: Northwestern University)H-Index: 83
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Background Interrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown.
Purpose The present study aimed to investigate the prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies in subjects with bicuspid aortic valves (BAVs).
#1Byung Joo Sun(CNU: Chungnam National University)H-Index: 11
#2Jin Kyung Oh(UOU: University of Ulsan)H-Index: 6
Last. Jae Kwan Song(UOU: University of Ulsan)H-Index: 35
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BACKGROUND: Although bicuspid aortic valve (BAV) is one of the most common congenital heart diseases, clinical outcome data regarding BAV are still limited. We evaluated clinical characteristics and mid-term clinical outcomes of asymptomatic Korean patients with bicuspid aortic valve. METHODS: We initiated a prospective registry in 2014 at a tertiary referral hospital. To develop a cohort of asymptomatic patients, we excluded patients who previously underwent open heart surgery (OHS) or who had ...