Unicuspid Aortic Valve in Infant.
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ABSTRACT:
SUBMITTER: Taksande AM
PROVIDER: S-EPMC5353412 | biostudies-literature | 2015 Jul-Sep
REPOSITORIES: biostudies-literature
Publications
Unicuspid Aortic Valve in Infant.
Journal of cardiovascular echography 20150701 3
The unicuspid aortic valve (UAV) is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Here, we present a case of UAV with moderate aortic stenosis detected by transthoracic echocardiography in the infant. ...[more]
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Project description:•UAV is a rare congenital anomaly that leads to severe symptomatic stenosis.•Echocardiography plays a critical role in the evaluation of aortic stenosis.•Correctly distinguishing between UAV and BAV is relevant in determining intervention.
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Project description:Unicuspid aortic valve is a rare congenital malformation that usually presents in the 3rd to 5th decade of life-and usually with severe aortic stenosis or regurgitation. It often requires surgical correction. Diagnosis can be made with 2- or 3-dimensional transthoracic or transesophageal echocardiography, cardiac computed tomography, or cardiac magnetic resonance imaging. We report the case of a 31-year-old man who presented with dyspnea on exertion due to severe aortic stenosis secondary to a unicuspid unicommissural aortic valve. After aortic valve replacement, this patient experienced complete heart block that required the placement of a permanent pacemaker.
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Project description:The epithelial growth factor receptor family of tyrosine kinases modulates embryonic formation of semilunar valves. We hypothesized that mice heterozygous for a dominant loss-of-function mutation in epithelial growth factor receptor, which are EgfrVel/+ mice, would develop anomalous aortic valves, valve dysfunction, and valvular cardiomyopathy.Aortic valves from EgfrVel/+ mice and control mice were examined by light microscopy at 2.5 to 4 months of age. Additional EgfrVel/+ and control mice underwent echocardiography at 2.5, 4.5, 8, and 12 months of age, followed by histologic examination. In young mice, microscopy revealed anatomic anomalies in 79% of EgfrVel/+ aortic valves, which resembled human unicuspid aortic valves. Anomalies were not observed in control mice. At 12 months of age, histologic architecture was grossly distorted in EgfrVel/+ aortic valves. Echocardiography detected moderate or severe aortic regurgitation, or aortic stenosis was present in 38% of EgfrVel/+ mice at 2.5 months of age (N=24) and in 74% by 8 months of age. Left ventricular enlargement, hypertrophy, and reversion to a fetal myocardial gene expression program occurred in EgfrVel/+ mice with aortic valve dysfunction, but not in EgfrVel/+ mice with near-normal aortic valve function. Myocardial fibrosis was minimal or absent in all groups.A new mouse model uniquely recapitulates salient functional, structural, and histologic features of human unicuspid aortic valve disease, which are phenotypically distinct from other forms of congenital aortic valve disease. The new model may be useful for elucidating mechanisms by which congenitally anomalous aortic valves become critically dysfunctional.
| S-EPMC6064885 | biostudies-other
Project description:To assess the impact of aortic valve morphology on aortic hemodynamics between normal tricuspid and congenitally anomalous aortic valves ranging from unicuspid to quadricuspid morphology.Aortic three-dimensional (3D) blood flow was evaluated by 4D flow MRI in 14 healthy volunteers with normal trileaflet valves and 14 patients with unicuspid (n = 3), bicuspid (n = 9, 3 "true" bicuspid, 3 right-left (RL), 3 right-noncoronary (RN) leaflet fusion, and quadricuspid aortic valves (n = 2). Data analysis included the co-registered visualization of aortic valve morphology with systolic 3D blood flow. The influence of valve morphology on aortic hemodynamics was quantified by valve flow angle.All RL-bicuspid aortic valve (BAV) were associated with flow jets directed toward the right anterior aortic wall while RN-fusion and unicuspid valves resulted in flow jet patterns toward the right-posterior or posterior wall. Flow angles were clearly influenced by valve morphology (47° ± 10, 28° ± 2, 29° ± 18, 18° ± 12, 15° ± 2 for unicuspid, true BAV, RN-BAV, RL-BAV, quadricuspid valves) and increased compared with controls (7.2° ± 1.1, P = 0.001).Altered 3D aortic hemodynamics are impacted by the morphology of congenitally malformed aortic valves.
| S-EPMC4864037 | biostudies-literature
Project description:Unicuspid aortic valve (UAV) is an extremely rare form of congenital aortic valvular abnormality. Although UAV shows similar clinical characteristics to bicuspid aortic valve, the clinical symptoms develop at earlier age and progress at a faster pace in UAV. In this report, we are presenting a 42-year-old male with severe aortic stenosis associated with unicommissural UAV. The patients underwent a successful Bentall operation.
| S-EPMC5050315 | biostudies-literature
Project description:BackgroundA unicuspid aortic valve is a rare congenital cardiac abnormality. Despite its uncommon finding on an initial presentation, aortic insufficiency is accompanied with unicuspid aortic valve and this might reflect the natural history of progression in the morphology of unicuspid aortic valve.Case presentationWe describe a 65-year-old Japanese man who was evaluated for endocarditis and found to have a unicuspid aortic valve concomitant with moderate aortic insufficiency, which was, owing to the lack of evidence of valve membrane destruction, independent of underlying infectious endocarditis. In addition, aortic insufficiency was progressed because of nonbacterial thrombotic endocarditis on the ventricular side, in areas of high turbulence around the heart valve.ConclusionsOur case is unusual given the unicuspid aortic valve concomitant with aortic insufficiency, which was presumably independent of underlying infectious endocarditis because of the location of the vegetation and the lack of evidence of valve destruction. Therefore, attention should be paid to a variety of complications in the setting of unicuspid aortic valve.
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Project description:BackgroundAortic stenosis is a progressive disease that frequently remains undiagnosed until late in the disease course. In patients that present with symptoms of heart failure and a systolic murmur at a young age, a congenital valvular abnormality must be on the differential. With patients that have accelerated symptoms of aortic stenosis and valvular dysfunction, a unicuspid aortic valve (UAV) could be present. A UAV is often difficult to distinguish from a bicuspid aortic valve (BAV) on transthoracic echocardiography. In patients with congenital valvular abnormalities an ascending aortic aneurysm can also be present. Aortic stenosis changes the jet of fluid emerging from the aortic valve leading to an increased risk for aortic aneurysm dissection and rupture. The gold standard treatment for aortic stenosis secondary to a congenital valvular abnormality is valve replacement. A known risk of aortic valve replacement is conduction abnormalities. In this case, we present a patient with a unicuspid valve who postoperatively develops complete heart block leading to pacemaker implantation.Case summaryWe present a case of a 46-year-old Caucasian male with no prior medical history who presented with progressively worsening exertional dyspnoea and palpitations for 7 months. Transthoracic echocardiogram showed a BAV, however, further work up confirmed a unicommissural aortic valve with severe aortic stenosis and moderate regurgitation along with an ascending aortic aneurysm. Aortic valve replacement and aortic aneurysm repair via the Bentall procedure was successfully completed with postoperative course being complicated by a complete heart block and subsequent permanent pacemaker placement.DiscussionWhen assessing patients with symptoms of heart failure with a systolic murmur that suggests aortic stenosis at a young age, a UAV must be kept on the differential. The symptoms of aortic stenosis and valvular dysfunction are accelerated in UAVs when compared with BAVs. Currently, the treatment for patients with congenital valvular abnormalities presenting with aortic stenosis is aortic valve replacement using traditional open surgery. A known sequelae of isolated aortic valve replacement is conduction abnormalities that can sometimes lead to permanent pacemaker placement. After the confirmation of unicuspid or bicuspid valve postoperatively, it is important to report any postoperative conduction abnormalities. This is because, currently, there is no literature that compares the incidence of conduction abnormalities after unicuspid replacement to that of other BAV syndromes.
| S-EPMC6458860 | biostudies-literature
Project description:BACKGROUND:Unicuspid aortic valve is an important subset of bicuspid aortic valve, and knowledge regarding its aortopathy pattern and surgical outcomes is limited. Our objectives were to characterize unicuspid aortic valve patients, associated aortopathy, and surgical outcomes. METHODS:From January 1990 to May 2013, 149 adult unicuspid aortic valve patients underwent aortic valve replacement or repair for aortic stenosis (n = 13), regurgitation (n = 13), or both (n = 123), and in 91 (61%) the aortic valve operation was combined with aortic repair. Data were obtained from the Cardiovascular Information Registry and medical record review. Three-dimensional imaging analysis was performed from preoperative computed tomography and magnetic resonance imaging scans. The Kaplan-Meier method was used for survival analysis. RESULTS:Patients had a mean maximum aortic diameter of 44 ± 8 mm and variably involved the aortic root, ascending, or arch, or both. Patients with valve operations alone were more likely to be hypertensive (p = 0.01) and to have severe aortic stenosis (p = 0.07) than those who underwent concurrent aortic operations. There were no operative deaths, strokes, or myocardial infarctions. Patients undergoing aortic repair had better long-term survival. Estimated survival at 1, 5, and 10 years was 100%, 100%, and 100% after combined operations and was 100%, 88%, and 88% after valve operations alone (p = 0.01). CONCLUSIONS:Patients with a dysfunctional unicuspid aortic valve frequently present with an ascending aneurysm that requires repair. Combined aortic valve operations and aortic repair was associated with significantly better long-term survival than a valve operation alone. Further study of this association may direct decisions about timing of surgical intervention.
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Project description: Not available
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