Severe Aortic Insufficiency in a Patient with a Functionally Bicuspid Quadricuspid Aortic Valve.
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ABSTRACT:
SUBMITTER: Malm BJ
PROVIDER: S-EPMC6058220 | biostudies-literature | 2017 Jun
REPOSITORIES: biostudies-literature
Publications
Severe Aortic Insufficiency in a Patient with a Functionally Bicuspid Quadricuspid Aortic Valve.
CASE (Philadelphia, Pa.) 20170511 3
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Project description:Quadricuspid aortic valve (QAV) is a rare congenital cardiac anomaly causing aortic regurgitation usually in the fifth to sixth decade of life. Earlier, the diagnosis was mostly during postmortem or intraoperative, but now with the advent of better imaging techniques such as transthoracic echocardiography, transesophageal echocardiography (TEE), and cardiac magnetic resonance imaging, more cases are being diagnosed in asymptomatic patients. We present a case of a 39-year-old male who was found to have QAV, with the help of TEE, while undergoing evaluation for a diastolic murmur. The patient was found to have Type B QAV with moderate aortic regurgitation. We also present a brief review of classification, pathophysiology, and embryological basis of this rare congenital anomaly. The importance of diagnosing QAV lies in the fact that majority of these patients will require surgery for aortic regurgitation and close follow-up so that aortic valve replacement/repair is done before the left ventricular decompensation occurs.
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Project description:Bicuspid aortic insufficiency (BAI) patients with root aneurysm often require aortic valve and root replacement in a composite procedure. The valve-sparing root replacement (VSARR) procedure is aimed at preserving the native valve when possible. This case highlights a successful transcatheter aortic valve replacement procedure in a BAI patient previously treated with VSARR. (Level of Difficulty: Intermediate.).
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| S-EPMC8453391 | biostudies-literature
Project description:ObjectivesBicuspid aortic valve, characterized by valve malformation and risk for aortopathy, displays profound alteration in systolic aortic outflow and wall shear stress distribution. The present study performed 4-dimensional flow magnetic resonance imaging in patients with bicuspid aortic valve with right-left cusp fusion, focusing on the impact of valve function on hemodynamic status within the ascending aorta.MethodsFour-dimensional flow magnetic resonance imaging was performed in 50 subjects with right-left bicuspid aortic valve and 15 age- and aortic size-matched controls with tricuspid aortic valve. Patients with bicuspid aortic valve were categorized into 3 groups according to their aortic valve function as follows: bicuspid aortic valve with no more than mild aortic valve dysfunction (bicuspid aortic valve control, n = 20), bicuspid aortic valve with severe aortic insufficiency (n = 15), and bicuspid aortic valve with severe aortic stenosis (n = 15).ResultsAll patients with right-left bicuspid aortic valve exhibited peak wall shear stress at the right-anterior position of the ascending aorta (bicuspid aortic valve vs trileaflet aortic valve at the right-anterior position: 0.91 ± 0.23 N/m2 vs 0.43 ± 0.12 N/m2, P < .001) with no distinct alteration between bicuspid aortic valve with severe aortic insufficiency and bicuspid aortic valve with severe aortic stenosis. The predominance of dilatation involving the tubular ascending aorta (82%, type 2 aortopathy) persisted, with or without valve dysfunction. Compared with bicuspid aortic valve control subjects, the bicuspid aortic valve with severe aortic insufficiency group displayed universally elevated wall shear stress (0.75 ± 0.12 N/m2 vs 0.57 ± 0.09 N/m2, P < .01) in the ascending aorta, which was associated with elevated cardiac stroke volume (P < .05). The bicuspid aortic valve with severe aortic stenosis group showed elevated flow eccentricity in the form of significantly increased standard deviation of circumferential wall shear stress, which correlated with markedly increased peak aortic valve velocity (P < .01).ConclusionsThe location of peak aortic wall shear stress and type of aortopathy remained homogeneous among patients with right-left bicuspid aortic valve irrespective of valve dysfunction. Severe aortic insufficiency or stenosis resulted in further elevated aortic wall shear stress and exaggerated flow eccentricity.
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| S-EPMC10876132 | biostudies-literature
Project description:BackgroundQuadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV).Case presentationWe diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained.ResultsIn QPV cases diagnosed at autopsy, Hurwitz's type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz's type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients.ConclusionBetween QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.
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| S-EPMC5385316 | biostudies-literature
Project description:BackgroundNon-calcified aortic stenosis (AS) is rare and is associated with a high risk of transcatheter valve embolization and migration (TVEM) because aortic valve complex calcification is important for stable anchoring of the prosthesis. Therefore, transcatheter aortic valve implantation (TAVI) for non-calcified AS is not preferred. However, a universally accepted strategy for TAVI in such patients is not yet established.Case summaryA 69-year-old woman with symptomatic severe AS and a high surgical risk was admitted to our institution for TAVI. Pre-procedural computed tomography (CT) revealed a non-calcified bicuspid aortic valve. Implantation of a 23 mm self-expandable valve (SEV) was planned according to the manufacturer's recommended optimal size based on CT measurements. Intraoperatively, the 23 mm SEV did not snugly fit at the aortic apparatus level. Thus, we deployed a 26 mm SEV with stable anchoring because of the stronger radial force. She was discharged without any complication. Echocardiography at 3 months follow-up showed a well-functioning transcatheter heart valve (THV) without migration or paravalvular leakage.DiscussionIn our patient with non-calcified bicuspid AS, an SEV that was one size larger than the optimal as measured on CT was successfully implanted without THV embolization. An upsized SEV may be considered when performing TAVI in patients with severe non-calcified AS.
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