scholarly journals A heart in a heart: a case report of spontaneous flail of bicuspid aortic valve

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Khadije Mohammadi ◽  
Mahsa Akrami ◽  
Marzieh Mirtajaddini
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Past Medical History ◽  
Severe Aortic Regurgitation ◽  
Case Summary ◽  
History Of ◽  
Old Persian ◽  
Acquired Abnormalities ◽  
Supporting Structures

Abstract Background Aortic regurgitation is attributed to congenital and acquired abnormalities of the aortic valve or aortic valve supporting structures. The most common cause of aortic regurgitation is atherosclerotic degeneration of the valve, especially in the presence of a bicuspid aortic valve. Case summary A 25-year-old Persian man with no past medical history, developed dyspnea since 1 week before his first visit to the physician. He was an active person up to this time, and had no history of trauma or chest pain. Transthoracic echocardiography showed severe aortic regurgitation in the context of flail bicuspid aortic valve, with no evidence of endocarditis. Laboratory tests including blood cultures were negative for infection. The patient underwent aortic valve replacement and the diagnosis was confirmed at time of surgery. Conclusion This case represents noninfective and nontraumatic spontaneous flail of bicuspid aortic valve.

scholarly journals Repair of Bicuspid Aortic Valve in the Presence of Endocarditis and Leaflet Perforation

2014 ◽  
Vol 41 (1) ◽  
pp. 67-69
Author(s):  
William D.T. Kent ◽  
Hadi D. Toeg ◽  
Jehangir J. Appoo
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Recurrent Infection ◽  
Aortic Valve Repair ◽  
Valve Repair ◽  
Simplified Approach ◽  
Severe Aortic Regurgitation ◽  
History Of ◽  
Leaflet Coaptation

Aortic valve repair can be a good option in younger patients who have severe aortic regurgitation. A systematic, disease-directed approach can simplify repair. This case report describes how a simplified approach can be successfully applied to complex pathologic conditions of the aortic valve. A 49-year-old man with a bicuspid aortic valve and a history of endocarditis presented with severe aortic regurgitation and evidence of recurrent infection. Intraoperatively, we found congenital and degenerative aortic anatomy with endocarditis and perforation. We performed aortic valve repair to enable leaflet coaptation and to adjust the coaptation height. After 24 months, the patient remained well, with an intact repair and trivial aortic regurgitation. We describe our systematic repair approach and rationales for targeting repairs to identified lesions. To our knowledge, this is the first description of complex aortic valve repairs in a patient who had simultaneous congenital, degenerative, and infectious conditions.

scholarly journals Transcatheter aortic valve implantation for severe aortic regurgitation in a patient with end-stage respiratory failure

2020 ◽  
Vol 26 (1) ◽  
pp. 58-70
Author(s):  
Aleksandra Cherneva ◽  
Zoran Stankov ◽  
Naidenka Zlatareva ◽  
Iveta Tasheva ◽  
Georgi Dobrev ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Functional Class ◽  
Severe Aortic Regurgitation ◽  
Transcatheter Aortic Valve ◽  
History Of ◽  
And Migration ◽  
End Stage ◽  
Valve Implantation

We report a case of a high-risk 73-year–old patient with a combined aortic valve disease with predominant severe, symptomatic aortic regurgitation and a history of an end-stage respiratory failure with prohibitive surgical risk who was successfully treated using a minimalist approach to implant off-label а self-expandable Medtronic Evolut R prosthesis. This case report demonstrates that the self-expandable prosthesis Medtronic Evolut R might be implanted without tissue damage and migration in a moderate-calcified tricuspid aortic valve with predominant regurgitation and mild stenosis with satisfactory hemodynamic results and improvement in functional class heart failure in a patient with concomitant severe respiratory failure.

scholarly journals Bicuspid Aortic Valve Associated with Severe Aortic Regurgitation and Large Aortic Root Aneurysm

Cureus ◽  
2020 ◽  
Author(s):  
Alejandro Sanchez-Nadales ◽  
Miguel Treminio Quezada ◽  
Valentina Celis ◽  
Jessica Navarro
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Severe Aortic Regurgitation ◽  
Aortic Root Aneurysm

P6501Recurrent genetic aberrations in bicuspid aortic valve disease patients with isolated severe aortic regurgitation

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Y Wang ◽  
B Wu ◽  
J Li ◽  
C Wang ◽  
X Shu ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Genetic Variants ◽  
Early Onset ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Genetic Defects ◽  
Severe Aortic Regurgitation ◽  
Rare Genetic Variants

Abstract Background The etiology of bicuspid aortic valve disease (BAVD) is still unclear. Recent studies have demonstrated elevated prevalence of genetic defects in BAV patients with root phenotype, which includes aortic regurgitation and root-predominant dilatation. Purpose The present study intended to illustrate the feature of genetic defects among early-onset BAV patients with isolated severe aortic regurgitation. Methods From June 2015 to December 2017, whole exome sequencing was performed upon 27 BAVD patients with isolated severe aortic regurgitation under 45 years in our institution. Patients were categorized into right-left (R-L, n=16) and non-RL (n=11) cusp fusion types, and those with complex cardiac defects were excluded from the present study. Results Among 27 patients with a median age of 30.5 (18–44) years, only one was female with a rare left-non-coronary cusp fusion type. The prevalence of root phenotype was markedly higher in RL patients (56.3% vs 9.1%, p=0.018). In RL patients, the numbers of rare genetic variants (RGVs) were 15 in extracellular matrix genes, 8 in TGF-β signaling pathway genes, 2 in smooth muscle cell contraction apparatus genes, and 3 in familiar BAV related genes. In non-RL patients, the number of RGVs were 15, 3, 4, and 5, respectively. On the other hand, the number of RGVs in above gene clusters were 9, 6, 3, 2 in patients with a root phenotype, and 21, 5, 3, 6 in those without. Eight recurrent genetic variants were identified in 6 genes (see Table). An interesting observation was that ADAMTS2 variants were exclusively found among non-RL patients without root phenotype, as FBN2 variants among RL patients with root phenoype. Recurrent Rare Genetic Variants Gene Reference sequence Variant 1000G 1000G-East Asia Patients TGFBR2 NM_001024847.2 p.Val216Ile/c.646G>A 0.004 0.018 A16, A23 TGFBR2 NM_001024847.2 p.Thr340Met/c.1019C>T 0.003 0.015 A03, A05, A07 ADAMTS2 NM_014244.4 p.Gly1169Val/c.3506G>T 0.0044 0.021 A03, A15 FBN2 NM_001999.3 p.Gly475Val/c.1424G>T 0.0004 0.002 A19, A24 ELN NM_001278939.1 p.Pro93Leu/c.278C>T 0.0014 0.0069 A22, A26 COL4A5 NM_033380.2 p.Gly953Val/c.2858G>T 0.0079 0.03 A11, A17 MYLK NM_053025.3 p.Ser243Trp/c.728C>G 0.0002 0.001 A01, A02 MYLK NM_053025.3 p.Asp717Tyr/c.2149G>T 0.0024 0.011 A04, A21 Conclusion Recurrent genetic variants could be identified in a cohort of early-onset BAVD patients with isolated severe aortic regurgitation and staggering male predominance. The incidence and clinical relevance of these variants should be validated in an extended real-world BAV cohort.

scholarly journals P915 Bicuspid aortic valve: differences between populations

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D Sharif ◽  
E Azzam ◽  
A Sharif-Rasslan ◽  
I Dobrecky Mery
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Aortic Regurgitation ◽  
Racial Differences ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Left Ventricular ◽  
Female Ratio ◽  
Severe Aortic Regurgitation ◽  
Male To Female

Abstract Congenital bicuspid aortic valve (BAV) is the most common congenital valve abnormality, with an estimated incidence of 0.5-2% in adults. However, BAV is much less prevalent in some populations and racial differences were reported. Aim: Comparison between Arab and Jewish patients with BAV in Israel. Methods: Screening of 18962 echocardiographic studies in Bnai Zion Medical Center and evaluation of demographic, atherosclerotic risk factors, clinical and echocardiographic parameters was performed. Results: Male to female ratio in Arab patients was 4.6:1 while in Jewish patients 1.6:1, p < 0.01. The age of both populations with BAV was similar 45 ± 21yeras, however larger prevalence was found of cigarette smoking and diabetes mellitus in Arabs 31.1% and 6.7% than in Jews 8.2% and 3.5% respectively, p < 0.05. Left ventricular, left atrial and aortic dimensions as well as Doppler parameters were similar in both groups except for large mitral peak A-wave velocities in Jewish patients. Prevalence of aortic valve stenosis in Jewish patients was 35% similar to that in Arabs 33% with similar severity and mean pressure gradients. Aortic valve regurgitation was fond in 53% of Arab patients and in 46% of Jewish patients, p = ns, however, moderately severe and severe aortic regurgitation was more prevalent in Arab patients 22% compared to 13% of Jewish patients, p < 0.05. During 10 years follow up, 25% of Arab patients needed aortic valve replacement while in Jewish patients 10% had surgery, p < 0.05. Conclusion: In Arab patients, male to female ratio was larger, with higher prevalence of moderately severe to severe aortic regurgitation and higher rate of aortic valve replacement compared to Jewish patients.

Acute severe aortic regurgitation due to rupture of a raphal cord on bicuspid aortic valve: a case report

Choonpa Igaku ◽  
2017 ◽  
Vol 44 (1) ◽  
pp. 49-54
Author(s):  
Kenji AIDA ◽  
Minako KINOSHITA ◽  
Nobuhiro SATO ◽  
Yuko YOSHIGAI ◽  
Keiichiro YAMANE ◽  
...  
Keyword(s):  
Case Report ◽  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Severe Aortic Regurgitation
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