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

Emergency aortic root replacement with the Bioconduit stentless aortic valve conduit

2020 ◽  
Vol 25 (6) ◽  
pp. 2055-2059
Author(s):  
ADRIAN TULIN ◽  
◽  
OVIDIU STIRU ◽  
MIRUNA LUANA MIULESCU ◽  
LAURA RADUCU ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Aortic Root ◽  
Aortic Root Replacement ◽  
Bentall Operation ◽  
Severe Aortic Regurgitation ◽  
Valve Conduit ◽  
Successful Case ◽  
Valved Conduit

This report concerns a 73-year-old woman who presented with asymptomatic aortic root an-eurysm with severe aortic regurgitation. The purpose of this article is to present our first successful case for emergency aortic root replacement (Bentall operation) that involves annular implantation of a pericardial valved conduit (Bioconduit TM, Biointegral Surgical, Inc., Ontario, Canada) and to discuss some essential technical clue issues related to this approach.

scholarly journals Uncommon functional ‘bicuspid’ aortic valve associated with an aortic root aneurysm

2009 ◽  
Vol 36 (3) ◽  
pp. 587-587
Author(s):  
Sylvain Rubin ◽  
Sebastien Sandu ◽  
Emmanuelle Durand ◽  
Bernard Baehrel
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Aortic Root Aneurysm

scholarly journals Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease

Heart ◽  
2017 ◽  
Vol 104 (7) ◽  
pp. 566-573 ◽  
Author(s):  
Arturo Evangelista ◽  
Pastora Gallego ◽  
Francisco Calvo-Iglesias ◽  
Javier Bermejo ◽  
Juan Robledo-Carmona ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Clinical Predictors ◽  
Valve Dysfunction ◽  
Tertiary Hospitals ◽  
Male Sex

ObjectiveBicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications.MethodsEight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established.ResultsThree BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN.ConclusionsNormofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

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.

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.

Sleeve Technique Combined With Aortic Valve Replacement: A Simpler Alternative to the Bentall Procedure

2020 ◽  
pp. 155698452096701
Author(s):  
Giuseppe De Cicco ◽  
Ana Paula Tagliari ◽  
Gerardo Di Matteo ◽  
Francesco Trinca ◽  
Fabrizio Rosati ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Aortic Regurgitation ◽  
Valve Replacement ◽  
Aortic Root ◽  
Surgical Techniques ◽  
Root Disease ◽  
Severe Aortic Regurgitation ◽  
Bentall Procedure ◽  
Aortic Root Disease

Aortic root disease can be treated with different surgical techniques. The surgical approach of choice depends on patients’ comorbidities and aortic valve conditions. We describe an operation combining a Sleeve aortic rootplasty with an aortic valve replacement, as an alternative to the classical Bentall procedure. The patient, a 63-year-old man, was admitted to our institution in April 2019 with severe aortic regurgitation, left ventricle dysfunction (ejection fraction = 44%), and aortic root ectasia (476 mm). Since a sparing aortic valve procedure was judged not feasible because of the thickness and retraction of the leaflets, we decided to perform a procedure through a sleeve operation to treat the aortic ectasia and a standard bioprosthetic aortic valve replacement to treat the aortic regurgitation. The patient had an uneventful postoperative course and was discharged on the sixth postoperative day with aspirin as sole antiplatelet treatment. This procedure may be considered an alternative to the Bentall operation. In fact, it may also offer complete treatment for the aortic root disease, avoiding coronary complications due to coronary ostia manipulation and reimplantation.

A rare case of a patient with aortic root aneurysm, bicuspid aortic valve, and Scimitar syndrome with anomalous venous return to the right superior pulmonary vein

2018 ◽  
Vol 28 (4) ◽  
pp. 595-597
Author(s):  
Kamran Ahmadov ◽  
Catherine A. Beigelman ◽  
Matthias Kirsch
Keyword(s):  
Aortic Valve ◽  
Pulmonary Vein ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Rare Case ◽  
Venous Return ◽  
Scimitar Syndrome ◽  
Aortic Root Aneurysm ◽  
Superior Pulmonary Vein ◽  
The Right

AbstractA rare case of Scimitar Syndrome with an abnormal drainage of the right lower pulmonary vein into the right superior pulmonary vein associated with aortic root aneurism and bicuspid aortic valve is reported.

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