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bicuspid aortic valve with associated aortopathy, significant left ventricular hypertrophy or concomitant hypertrophic cardiomyopathy: A diagnostic and therapeutic challenge

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World Journal of Clinical Cases 2023年第18期11卷 4251-4257页

作者： Ivana Sopek Merkaš Nenad Lakušić Mladen Predrijevac KrešimirŠtambuk Maja Hrabak Paar Department of Cardiology Special Hospital for Medical Rehabilitation Krapinske TopliceKrapinske Toplice 49217Croatia Department of Clinical Medicine Faculty of Dental Medicine and Health OsijekJ.J.Strossmayer University of OsijekOsijek 31000Croatia Department of Internal Medicine Family Medicine and History of MedicineFaculty of Medicine OsijekJ.J.Strossmayer University of OsijekOsijek 31000Croatia Department of Cardiovascular Surgery Magdalena Clinic for Cardiovascular DiseasesKrapinske Toplice 49217Croatia Department of Cardiology Magdalena Clinic for Cardiovascular DiseasesKrapinske Toplice 49217Croatia Department of Diagnostic and Interventional Radiology University Hospital Centre ZagrebZagreb 10000Croatia School of Medicine University of ZagrebZagreb 10000Croatia

Due to its prevalence of 0.5%to 2%in the general population,with a 75%predominance among men,bicuspid aortic valve is the most common congenital heart *** is frequently accompanied by other cardiac congenital anomalies,and clinical presentation can vary significantly,with stenosis being the most common manifestation,often resulting in mild to moderate concentric hypertrophy of the left *** is the primary diagnostic modality utilized for establishing the diagnosis,and it is often the sole diagnostic tool relied upon by ***,due to the heterogeneous clinical presentation and possible associated anomalies(which are often overlooked in clinical practice),it is necessary to employ various diagnostic methods and persist in finding the accurate diagnosis if multiple inconsistencies *** employing this approach,we can effectively manage these patients and provide them with appropriate *** a clinical case from our practice,we provide an overview of the literature on bicuspid aortic valve with aortophaty and the possible association with hypertrophic cardiomyopathy,diagnostic methods,and treatment *** review article highlights the critical significance of achieving an accurate diagnosis in patients with bicuspid aortic valve and significant left ventricular *** is crucial to exclude other possible causes of left ventricular outflow tract obstruction,such as sub-or supra-aortic obstructions,and hypertrophic cardiomyopathy.

关键词： bicuspid aortic valve Left ventricular hypertrophy Left ventricular tract obstruction Hypertrophic cardiomyopathy Aortopathy Multimodal imaging

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Prevalence of bicuspid aortic valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis

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Congenital Heart Disease 2022年第2期17卷 129-140页

作者： Pengzhu Li Martina Bacova Robert Dalla-Pozza Nikolaus Alexander Haas Felix Sebastian Oberhoffer Department of Pediatric Cardiology and Intensive Care Medical Hospital of the University of MunichLudwig Maximilians University MunichMunichGermany

Turner syndrome(TS)is a rare disorder affecting 25–50 in 100000 female *** aortic valve(BAV)is assumed to be the most common congenital heart defect(CHD)in *** literature,reported BAV prevalence in TS ranges between 14%and 34%.The specific BAV prevalence in TS is still *** aim of this study was to give a more precise estimation of BAV prevalence in TS by conducting a meta-analysis of TS-studies,which detected BAV by either cardiac magnetic resonance imaging(MRI)or cardiac computed tomography(CT).We searched PubMed,Cochrane Library,and Web of Science databases to collect observational studies including the prevalence of BAV identified by cardiac MRI or cardiac CT in TS patients up to June 4th,*** screening for inclusion,data extraction,and quality assessment by two independent reviewers,the meta-analysis was performed with R 4.1.1 *** are shown as proportion and weighted mean difference with 95%confidence intervals(95%CI).In total,11 studies involving 1177 patients were *** data showed that the prevalence of BAV in TS patients was 23.7%(95%CI:21.3%to 26.1%).No high heterogeneity was found between the included *** current meta-analysis reveals that BAVcan be detected in 23.7%of TS patients receiving cardiac MRI or cardiac ***,BAV can be considered as the most common CHD in *** to TTE,cardiac MRI and cardiac CT might represent superior imaging modalities in BAV assessment of adult TS patients.

关键词： Turner syndrome bicuspid aortic valve magnetic resonance imaging(MRI) tomography X-ray computed(CT)

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bicuspid aortic valve Disease in Turner Syndrome: A Meta-Analysis of Prevalence

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International Journal of Clinical Medicine 2022年第7期13卷 276-286页

作者： Erick Thokerunga Yahya-Abdullahi Ali Christopher Ntege Department of Clinical Laboratory Medicine Center for Gene Diagnosis Zhongnan Hospital of University Wuhan China Department of Cardiology First Affiliated Renmin Hospital of Wuhan University Wuhan China Department of Radiology and Nuclear Medicine Zhongnan Hospital of Wuhan University Wuhan China

Turner syndrome patients partially or completely lack the X chromosome. 1 - 2500 female live births are affected. Clinical features include webbed neck, short stature, broad chest etc. bicuspid aortic valve disease (BAV) occurs in more than 30% of Turner syndrome patients causing significant morbidity and mortality. We aimed to establish a more reliable estimate of the prevalence of BAV in Turner syndrome. PubMed, Embase and PsycINFO databases were searched until 2022. Review Manager (RevMan 5.4.1) and the JASP software (0.16.00) were used for meta-analysis. 15 studies with a total of 3189 patients were combined. The pooled prevalence of BAV in Turner syndrome was 22.0% (95% CI: 15.0% - 29.0%). Sub group analysis by 45, X0 karyotype and age had prevalence of 24.0% and 8% respectively. The studies had high heterogeneity and possible publication biases. In summary, the study established that the prevalence of BAV in Turner syndrome patients diagnosed by echocardiogram, CT and MRI scans, is 22.0%, and 24% in patients with true monosomy 45, X0 karyotypes. Routine BAV exam should pay particular attention to monosomy 45, X0 karyotype patients, and where possible, CT and MRI should always accompany echocardiography for BAV screening, especially for pediatrics.

关键词： bicuspid aortic valve Turner Syndrome Meta-Analysis Prevalence

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Ascending aortic Aneurysm and Dissection Secondary to bicuspid aortic valve with Concomitant Coarctation of Descending Aorta Successfully Repaired with Extracorporeal Membrane Oxygenation Support: A Case Report

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Cardiology Discovery 2022年第2期2卷 124-126页

作者： Qin Jiang Juan Du Tao Yu Xiaobo Huang Mingliang Zuo Keli Huang Department of Cardiac Surgery Sichuan Provincial People’s HospitalAffiliated Hospital of University of Electronic Science and TechnologyChengduSichuan 610072China Department of Operating Room Sichuan Provincia People’s HospitalAffiliated Hospital of University of Electronic Science and TechnologyChengduSichuan 610072China Department of Surgical Intensive Care Unit Sichuan Provincia People’s HospitalAffiliated Hospital of University of Electronic Science and TechnologyChengduSichuan 610072China Department of Medica Ultrasonics Sichuan Provincia People’s HosptalAffiliated Hospital of University of Electronic Science and TechnologyChengduSichuan 610072China

Type A aortic aneurysm and dissection secondary to bicuspid aortic valve(BAV)with untreated coarctation of the aorta(CoA)in adults is a rare finding because there are almost no“abnormalities”in these patients’medical ***,we report on a 47-year-old man with unexplained weakness followed by *** was diagnosed with cardiogenic shock and underwent venoarterial extracorporeal membrane oxygenation(VA-ECMO).BAV,ascending aortic aneurysm and dissection(based on bedside echocardiography)and concomitant CoA(based on computed tomography angiography)were *** patient then underwent emergency surgery including aortic root replacement with a composite mechanical valve conduit,additional ascending-to-descending aortic bypass,and coronary artery bypass grafting with a saphenous vein graft to the right coronary *** treatment,he recovered ***,here we present a case involving a patient in a critical condition with ascending aortic aneurysm and dissection secondary to complex congenital heart defects,who was successfully treated with composite surgical procedures combined with life-saving VA-ECMO.

关键词： aortic aneurysm Dissection bicuspid aortic valve Case report Coarctation of the aorta Extracorporeal membrane oxygenation

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The Impact of a bicuspid aortic valve on aortic Geometry and Function in Patients with aortic Coarctation:A Comprehensive CMR Study

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Congenital Heart Disease 2021年第6期16卷 551-560页

作者： Laura Schweikert Dominik Gabbert Sylvia Krupickova Inga Voges Department of Congenital Heart Disease and Paediatric Cardiology University Hospital Schleswig-HolsteinKielGermany Royal Brompton Hospital LondonUK

Background:An isolated bicuspid aortic valve(BAV)is associated with structural and functional abnormalities of the aorta and the left ventricle(LV).Although~50%of patients with aortic coarctation(CoA)have a BAV,less is known about its impact on LV function and aortic geometry and function in CoA *** this cardiovascular magnetic resonance imaging(CMR)study,we analysed markers of LV and aortic function as well as aortic geometry in a large cohort of CoA patients with a BAVand compared them with CoA patients with a tricuspid aortic valve(TAV).Methods:We included 48 patients with a BAV(18.4±9.3 years)and 45 patients with TAV(20.7±9.9 years).LV volumes,mass and ejection fraction as well as aortic distensibilty,pulse wave velocity(PWV)were measured from standard cine CMR and phase-contrast CMR images.2-dimensional CMR feature tracking(2DCMR-FT)was performed to measure longitudinal,circumferential and radial strain and strain rate of the *** arch geometry was classified as romanic,gothic and ***:LV volumes,mass and ejection fraction as well as aortic distensibility and PWV did not significantly differ between the BAV and the TAV *** was also no significant difference for LV global longitudinal,radial and circumferential strain and strain rate between both *** with a BAV had more commonly a gothic aortic arch compared to TAV patients,but this difference was not statistically significant(22 vs.14,p=0.2).Ascending and descending aortic distensibility correlated with LV mass in the entire patient group(paortic function in children and young adults with *** correlation of global circumferential,longitudinal and radial strain values with LV ejection fraction dem

关键词： aortic coarctation bicuspid aortic valve cardiovascular magnetic resonance feature tracking

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Acquired Gerbode Defect Secondary to Severe bicuspid aortic valve Endocarditis

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Case Reports in Clinical Medicine 2022年第3期11卷 55-62页

作者： Mohamed Elbayomi Michael Weyand Ehab Nooh Jö rg Fechner Frank Harig Department of Cardiac Surgery Friedrich-Alexander University Erlangen Germany Department of Anaesthesiology Friedrich-Alexander University Erlangen Germany

An annular aortic abscess is a fatal complication of infective endocarditis. Echocardiography is the initial imaging modality to confirm the diagnosis in suspected infective endocarditis. Here, we present a case of a bicuspid aortic valve infective endocarditis caused by Staphylococcus aureus and complicated with annular aortic abscess resulting in acquired Gerbode defect (type II) with tricuspid valve vegetation which was undiagnosed preoperatively. The intraoperative transoesophageal echocardiography yields a new finding prior to the surgical incision, which impacted the clinical decision-making and increased the burden of the procedure.

关键词： Acquired Gerbode Defect Infective Endocarditis Staphylococcus aureus bicuspid aortic valve

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High-Risk Congenital Coronary Abnormalities in Patients with bicuspid aortic valve

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Congenital Heart Disease 2021年第2期16卷 141-146页

作者： Félix Collard Dimitrios Buklas Pascale Maragnes Fabien Labombarda Department of Cardiology CHU de CaenCaen14000France Department of Heart Surgery CHU de CaenCaen14000France

Objective:Abnormal coronary artery origin(ACAO)from the opposite sinus with inter-arterial course of the ectopic proximal vessel is associated with the greatest potential for clinical manifestations,specifically sudden *** remain limited regarding the association between bicuspid aortic valve(BAV)and this potentially dangerous coronary variant reported in up to 0.6%in the general *** investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV *** and Results:We conducted a retrospective study to identify BAV patients with ACAO and inter-arterial course who underwent elective aortic valve/root surgery between 2010 and 2019 in our tertiary center.A total of 279 consecutive patients with BAV were *** these,four patients(1.4%)had ACAO with inter-arterial *** patients had abnormal right coronary artery arising from the theoretical opposite coronary sinus with intramural *** fourth patient presented an abnormal left coronary artery from a single coronary artery arising from the theoretical right coronary sinus with a long inter-arterial ***:Our results from a single-center experience support high-risk ACAOS may be more frequently diagnosed in BAV patients,especially in surgical series,justifying a careful preoperative assessment.

关键词： Congenital heart disease coronary artery bicuspid aortic valve

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Infective bicuspid aortic valve endocarditis causing acute severe regurgitation and heart failure:A case report

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World Journal of Clinical Cases 2021年第5期9卷 1221-1227页

作者： Chang Hou Wu-Chao Wang Hong Chen Yuan-Yuan Zhang Wei-Min Wang Department of Cardiology Peking University People’s HospitalBeijing 100044China Department of Emergency Peking University People’s HospitalBeijing 100044China Department of Pathology Peking University People’s HospitalBeijing 100044China

BACKGROUND Infective endocarditis(IE)is an uncommon but potentially life-threatening infection,which occasionally develops into acute severe valve insufficiency leading to the onset of heart failure,and necessitates timely ***,the variable and atypical clinical manifestations always make the early detection of IE difficult and *** SUMMARY A 45-year-old female who was previously healthy presented with exertional shortness of breath and paroxysmal nocturnal *** also suffered from a significant decrease in exercise capacity,whereas her body temperature was *** had severe hypoxemia and hypotension along with a marked aortic valve *** pulmonary edema and bilateral pleural effusion were observed on both chest X-ray and computed tomography *** echocardiography was performed immediately and revealed severe regurgitation of the bicuspid aortic *** echocardiography was further performed and vegetations were *** addition to adequate medical therapy and ventilation support,the patient underwent urgent and successful aortic valve *** symptoms were significantly relieved and the postoperative chest X-ray showed that pulmonary edema was significantly *** of the resected valve and positive microorganism culture of the surgical specimen provided evidence of definite *** IE should be considered in critical patients with refractory heart failure caused by severe bicuspid aortic valve regurgitation.

关键词： Infective endocarditis Acute heart failure Acute severe aortic regurgitation bicuspid aortic valve Echocardiography Case report

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Effect of transcatheter aortic valve replacement on bicuspid coronary hemodynamics:A numerical study

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Medicine in Novel Technology and Devices 2023年第2期18卷 191-197页

作者： Hongge Wei Zhuoran Qu Ziyao Geng Qianwen Hou Tianming Du Xinjin Luo Juntao Qiu Aike Qiao Faculty of Environment and Life Beijing University of TechnologyBeijing100124China Cardiovascular Surgery Fuwai HospitalPeking Union Medical CollegeChinese Academy of Medical SciencesBeijing100037China

To investigate the effects of transcatheter heart valve(THV)poses and bicuspid aortic valve(BAV)subtypes on coronary hemodynamics after transcatheter aortic valve replacement(TAVR).The computational models for BAV included left-right fusion(LR),non-coronary-left fusion(LN)and non-coronary-right fusion(RN).THV deployment height is defined as the distance below the aortic annulus of the lowest point of the stent,H_(1)=2mm,H_(2)=5mm and H_(3)=8mm,Orientations include O_(1)(one commissure is aligned with the raphe)and O_(2)(one commissure is aligned with the axis of non-fused leaflet symmetry).The maximum flow velocity(Vmax),mean wall shear stress(mWSS)and coronary perfusion pressure(CPP)of coronary were obtained by computational fluid dynamics(CFD)*** CPP was 59%in left coronary and 82%in right coronary higher than that before *** O_(1),the CPP of the LN left coronary and the RN right coronary was 74%and 79%higher than that before *** O_(2),the CPP of the LN right coronary and the RN left coronary was 83%and 82%higher than that before *** the THV deployment height is less than 2mm,Vmax and CPP of coronary arteries do not return to healthy TAV levels or changed *** overlap of the THV commissure with the coronary ostium makes the coronary CPP so large that it exceeds the level of a healthy TAV.

关键词： Transcatheter aortic valve replacement bicuspid aortic valve Coronary artery Hemodynamics Computational fluid dynamics

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Trans-Frame aortic Regurgitation of New-Generation aortic Bioprosthesis

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World Journal of Cardiovascular Surgery 2024年第4期14卷 41-44页

作者： Michael Timothy Simpson Kasmir Ramo Vinayak Bapat Andrea Miltiades Isaac George Division of Cardiothoracic Surgery New York-Presbyterian Hospital Columbia University Medical Center New York USA Division of Cardiothoracic Anesthesia New York-Presbyterian Hospital Columbia University Irving Medical Center New York USA Division of Cardiothoracic Surgery Abbott Northwestern Hospital Minneapolis Heart Institute Minneapolis USA

A widely used aortic valve bioprosthesis is susceptible to regurgitation between the sewing ring and the frame of the valve due to its relatively thin fabric coverage. In some cases this leak has been shown to resolve with administration of protamine, however, tension on this area from annular sutures placed in an asymmetric bicuspid valve annulus may exacerbate the defect.

关键词： aortic valve Replacement bicuspid aortic valve Paravalvular Leak

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