Pengzhu Li, Martina Bačová, Robert Dalla-Pozza, Nikolaus Alexander Haas, Felix Sebastian Oberhoffer^*

Congenital Heart Disease, Vol.17, No.2, pp. 129-141, 2022, DOI:10.32604/CHD.2022.018300

Abstract Turner syndrome (TS) is a rare disorder affecting 25–50 in 100000 female newborns. Bicuspid aortic valve (BAV) is assumed to be the most common congenital heart defect (CHD) in TS. In literature, reported BAV prevalence in TS ranges between 14% and 34%. The specific BAV prevalence in TS is still unknown. The 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… More >

Laura Schweikert¹, Dominik Gabbert¹, Sylvia Krupickova², Inga Voges^1,*

Congenital Heart Disease, Vol.16, No.6, pp. 551-560, 2021, DOI:10.32604/CHD.2021.016635

Abstract 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 patients. In 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 BAV and compared them with CoA patients with a tricuspid aortic valve (TAV). Methods: We included 48 patients with a… More >

Félix Collard¹, Dimitrios Buklas², Pascale Maragnes¹, Fabien Labombarda^1,*

Congenital Heart Disease, Vol.16, No.2, pp. 141-146, 2021, DOI:10.32604/CHD.2021.013180

Abstract 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 death. Data 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 population. We investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV patients. Methods 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… More >

Joseph M. Krepp¹, Mary J. Roman², Richard B. Devereux², Adrienne Bruce¹, Siddharth K. Prakash³, Shaine A. Morris⁴, Dianna M. Milewicz³, Kathryn W. Holmes⁵, William Ravekes⁵, Ralph V. Shohet⁶, Reed E. Pyeritz⁷, Cheryl L. Maslen⁸, Barbara L. Kroner⁹, Kim A. Eagle¹⁰, Liliana Preiss⁹, GenTAC Investigators^*, Federico M. Asch¹

Congenital Heart Disease, Vol.12, No.6, pp. 740-745, 2017, DOI:10.1111/chd.12520

Abstract Background: Unicuspid aortic valve (UAV) is a rare disorder, often difficult to distinguish from bicuspid aortic valve (BAV). BAV and UAV share valve pathology such as the presence of a raphe, leaflet fusion, aortic stenosis, aortic regurgitation, and/or ascending aortic dilatation, but a comprehensive echocardiographic comparison of patients with UAV and BAV has not been previously performed.
Methods: We investigated UAV and BAV patients at an early stage of disease included in GenTAC, a national registry of genetically related aortic aneurysms and associated cardiac conditions. Clinical and echocardiographic data from the GenTAC Registry were compared between 17 patients with UAV… More >

Natalie M. Hoeting¹, Courtney E. McCracken¹, Michael McConnell^1,2, Denver Sallee^1,2, Glen J. Iannucci^1,2, Matthew E. Oster^1,2

Congenital Heart Disease, Vol.12, No.4, pp. 417-420, 2017, DOI:10.1111/chd.12460

Abstract Objective: Bicuspid aortic valve (BAV) disease is associated with potential lifetime complications, but auscultation of a BAV click is commonly missed or mistaken for a benign split first heart sound. Our objective was to determine whether pediatric cardiologists could reliably distinguish between BAV clicks and benign split first heart sounds.
Design: Quality evaluation project using de-identified recordings from an outpatient pediatric cardiology clinic.
Outcome Measures: Twenty-one cardiologists listened to five de-identified recordings of pediatric heart sounds (three with BAV clicks, two with mitral components of benign split first heart sounds) and indicated whether they believed each recording was a BAV… More >

Talha Niaz¹, Joseph T. Poterucha¹, Jonathan N. Johnson^1,2, Cecilia Craviari¹, Thomas Nienaber¹, Jared Palfreeman¹, Frank Cetta^1,2, Donald J. Hagler^1,2

Congenital Heart Disease, Vol.12, No.3, pp. 261-269, 2017, DOI:10.1111/chd.12429

Abstract Background: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV.
Methods: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention.
Results: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and… More >

Melissa S.W. Yamauchi^1,2, Michael D. Puchalski^1,2, Hsin Ti Weng¹, Nelangi M. Pinto^1,2, Susan P. Etheridge^1,2, Angela P. Presson¹, Lloyd Y. Tani^1,2, L. LuAnn Minich^1,2, Richard V. Williams^1,2

Congenital Heart Disease, Vol.13, No.3, pp. 432-439, 2018, DOI:10.1111/chd.12591

Abstract Background: Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices.
Methods: Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/ 2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation,… More >

Ersin Çagrı Şimşek¹, Selcen Yakar Tülüce², Kamil Tülüce³, Sadık Volkan Emren², Serap Çuhadar⁴, Cem Nazlı²

Congenital Heart Disease, Vol.14, No.2, pp. 256-263, 2019, DOI:10.1111/chd.12718

Abstract Objective: The bicuspid aortic valve (BAV) is the most common congenital heart dis‐ ease. The process of aortic dilatation is not completely clear in patients with the BAV. Apelin is a peptide found at high levels in vascular endothelial cells which has a role in vascular regulation and cardiovascular function. The aim of this study was to de‐ termine the relationship between serum apelin levels and ascending aortic dilatation in adult patients with BAV.
Design: This cross‐sectional study included 62 patients with isolated BAV and to an age, gender, and body mass index‐matched control group of 58 healthy volunteers with… More >

Raffaele Giordano¹, Massimiliano Cantinotti², Giuseppe Comentale¹, Luigi Di Tommaso¹, Gabriele Iannelli¹¹, Emanuele Pilato¹, Gaetano Palma¹

Congenital Heart Disease, Vol.14, No.2, pp. 162-166, 2019, DOI:10.1111/chd.12680

Abstract Background: In this study, we compared our experience about early and midterm follow‐up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternot‐ omy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV).
Methods: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on indi‐ vidual surgeon’s preferences or when expressly requested by patient that was in‐ formed of nonconventional approach.
Results: We enrolled 61 patients, 23 in… More >