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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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Splanchnic-aortic inflammatory axis in experimental portal hypertension

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World Journal of Gastroenterology 2013年第44期19卷 7992-7999页

作者： Maria-Angeles Aller Natalia de las Heras Maria-Paz Nava Javier Regadera Jaime Arias Vicente Lahera Surgery Department School of Medicine Complutense University of Madrid 28040 MadridSpain Department of Physiology School of Medicine Complutense University of Madrid28040 Madrid Spain Animal Physiology II School of BiologyComplutense University of Madrid 28040 Madrid Spain Department of Histology and Neuroscience School of Medicine Universidad Autonoma Madrid 28046Spain

Splanchnic and systemic low-grade inflammation has been proposed to be a consequence of long-term prehepatic portal *** experimental model causes minimal alternations in the liver,thus making a more selective study possible for the pathological changes characteristic of prehepatic portal ***-grade splanchnic inflammation after longterm triple partial portal vein ligation could be associated with liver steatosis and portal hypertensive intestinal *** fact,we have previously shown that prehepatic portal hypertension in the rat induces liver steatosis and changes in lipid and carbohydrate metabolism similar to those produced in chronic inflammatory conditions described in metabolic syndrome in *** and bacterial translocation in this experimental model suggest the existence of a portal hypertensive intestinal microbiome implicated in both the splanchnic and systemic alterations related to prehepatic portal *** the systemic impairments,aortopathy characterized by oxidative stress,increased levels of proinflammatory cytokines and profibrogenic mediators stand *** this experimental model of long-term triple portal vein ligated-rats,the abdominal aortic proinflammatory response could be attributed to oxidative ***,the increased aortic reduced-nicotinamide-adenine dinucleotide phosphate[NAD(P)H]oxidase activity could be associated with reactive oxygen species production and promote aortic ***,oxidative stress mediated by NAD(P)H oxidase has been associated with risk factors for inflammation and *** splanchnic and systemic pathology that is produced in the long term after triple partial portal vein ligation in the rat reinforces the validity of this experimental model to study the chronic low-grade inflammatory response induced by prehepatic portal hypertension.

关键词： Portal hypertension Inflammation aortopathy Hepatic steatosis

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Predictors for Dilated Aorta in Repaired and Unrepaired Tetralogy of Fallot

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World Journal of Cardiovascular Diseases 2015年第8期5卷 233-253页

作者： Khaled R. Abd El Meguid Hesham B. Mahmoud Mostafa M. Mohammad Division of Cardiology Benisuef University Hospital Benisuef Egypt Division of Cardiology National Heart Institute Cairo Egypt

Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13

关键词： Tetralogy of Fallot aortopathy Ventricular Septal Defect Aortic Regurgitation Pulmonary Regurgitation Pulmonary Atresia

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Effect of Bicuspid Aortic Valve Cusp Fusion on Aorta Wall Shear Stress: Preliminary Computational Assessment and Implication for Aortic Dilation

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World Journal of Cardiovascular Diseases 2015年第6期5卷 129-140页

作者： Kai Cao Philippe Sucosky Department of Aerospace and Mechanical Engineering University of Notre Dame Notre Dame USA

The bicuspid aortic valve (BAV) is a major congenital valvular abnormality and is associated with a high prevalence of aortic dilation, whose expression depends on the type of leaflet fusion. Although BAV hemodynamics is considered a potential pathogenic contributor, the impact of BAV fusion on ascending aorta (AA) wall shear stress (WSS) remains largely unknown. A fluid-structure interaction approach was implemented to predict the hemodynamics and WSS characteristics in realistic AA models subjected to the flow of a normal tricuspid aortic valve (TAV) and three BAV morphotypes (left-right coronary cusp fusion (LR), right-non coronary cusp fusion (RN) and non-left coronary cusp fusion (NL)). TAV flow conditions subjected the proximal and middle AA to a streamlined flow typical of flows in bends, while BAV flow conditions generated increased flow helicity. The LR-BAV orifice jet generated flow abnormalities primarily in the proximal AA, which were marked by a uniform WSS overload along the wall circumference and contrasted WSS directionalities on the wall convexity and concavity. Flow abnormalities generated by the RN-BAV and NL-BAV inlet flow conditions were more diffuse and consisted of WSS overloads in the convexity of the proximal and middle AA and contrasted WSS directionalities in the anterior and posterior wall regions. This study demonstrates the impact of the BAV morphotype on AA hemodynamics and provides quantitative evidence for the existence of WSS abnormalities in aortic wall regions prone to dilation.

关键词： Bicuspid Aortic Valve (BAV) Aorta aortopathy Hemodynamics Fluid-Structure Interaction (FSI)

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Bicuspid aortic valve hemodynamics does not promote remodeling in porcine aortic wall concavity

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World Journal of Cardiology 2016年第1期8卷 89-97页

作者： Samantha K Atkins Alison N Moore Philippe Sucosky Department of Aerospace and Mechanical Engineering University of Notre Dame Department of Mechanical and Materials Engineering Wright State University

AIM: To investigate the role of type-I left-right bicuspid aortic valve(LR-BAV) hemodynamic stresses in the remodeling of the thoracic ascending aorta(AA) concavity, in the absence of underlying genetic or structural ***: Transient wall shear stress(WSS) profiles in the concavity of tricuspid aortic valve(TAV) and LR-BAV AAs were obtained computationally. Tissue specimens excised from the concavity of normal(nondilated) porcine AAs were subjected for 48 h to those stress environments using a shear stress bioreactor. Tissue remodeling was characterized in terms of matrix metalloproteinase(MMP) expression and activity via immunostaining and gelatin ***: Immunostaining semi-quantification results indicated no significant difference in MMP-2 and MMP-9 expression between the tissue groups exposed to TAV and LR-BAV AA WSS(P = 0.80 and P = 0.19, respectively). Zymography densitometry revealed no difference in MMP-2 activity(total activity, active form and latent form) between the groups subjected to TAV AA and LR-BAV AA WSS(P = 0.08, P = 0.15 and P = 0.59, respectively).CONCLUSION: The hemodynamic stress environment present in the concavity of type-I LR-BAV AA does not cause any significant change in proteolytic enzyme expression and activity as compared to that present in the TAV AA.

关键词： Bicuspid aortic valve Fluid shear stress aortopathy Remodeling Matrix metalloproteinases

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