Project description:Objectives:We aimed to assess the procedural and clinical results of transcatheter aortic valve replacement (TAVR) for nonraphe bicuspid aortic stenosis (AS) with coronary vs mixed cusp fusion. Background:It remains unclear whether cusp fusion morphology affects TAVR outcomes in patients with nonraphe bicuspid AS. Methods:This retrospective study enrolled consecutive patients with severe symptomatic AS and type-0 bicuspid aortic valve, who underwent TAVR at our institution between 2012 and 2017. TAVR outcomes were defined based on the Valve Academic Research Consortium-2 recommendations. Results:Compared to patients with mixed cusp fusion (44/71), those with coronary cusp fusion (27/71) had a larger ellipticity index for the aortic annulus (21.9%?±?9.0% vs 15.6%?±?9.3%, p=0.007) and increased left ventricular outflow tract obstruction (31.1%?±?9.4% vs 26.9%?±?7.5%, p=0.04) but comparable rates of second valve implantation (15.9% vs 14.8%), mild paravalvular leakage (PVL, 38.5% vs 30.2%), permanent pacemaker implantation (PPM, 25.9% vs 15.9%), and 30-day mortality (7.4% vs 6.8%). Use of a first-generation transcatheter heart valve was associated with higher risk for mild PVL (odds ratio (OR)?=?4.37; 95% confidence interval (95% CI)?=?1.14-16.75; p=0.03) but not PPM (OR?=?0.77; 95% CI?=?0.22-2.62; p=0.67), whereas a larger oversizing ratio tended to be associated with a higher PPM rate (OR?=?1.49; 95% CI?=?0.46-4.86; p=0.51) but lower incidence of mild PVL (OR?=?0.51; 95% CI?=?0.19-1.35; p=0.17). Conclusions:In AS patients with type-0 bicuspid valves, cusp fusion morphology does not affect the procedural or clinical results of TAVR. Use of second-generation transcatheter heart valves may provide more favorable results in such patients. This trial is registered with NCT01683474.
Project description:BACKGROUND:Aortic 3-dimensional blood flow was analyzed to investigate altered ascending aorta (AAo) hemodynamics in bicuspid aortic valve (BAV) patients and its association with differences in cusp fusion patterns (right-left, RL versus right-noncoronary, RN) and expression of aortopathy. METHODS AND RESULTS:Four-dimensional flow MRI measured in vivo 3-dimensional blood flow in the aorta of 75 subjects: BAV patients with aortic dilatation stratified by leaflet fusion pattern (n=15 RL-BAV, mid AAo diameter=39.9±4.4 mm; n=15 RN-BAV, 39.6±7.2 mm); aorta size controls with tricuspid aortic valves (n=30, 41.0±4.4 mm); healthy volunteers (n=15, 24.9±3.0 mm). Aortopathy type (0-3), systolic flow angle, flow displacement, and regional wall shear stress were determined for all subjects. Eccentric outflow jet patterns in BAV patients resulted in elevated regional wall shear stress (P<0.0125) at the right-anterior walls for RL-BAV and right-posterior walls for RN-BAV in comparison with aorta size controls. Dilatation of the aortic root only (type 1) or involving the entire AAo and arch (type 3) was found in the majority of RN-BAV patients (87%) but was mostly absent for RL-BAV patients (87% type 2). Differences in aortopathy type between RL-BAV and RN-BAV patients were associated with altered flow displacement in the proximal and mid AAo for type 1 (42%-81% decrease versus type 2) and distal AAo for type 3 (33%-39% increase versus type 2). CONCLUSIONS:The presence and type of BAV fusion was associated with changes in regional wall shear stress distribution, systolic flow eccentricity, and expression of BAV aortopathy. Hemodynamic markers suggest a physiological mechanism by which the valve morphology phenotype can influence phenotypes of BAV aortopathy.
Project description:Based on the natural mathematical relationships between the components of the human tri-leaflet aortic valve, new calibrated cusp sizers were developed in order to facilitate aortic valve assessment in the operating room and enhance the chance for a perfect restoration of aortic valve competence. These sizers were used clinically to guide the implementation of established aortic valve repair techniques in 10 consecutive patients with severe aortic valve regurgitation. Valve repair was successful in all cases, and at a median follow-up was 5.5 months, aortic valve function remained stable, with aortic regurgitation ?1+ in every patient and no significant gradient across the aortic valves. This preliminary clinical experience indicates that the calibrated cusp sizers can provide reliable insight into the mechanism of aortic valve insufficiency, and can guide aortic valve repair techniques successfully. We hope that the simplicity and reproducibility of this method would assist in its dissemination and further increase the percentage of aortic valves that are repaired when compared with current practice.
Project description:Bicuspid aortic valve (BAV) is the most common adult congenital heart defect and is found in 0.5% to 2.0% of the general population. The term "BAV" refers to a heterogeneous group of disorders characterized by diverse aortic valve malformations with associated aortopathy, congenital heart defects, and genetic syndromes. Even after decades of investigation, the genetic determinants of BAV and its complications remain largely undefined. Just as BAV phenotypes are highly variable, the genetic etiologies of BAV are equally diverse and vary from complex inheritance in families to sporadic cases without any evidence of inheritance. In this paper, the authors discuss current concepts in BAV genetics and propose a roadmap for unraveling unanswered questions about BAV through the integrated analysis of genetic and clinical data.
Project description:Although it is attractive, a limitation of aortic valve (AV) replacement (AVR) through a mini-thoracotomy approach (mini-AVR) is the limited exposure of the AV. Here, we present a simple exposure technique named "suspending commissural sutures" for a more efficient mini-AVR. The technique involves making 3 half-depth stitches with 1-0 silk at each of the commissures, which are anchored to each corresponding pericardial surface. These stitches are tightened up so that the aortic root is axially expanded and is pulled upward. The technique of suspending commissural stitches seems to offer reasonable exposure of the AV in mini-AVR, and shows excellent early surgical outcomes.
Project description:A 37-year-old male presented to the Emergency Department with acute worsening of back pain and new onset dyspnea. Transthoracic echocardiography revealed moderate left ventricular dysfunction and a bicuspid aortic valve (BAV). In addition, he was noted to have a dilated thoracic aorta concerning for a dissection, severe aortic insufficiency (AI), and both a pericardial and pleural effusion. Magnetic resonance imaging revealed a Type A ascending aortic dissection. He was taken emergently to the operating room for repair. An intraoperative transesophageal echocardiography examination was performed which revealed a normal trileaflet AV with a Type A aortic dissection flap masquerading as a BAV. The dissection flap interfered with both the valve's function, causing severe AI, as well as the valve's appearance, causing it to look bicuspid on echocardiography.
Project description:We report the successful repair of a bicuspid aortic valve with vegetation on its thickened raphe by using two pericardial patches. After excising the vegetation and thickened raphe, the first patch was sewn between the remaining leaflets. Another patch was then sewn at the base of the cusp to create sufficient geometrical height for good coaptation. Our two-patch technique may facilitate intraoperative accommodation of the 3-D shape of the new cusp.
Project description:ObjectivesA bicuspid aortic valve (BAV) is characterized by variable phenotypic manifestations, as well as longitudinal evolution of valve dysfunction and ascending aorta dilatation. The present study investigated the impact of severe aortic stenosis (AS) on the flow patterns and wall shear stress (WSS) distribution in BAV patients with right-left (RL) and right-non-coronary (RN) cusp fusion types, and the study aimed to reveal whether aortic dysfunction could further alter intrinsic aortic haemodynamic aberrations generated by abnormal BAV cusp fusion patterns.MethodsFour-dimensional flow magnetic resonance imaging was performed in 120 BAV subjects and 20 tricuspid aortic valve controls. BAV patients were evenly categorized into 4 cohorts, including RL and RN BAV with no more than mild aortic dysfunction as well as RL and RN BAV-AS with isolated severe AS.ResultsBAV subjects exhibited eccentric outflow jets resulting in regional WSS elevation at the right-anterior position of the ascending aorta in the RL group and the right-posterior location in the RN group (P?<?0.005). The presence of severe AS resulted in accelerated outflow jets and more prominent flow and WSS eccentricity (P?<?0.005) by marked helical (P?=?0.014) and vortical flow formation (P?<?0.005), as well as increased prevalence of tubular and transverse arch dilatation. The changes to the flow jet in BAV-AS subjects blurred the differences in peak flow velocity and WSS distribution between RL and RN BAV. Differences in the phenotypes of aortopathy were associated with changes in functional haemodynamic parameters such as flow displacement and WSS eccentricity.ConclusionsSevere AS markedly exacerbated aortic flow aberrations in BAV patients and masked the existing distinct flow features deriving from RL and RN fusion types. Longitudinal studies are needed to investigate the evolution of ascending aortic dilatation relative to the interaction between intrinsic cusp fusion types and acquired severe valve dysfunction.
Project description:OBJECTIVES: Continuous-flow left ventricular assist devices (cf-LVADs) may induce commissural fusion of the aortic valve leaflets. Factors associated with this occurrence of commissural fusion are unknown. The aim of this study was to examine histological characteristics of cf-LVAD-induced commissural fusion in relation to clinical variables. METHODS: Gross and histopathological examinations were performed on 19 hearts from patients supported by either HeartMate II (n = 17) or HeartWare (n = 2) cf-LVADs and related to clinical characteristics (14 heart transplantation, 5 autopsy). RESULTS: Eleven of the 19 (58%) aortic valves showed fusion of single or multiple commissures (total fusion length 11 mm [4-20] (median [interquartile range]) per valve), some leading to noticeable nodular displacements or considerable lumen diameter narrowing. Multiple fenestrations were observed in one valve. Histopathological examination confirmed commissural fusion, with varying changes in valve layer structure without evidence of inflammatory infiltration at the site of fusion. Commissural fusion was associated with continuous aortic valve closure during cf-LVAD support (P = 0.03). LVAD-induced aortic valve insufficiency developed in all patients with commissural fusion and in 67% of patients without fusion. Age, duration of cf-LVAD support and aetiology of heart failure (ischaemic vs dilated cardiomyopathy) were not associated with the degree of fusion. CONCLUSIONS: Aortic valve commissural fusion after support with cf-LVADs is a non-inflammatory process leading to changes in valve layer structure that can be observed in >50% of cf-LVAD patients. This is the first study showing that patients receiving full cf-LVAD support without opening of the valve have a significantly higher risk of developing commissural fusion than patients on partial support.