Bicuspid Aortic Valve Stenosis Research Articles

Comparison of One- and 12-Month Outcomes of Transcatheter Aortic Valve Replacement in Patients With Severely Stenotic Bicuspid Versus Tricuspid Aortic Valves (Results from a Multicenter Registry)

The aim of the study was to evaluate the transcatheter aortic valve replacement (TAVR) in high-risk patients with severe bicuspid aortic valve (BAV) stenosis and to compare the outcomes with a matched group of patients with tricuspid aortic valve. TAVR became an alternative treatment method in high-risk patients with symptomatic aortic stenosis; however, BAV stenosis is regarded as a relative contraindication to TAVR. The study population comprised 28 patients with BAV who underwent TAVR. BAV was diagnosed based on a transesophageal echocardiography. CoreValve and Edwards SAPIEN prostheses were implanted. The control group consisted of 84 patients (3:1 matching) with significant tricuspid aortic valve stenosis treated with TAVR. There were no significant differences between patients with and without BAV in device success (93% vs 93%, p= 1.0), risk of annulus rupture (0% in both groups), or conversion to cardiosurgery (4% vs 0%, respectively, p= 0.25). The postprocedural mean pressure gradient (11.5 ± 6.4 vs 10.4 ± 4.5mm Hg, p=0.33), aortic regurgitation grade ≥2 of 4 (32% vs 23%, p= 0.45), 30-day mortality (4% vs 7%, p= 0.68), and 1-year all-cause mortality (19% vs 18%, p= 1.00) did not differ between the groups. Echocardiography showed well-functioning valve prosthesis with a mean prosthetic valve area of 1.6 ± 0.4cm(2) versus 1.7 ± 0.3cm(2) (p= 0.73), a mean pressure gradient of 10.3 ± 5.4 versus 9.8 ± 2.8mm Hg (p= 0.64), and aortic regurgitation grade ≥2 of 4 (22% vs 22%, p= 1.00) for the 2 groups. In conclusion, selected high-risk patients with BAV can be successfully treated with TAVR, and their outcomes are similar to those reported in patients without BAV.

Choc cardiogénique sur bicuspidie aortique sténosante traité par remplacement valvulaire aortique percutané en urgence

Transcatheter aortic valve implantation is a therapeutic option for high-risk patients with severe aortic valve stenosis and with cardiac symptoms. This procedure requires the preliminary evaluation by a “heart team” and presents some contraindications. We report the case of a 58-year-old man with severe bicuspid aortic valve stenosis and cardiogenic shock. In spite of contraindications and because of the failure of balloon aortic valvuloplasty, transcatheter aortic valve implantation was performed in emergency.

Fulminant diffuse systemic sclerosis following aortic valve replacement

We present a case of fulminant diffuse systemic sclerosis (dSSc) developed after the aortic valve replacement followed by fatal congestive heart failure within the 6months from the initial symptoms. A 61-year-old male developed rapidly progressive diffuse systemic sclerosis following aortic valve replacement due to stenosis of bicuspid aortic valve. He presented with diarrhoea, weight loss, mialgia and arthralgia after cardiac surgery. Heart failure, due to myocardial fibrosis, was noted as a cause of death. We hypothesize that artificial materials like the ones used in mechanical valves or silicon materials in breast implants may induce fulminant course of pre-existing systemic sclerosis or create a new onset in predisposed individual.

Mixed partial anomalous pulmonary venous drainage coexistent with an aortic valve abnormality – analysis of ultrasound diagnostics in a 10-year-old girl with Turner syndrome

The authors present a case of echocardiographic diagnosis of a rare congenital cardiovascular anomaly in the form of mixed partial anomalous pulmonary veins connection in a 10-year-old girl with Turner syndrome and congenital mild stenosis of insufficient bicuspid aortic valve, made while diagnosing the causes of intestinal tract bleeding. The article presents various diagnostic difficulties leading to the delayed determination of a correct diagnosis, resulting from the absence of symptoms of circulatory failure in the early stage of the disease and the occurrence of severe and dominant auscultatory phenomena typical for congenital aortic valve defect which effectively masked the syndromes of increased pulmonary flow. The authors discuss the role of the impact of phenotypic characteristics of the Turner syndrome, in particular a short webbed neck restricting the suprasternal echocardiographic access and the presence of psychological factors associated with a long-term illness. The importance of indirect echocardiographic symptoms suggesting partial anomalous pulmonary veins connection in the presence of bicuspid aortic valve, e.g. enlargement of the right atrium and right ventricle, and paradoxical interventricular septum motion were emphasized in patients lacking ASD, pulmonary hypertension or tricupid and pulmonary valve abnormalities. The methodology of echocardiographic examination enabling direct visualization of the abnormal vascular structures was presented. Special attention was paid to the significance of highly sensitive echocardiographic projections: high right and left parasternal views in sagittal and transverse planes with patient lying on the side, with the use of two-dimensional imaging and color Doppler. Finally, the limitations of echocardiography resulting from the visualization and tracking of abnormal vascular structures hidden behind ultrasound non-conductive tissues were indicated, as was the role of other diagnostic modalities, such as angio-CT and/or nuclear magnetic resonance.

PM100 Systemic inflammatory activation and endothelial dysfunction predict development of aortic valve stenosis in bicuspid aortic valve

PM100 Systemic inflammatory activation and endothelial dysfunction predict development of aortic valve stenosis in bicuspid aortic valve

M1/M2 macrophages and associated mechanisms in congenital bicuspid aortic valve stenosis

The aim of this study was to observe macrophage infiltration in congenital bicuspid aortic valve (CBAV) stenosis. M1/M2 macrophage distribution, inflammatory cytokine expression and the role of M1 macrophages during CBAV stenosis were also explored. The experimental and control groups comprised 30 severely stenotic CBAVs and 30 severely stenotic tricuspid aortic valves (TAVs), respectively. Histological and morphological changes were assessed using hematoxylin-eosin (HE) staining and mRNA levels of vascular endothelial growth factor (VEGF) were examined using the quantitative polymerase chain reaction. Nonspecific, M1 and M2 macrophages were monitored using cluster of differentiation (CD)68, inducible nitric oxide synthase (iNOS) and CD163 staining, respectively. Endothelial nitric oxide synthase (eNOS), interleukin (IL)-10, arginase (Arg)-1 and macrophage colony-stimulating factor (M-CSF) were also examined using immunohistochemical staining. Of note, HE staining revealed a higher cell density and neovascularization was more common in CBAVs than TAVs. At the mRNA level, VEGF expression was two-fold higher in CBAVs relative to that in TAVs (P=0.02). Furthermore, CD68 and iNOS were significantly higher in CBAVs compared with TAVs (P=0.029 and 0.021, respectively), while CD163 expression was lower in CBAVs (P=0.033). In addition, eNOS expression was higher and Arg-1, IL-10 and M-CSF expression were lower in CBAVs compared with TAVs (all P<0.0001). The present study suggested that CBAVs are associated with a higher total and M1 macrophage density and a lower M2 macrophage density than TAVs, and that M1 macrophage infiltration may contribute to calcification of CBAVs.

Is there any difference in aortic wall quality between patients with bicuspid aortic valve stenosis and those with bicuspid aortic valve insufficiency?

We read with a great interest the manuscript by Benedik et al. [1] published in the recent issue of European Journal of CardioThoracic Surgery. Benedik et al. should be congratulated for prospectively addressing the issue of proximal aortic disease in aortic valve stenosis vs aortic valve insufficiency in a reasonable number of consecutively enrolled patients. The background of this study is of great interest and absolutely well-taken, as patients with aortic valve insufficiency have been repeatedly demonstrated to be at higher risk of late aortic events after isolated aortic valve replacement (AVR) surgery [2]. The findings of this study are in line with the previous reports and support once again common surgical observation that, in terms of aortopathy, aortic valve insufficiency is a different clinical entity when compared with valve stenosis. However, we would like to focus our comment on the bicuspid aortic valve (BAV) entity. The authors addressed this issue in their previous manuscript [3] and were unable to find any difference in the aortic wall cohesion between patients with tricuspid aortic valve (TAV) and those with BAV disease. Results from their previous study [3] may explain the fact that the authors did not split their study population into TAV and BAV cohorts in their current series [1]. However, aortic valve stenosis was the predominant valve pathology in the majority of patients in both (i.e. TAV and BAV) cohorts in their comparative analysis [3] and barely 9 (5%) patients with BAV insufficiency were included. Our recent data strongly suggest that aortopathy in BAV stenosis is predominantly of haemodynamic origin and has a comparable clinical course as an aortopathy in TAV stenosis [4]. However, BAV is a heterogeneous disorder [5] and patients with an isolated BAV insufficiency (so-called root phenotype) may have a completely distinct form of aortopathy that may follow a different clinical course after AVR surgery [6]. Therefore, the results from comparative analysis of aortopathy between patients with BAV and those with TAV stenosis may not be generalizable to the whole BAV population. As the recent study [1] includes a reasonable number of patients with aortic valve insufficiency and a significant proportion of them are presumably bicuspid (i.e. share the typical features of root phenotype), differential analysis of BAV and TAV aortopathy in the stenosis and insufficiency subgroups would be very welcoming. Potentially, this supplemental analysis would be a nice contribution to the controversial area of BAV-associated aortopathy.

Comparison of Results of Transcatheter Aortic Valve Implantation in Patients With Severely Stenotic Bicuspid Versus Tricuspid or Nonbicuspid Valves

Data on transcatheter aortic valve implantation (TAVI) for severe bicuspid aortic valve (BAV) stenosis and how this compares to that for tricuspid aortic valve (TAV) stenosis are limited. Twenty-one consecutive patients with BAV were treated with the Edwards or CoreValve bioprosthesis from November 2007 to December 2012 at San Raffaele Scientific Institute and Clinical Institute S. Ambrogio, Milan, Italy. Results were compared with a cohort of patients with TAV (n=447) treated with the same bioprostheses over the same period. Procedural 1- and 12-month outcomes were examined as defined by the Valve Academic Research Consortium criteria. Patients with BAV were younger (76.7±7.1 vs 79.8±7.4 years, p=0.06) and with a larger aortic annulus (25.0±1.8 vs 23.6±2.1 mm, p=0.01). Device success (85.7% vs 94.4%, p=0.10) was lower in patients with BAV. Although the 30-day composite safety end point (23.8% vs 21.0%, p=0.76) was similar between the 2 groups, mortality rate at 30 days was higher (14.2% vs 3.6%, p=0.02) in the BAV group. Cardiovascular mortality at 1 year did not differ significantly between the 2 groups (10.5% vs 7.4%, p=0.62). In conclusion, transcatheter aortic valve implantation in high surgical risk patients with severe BAV stenosis appears to be feasible with mid-term cardiovascular mortality similar to that for patients with TAV. Early survival and device success, however, were lower for patients with BAV demonstrating that further studies are required to identify which subset of patients with BAV is best suited for transcatheter treatment.

Correlation between systolic transvalvular flow and proximal aortic wall changes in bicuspid aortic valve stenosis

The purpose of this study was to analyse the correlation between preoperative systolic transvalvular flow patterns and proximal aortic wall lesions in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. A total of 48 consecutive patients with BAV stenosis (mean age 58 ± 9 years, 65% male) underwent aortic valve replacement (AVR) ± proximal aortic surgery from January 2012 through February 2013. Preoperative cardiac phase-contrast cine magnetic resonance imaging (MRI) assessment was performed in all patients in order to detect the area of maximal flow-induced stress in the proximal aorta. Based on these MRI data, two aortic wall samples (i.e. area of the maximal stress (jet sample) and the opposite aortic wall (control sample)) were collected during AVR surgery. Aortic wall changes were graded based on a summation of seven histological criteria (each scored from 0 to 3). Histological sum score (0-21) was separately calculated and compared between the two aortic samples (i.e. jet sample vs control sample). An eccentric transvalvular flow jet hitting the proximal aortic wall could be identified in all 48 (100%) patients. The mean histological sum score was significantly higher in the jet sample vs control sample areas of the aorta (i.e. 4.1 ± 1.8 vs 2.2 ± 1.5, respectively) (P = 0.02). None of the patients had a higher sum score value in the control sample. Our study demonstrates a strong correlation between the systolic pattern of the transvalvular flow jet and asymmetric proximal aortic wall changes in patients undergoing AVR for BAV stenosis.

Thoracic aortic wall shear stress atlases in patients with bicuspid aortic valves

Background Wall shear stress (WSS) may be associated with the onset and development of aortopathy in the presence of bicuspid aortic valve (BAV) [Michelena, JAMA (2011)]. The use of ‘aortic atlases’ of 3D WSS vectors allows for systematic analysis of WSS differences between large patient cohorts. In this study, a comparison is performed between WSS atlases of BAV patients, BAV patients with aortic dilation, patients with BAV stenosis, and healthy controls with tricuspid valves. The aim is to test the hypothesis that WSS atlases can identify regions of significantly altered WSS that are associated with different expression of BAV and aortopathy.

Aortic balloon valvuloplasty in corrected transposition of the great arteries with severe valvular aortic stenosis of congenital bicuspid aortic valve.

Aortic balloon valvuloplasty in corrected transposition of the great arteries with severe valvular aortic stenosis of congenital bicuspid aortic valve.

Outcome of pregnancy in patients with bicuspid aortic valve – a study of 89 patients (RCD code: VII-IV-1D.2o)

The changes in hemodynamics, as well as changes in the aortic media during pregnancy, put the women with bicuspid aortic valve (BAV) and significant aortic stenosis (AS) and/or dilated aortic roots at risk for complications during this period. From 1988–2014,89 pregnant patients with BAV, mean 26.8 ±4.6 years, were observed. There were 52 patients with AS: 22 with mild AS, 30 with moderate to severe AS and 37 patients with aortic insufficiency (AI). Medical history, physical examination, NYHA class assessment, ECG and echocardiography were performed during each trimester (TR) of pregnancy and after delivery. During pregnancy all patients with mild AS remained in NYHA class I. All of them delivered vaginally healthy babies on term. Patients with moderate to severe AS in I TR remained in NYHA class I-II, in 6/30 patients clinical deterioration was observed within III TR. Seventeen women with severe AS delivered by cesarean section, the remaining vaginally. In the group of IA clinical deterioration was observed in III TR in 5/37 patients with severe AI, enlarged left ventricle (LV) and depressed systolic function. Six of them delivered by cesarean section, the remaining vaginally. Conclusions: Patients with mild AS tolerate well the increased cardiovascular demand of pregnancy. In patients with severe AS pregnancy intensifies the hemodynamic disorders. Volume overload of pregnancy is well tolerated in patients with mild and moderate IA. Cardiac complications can be expected in patients with severe IA, LV enlargement and impaired systolic function. In the observed group of BAV patients there was no pregnancy-associated dissection. JRCD 2014; 2 (1): 9–14

Risk of proximal aortic dissection in patients with bicuspid aortic valve: how to address this controversy?

The risk of acute aortic events in patients with bicuspid aortic valve (BAV) disease is a controversial issue. The real risk of aortic dissection in patients with BAV disease is unknown. An indirect assessment of this risk, however, could be gained with a more detailed understanding of the pathogenesis of BAV aortopathy. There are two major issues that should be clarified before one addresses the question of aortic dissection risk in BAV patients. The first issue, when analysing the data from previous BAV cohorts, is to determine what stage of BAV disease was present in the described patient population. In particular, was the risk of aortic dissection in BAV patients determined before or after aortic valve replacement (AVR) surgery? The second issue to consider is the functional state of the pathological valve within the observed population. In particular, did patients predominantly suffer from BAV stenosis or BAV insufficiency? Unfortunately, the vast majority of published reports do not separate between the different BAV phenotypes, thereby complicating interpretation of the results. Considering these two important clinical variables (i.e. the stage of BAV disease and the functional phenotype), we herein aim to explain the inconsistency of the published data with regard to the risk of aortic dissection in patients with BAV disease.

EComment. Evaluating aortic media changes in ascending aorta aneurysm with bicuspid aortic valve disease

We read with great interest the paper by Girdauskas and colleagues who found that patients with bicuspid aortic valve (BAV) insufficiency and a proximal aorta of ≥50 mm had a significantly higher rate of moderate/severe proximal aortic media elastic fibre loss as compared to their counterparts with BAV stenosis [1]. We would like to add some thoughts about other fields of investigation between patients with BAV stenosis versus BAV insufficiency and ascending aorta aneurysms. The role of transforming growth factor β (TGFβ) pathway (expression of TGFβR1 and TGFβR2) could be evaluated in BAV stenosis versus BAV insufficiency groups with ascending aorta aneurysms. Forte et al. showed an increased TGFβ and TGFβR2 expression in mildly dilated ascending aortas in BAV stenosis and tricuspid aortic valve stenosis groups and also a decreased TGFβR1 expression only in BAV [2]. The existence of difference or not of the expression of extra domain-A splicing variant of fibronectin in BAV stenosis versus BAV insufficiency groups is an interesting issue as it was shown that its expression was activated in the media of mildly dilated BAV and tricuspid aortic valve (TAV) [2]. Another topic is the evaluation of smooth muscle cell (SMCs) phenotype and orientation in ascending aorta samples (both aortic curvatures) in BAV stenosis versus BAV insufficiency groups as different flow derangements and shear stress anomalies might be observed in these patients. Is there a different pattern of ascending aorta medial remodelling in these groups? Smooth muscle cells switched their phenotype in mildly dilated BAV and tricuspid aortic valve (TAV) [2]. Smoothelin and myocardin mRNAs decreased in all samples from BAV and TAV, with the exception of BAV convexity. There was also a change in orientation of smoothelin-positive SMCs and an increase of α-smooth muscle actin mRNA [2]. The expression of genes that are involved in extracellular matrix remodelling is another field which we could search for differences of expression of matrix metalloproteinase-2 (MMP-2), tissue inhibitor of metelloproteinases-2, MMP-9 and other MMPs in BAV stenotic versus BAV insufficiency groups. The MMP-2 mRNA was increased in the convexity of mildly dilated ascending aortas of BAV and TAV groups [2]. Differences in the biosynthesis of collagen and cross-linking in ascending aorta aneurysm of patients with BAV stenosis versus BAV insufficiency is another field of investigation. Are there any differences in collagen turnover in BAV stenosis versus BAV insufficiency groups? It was found that similar collagen turnover rates were observed in dilated and non-dilated aortas of BAV patients [3]. Is the nature of proximal aortic aneurysm (collagen biosynthesis and cross-linking) similar in BAV stenotic and BAV insufficiency groups? Is the collagen network similar in both groups? What about the ratio of hydroxylysyl pyridiholine to lysyl pyridinoline, two distinct forms of collagen cross-linking? Is there any defect in post-translational collagen modification? In dilated aortas from patients with BAV a deficiency was found at the level of lysyl hydroxylase (PLOD1) which showed reduced PLOD1 expression [3]. Could microRNA (miR)-29 expression be involved in the ascending thoracic aorta with aneurysm progression in BAV patients? It was demonstrated that decreased levels of miR-29b in the aortic wall could attenuate aortic aneurysm progression in two different mouse models of abdominal aortic aneurysms [4]. BAV-related ascending aorta dilation represent a potential field of investigation for future research. Conflict of interest: none declared.

Newly diagnosed aortic arch interruption in an adult presenting with heart failure

A 52-year-old man diagnosed in childhood with bicuspid aortic valve (BAV) was admitted with heart failure. Echo showed severe BAV stenosis ( Panel A ) (gradient 80/50 mmHg, AVA 0.8 cm2), significant LV dysfunction [ejection fraction (EF) 27%, global longitudinal strain −8%], severe pulmonary hypertension (RVSP ≈ 115 mmHg), and narrowing/interruption of the descending aorta with holodiastolic forward flow …

Alterations in Aortic Root Geometry in Bicuspid Aortic Insufficiency Versus Stenosis: Implications for Valve Repair

The contribution of aortic annular and root disease in bicuspid aortic valve (BAV) insufficiency remains unclear. We compare the aortic root geometry between BAV stenosis (AS) and insufficiency (AI), before and after AV repair.

078 * CORRELATION BETWEEN SYSTOLIC TRANSVALVULAR FLOW AND PROXIMAL AORTIC WALL CHANGES IN BICUSPID AORTIC VALVE STENOSIS

OBJECTIVES: The purpose of this study was to analyse the correlation between preoperative systolic transvalvular flow patterns and proximal aortic wall lesions in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. METHODS: A total of 48 consecutive patients with BAV stenosis (mean age 58 ± 9 years, 65% male) underwent aortic valve replacement (AVR) ± proximal aortic surgery from January 2012 through February 2013. Preoperative cardiac phase-contrast cine magnetic resonance imaging (MRI) assessment was performed in all patients in order to detect the area of maximal flow-induced stress in the proximal aorta. Based on these MRI data, two aortic wall samples (i.e. area of the maximal stress ( jet sample) and the opposite aortic wall (control sample)) were collected during AVR surgery. Aortic wall changes were graded based on a summation of seven histological criteria (each scored from 0 to 3). Histological sum score (0–21) was separately calculated and compared between the two aortic samples (i.e. jet sample vs control sample). RESULTS: An eccentric transvalvular flow jet hitting the proximal aortic wall could be identified in all 48 (100%) patients. The mean histological sum score was significantly higher in the jet sample vs control sample areas of the aorta (i.e. 4.1 ± 1.8 vs 2.2 ± 1.5, respectively) (P = 0.02). None of the patients had a higher sum score value in the control sample. CONCLUSIONS: Our study demonstrates a strong correlation between the systolic pattern of the transvalvular flow jet and asymmetric proximal aortic wall changes in patients undergoing AVR for BAV stenosis.

Comparison of aortic media changes in patients with bicuspid aortic valve stenosis versus bicuspid valve insufficiency and proximal aortic aneurysm

The aim of this study was to evaluate aortic media changes in bicuspid aortic valve (BAV) patients who underwent aortic valve replacement (AVR) and simultaneous replacement of the proximal aorta for BAV stenosis vs BAV insufficiency. Review of our institutional BAV database identified a subgroup of 79 consecutive BAV patients (mean age 52.3 ± 13 years, 81% men) with BAV stenosis or insufficiency and concomitant proximal aortic dilatation of ≥50 mm who underwent AVR and simultaneous replacement of proximal aorta from 1995 through 2005. All cases of BAV disease and concomitant ascending aortic dilatation of 40-50 mm underwent isolated AVR and therefore were excluded from this analysis. Proximal aortic media elastic fibre loss (EFL) was assessed (graded 0 to 3+) and compared between patients with BAV stenosis (Group I, n = 44) vs BAV insufficiency (Group II, n = 35). Follow-up (690 patient-years) was 100% complete and 9.1 ± 4.6 years long. Mean aortic media EFL was 1.3 ± 0.7 in Group I vs 2.5 ± 0.8 in Group II (P = 0.03). Moderate/severe EFL (i.e. defined as grade 2+/3+) was found in 13 patients (29%) in Group I vs 28 patients (80%) in Group II (P < 0.001). Logistic regression identified BAV insufficiency as the strongest predictor of moderate/severe EFL (OR 9.3; 95% CI 3.2-29.8, P < 0.001). Valve-related event-free survival was 64 ± 8% in Group I vs 93% ± 5% in Group II at 10 years postoperatively (P = 0.05). A total of 4 patients (5%, 3 from Group I and 1 from Group II) underwent redo aortic root surgery for prosthetic valve endocarditis during follow-up. Patients with BAV insufficiency and a proximal aorta of ≥50 mm have a significantly higher rate of moderate/severe EFL as compared to their counterparts with BAV stenosis.

GW24-e1746 Transcatheter aortic valve implantation in high-risk patients with severe bicuspid aortic valve stenosis: Feasibility, safety and efficacy

ObjectivesWe sought to assess the feasibility and outcomes of transcatheter aortic valve implantation (TAVI) in the setting of bicuspid aortic valves (BAVs), which were regarded as a relative contraindication.MethodsConsecutive patients...

Successful Single-Stage Repair of an Interrupted Aortic Arch with a Bicuspid Aortic Valve in a Young Adult

Interrupted aortic arch is a rare congenital malformation. We describe the case of an interrupted aortic arch with a bicuspid aortic valve and severe aortic stenosis. The patient underwent a single-stage aortic valve replacement (AVR) and ventral aorta repair through a median sternotomy-laparotomy.