Bicuspid Aortic Valve Morphology Research Articles

The Impact of Bicuspid Aortic Valve on Long-term Outcomes After Bioprosthetic Valve Replacement

There is limited data comparing the outcomes of aortic valve replacement surgery between patients with bicuspid versus tricuspid aortic valve morphology. From January 2000 to June 2022, 1122 patients with either tricuspid aortic valve (TAV, n=562) or bicuspid aortic valve (BAV, n=560) underwent surgical aortic valve replacement with the same type of bovine pericardial stented bioprosthesis for aortic stenosis. Propensity score matching identified 350 pairs by matching for age, sex, operative status, chronic lung disease, prior stroke, diabetes, ejection fraction, renal failure on dialysis, coronary artery disease, prior cardiac surgery, and concomitant procedures. The primary endpoints were long-term survival and reoperation. Perioperative outcomes including reoperation for bleeding, atrial fibrillation, heart block requiring pacemaker, stroke, need for dialysis, and operative mortality were similar between the matched groups. 10-year survival in the BAV group was 67% (95% CI: 59% to 74%) compared to 54% (95% CI: 46% to 61%) in the TAV group (p=0.001). Bicuspid aortic valve was a significant protective factor for late mortality with a hazard ratio of 0.60 (95% CI: 0.45 to 0.81, p<0.001). Risk factors for late mortality included age, chronic lung disease, low ejection fraction, and renal failure on dialysis. Cumulative incidence of aortic valve reintervention at 10 years was similar between the groups at 10% in the BAV group and 4.9% in the TAV group (p=0.55). Bicuspid aortic valve patients likely could not be considered the same as patients with tricuspid aortic valve when deciding on the approach of aortic valve intervention.

Bicuspid Aortic Valve Function and Aortopathy on Presentation and Progression in Children: Does Sex Difference Have Any Implications?

Bicuspid aortic valve (BAV) is the most common congenital heart defect. It can be accompanied by aortic regurgitation or stenosis with aortopathies. Studies in adults showed a sex difference, but there are limited number of reports in the pediatric population. To evaluate the difference in bicuspid aortic valve morphology and functionality between sexes, and the presence and progression of aortopathies, a retrospective chart review study was performed at a tertiary referral care center in the Midwest. In our study, we analyzed a cohort of 476 pediatric patients diagnosed with BAV who presented between January 2007 and February 2018. During the follow-up period spanning 2 to 10years, male patients (n = 314, 66%) had larger aortic valve annulus (AVA) and sinus of Valsalva (SOV) at the time of initial presentation with more likelihood for progression. In the subgroup analysis, the larger SOV in males was observed in isolated BAV patients without genetic syndromes or cardiac malformations, and there were no significant differences between both sexes in the ascending aorta dimension, valve functionality, valve morphology, and the need for intervention in any of the studied groups. As such, these findings may alter the follow-up focus and frequency for patients with BAV, particularly before adulthood, and warrant further studies.

Effect of beta-blockers and angiotensin receptor blockers in reducing the aortic growth rate in children with bicuspid aortic valve-related aortopathy

AimsThe aim of this study is to evaluate the effect of beta-blockers and angiotensin receptor blockers in reducing the aortic growth rate in children with bicuspid aortic valve (BAV)-related aortopathy and ascending phenotype. MethodsConsecutive paediatric patients (≤16 years) with BAV and ascending aorta (AsAo) dilation (z-score > 3) were enrolled in this observational retrospective cohort study. Patients receiving prophylactic treatment with either atenolol (0.5 to 1.0 mg/kg/daily) or losartan (0.7 to 1.4 mg/kg/daily) were compared with those who did not receive medical prophylaxis (control group). The primary outcome of interest was the annual rate of change in maximal AsAo diameter z-score in the treatment and control groups. ResultsFrom a cohort of 1005 patients, 120 (mean age 11.3 ± 4.5 years, 82% males) fulfilled the inclusion criteria and were included in the study. Patients in the treatment and control group had similar age, sex, family history of BAV, BAV morphology, and baseline AsAo diameter. During a median follow-up of 7.1 years (interquartile range 3.8–10.2), no differences were observed in the annual growth rate of aortic diameter z-score between patients on treatment and controls. The prevalence of aortic diameter progression was similar in the treatment and control groups, and treatment with atenolol or losartan was not associated with a lower rate of aortic disease progression. ConclusionsThe findings revealed no significant difference in the annual aortic growth rate between treated and untreated patients. Larger cohort studies or, ideally, randomized clinical controlled trials are needed to validate these findings.

Bicuspid Morphology and Rapid Deployment Valve Replacement: Is This Still a Contraindication?

When using rapid deployment surgical aortic valve replacement (RD SAVR) in treating bicuspid aortic valve morphology (BAV), several challenges are faced due to annular asymmetry. The Sievers classification has been traditionally used for the description of BAV morphology. In this study, we aimed to conduct a retrospective feasibility analysis of RD SAVR in relation to the Sievers classification. From January 2014 to March 2022, 31 patients with BAV morphology (15 patients with Sievers type 0 BAV and 16 with Sievers type 1 BAV) underwent RD SAVR. Specific surgical techniques were applied depending on the BAV morphology. Comparable clinical outcomes were observed. No paravalvular leaks and no valvular re-interventions occurred in either group. CPB and cross-clamping times, as well as the prosthesis sizes used, were also not significantly different. Postoperative mean gradients were comparable in both groups. No significant distinction was found between the groups in terms of postoperative pacemaker indication, postoperative stroke, or death. Annular symmetry can be adequately restored through precise prosthesis sizing and placement according to an individual’s valve morphology regardless of the Sievers classification of BAV by choosing a different landmark for the initial suture. RD SAVR seems to be a safe approach for any bicuspid morphology, with good hemodynamic results and time-saving potential in experienced hands.

The effect of aortic valve replacement on 4D flow-measured flow displacement in patients with bicuspid and tri-leaflet aortic valves with severe aortic stenosis

Abstract Introduction Eccentric outflow jet direction in the ascending aorta is correlated with the altered haemodynamic flow patterns thought to contribute to bicuspid aortic valve (BAV)-associated aortopathy. Purpose We aimed to analyse the impact of surgical aortic valve replacement (AVR) on outflow jet displacement in the ascending aorta, and determine if differences exist between individuals with BAV and tri-leaflet aortic valves (TAV). Method Four-dimensional flow-cardiac magnetic resonance imaging (4D Flow) of the aorta was performed in prospectively recruited patients with BAV and TAV and severe symptomatic aortic stenosis (AS), before and 12 months after AVR. Healthy individuals with BAV and TAV morphology, but no significant valve disease, were also recruited as controls. Outflow direction was calculated from the in-plane, normalised flow displacement (NFD), defined as the distance between the centre of the aortic lumen (Clum) and the centre of velocity (Cvel) of the forward flow and normalised to the lumen diameter (Figure 1). Mean NFDs were evaluated at peak systole on planes perpendicular to 25 equidistant nodes along the centreline between the aortic root to the level of the pulmonary artery bifurcation in all subjects using custom Matlab code. Results 32 subjects with severe symptomatic AS (BAV n=11, TAV n=21; mean age 68±10 years) and 17 controls (BAV n=8, TAV n=9; mean age 57±7 years) were assessed (Table 1). In those with TAV, there were no differences in NFD between patients with severe AS pre-AVR compared to post-AVR, or compared to TAV controls. However, in BAV subjects, pre-AVR BAV patients with severe AS had a significantly higher mean NFD compared to BAV controls, and compared to pre-AVR TAV patients with severe AS. Post-AVR, there was no longer a significant difference in mean displacement between BAV vs TAV. Conclusion Our observations suggest that the BAV morphology plays a predominant role in the abnormal eccentric flow displacement in the ascending aorta, which may be normalised following bioprosthetic valve replacement. This may have implications for monitoring aortas post-AVR in BAV subjects.

Determinants of device success after transcatheter aortic valve replacement in patients with type-0 bicuspid aortic stenosis.

Clinical evidence of transcatheter aortic valve replacement in patients with type-0 bicuspid aortic valve was relatively scarce. Our goal was to explore determinants of device success after transcatheter aortic valve replacement in patients with type-0 bicuspid aortic valve morphology. In this retrospective multicenter analysis, we included 59 patients with symptomatic severe aortic stenosis with type-0 bicuspid aortic valve morphology who underwent transcatheter aortic valve replacement. Type-0 bicuspid aortic valve was identified with multidetector computed tomography scans. The technical success rate was 89.8%, and the device success rate was 81.4%. Patients were divided into a device success group and a device failure group according to Valve Academic Research Consortium- 3 criteria. When we compared the two groups, we found that the ellipticity index of the aortic root and the presence of bulky calcifications at the commissure were statistically different (ellipticity index 35.7 ± 1.7 vs. 29.7 ± 1.1, p = 0.018; bulky calcification at the commissure, 54.5% vs. 4.5%, p < 0.001). Further multivariate logistic analysis showed that bulky calcification at the commissure had a negative correlation with device success (odds ratio 0.030, 95% confidence interval 0.003-0.285, p = 0.002). Yet there was no statistical correlation between the ellipticity index and device success (odds ratio 0.818, 95% confidence interval 0.667-1.003, p = 0.053). The presence of bulky calcifications at the commissure is negatively correlated with device success after transcatheter aortic valve replacement in patients with type-0 bicuspid aortic valve.

Computed tomography calcium scoring in aortic stenosis: bicuspid versus tricuspid morphology

ObjectiveCT aortic valve calcium score (AVCscore) and density (AVCdensity) thresholds have been recommended for aortic stenosis (AS) severity assessment in tricuspid aortic valve (TAV). We aimed to compare AVCscore and...

Snaring Self-Expanding Devices to Facilitate Transcatheter Aortic Valve Replacement in Patients with Complex Aortic Anatomies.

The snare-assisted technique has been described to facilitate transcatheter aortic valve replacement (TAVR) delivery system advancement in complex aortic anatomies. However, the evidence is limited to case reports. To evaluate the safety profile of the snare-facilitated approach and its impact on self-expanding (SE) TAVR outcomes, we collected consecutive patients who underwent transfemoral SE-TAVR for aortic stenosis, using propensity score matching (PSM) separately in tricuspid and type-0 and type-1 bicuspid aortic valve morphology between the snare and non-snare groups. In 766 patients, despite the snare group having significantly larger annulus angulation and maximal ascending aortic diameter, both groups achieved comparable 30-day device success rates, regardless of first-generation or new-generation valve use. After PSM, the snare group had a significantly lower new permanent pacemaker implantation rate among 193 type-0 patients (3.3% vs. 18.3%, p = 0.01). The ipsilateral group used new-generation valves less frequently (23.0% vs. 75.4%, p < 0.001), but there were no significant inter-group differences in procedure-related events, except for a lower incidence of PVL ≥ mild in the ipsilateral group (14.9% vs. 32.3%, p = 0.01). In conclusion, the snare-assisted technique appears useful in SE-TAVR with angulated aortic root anatomy, and the benefits were comparable between ipsilateral and contralateral snare techniques.

Outcomes of aortic valve repair with the reimplantation technique and maintenance of commissural orientation.

Debate still persists on whether valve-sparing root reimplantation (VSRR) of a very asymmetric bicuspid aortic valve (BAV) should be completed such that the asymmetry of the native commissural orientation is retained, or if it should be made symmetric (180°-180°). Herein, we present our approach, in which the native asymmetry is preserved, and the valve is reimplanted in a 210°-150° orientation. A retrospective review was performed of 130 patients with BAV who underwent VSRR between January 1, 2004 and March 1, 2023 at a single institution. Of this total, 37 were reimplanted asymmetrically (210°-150°). The primary outcome was > moderate aortic insufficiency (AI). Secondary outcomes included severe aortic stenosis (AS), reintervention, and survival. The included 37 patients were mostly male [94.6% (35/37)] with mean age of 46.3 years, and with low rates of comorbidities. At least moderate AI was present in 40.5% (15/37) prior to surgery. All BAV in this series were Sievers Type 1 with a mean commissural angle of 128.2°. Leaflet repair was required in 81.1% (30/37), most commonly involving central plication of the conjoined cusp [96.7% (29/30)] and raphe release [73.3% (22/30)]. There was no 30-day mortality or stroke. At 10 years, the cumulative incidences of > moderate AI, severe AS, and reintervention were 7.6% (0-17.2%), 7.1% (0-19.7%), and 5.3% (0.3-22%), respectively. There was no mortality for the entire duration of the study period. This series demonstrates excellent 10-year outcomes of maintaining commissural orientation in asymmetric BAV reimplantation procedures. However, further study with additional patients, longer follow-up, and direct comparison to symmetric reimplantation for similar BAV morphology is required.

Surgical aortic valvuloplasty is a better primary intervention for isolated congenital aortic stenosis in children with bicuspid aortic valve than balloon aortic valvuloplasty

ObjectivesSurgical aortic valvuloplasty (SAV) and balloon aortic valvuloplasty (BAV) are two main treatments for children with isolated congenital aortic stenosis (CAS). We aim to compare the two procedures' midterm outcomes, including valve function, survival, reintervention, and replacement. MethodsFrom January 2004 to January 2021, children with isolated CAS undergoing SAV (n = 40) and BAD (n = 49) at our institution were included in this study. Patients were also categorized into subgroups based on the aortic leaflet number(Tricuspid = 53, Bicuspid = 36) to compare the two procedures’ outcomes. Clinical and echocardiogram data were analyzed to identify risk factors for suboptimal outcomes and reintervention. ResultsPostoperative peak aortic gradient (PAG) and PAG at follow-up in the SAV group were lower compared with the BAV group (p < 0.001, p = 0.001, respectively). There was no difference in moderate or severe AR in the SAV group compared with the BAV group before discharge (5.0% vs 12.2%, p = 0.287) and at the last follow-up (30.0% vs 32.7%, p = 0.822). There were no early death but three late deaths (SAV = 2, BAV = 1). Kaplan-Meier estimated survivals were 86.3% and 97.8% in SAV and BAV groups respectively at 10 years (p = 0.54). There was no significant difference in freedom from reintervention (p = 0.22). For patients with bicuspid aortic valve morphology, SAV achieved higher freedom from reintervention (p = 0.011) and replacement (p = 0.019). Multivariate analysis indicated that residual PAG was a risk factor for reintervention (p = 0.045). ConclusionsSAV and BAV achieved excellent survival and freedom from reintervention in patients with isolated CAS. SAV performed better in PAG reduction and maintenance. For patients with bicuspid AoV morphology, SAV was the preferred choice.

Sex-Specific Differences in Aortic Valve Calcification Between Bicuspid and Tricuspid Severe Aortic Stenosis

Sex-specific thresholds of aortic valve calcification (AVC) correlate with aortic stenosis (AS) and may complement echocardiography to determine AS severity. Importantly, current guideline-recommended thresholds of AVC scores derived by multislice computed tomography do not distinguish between bicuspid and tricuspid aortic valves. The objective of this study was to evaluate the sex-specific differences in the amount of AVC in patients with severe AS and tricuspid (TAV) versus bicuspid (BAV) aortic valve morphologies, retrospectively evaluated by 2 tertiary care institutions. The inclusion criteria comprised patients with severe AS and a left ventricular ejection fraction ≥50% and suitable imaging examinations. The study included 1,450 patients (723 men; 49.9%) with severe AS, including 1,335 patients with TAV (92.1%) and 115 with BAV (17.9%). The calculated Agatston score was higher in BAV patients (men: BAV 4,358 [2,644 to 6,005] AU vs TAV 2,643 [1,727 to 3,794] AU, p <0.01; women: BAV 2,174 [1,330 to 4,378] AU vs TAV 1,703 [964 to 2,534] AU, p <0.01), also when indexed for valve dimensions and body surface area (men: BAV 2,227 [321 to 3,105] AU/m2 vs TAV 1,333 [872 to 1,913] AU/m2, p <0.01; women: BAV 1,326 [782 to 2,148] AU/m2 vs TAV 930 [546 to 1,456] AU/m2, p <0.01). Differences between the BAV- and TAV-derived Agatston score was more prominent in concordant severe AS. In conclusion, sex-specific Agatston scores in severe AS were approximately 1/3 higher in patients with BAV than in patients with TAV for both women and men. Optimal AVC thresholds should be adjusted for BAV, also respecting considerable prognostic implications.

Surgical treatment of bicuspid aortic valve with aortic stenosis

Background: Patients with bicuspid aortic valve may develop isolated aortic valve disease, including isolated aortic stenosis. This disease progresses over a long period of time leading to simultaneous damage of the valves and aorta. Surgery is an effective treatment of both lesions simultaneously. This study objective is to describe the characteristic features of the diagnosis and surgery results of this disease.&#x0D; Methods: All of patients diagnosed aortic stenosis due to bicuspid aortic valve were performed aortic valve replacement and asscociated aortic lesions. This retrospective descritptive study used data from the Hanoi Medical Uninversity Hospital from 3/2020 to 3/2023.&#x0D; Results: This study include 25 patients who met review criteria. Aortic stenosis was in all of patients associated with ascending aortic aneurysm. Mean aortic gradient was 56,7mmHg and 36,0% of patients had ascending aortic diameter above 45mm. The most common morphology of bicuspid aortic valve was right – left coronary leaflet. 8,0% of patients had poor left ventricle function (EF &lt; 30%). 36,0% of patients were perfomed single aortic valve replacement, 60,0% were corrected simultaneously lesions of aortic valve and ascending aorta in which 24,0% using wrapping with dacron graft. Complications: One death due to intraoperative Stanford A aortic dissection; one bleeding due to lateral wound close distal aortic anastomosis required an operation and one of sternal instability require a sternal plasty. 96,0% of patients have discharged uneventfully.&#x0D; Conclusions: Surgery is an effective treatment of both lesions simultaneously of aortic valve and descending aorta in aortic stenosis due to bicuspid aortic valve malformation. Wrapping of the ascending aorta may be useful in patients who need to undergo aortic valve surgery without increasing the risk of surgery.

Apoptotic Cell Death in Bicuspid-Aortic-Valve-Associated Aortopathy.

The bicuspid aortic valve (BAV) is the most common cardiovascular congenital abnormality and is frequently associated with proximal aortopathy. We analyzed the tissues of patients with bicuspid and tricuspid aortic valve (TAV) regarding the protein expression of the receptor for advanced glycation products (RAGE) and its ligands, the advanced glycation end products (AGE), as well as the S100 calcium-binding protein A6 (S100A6). Since S100A6 overexpression attenuates cardiomyocyte apoptosis, we investigated the diverse pathways of apoptosis and autophagic cell death in the human ascending aortic specimen of 57 and 49 patients with BAV and TAV morphology, respectively, to identify differences and explanations for the higher risk of patients with BAV for severe cardiovascular diseases. We found significantly increased levels of RAGE, AGE and S100A6 in the aortic tissue of bicuspid patients which may promote apoptosis via the upregulation of caspase-3 activity. Although increased caspase-3 activity was not detected in BAV patients, increased protein expression of the 48 kDa fragment of vimentin was detected. mTOR as a downstream protein of Akt was significantly higher in patients with BAV, whereas Bcl-2 was increased in patients with TAV, assuming a better protection against apoptosis. The autophagy-related proteins p62 and ERK1/2 were increased in patients with BAV, assuming that cells in bicuspid tissue are more likely to undergo apoptotic cell death leading to changes in the wall and finally to aortopathies. We provide first-hand evidence of increased apoptotic cell death in the aortic tissue of BAV patients which may thus provide an explanation for the increased risk of structural aortic wall deficiency possibly underlying aortic aneurysm formation or acute dissection.

Calcific aortic valve disease: mechanisms, prevention and treatment.

Calcific aortic valve disease (CAVD) is the most common disorder affecting heart valves and is characterized by thickening, fibrosis and mineralization of the aortic valve leaflets. Analyses of surgically explanted aortic valve leaflets have shown that dystrophic mineralization and osteogenic transition of valve interstitial cells co-occur with neovascularization, microhaemorrhage and abnormal production of extracellular matrix. Age and congenital bicuspid aortic valve morphology are important and unalterable risk factors for CAVD, whereas additional risk is conferred by elevated blood pressure and plasma lipoprotein(a) levels and the presence of obesity and diabetes mellitus, which are modifiable factors. Genetic and molecular studies have identified that the NOTCH, WNT-β-catenin and myocardin signalling pathways are involved in the control and commitment of valvular cells to a fibrocalcific lineage. Complex interactions between valve endothelial and interstitial cells and immune cells promote the remodelling of aortic valve leaflets and the development of CAVD. Although no medical therapy is effective for reducing or preventing the progression of CAVD, studies have started to identify actionable targets.

Clinical and Echocardiographic Features of Patients With Infective Endocarditis and Bicuspid Aortic Valve According to Echocardiographic Definition of Valve Morphology

The influence of different bicuspid aortic valve (BAV) morphology in the clinical course of infective endocarditis (IE) has not yet been investigated. This study aimed to describe the clinical and echocardiographic features of IE in patients with BAV (BAVIE) according to valve morphology. Patients with definite BAVIE prospectively enrolled in 4 high-volume referral centers from 2000 to 2019 were evaluated and divided into 2 groups according to the echocardiographic definition of fused BAV morphology: right-left coronary (RL type) and right noncoronary or left noncoronary (non-RL type) cusp fusion. All patients were followed up for 1year. One hundred thirty-eight patients with BAVIE were included (77.7% male; median age, 52 [36.83-61.00]years): 112 patients with RL type (81%) and 26 patients with non-RL type BAV (19%), with no significant differences in age, sex, and comorbidities between groups. Although 43% of the cohort had known BAV, the referral was late after symptom onset, particularly for the RL phenotype; time from symptom onset to hospitalization >30days (31.3% vs 11.5%; P=.032) and New York Heart Association class≥II (64.3% vs 42.3%; P=.039) were more frequent in patients with RL type BAV than in patients with non-RL type BAV. Conversely, patients with non-RL type BAV had a higher incidence of hemorrhagic stroke (19.2% vs 5.4%; P=.034) and high-grade atrioventricular block (11.5% vs 0.9%; P=.021). Streptococcus viridans was more frequently isolated in patients with non-RL type BAV than in patients with RL type BAV (44% vs 24.1%; P=.045). No difference in short- and intermediate-term mortality was observed between groups. Clinical profile and echocardiographic features in BAVIE patients may differ according to valve morphology, and patients with BAVIE appear to be referred late, even when BAV disease is previously known.

Clinical outcomes of bicuspid versus tricuspid aortic valve stenosis after transcatheter aortic valve replacement with self-expandable valves

BackgroundThere is a lack of available data on specific prognostic comparisons between transcatheter aortic valve replacement (TAVR) using self-expandable valves (SEV) in patients with stenotic Type 0, Type 1 bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV).ObjectivesTo evaluate the association between aortic valve morphology and outcomes following self-expandable TAVR.MethodsConsecutive patients with aortic stenosis(AS) undergoing self-expandable TAVR were enrolled and categorized into three groups (Type 0/Type 1 BAV or TAV) according to the Sievers classification. The primary endpoint was a composite of all-cause mortality and rehospitalization for heart failure (HF) within 2 years. Secondary outcomes included procedural complications and major cardiovascular events observed in clinical follow-ups. Clinical outcomes at 2 years following TAVR were compared among three groups using Kaplan-Meier curve and multivariable Cox proportional hazards regression models.ResultsA total of 344 AS patients (Type 0: 86; Type 1: 109; TAV: 149) were enrolled. The presence of moderate or severe paravalvular leak (PVL) was significantly higher in patients with Type 0 and Type 1 BAV versus TAV (10.47% vs. 16.51% vs. 6.71%, p = 0.043). All-cause 30-day mortality (2.33% vs. 0.92% vs. 2.68%, p = 0.626) and 2-year mortality (3.49% vs. 5.50% vs. 6.71%, p = 0.657) was comparable among the three groups. However, rehospitalization for HF within 2 years was significantly higher in Type 1 BAV (11.63% vs. 20.18% vs. 8.72%, p = 0.020). Multivariate Cox analysis showed that a higher STS score, Type 1 BAV morphology and excess leaflet calcification (≥ median calcium volume (CV) of the entire population) were independent predictors for HF rehospitalization. Additional intragroup Kaplan‒Meier analysis showed that excess leaflet calcification could predict higher long-term mortality and rehospitalization risk for HF(HR (95% CI): 3.430 (1.166–10.090), log rank p = 0.017) in Type 1 BAV patients.ConclusionOutcomes of self-expandable TAVR in BAV-AS patients might vary depending on valve subtypes. BAV patients with excess leaflet calcification and a raphe, especially calcified, had an increased risk of moderate PVL and HF readmission in mid-to-long term follow-ups.

Abstract 13173: Impact of Bicuspid Aortic Valve Morphology on Computed Tomography Valvular Calcification in Aortic Stenosis and Sex Differences

Introduction: Aortic valve calcification (AVC) is the key pathophysiology underlying aortic stenosis (AS). We aimed to assess computed tomography AVC in AS by valve morphology (bicuspid [BAV] vs. tricuspid aortic valve[TAV]) and sex. Methods: Retrospective study of patients with echocardiographic AS severity and AVC assessments within six months. Patients with &gt; moderate aortic regurgitation, prosthetic aortic valve, or indeterminate aortic valve morphology were excluded. Severe AS with high mean gradient (MG) was defined as aortic valve area (AVA)≤1cm2 or indexed AVA≤0.6 cm2/m2 + MG≥40 mmHg. Results: Of 2647 patients, 361(65% men) had BAV and 2286(59% men) had TAV, age 65±11 vs. 80±9 years(p&lt;0.001), AVA 1.07±0.42 vs. 0.90±0.25 cm2(p&lt;0.001), MG 41±16 vs.42±14 mmHg(p=0.4) for BAV and TAV, respectively. For both BAV and TAV groups, age, MG, and severe AS frequency were similar between women and men(all p≥0.06). BAV morphology affected AVC-AS severity and AVC-sex associations (p for interactions &lt;0.001): as AS severity increased, mean AVC difference became larger between BAV-men and TAV-men, but smaller between BAV-women and TAV-women. Similar patterns were observed in AVC density (AVCd, AVC divided by left ventricular outflow tract diameter)-AS severity and AVCd-sex associations (p for interactions &lt; 0.001). In patients with concordant AS severity and MG levels, the best BAV AVC-cutoff for detecting severe AS + high MG by area-under-the-curve analysis was 924 AU for BAV-women and 2851 AU for BAV-men, different from guideline-recommended AVC-thresholds (1200 for women and 2000 for men). The best BAV AVCd cutoff was 289 for BAV-women and 476 for BAV-men, similar to the cutoffs for patients predominantly with TAV (292 for women and 476 for men from Clavel MA JACC 2013). The BAV-AVC threshold identified more patients with severe AS than guideline-recommended threshold in BAV-women (84% vs 74%, p&lt;0.001), but not in BAV-men. Conclusions: Valvular calcium accretion in AS was different by valve morphology and sex. As compared to TAV, BAV-women exhibited the least calcification while BAV-men exhibited the highest. Such patterns may impact the sensitivity of guideline-recommended AVC thresholds for detecting severe AS in BAV patients.

The impact of bicuspid aortic valve morphology on von Willebrand factor function in patients with severe aortic stenosis and its change after TAVI

BackgroundAortic stenosis (AS) can cause acquired von Willebrand syndrome (AVWS) and valve replacement has been shown to lead to von Willebrand factor (vWF) recovery. The aim of the current study was to investigate the prevalence of AVWS in different severe AS phenotypes and its course after transcatheter aortic valve implantation (TAVI).Methods143 patients with severe AS undergoing TAVI were included in the study. vWF function was assessed at baseline, 6 and 24 h after TAVI. AVWS was defined as a reduced vWF:Ac/Ag ratio ≤ 0.7. Phenotypes were classified by tricuspid (TAV) and bicuspid (BAV) valve morphology, mean transvalvular gradient (Pmean), stroke volume index (SVI), ejection fraction (EF) and indexed effective orifice area (iEOA).ResultsAVWS was present in 36 (25.2%) patients before TAVI. vWF:Ac/Ag ratio was significantly lower in high gradient compared to low-gradient severe AS [0.78 (IQR 0.67–0.86) vs. 0.83 (IQR 0.74–0.93), p < 0.05] and in patients with BAV compared to TAV [0.70 (IQR 0.63–0.78) vs. 0.81 (IQR 0.71–0.89), p < 0.05]. Normalization of vWF:Ac/Ag ratio was achieved in 61% patients 24 h after TAVI. As in the overall study cohort, vWF:Ac/Ag ratio increased significantly in all severe AS subgroups 6 h after TAVI (each p < 0.05). Regarding binary logistic regression analysis, BAV was the only significant predictor for AVWS.ConclusionsBAV morphology is a strong predictor for AVWS in severe AS. TAVI restores vWF function in most patients with severe AS independently of AS phenotype and valve morphology.Graphical abstract

Outcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?

We aimed to assess outcomes after aortic valve repair leading to bicuspid valve anatomy in children. This is a retrospective study of patients who underwent aortic valve repair with creation/preservation of bicuspid aortic valve anatomy or tricuspidization of bicuspid valves between 1980 and 2016. Overall, 127 patients underwent bicuspid repair. Median age was 0.73years (interquartile range, 0.1-8.9), and median weight was 8.15kg (interquartile range, 3.9-31.7). The cohort included 22.8% neonates (n=29), 28.3% infants (n=36), and 48.8% children (n=62). Repair was performed without a patch in 54.3% (n=69). Survival at 10years was 94.8% (95% confidence interval, 87.5-97.85). Freedom from aortic valve reoperation at 5 and 10years was 79.9% (95% confidence interval, 71.2-86.2) and 65.6% (95% confidence interval, 53.4-75.3), respectively. Re-repair was undertaken in 53.7% (22/41). Freedom from aortic valve replacement at 5 and 10years was 90.3% (95% confidence interval, 83.1-94.5) and 75.8% (95% confidence interval, 63-84.7), respectively. Risk factors for reoperation were age less than 1year, unicuspid valve, and the presence of Shone complex and concomitant aortic arch repair. There were 107 patients (107/127, 84.25%) with preoperative bicuspid aortic valve morphology that was preserved. They were compared with a separate cohort of 44 patients who underwent tricuspidization of bicuspid aortic valve during the same period. There was no difference in survival or freedom from aortic valve reoperation. However, freedom from aortic valve replacement was lower after tricuspidization with 49.7% (95% confidence interval, 26.3-69.3) versus 75.8% (95% confidence interval, 62.1-85.1) after 10years (P=.0118). Aortic valve repair leading to bicuspid valve anatomy in children hassatisfactory long-term results. Reoperation remains common, but the need for early valve replacement can be effectively delayed.

Transcatheter aortic valve implantation in patients with bicuspid valve morphology: a roadmap towards standardization.

Indications for transcatheter aortic valve implantation (TAVI) have expanded in many countries to include patients with aortic stenosis who are at low surgical risk, and a similar expansion to this cohort is anticipated elsewhere in the world, together with an increase in the proportion of patients with bicuspid aortic valve (BAV) morphology as the age of the patients being treated decreases. To date, patients with BAV have been excluded from major randomized trials of TAVI owing to anatomical considerations. As a consequence, BAV has been a relative contraindication to the use of TAVI in international guidelines. Although clinical experience and observational data are accumulating, BAV presents numerous anatomical challenges for successful TAVI, despite advances in device design. Furthermore, in those with BAV, substantial geographical variation exists in patient characteristics, clinical approach and procedural strategy. Therefore, in this Roadmap article, we summarize the existing evidence and provide consensus recommendations from an international group of experts on the application of TAVI in patients with BAV in advance of the anticipated growth in the use of this procedure in this challenging cohort of patients.