Abstract 18719: Relationship Between Proximal Aorta Morphology and Progression Rate of Aortic Stenosis

Background: Morphological changes of the proximal aorta, such as effacement of the sinotubular junction (STJ), may result in increased mechanical stress on the aortic valve leaflets and contribute to calcification and progression of aortic stenosis (AS). The aim of this study was to examine the association between abnormal morphology of proximal aorta and AS progression rate. Methods: Between 2010 and 2012, 426 patients with mild to moderate AS (peak aortic jet velocity >2.5 and <4 m/s) and LVEF≥50% with at least two years of follow up were included in this study. Aortic dimensions were measured at 3 different levels: sinus of Valsalva (SVal), STJ and ascending aorta (Aa). The ratios of SVal by STJ (SVal/STJ) and Aa by STJ (Aa/STJ) were used to determine degree of aortic deformity with smaller ratios consistent with greater perturbation of normal geometry. SVal/STJ<1.13 and Aa/STJ<1.09 were defined as significant low ratios per normal range reported in Guidelines. AS progression rate was assessed by annualized increase in mean gradient (MG; follow-up time = 3.2±0.8 yrs). Results: Mean age was 71±13 yrs and 64% were male. 16% had bicuspid aortic valve and MG was 21±8 mmHg. SVal, STJ and Aa dimensions were respectively 33±4 mm, 27±4 mm and 36±5 mm. Mean SVal/STJ ratio was 1.21±0.15 and Aa/STJ ratio was 1.29±0.19. Patients with significant low ratios had faster AS progression (p≤0.05; figure). After adjustment for age, gender, hypertension, diabetes, renal disease, bicuspid aortic valve, baseline MG, LVEF, aortic regurgitation and indexed STJ, SVal/STJ (p=0.025) or Aa/STJ (p=0.027) were independently associated with faster AS progression. Conclusion: Abnormal aortic root geometry such as effacement of the sinotubular junction is a strong and independent predictor of faster AS progression, regardless of arterial hemodynamics, aortic valve phenotype and baseline AS severity. This finding suggests an interrelation between proximal aorta morphology and stenosis progression.

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Progression of Aortic Stenosis in Patients with Bicuspid Aortic Valve

10.22541/au.162818853.34969761/v1 ◽

2021 ◽

Author(s):

Michael Shang ◽

Arianna Kahler-Quesada ◽

Makoto Mori ◽

Sameh Yousef ◽

Arnar Geirsson ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Stenosis ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Bicuspid Aortic Valve ◽

Proportional Hazards Model ◽

Cox Proportional Hazards Model ◽

Rapid Progression ◽

Progression Rate

Background: Bicuspid aortic valve is the most common congenital heart defect and predisposes patients to developing aortic stenosis more frequently and at a younger age than the general population. However, the influence of bicuspid aortic valve on the rate of progression of aortic stenosis remains unclear. Methods: In 236 patients (177 tricuspid aortic valve, 59 bicuspid aortic valve) matched by initial severity of mild or moderate aortic stenosis, we retrospectively analyzed baseline echocardiogram at diagnosis with latest available follow-up echocardiogram. Baseline comorbidities, annualized progression rate of hemodynamic parameters, and hazard of aortic valve replacement were compared between valve phenotypes. Results: Median echocardiographic follow-up was 2.6 (IQR 1.6-4.2) years. Patients with tricuspid aortic stenosis were significantly older with more frequent comorbid hypertension and congestive heart failure. Median annualized progression rate of mean gradient was 2.3 (IQR 0.6-5.0) mmHg/year vs. 1.5 (IQR 0.5-4.1) mmHg/year (p=0.5), and that of peak velocity was 0.14 (IQR 0-0.31) m/s/year vs. 0.10 (IQR 0.04-0.26) m/s/year (p=0.7) for tricuspid vs. bicuspid aortic valve, respectively. On multivariate analyses, bicuspid aortic valve was not significantly associated with more rapid progression of aortic stenosis. In a stepwise Cox proportional hazards model adjusted for baseline mean gradient, bicuspid aortic valve was associated with increased hazard of aortic valve replacement (HR: 1.7, 95% CI [1.0, 3.0], p=0.049). Conclusion: Bicuspid aortic valve may not significantly predispose patients to more rapid progression of mild or moderate aortic stenosis. Guidelines for echocardiographic surveillance of aortic stenosis need not be influenced by valve phenotype.

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Left ventricular outflow tract obstruction (LVOTO)

10.1093/med/9780199228188.003.0008 ◽

2011 ◽

Keyword(s):

Aortic Valve ◽

Aortic Stenosis ◽

Bicuspid Aortic Valve ◽

Left Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Ventricular Outflow Tract Obstruction ◽

Left Ventricular Outflow ◽

Different Levels

Introduction 68Subvalvar aortic stenosis (AS) 70Bicuspid aortic valve 72Supravalvar AS 74LVOTO may occur at different levels: • Subvalvular.• Valvular—including bicuspid aortic valve.• Supravalvular.• Coarctation— see p.118.Effects of LVOTO, irrespective of site of lesion, are: • ↑ afterload on LV....

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Effect of bicuspid aortic valve phenotype on progression of aortic stenosis

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa068 ◽

2020 ◽

Vol 21 (7) ◽

pp. 727-734 ◽

Cited By ~ 3

Author(s):

Mylène Shen ◽

Lionel Tastet ◽

Romain Capoulade ◽

Marie Arsenault ◽

Élisabeth Bédard ◽

...

Keyword(s):

Risk Factors ◽

Aortic Valve ◽

Aortic Stenosis ◽

Bicuspid Aortic Valve ◽

Density Ratio ◽

Progression Rate ◽

Aortic Valve Calcification ◽

Unique Identifier ◽

Clinical Trial Registration ◽

Valve Calcification

Abstract Aims To compare the progression of aortic stenosis (AS) in patients with bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV). Methods and results One hundred and forty-one patients with mild-to-moderate AS, recruited prospectively in the PROGRESSA study, were included in this sub-analysis. Baseline clinical, Doppler echocardiography and multidetector computed tomography characteristics were compared between BAV (n = 32) and TAV (n = 109) patients. The 2-year haemodynamic [i.e. peak aortic jet velocity (Vpeak) and mean transvalvular gradient (MG)] and anatomic [i.e. aortic valve calcification density (AVCd) and aortic valve calcification density ratio (AVCd ratio)] progression of AS were compared between the two valve phenotypes. The 2-year progression rate of Vpeak was: 16 (−0 to 40) vs. 17 (3–35) cm/s, P = 0.95; of MG was: 1.8 (−0.7 to 5.8) vs. 2.6 (0.4–4.8) mmHg, P = 0.56; of AVCd was 32 (2–109) vs. 52 (25–85) AU/cm2, P = 0.15; and of AVCd ratio was: 0.08 (0.01–0.23) vs. 0.12 (0.06–0.18), P = 0.16 in patients with BAV vs. TAV. In univariable analyses, BAV was not associated with AS progression (all, P ≥ 0.26). However, with further adjustment for age, AS baseline severity, and several risk factors (i.e. sex, history of hypertension, creatinine level, diabetes, metabolic syndrome), BAV was independently associated with faster haemodynamic (Vpeak: β = 0.31, P = 0.02) and anatomic (AVCd: β = 0.26, P = 0.03 and AVCd ratio: β = 0.26, P = 0.03) progression of AS. Conclusion In patients with mild-to-moderate AS, patients with BAV have faster haemodynamic and anatomic progression of AS when compared to TAV patients with similar age and risk profile. This study highlights the importance and necessity to closely monitor patients with BAV and to adequately control and treat their risk factors. Clinical trial registration https://clinicaltrials.gov Unique identifier: NCT01679431.

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Bicuspid aortic valve disease and competitive sports: key considerations and challenges

The ESC Textbook of Sports Cardiology ◽

10.1093/med/9780198779742.003.0027 ◽

2019 ◽

pp. 233-238

Author(s):

Benjamin S. Wessler ◽

Natesa G. Pandian

Keyword(s):

Aortic Valve ◽

Aortic Stenosis ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Aortic Dilation ◽

Valvular Stenosis ◽

Valvular Function ◽

A Minor ◽

Valve Lesion

Bicuspid aortic valve (BAV) is a common congenital disorder. It could simply be a minor anatomic abnormality or be associated with progressive aortic stenosis, aortic regurgitation, and aortic dilation. If an athlete is recognized to have a BAV, questions arise with regard to whether they can pursue their selected sports, particularly elite athletic activity, and what type of follow-up examinations are necessary and how often should be done. Valvular disorders such as the degree of aortic stenosis and aortic regurgitation, aortic size, and coexisting disorders are also influencing factors. The absence of robust controlled studies, which are difficult to perform, make decision-making difficult, although recommendations by expert panels provide some guidance. The general consensus is that athletes with BAV with normal valvular function and no aortic dilation can participate in all athletic activities. Those with mild aortic dilation should undergo annual screening, some more frequently than others. Those with moderate or severe valvular stenosis or regurgitation should be managed based on the haemodynamic impact of the valve lesion. Athletes with coexisting lesions or syndromes should be evaluated comprehensively. The overall recommendation to an individual athlete should incorporate many factors and employ a multidisciplinary approach.

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Bicuspid aortic valve repair: outcomes after 17 years of experience

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezab176 ◽

2021 ◽

Author(s):

Marek J Jasinski ◽

Kinga Kosiorowska ◽

Radoslaw Gocol ◽

Jakub Jasinski ◽

Rafal Nowicki ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Left Ventricular ◽

Aortic Valve Repair ◽

Aortic Dilatation ◽

Years Of Experience ◽

Valve Repair ◽

Sinotubular Junction ◽

Reverse Remodelling

Abstract OBJECTIVES This study presents the results of 17 years of experience with bicuspid aortic valve (BAV) repair and the analysis of factors associated with repair failure and early echocardiographic outcome. METHODS Between 2003 and 2020, a total of 206 patients [mean age: 44.5 ± 15.2 years; 152 males (74%)] with BAV insufficiency with or without aortic dilatation underwent elective aortic valve repair performed by a single surgeon with a mean follow-up of 5 ± 3.5 years. The transthoracic echocardiography examinations were reported. RESULTS There were no deaths during the hospital stay, and all but 1 patient survived the follow-up period (99.5%). Overall, 10 patients (5%) developed severe insufficiency and 2 (1%) developed aortic dilatation requiring reoperation. Freedom from reoperation at 7 years reached 91.8%. Type 2 BAV configuration [hazard ratio (HR) 3.9; 95% confidence interval (CI): 1.01–60; P = 0.049], no sinotubular junction remodelling (HR 7; 95% CI: 1.7–23; P = 0.005), no circumferential annuloplasty (HR 3.9; 95% CI: 1.01–64; P = 0.047) and leaflet resection (HR 5.7; 95% CI 1.2–13. P = 0.017) have been identified as a risk factor of redo operation. Parameters of the postoperative left ventricle reverse remodelling improved significantly early after the operation and later at 2 years evaluation. CONCLUSIONS The repair of BAV offers good short- and mid-term results providing a significant reverse left ventricular remodelling. Type 0 BAV preoperative configuration, circumferential annuloplasty and sinotubular junction remodelling are associated with better repair durability.

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Aortic stenosis of a bicuspid aortic valve in a patient with Klippel–Feil syndrome: a case report

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytaa037 ◽

2020 ◽

Vol 4 (3) ◽

pp. 1-4

Author(s):

Rory F L Hammond ◽

Sara Jasionowska ◽

Wael I Awad

Keyword(s):

Cervical Spine ◽

Aortic Valve ◽

Aortic Stenosis ◽

Valve Replacement ◽

Bicuspid Aortic Valve ◽

Uneventful Recovery ◽

Caucasian Woman ◽

Surgical Aortic Valve Replacement ◽

First Case

Abstract Background Klippel–Feil syndrome (KFS) is a rare congenital anomaly of the cervical spine, which is associated with a number of cardiovascular malformations, including coarctation of the aorta, bicuspid aortic valve (BAoV), and aortic aneurysm. Operative management of aortic stenosis of a BAoV in a patient with KFS has not been previously reported. Case summary A 54-year-old Caucasian woman with known KFS presented to her local hospital for elective cholecystectomy. An ejection systolic murmur was found incidentally on preoperative workup, which was confirmed to be due to a severely stenosed BAoV. The cholecystectomy was cancelled, and the patient was referred to our centre and accepted for surgical aortic valve replacement (AVR) based on symptomatic and prognostic grounds. Anaesthetic review of cervical spine imaging showed fusion of the C2–C6 vertebral bodies and a desiccated bulging disc at C4–C5 but no significant foraminal narrowing in the lower cervical spine. Valve replacement with a mechanical aortic prosthesis resulted in an uneventful recovery and the patient was discharged home to follow-up. Discussion We report the first case of severe aortic valve stenosis requiring AVR in a Klippel–Feil patient, in whom the aortic valve was confirmed to be bicuspid. This report provides further evidence of an association of KFS with BAoV and strengthens the case for screening and follow-up of KFS patients for BAoV and other cardiovascular pathologies, the consequences of which may be serious.

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Abstract 2131: The Association of Coarctation of Aorta Diminishes the Occurrence of Ascending Aorta Dilation in Children and Young Adults with Bicommissural Aortic Valves.

Circulation ◽

10.1161/circ.116.suppl_16.ii_462 ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Thieu Nguyen ◽

Andrea Z Beaton ◽

Wyman W Lai ◽

Prema Ramaswamy ◽

Ira A Parness ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Ascending Aorta ◽

Aortic Annulus ◽

Sinotubular Junction ◽

Z Scores ◽

Group A ◽

Group B ◽

Aortic Dimensions

Objectives: To explore the difference in ascending aortic dilatation between subgroups of bicuspid aortic valve (BAV) patients with and without coarctation of the aorta (CoA). Methods: Our echocardiographic database (1993–2006) was searched for BAV patients with CoA (Group A) and without CoA (Group B). Measurements at the aortic annulus, root, sinotubular junction, and ascending aorta were obtained for each patient, and body surface area-adjusted Z-score values were compared. Exclusion criteria included more than mild aortic stenosis or regurgitation, previous balloon aortic valvuloplasty, or complex left heart disease; plus Turner, Noonan, Williams, and Marfan Syndromes. Results: The median age in Group A (n=53) was 11.3 yrs (range 0 to 30) with median follow-up of 7 yrs (0 to 12.7); median age in Group B (n=145) was 8.7 yrs (0 to 29) with median follow-up of 4 yrs (0 to 13.1). Group B patients had significantly greater aortic annulus, sinotubular junction, and ascending aortic dimensions (ascending aorta Z-scores shown in Figure , p<0.0001). Group A ascending aortic dimensions did not differ significantly from the normal population. The rate of growth of the ascending aorta in Group B was higher in the first 10 years of life. Conclusion: The ascending aorta in patients with bicuspid aortic valve and coarctation does not dilate to the same degree as patients with isolated bicuspid aortic valve. This may reflect an inherent difference in aortic wall properties between the two groups. Comparison of Ascending Aorta Z Scores

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P811 More than aortic measurements: evaluation with TTE and angioCT in bicuspid aortic valve yields useful information about valvular compromise

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.464 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

M C Carrero ◽

L De Stefano ◽

I Constantin ◽

G Masson ◽

M Mezzadra ◽

...

Keyword(s):

Aortic Valve ◽

Thoracic Aorta ◽

Bicuspid Aortic Valve ◽

Aortic Diameter ◽

Time Interval ◽

Aortic Dilatation ◽

Coronary Anomalies ◽

Sinotubular Junction ◽

Valve Morphology

Abstract Background 2D transthoracic echocardiogram (TTE) is the technique of choice in the diagnosis of bicuspid aortic valve (BAV). Computed tomography (CT) is widely used in BAV to measure aortic diameters. However, in some cases CT or magnetic resonance (MRI) can add important information to TTE regarding valvular degeneration and morphotype. We designed the present prospective study to determine the agreement between TTE and gated CT in the assessment of aortic valve morphology, fibrosis, calcification and measurements of thoracic aorta. We also aimed to analyze the utility of CT in the evaluation of BAV patients, in addition to aortic measurements. Methods We included 30 consecutive patients with BAV (mean age 45 ± 15.7 years ; 73.3% men) who underwent both TTE and ECG-gated cardiac and aortic CT for valvular and aortic assessment in a follow-up protocol with a time interval between TTE and CT of 4 ± 2.6 months. We performed measurements of thoracic aorta at 6 levels (annulus, Valsalva sinus: VS, sinotubular junction, ascendent, arch and isthmus) with both techniques following guideline recommendations blinded to the results of the other technique. Several measurements of VS were performed in short-axis view (double-oblique method) (maximal diameter perpendicular to the valve opening, sinus to sinus, raphe to sinus, commissure to commissure) at systole and diastole with CT. Valve phenotype, presence of raphe, calcification scoring, aortic valve prolapse and fibrosis were also determined with both techniques and maximum aortic diameters were compared. An indexed aortic diameter > 21 mm/m2 was considered as aortic dilation and assimetryc root was defined when differences between CT measurements were ≥5mm. Results In 7 patients (23.3%) aortic diameter differences at Sinus by TTE and CT were ≥3mm. Concerning ascending aorta measurements, there was better agreement and only 2 cases (6.6%) showed differences ≥ 3mm. In 2 patients with severe calcification valve morphology was identified only with CT. There was good agreement between TTE and CT in calcium quantification in patients with valvular calcium score over 2000 AU (n = 5) and in those without calcification. However, TTE failed in identification of valvular fibrosis in 5 patients. 10 patients (30%) had aortic dilatation with CT and 7 according to TTE measurements. The 3 patients that were not identified as dilated in TTE had dilatation at the distal tubular portion. CT led to identification of coronary anomalies in 5 patients (16.7%), most of them anomalous high origin above the sinotubular junction. Conclusions Although TTE is the gold-standard in the diagnosis and follow-up of patients with BAV, CT was useful to confirm aortic measurements and to identify valvular fibrosis, assimetry, coronary anomalies and dilatation at the tubular portion. CT can add important information to TTE regarding valvular morphotype and aortic measurements, although radiation and cost should be evaluated. Abstract P811 Figure. Calcification agreement TTE and CT

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