Abstract 17361: Phenotype of the Aortic Valve in Patients With Filamin-A Mutations: Echocardiographic Features and Clinical Outcomes

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Romain Capoulade ◽  
Caroline Cueff ◽  
Nicolas Piriou ◽  
Claire Toquet ◽  
Stéphanie Blandin ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Clinical Outcomes ◽  
Aortic Root ◽  
Ventricular Outflow Tract ◽  
Underlying Disease ◽  
Left Ventricular ◽  
Filamin A ◽  
Sinotubular Junction ◽  
The Impact

Background: Filamin-A ( FLNA ) mutations have been associated with the development of mitral valve prolapse and a unique mitral valve features described as a paradoxical restrictive leaflets motion in diastole has been recently described using a comprehensive echocardiographic screening. Polyvalvular diseases have also been reported in these patients, especially affecting the aortic valve. Objectifs: The objective of this study was to perform a comprehensive echocardiographic analysis of the aortic valve (AV) and the proximal aortic root of patients with FLNA mutations, and assess the impact of the aortic disease on outcomes. Methods: We included in this analysis 256 subjects (42±22 years, 136 men, 76 mutated: FLNA+) with confirmed genetic status, from 5 FLNA families. Comprehensive echocardiographic characterization of the aortic valve and the proximal aortic root, including the measurement of the aortic annulus, sinuses of Valsalva, sinotubular junction and ascending aorta, was performed in FLNA+ patients vs control relatives. Results: Overall, 47 subjects (18%) presented an aortic valve alteration: 40 (53%) of FLNA+ compared to 7 (4%) FLNA- subjects (p<0.001). Among the 76 FLNA+ patients, 7 (9%) had a bicuspid aortic valve phenotype as opposed to 2 (1%) in control relatives (p=0.02). The underlying disease affecting the aortic valve was AV sclerosis, stenosis and AV regurgitation with either prolapse or restricted cusps motion. A restrictive opening of the AV was also reported in some patients. Aortic valve mean gradient was slightly increased in FLNA+ compared with FLNA- subjects (5.7±5.1 vs 4.2±1.8 mmHg, P= 0.02). In adults, left ventricular outflow tract diameter (12.5±1.4 vs 12.0±1.0 mm/m 2 ; p=0.02), sinuses of Valsalva (17.8±2.5 vs 16.2±1.9 mm/m 2 ; p<0.001) and sinotubular junction (15.0±2.0 vs 13.7±1.6 mm/m 2 ; p<0.001) were larger in FLNA+ subjects as compared to control relatives. 8 FLNA+ subjects (11%; 6 males) underwent aortic valve-related surgery versus 0 in controls (p<0.001). Survival was also impaired in FLNA+ male subjects (70 year old: 72% vs 64%, p=0.03). Conclusion: The FLNA -mutated patients presented aortic valve disease more frequently, including a higher prevalence of bicuspid valve, stenosis, and regurgitation owing to either cusp prolapsed or restrictive motion. This unique features described in this population was associated with worse clinical outcomes, especially in FLNA+ males. Management and decision making should be done according to the features of these patients with polyvalvular diseases

scholarly journals Impact of left ventricular outflow tract flow acceleration on aortic valve area calculation in patients with aortic stenosis

2019 ◽  
Vol 6 (4) ◽  
pp. 97-103 ◽  
Author(s):  
Andaleeb A Ahmed ◽  
Robina Matyal ◽  
Feroze Mahmood ◽  
Ruby Feng ◽  
Graham B Berry ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Continuity Equation ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Area Measurement ◽  
Aortic Valve Area ◽  
Flow Acceleration ◽  
The Impact ◽  
Valve Area

Objective Due to its circular shape, the area of the proximal left ventricular tract (PLVOT) adjacent to aortic valve can be derived from a single linear diameter. This is also the location of flow acceleration (FA) during systole, and pulse wave Doppler (PWD) sample volume in the PLVOT can lead to overestimation of velocity (V1) and the aortic valve area (AVA). Therefore, it is recommended to derive V1 from a region of laminar flow in the elliptical shaped distal LVOT (away from the annulus). Besides being inconsistent with the assumptions of continuity equation (CE), spatial difference in the location of flow and area measurement can result in inaccurate AVA calculation. We evaluated the impact of FA in the PLVOT on the accuracy of AVA by continuity equation (CE) in patients with aortic stenosis (AS). Methods CE-based AVA calculations were performed in patients with AS once with PWD-derived velocity time integral (VTI) in the distal LVOT (VTILVOT) and then in the PLVOT to obtain a FA velocity profile (FA-VTILVOT) for each patient. A paired sample t-test (P < 0.05) was conducted to compare the impact of FA-VTILVOT and VTILVOT on the calculation of AVA. Result There were 46 patients in the study. There was a 30.3% increase in the peak FA-VTILVOT as compared to the peak VTILVOT and AVA obtained by FA-VTILVOT was 29.1% higher than obtained by VTILVOT. Conclusion Accuracy of AVA can be significantly impacted by FA in the PLVOT. LVOT area should be measured with 3D imaging in the distal LVOT.

scholarly journals Effect of Eccentric Calcification of an Aortic Valve on the Implant Depth of a Venus-A Prosthesis During Transcatheter Aortic Valve Replacement: A Retrospective Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Lanlan Li ◽  
Yang Liu ◽  
Ping Jin ◽  
Jiayou Tang ◽  
Linhe Lu ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Transcatheter Aortic Valve Replacement ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Release Process ◽  
Standard Position ◽  
Transcatheter Aortic Valve

ObjectOur goal was to assess the implant depth of a Venus-A prosthesis during transcatheter aortic valve replacement (TAVR) when the areas of eccentric calcification were distributed in different sections of the aortic valve.MethodsA total of 53 patients with eccentric calcification of the aortic valve who underwent TAVR with a Venus-A prosthesis from January 2018 to November 2019 were retrospectively analyzed. The patients were divided into three groups (A, B, and C) according to the location of the eccentric calcification, which was determined by preprocedural computerized tomography angiography (CTA) images. The prosthesis release process and position were evaluated by contrast aortography during TAVR, and the differences in valve implant depths were compared among the three groups. The effects of different aortic root structures and procedural strategies on prosthesis implant depth were analyzed.ResultsEleven patients had eccentric calcification in region A; 19 patients, in region B; and 23 patients, in region C. The patients with eccentric calcification in region B had a higher risk of prosthesis migration (10.5% upward and 21.1% downward), and the position of the prosthesis after TAVR in group B was the deepest among the three groups. When eccentric calcification was located in region A or C, the prosthesis was released at the standard position with more stability, and the location of the prosthesis was less deep after TAVR (region A: 4.12 ± 3.4 mm; region B: 10.2 ± 5.3 mm; region C: 8.4 ± 4.0 mm; region A vs. region B, P = 0.0004; region C vs. region B; and P = 0.0360). In addition, the left ventricular outflow tract (LVOT) (P = 0.0213) and aortic root angulation (P = 0.0263) also had a significant effect on implant depth in the aortic root structure of the patients. The prosthesis size was 28.3 ± 2.4 in the deep implant group and 26.4 ± 2.0 in the appropriate implant group (P = 0.0068).ConclusionThe implant depth of the Venus-A prosthesis is closely related to the distribution of eccentric calcification in the aortic valve during TAVR. Surgeons should adjust the surgical strategy according to aortic root morphology to prevent prosthesis migration.

scholarly journals Redo aortic root operations in late prosthetic endocarditis: clinical case series

2019 ◽  
Vol 23 (4) ◽  
pp. 73
Author(s):  
I. I. Skopin ◽  
P. V. Kakhktsyan ◽  
M. S. Latyshev ◽  
D. V. Murysova ◽  
T. A. Kupriy ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Conflict Of Interest ◽  
Aortic Root ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Infectious Endocarditis ◽  
Infectious Focus ◽  
Left Ventricular Outflow

<p>Prosthetic aortic valve endocarditis is a severe disease that quickly leads to heart failure. Owing to microorganisms and their toxins constantly entering the bloodstream, bypassing biological barriers, and hemodynamic disturbances, systemic embolism develops quite quickly, leading to sepsis and multi-organ failure. Conservative antibiotic therapy is often not effective because the infectious focus is located in the avascular zone. The presence of an implanted foreign body promotes adhesion of bacteria on the surface of the prosthetic tissue with simultaneous isolation from the action of phagocytes. Conservative treatment of prosthetic infectious endocarditis has an extremely unfavourable prognosis. Hospital mortality without operation is approximately 80%. Operations for prosthetic infectious endocarditis of the aortic valve are technically complex and require a highly qualified operating surgeon. The most difficult operations involve extension of the abscess to the aortic root, area of mitralaortic continuity and left ventricular outflow tract. In such situations, it is necessary to perform complex reconstructive operations on the aortic root, mitral-aortic continuity and left ventricular outflow tract. This study presents an overview of a series of complex redo operations on the aortic root and the ascending aorta in late prosthetic infectious endocarditis, with an analysis of the main tactical and technical aspects of the operations. Moreover, similar operations can be performed with good results by an experienced cardiac surgeon. In this case, it is necessary that prior to operation, the surgeon develops an algorithm of actions and determines 1) optimal access to the heart, 2) perfusion scheme, 3) type of implantable conduit, 4) cardiolysis performance features, 5) myocardial protection scheme and 6) features of the treatment of the infectious focus.</p><p>Received 29 October 2019. Revised 19 December 2019. Accepted 23 December 2019.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p><p><strong>Author contributions</strong><br />Drafting the article: I.I. Skopin, P.V. Kakhktsyan, M.S. Latyshev, D.V. Murysova, T.A. Kupriy, I.A. Zhangeriev<br />Critical revision of the article: I.I. Skopin, P.V. Kakhktsyan, M.S. Latyshev, D.V. Murysova, T.A. Kupriy, I.A. Zhangeriev, E.V. Khasigova, L.Zh. Enokyan<br />Surgical treatment I.I. Skopin, P.V. Kakhktsyan<br />Diagnostics: L.Zh. Enokyan<br />Treatment: D.V. Murysova, T.A. Kupriy<br />Assistance in surgery: M.S. Latyshev, E.V. Khasigova<br />Final approval of the version to be published: I.I. Skopin, P.V. Kakhktsyan, M.S. Latyshev, D.V. Murysova, T.A. Kupriy, I.A. Zhangeriev, E.V. Khasigova, L.Zh. Enokyan</p>

scholarly journals 1161 Baseline echocardiographic parameters associated with reintervention in children with aortic valve stenosis following balloon aortic valvuloplasty

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Papneja ◽  
Z Blatman ◽  
I D Kawpeng ◽  
J Wheatley ◽  
H Osce ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Circumferential Strain ◽  
Ventricular Outflow Tract ◽  
Aortic Insufficiency ◽  
Left Ventricular ◽  
Balloon Aortic Valvuloplasty ◽  
Aortic Valvuloplasty ◽  
The Impact

Abstract Introduction Aortic valve (AV) stenosis is the most common type of congenital left ventricular outflow tract obstruction. Short-term outcomes following balloon aortic valvuloplasty (BAV) including residual aortic stenosis, aortic insufficiency, and procedural complications have been established. The impact of pre-intervention AV characteristics on long-term outcomes has not been well studied. Purpose The aim of this study was to determine the relationship between the initial parameters on baseline echocardiogram and the time to reintervention in children with AV stenosis following BAV. Methods Children from the newborn period to 18 years of age with AV stenosis who underwent BAV from 2004-2012 were included. Patients with aortic insufficiency prior to BAV, complex congenital heart lesions, or less than two accessible follow-up echocardiograms were excluded. Baseline and serial echocardiographic data pertaining to aortic valve and LV size and function was retrospectively collected until December 2017 or until the first reintervention. Time to reintervention or death was evaluated. Results Among the 98 enrolled patients, the median [IQR] age at BAV was 2.8 months [0.2-75]. The median [IQR] duration of follow-up was 6.8 [1.9-9.0] years. Eighty-nine (83%) patients had bicuspid valve morphology and the median [IQR] peak-to-peak catheterization gradient prior to BAV was 49 [34-65] mmHg. The cumulative proportion [95% CI] of reintervention at 5 years following BAV was 33.7% [23.6%, 42.4%]. Primary indications for reintervention were aortic stenosis (57%), aortic insufficiency (14%), or mixed valve disease (30%). Reinterventions included repeat BAV (49%), AV repair (15%), and AV replacement (36%). Increased LVEF at baseline as well as increased mean LV circumferential strain at baseline were associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.974 [0.959-0.989], p &lt; 0.001; 0.939 [0.884-0.997], p = 0.041 respectively). Increased AV annulus z-score was also associated with decreased risk of reintervention (HR [95% CI] (1 unit increments): 0.806 [0.698-0.93], p = 0.003). Conclusions Our results demonstrate that better left ventricular function at baseline, measured by LVEF and mean LV circumferential strain, is associated with a decreased risk of reintervention in neonates and children following BAV. We have also shown that a bigger AV annulus prior to BAV is associated with a decreased risk of reintervention.

Progressive dilation of the diameter of the aortic root in adults with a bicuspid aortic valve

2003 ◽  
Vol 13 (6) ◽  
pp. 526-531 ◽  
Author(s):  
Annie Dore ◽  
Marie-Claude Brochu ◽  
Jean-François Baril ◽  
Marie-Claude Guertin ◽  
Lise-Andrée Mercier
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Left Ventricular Outflow Tract ◽  
Normal Population ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Ascending Aorta ◽  
Rate Of Progression ◽  
Left Ventricular Outflow

Background: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. Methods: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. Results: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. Conclusion: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

scholarly journals Clinical and echocardiographic characteristics of patients with pathology proven left-sided non-valvular papillary fibroelastoma

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E El-Am ◽  
A Ahmad ◽  
R Kurmann ◽  
A Sorour ◽  
M Bois ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Aortic Valve ◽  
Left Ventricle ◽  
Left Atrium ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Papillary Fibroelastoma ◽  
Left Ventricular Outflow

Abstract Funding Acknowledgements Type of funding sources: None. Background Papillary fibroelastoma (PFE) is now regarded as the most common primary tumor of the heart. Although benign, they are clinically significant for their high risk of embolization. They are most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. The aim of this study was to better characterize patients with left-sided non-valvular PFE and its clinical sequelae. Methods We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with left-sided non-valvular PFE were analyzed. Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative transesophageal echocardiograms were manually reviewed to estimate overall size and location. Results During the study period, we identified 37 patients with left-sided non-valvular PFE (mean age 61 ± 14 years; 62% females) (Table). PFEs were located on the left ventricle in 41%, left atrium in 35%, and left ventricular outflow tract in 24% of patients. Around a quarter of patients (27%) had a diagnosis of hypertrophic cardiomyopathy, 19% had prior cardiac surgery, and 27% had cancer diagnosed prior to PFE diagnosis. Transient ischemic attack or stroke was the presenting symptom in 22% of patients, myocardial infarction in 6% and peripheral embolization in 6%. Median maximal length for PFE on the left ventricle was 11.1 mm [3;18], on the left atrium 9 mm [2;25], and left ventricular outflow tract 8 mm [6;13]. A minority of patients (9/37 [24%]) had associated valvular PFE on the mitral valve and/or aortic valve (1 patient had both mitral valve and aortic valve PFE, 7 had aortic valve PFE and 1 had Mitral valve PFE). Only 13 patients had follow up transthoracic/transesophageal echocardiogram 1 year after PFE removal; 4/13 (31%) had documented PFE recurrence (3 PFE recurred in the same location as the original; 1 in a different location). Conclusion Left-sided non-valvular PFE is associated with thromboembolic events and at least in those that had follow-up echocardiograms, had a high recurrence rate. More studies are needed to evaluate the management of patients with asymptomatic PFE. Abstract Figure. Baseline Characteristics

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