scholarly journals Family screening in patients with isolated bicuspid aortic valve

2021 ◽  
Author(s):  
L. Cozijnsen ◽  
R. L. Braam ◽  
M. Bakker-de Boo ◽  
A. M. Otten ◽  
J. G. Post ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Risk Ratio ◽  
Heart Association ◽  
Recurrence Risk ◽  
Aortic Dilatation ◽  
Family Screening ◽  
Aortic Replacement ◽  
Increased Risk

Abstract Aim To determine the prevalence of undiagnosed bicuspid aortic valve (BAV) and isolated aortic dilatation in first-degree relatives (FDRs) of patients with isolated BAV and to explore the recurrence risk of BAV in different subgroups of probands with BAV. Recent American College of Cardiology (ACC)/American Heart Association (AHA) Guidelines recommend family screening in patients with associated aortopathy only. Methods During follow-up visits, patients with isolated BAV received a printed invitation for their FDRs advising cardiac screening. Results From 2012–2019, 257 FDRs of 118 adult BAV patients were screened, among whom 63 (53%) index patients had undergone aortic valve surgery (AVS), including concomitant aortic replacement in 25 (21%). Of the non-operated index patients, 31 (26%) had aortic dilatation (> 40 mm). Mean age of the FDRs was 48 years (range 4–83) and 42% were male. The FDR group comprised 20 parents, 103 siblings and 134 offspring. Among these FDRs, 12 (4.7%) had a previously undiagnosed BAV and 23 (8.9%) had an isolated aortic dilatation. FDRs of the probands with previous AVS (n = 147) had a risk ratio for BAV of 2.25 (95% confidence interval (CI) 0.62–8.10). FDRs of the probands with BAV and repaired or unrepaired aortic dilatation (n = 127) had a risk ratio for BAV of 0.51 (95% CI 0.16–1.66). Conclusion Screening FDRs of patients with isolated BAV resulted in a reasonable yield of 14% new cases of BAV or isolated aortic dilatation. A trend towards an increased risk of BAV in FDRs was observed in the probands with previous AVS, whereas this risk seemed to be diminished in the probands with associated aortic dilatation. This latter finding does not support the restrictive ACC/AHA recommendation.

scholarly journals Fate of mild-to-moderate bicuspid aortic valve disease untreated during ascending aorta replacement

2020 ◽  
Author(s):  
Alessandro Verzini ◽  
Marta Bargagna ◽  
Guido Ascione ◽  
Alessandra Sala ◽  
Davide Carino ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Term Survival ◽  
Kaplan Meier ◽  
Aortic Root Surgery ◽  
Increased Risk ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.

scholarly journals Integrated Aortic-Valve-And-Ascending-Aortic Replacement vs. Partial Replacement in Bicuspid Aortic Valve-Related Aortopathy

2021 ◽  
Vol 8 ◽  
Author(s):  
Mi Chen ◽  
Wangli Xu ◽  
Yan Ding ◽  
Honglei Zhao ◽  
Pei Wang ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Heart Association ◽  
Aortic Diameter ◽  
Partial Replacement ◽  
Native Valve ◽  
Aortic Replacement ◽  
Ascending Aortic Replacement ◽  
Long Term Mortality

Objective: We sought to evaluate the outcomes of integrated aortic-valve and ascending-aortic replacement (IR) vs. partial replacement (PR) in patients with bicuspid aortic valve (BAV)-related aortopathy.Methods: We compared long-term mortality, reoperation incidence, and the cumulative incidence of stroke, bleeding, significant native valve or prosthetic valve dysfunction, and the New York Heart Association (NYHA) functional classes II-IV between inverse probability-weighted cohorts of patients who underwent IR or PR for BAV-related aortopathy in a single center from 2002 to 2019. Patients were stratified into different aortic diameter groups (“valve type” vs. “aorta type”).Results: Among patients with “valve type,” aortic valve replacement in patients with an aortic diameter > 40 mm was associated with significantly higher 10-year mortality than IR compared with diameter 35–40 mm [17.49 vs. 5.28% at 10 years; hazard ratio (HR), 3.22; 95% CI, 1.52 to 6.85; p = 0.002]. Among patients with “aorta type,” ascending aortic replacement in patients with an aortic diameter 52–60 mm was associated with significantly higher 10-year mortality than IR compared with diameter 45–52 mm (14.49 vs. 1.85% at 10 years; HR, 0.04; 95% CI, 1.06 to 85.24; p = 0.03).Conclusion: The long-term mortality and reoperation benefit that were associated with IR, as compared with PR, minimizing to 40 mm of the aortic diameter among patients with “valve type” and minimizing to 52 mm of the aortic diameter among patients with “aorta type.”Trial Registration: Treatment to Bicuspid Aortic Valve Related Aortopathy (BAVAo Registry): ChiCTR.org.cn no: ChiCTR2000039867.

Abstract 2069: Aortic Valve Replacement In Patients With Bicuspid Aortic Valve Disease Is Not Followed By Progressive Aortic Dilatation: A 15-year Study

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ayyaz Ali ◽  
Amit Patel ◽  
Darren Freed ◽  
Yasir Abu-Omar ◽  
Ahmad Y Sheikh ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Aortic Diameter ◽  
Implantation Technique ◽  
Aortic Dilatation ◽  
Aortic Pathology ◽  
Ascending Aortic Diameter

Objectives A bicuspid aortic valve may be associated with an aortopathy, this may lead to progressive aortic dilatation over time. It is uncertain whether the ascending aorta should be replaced prophylactically during AVR in these patients. We analyzed change in ascending aortic diameter following AVR, to determine whether a clinically important aortic pathology exists in patients with bicuspid aortic valve disease. Methods Demographic, operative and clinical data were obtained retrospectively through casenote review. AVR was performed using a homograft or porcine stentless valve using the subcoronary implantation technique. Patients were grouped according to whether their native aortic valve was identified as tricuspid (TC) or bicuspid (BC) at operation. Serial transthoracic echocardiograms were analyzed to measure pre-operative and post-operative ascending aortic diameter. Results 217 patients underwent AVR between 1 st January 1991 and 1 st January 2001. Ninety patients had a bicuspid aortic valve, in the remaining 127 the valve was tricuspid. The bicuspid group was younger ( BC 62yr +/− 15, TC 71yr +/− 12 yrs; p < 0.001). Follow-up echocardiography was performed 6.0 +/− 4.3 years post-operatively. Pre-operative ascending aortic diameter was similar (BC 3.2 +/− 0.5, TC 3.2 +/− 0.5 cm; p = 0.56) There was no difference in the increase in ascending aortic diameter over follow-up (BC 0.1 +/− 0.5, TC 0.0 +/− 0.5 cm; p = 0.34) Conclusion The clinical importance of “bicuspid aortopathy” in an older age group appears to be minimal. Additional aortic procedures designed to protect against progressive aortic aneurysmal disease in this setting are not justified.

Three cusps are better than two: bicuspid aortic valve and implications for military service

2018 ◽  
Vol 166 (3) ◽  
pp. 167-170
Author(s):  
Andrew Williams ◽  
M Awadalla
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Military Service ◽  
Heart Association ◽  
Time Frame ◽  
Valve Disease ◽  
Aortic Dilatation ◽  
Military Career ◽  
Congenital Cardiac Malformation ◽  
Better Than

Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation. It is an aortopathy and is associated with other congenital heart disease. Although there is no mortality increase with BAV, the natural history increases the risk of aortic valve disease, aortic dilatation and infective endocarditis over the time frame of a full military career. Military service theoretically increases the risk of aortic dilatation and endocarditis in BAV. Conversely, there are some who have BAV who would not suffer any complications during their military career. Currently, potential UK Army recruits undergo personal/family history and physical examination plus an ECG and, although this goes beyond American Heart Association guidelines, it does not screen specifically for BAV. This would necessitate a transthoracic echo for each potential recruit but would be a considerable increase in resources–both time and financial. In addition to the recruitment medical, military personnel undergo frequent medicals, which could identify those who develop significant valvular disease. Those with mild valve disease are at lowest risk of complication. Those with aortic dilatation only remain a concern.

scholarly journals Exercise Training in Athletes with Bicuspid Aortic Valve Does Not Result in Increased Dimensions and Impaired Performance of the Left Ventricle

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Laura Stefani ◽  
Giorgio Galanti ◽  
Gabriele Innocenti ◽  
Roberto Mercuri ◽  
Nicola Maffulli
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Bicuspid Aortic Valve ◽  
Negative Impact ◽  
Large Population ◽  
Aortic Dilatation ◽  
Cross Sectional ◽  
Valve Morphology ◽  
Progressive Enlargement

Background.Bicuspid aortic valve (BAV) is one of the most common congenital heart disease (0.9%–2%) and is frequently found in the athletes and in the general population. BAV can lead to aortic valve dysfunction and to a progressive aortic dilatation. Trained BAV athletes exhibit a progressive enlargement of the left ventricle (LV) compared to athletes with normal aortic valve morphology. The present study investigates the possible relationship between different aortic valve morphology and LV dimensions.Methods.In the period from 2000 to 2011, we investigated a total of 292 BAV subjects, divided into three different groups (210 athletes, 59 sedentaries, and 23 ex-athletes). A 2D echocardiogram exam to classify BAV morphology and measure the standard LV systo-diastolic parameters was performed. The study was conducted as a 5-year follow-up echocardiographic longitudinal and as cross-sectional study.Results.Typical BAV was more frequent in all three groups (68% athletes, 67% sedentaries, and 63% ex-athletes) than atypical. In BAV athletes, the typical form was found in 51% (107/210) of soccer players, 10% (21/210) of basketball players, 10% track and field athletics (20/210), 8% (17/210) of cyclists, 6% (13/210) swimmers, and 15% (32/210) of rugby players and others sport. Despite a progressive enlargement of the LV (P<0.001) observed during the follow-up study, no statistical differences of the LV morphology and function were evident among the diverse BAV patterns either in sedentary subjects or in athletes.Conclusion.In a large population of trained BAV athletes, with different prevalence of typical and atypical BAV type, there is a progressive nonstatistically significant enlargement of the LV. In any case, the dimensions of the LV remained within normal range. The metabolic requirements of the diverse sport examined in the present investigations do not seem to produce any negative impact in BAV athletes

scholarly journals Aortic Dilatation in Patients With Bicuspid Aortic Valve

2021 ◽  
Vol 12 ◽  
Author(s):  
Jing Wang ◽  
Wenhui Deng ◽  
Qing Lv ◽  
Yuman Li ◽  
Tianshu Liu ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Surgical Intervention ◽  
Current Knowledge ◽  
Aortic Diameter ◽  
Aortic Dilatation ◽  
Frequent Monitoring ◽  
Increased Risk ◽  
Clinically Significant ◽  
Medical Burden

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and hence a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available.

scholarly journals Variability in surveillance practice for patients with diagnosis of bicuspid aortic valve syndrome

2021 ◽  
Author(s):  
Arianna Kahler-Quesada ◽  
Ishani Vallabhajosyula ◽  
Sameh Yousef ◽  
Makoto Mori ◽  
Roland Assi ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Care Center ◽  
Aortic Surgery ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Aortic Pathology ◽  
Increased Risk ◽  
Surveillance Practice

Background/Aim: In patients with bicuspid aortic valves, guidelines call for regular follow-up to monitor disease progression and guide timely intervention. We aimed to evaluate how closely these recommendations are followed at a tertiary care center. Methods: This was retrospective cohort study at a tertiary care center. Among 48,504 patients who received echocardiograms between 2013-2018, 245 patients were identified to have bicuspid aortic valve. Bivariate analyses compared patient and echocardiographic characteristics between patients who did and did not receive follow-up by a cardiovascular specialist. Results: The mean age of the cohort was 55.2  15.6 years and 30.2% were female. During a median follow-up of 3.5  2.2 years, 72.7% of patients had at least one visit with a cardiovascular specialist after diagnosis of bicuspid aortic valve by echocardiogram. Patients followed by specialists had a higher proportion of follow-up surveillance by echocardiogram (78.7% vs. 34.3%, p< .0001), or by CT or MRI (41.0% vs. 3.0%, p < .0001), and were more likely to undergo valve or aortic surgery compared with patients not followed by specialists. Patients with moderate to severe valvular or aortic pathology (aortic stenosis/regurgitation, dilated ascending aorta) were not more likely to be followed by a cardiovascular specialist or receive follow-up echocardiograms. Conclusions: Follow-up care for patients with bicuspid aortic valve was highly variable, and surveillance imaging was performed sparsely despite guidelines. There is an urgent need for surveillance and clinical follow-up mechanisms to monitor this patient population with increased risk of progressive valvulopathy and aortopathy.

Aortic assessment of bicuspid aortic valve patients and their first-degree relatives

2017 ◽  
Vol 25 (3) ◽  
pp. 192-198
Author(s):  
Pablo Straneo ◽  
Gabriel Parma ◽  
Natalia Lluberas ◽  
Alvaro Marichal ◽  
Gerardo Soca ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Endothelial Function ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Inverse Correlation ◽  
Hereditary Disease ◽  
Root Diameter ◽  
Aortic Dilatation ◽  
First Degree Relatives ◽  
Increased Risk

Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m−2, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = −0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.

scholarly journals Bicuspid aortic valve repair: outcomes after 17 years of experience

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.

Sign in / Sign up

Export Citation Format

Share Document