Aortic replacement in the setting of bicuspid aortic valve: How big? How much?

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.

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Commentary: Aortic replacement for bicuspid aortic valve disease—How much is too much (or too little)?

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2019.09.114 ◽

2021 ◽

Vol 161 (1) ◽

pp. 21-22

Author(s):

Peter J. Altshuler ◽

Pavan Atluri

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Aortic Valve Disease ◽

Valve Disease ◽

Aortic Replacement

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Dynamically-Loaded 3-D Model to Study the ECM Organization of Aortic Smooth Muscle Cells in Aneurysmal Patients With Bicuspid Aortic Valve

ASME 2011 Summer Bioengineering Conference, Parts A and B ◽

10.1115/sbc2011-53424 ◽

2011 ◽

Author(s):

Wei He ◽

Julie Phillippi ◽

Christopher E. Miller ◽

David A. Vorp ◽

Thomas G. Gleason

Keyword(s):

Smooth Muscle ◽

Aortic Valve ◽

Smooth Muscle Cells ◽

Bicuspid Aortic Valve ◽

Elective Surgery ◽

Muscle Cells ◽

The United States ◽

Aortic Aneurysms ◽

Aortic Replacement ◽

Valvular Stenosis

Rupture of aortic aneurysms and dissections are the fifteenth leading cause of a death in the United States [1]. Over 40% of patients undergoing elective surgery for ascending aortic replacement due to thoracic aortic aneurysm (TAA) have a congenital defect in the aortic valve know as bicuspid aortic valve (BAV) [2]. BAV patients have uniformly larger diameter aortic roots and ascending aortas compared to age- and sex-matched controls [3] and abnormal elasticity even in the absence of valvular stenosis or aneurysm [4] and this greatly increases the risk of aortic dissection and sudden death [5]. The cause of TAA is uncertain, but recent studies suggest that oxidative stress may play a role in the pathogenesis of TAAs by degrading the extracellular matrix (ECM). We identified that BAV smooth muscle cells (SMCs) lack sufficient resistance to reactive oxygen species to maintain ECM homeostasis [6, 7].

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Aortic replacement for bicuspid aortic valve aortopathy: When and why?

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2018.06.023 ◽

2019 ◽

Vol 157 (2) ◽

pp. 520-525 ◽

Cited By ~ 5

Author(s):

Alan C. Braverman

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Aortic Replacement

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Does the 45 mm Size Cutoff for Ascending Aortic Replacement Predict Better Early Outcomes in Bicuspid Aortic Valve?

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0040-1722197 ◽

2021 ◽

Author(s):

Yi-Jia Li ◽

Wei-Guo Ma ◽

Yue Qi ◽

Jun-Ming Zhu ◽

Ya Yang ◽

...

Keyword(s):

Aortic Valve ◽

Adverse Events ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Bicuspid Aortic Valve ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Aortic Replacement ◽

Ascending Aortic Replacement ◽

Size Criterion

Abstract Background The aim of this study is to test if the newly proposed 45 mm size criterion for ascending aortic replacement (AAR) in bicuspid aortic valve (BAV) patients undergoing aortic valve replacement (AVR) is predictive of improved early outcomes. Methods Data of 306 BAV patients with an aortic diameter of ≥45 mm undergoing AVR alone or with AAR were retrospectively analyzed. Patients were divided into groups of AVR + AAR (n = 220) and AVR only (n = 86) based on if surgery was performed according to the 45 mm criterion. End point was early adverse events, including 30-day and in-hospital mortality, cardiac events, acute renal failure, stroke, and reoperation for bleeding. Cox regression was used to assess if conformance to 45 mm criterion could predict fewer early adverse events. Results AVR + AAR group had significantly higher postoperative left ventricular ejection fraction (LVEF) (0.59 ± 0.09 vs. 0.55 ± 0.11, p = 0.006) and longer cardiopulmonary bypass (CPB) time (128 vs. 111 minutes, p = 0.002). Early adverse events occurred in 45 patients (14.7%), which was more prevalent in the AVR-only group (22.1% vs. 11.8%, p = 0.020). Conformance to the 45 mm criterion predicted lower rate of early adverse events (hazard ratio [HR]: 0.53, 95% confidence interval [CI]: 0.28–0.98, p = 0.042). After adjustment for gender, age, AAo diameter, sinuses of Valsalva diameter, preoperative LVEF, Sievers subtypes, BAV valvulopathy, and CPB and cross-clamp times, conformance to the 45 mm size criterion still predicted lower incidence of early adverse events (HR: 0.37, 95% CI: 0.15–0.90, p = 0.028). Conclusions This study shows that conformance to 45 mm size cutoff for preemptive AAR during aortic valve replacement in patients with BAV was not associated with increased risk for adverse events and may improve early surgical outcomes.

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Family screening in patients with isolated bicuspid aortic valve

Netherlands Heart Journal ◽

10.1007/s12471-021-01621-3 ◽

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.

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The length of the fusion between leaflets in bicuspid aortic valve is independently related to ascending aorta dilation and flow dynamics alterations assessed by 4D-flow CMR

European Heart Journal ◽

10.1093/ehjci/ehaa946.2334 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Guala ◽

A Evangelista ◽

L La Mura ◽

G Teixido-Tura ◽

L Dux-Santoy ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Statistical Significance ◽

Ascending Aorta ◽

Funding Source ◽

Aortic Dilation ◽

4D Flow ◽

National Budget ◽

4D Flow Cmr ◽

Aortic Replacement

Abstract Background Aortic dilation in bicuspid aortic valve (BAV) patients has been related to altered flow patterns, which contribute to aortic wall degeneration. However, preventive aortic replacement is currently based on a diameter threshold. Several studies on excised BAV reported wide variability of fusion extent. Purpose To unveil whether leaflet fusion extent can be quantified by CMR and is related to aortic dilation and flow abnormalities in non-dysfunctional BAV. Methods One hundred and twenty adults with non-dysfunctional BAV and no previous aortic or aortic valve surgery and 28 healthy volunteers underwent double-oblique cine and 4D flow CMR. BAV patients with two sinuses of Valsalva or left and non-coronary cusps fusion were excluded. Peak systolic circumferential wall shear stress (WSSc) and pulse wave velocity (PWV) in the ascending aorta (AAo) were assessed by 4D flow CMR. Fusion length between leaflets was measured using a stack of double-oblique cine CMR images of the aortic valve. Results The length of the fusion was effectively measured in 112/120 (93%) patients with good reproducibility (ICC = 0.826) and showed great variability (range 2.3–15.4 mm, 7.8±3.2 mm and tertiles cut-off points 6 and 9.3 mm). In multivariate analysis adjusted for clinical and demographic characteristics and PWV, fusion length was independently associated with the diameter at the sinus of Valsalva (p=0.002) and the AAo (p=0.02) (Table). WSSc progressively increased with larger fusion length (Figure), with statistical significance (p<0.05) in the right and outer regions of the proximal and mid AAo. Conclusions Bicuspid aortic leaflet fusion length varies considerably, and it is independently associated with AAo and aortic root dilation, possibly through flow alterations. Figure 1. Maps of circumferential WSS Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been partially funded by Instituto Carlos III, Spanish Ministry of Science and Innovation (PI17/00381). Guala A. has received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).

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