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Objective: We investigated long-term outcomes of bicuspid aortic valve (BAV) repair, with external annuloplasty, according to aorta phenotype., Methods: Between 2003 and 2020, all patients with BAV operated on for aortic insufficiency (AI) and/or aneurysm were included. Repairs included isolated AI repair with subvalvular with or without sinotubular junction (STJ) (single or double) annuloplasty, supracoronary aorta replacement (with or without hemiroot remodeling), and root remodeling with external subvalvular ring annuloplasty., Results: Among 343 patients operated, reparability rate was 81.3% (n = 279; age 46 ± 13.3 years). At 10 years (median follow-up: 3.42 years; interquartile range, 1.1, 5.8), survival was 93.9% (n = 8 deaths, similar to general population), cumulative incidence of reoperation was 6.2% (n = 10), AI grade >2 was 5.8% (n = 9), and grade >1 was 23.0% (n = 30). BAV repair stabilizing both the annulus and STJ with annuloplasty, compared with nonstabilized STJ repair (single annuloplasty), had lower incidence of reoperation (2.6% vs 22.5%, P = .0018) and AI grade >2 (1.2% vs 23.6%, P < .001) at 9 years. Initial commissural angle <160° was not a risk factor for reoperation, compared with angle ≥160° if symmetrical repair was achieved (2.7% and 4.1%, respectively, at 6 years, P = .85). Multivariable model showed that absence of STJ stabilization (odds ratio, 6.7; 95% confidence interval, 2.1-20, P = .001) increased recurrent AI, but not initial commissural angle <160° (odds ratio, 1.01; 95% confidence interval, 0.39-2.63, P = .98). Commissures adjusted symmetrically led to lower transvalvular gradient, compared with nonsymmetrical repair (8.7 mm Hg vs 10.2 mm Hg, P = .029)., Conclusions: BAV repair, tailored to aorta phenotype, is associated with excellent durable outcomes if both annulus and STJ are reduced and stabilized with external ring annuloplasty. Commissural angle <160° is not associated with reoperation if symmetrical repair is achieved., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Class I triggers for severe and chronic aortic regurgitation surgery mainly rely on symptoms or systolic dysfunction, resulting in a negative outcome despite surgical correction. Therefore, US and European guidelines now advocate for earlier surgery. We sought to determine whether earlier surgery leads to improved postoperative survival., Methods: We evaluated the postoperative survival of patients who underwent surgery for severe aortic regurgitation in the international multicenter registry for aortic valve surgery, Aortic Valve Insufficiency and Ascending Aorta Aneurysm International Registry, over a median follow-up of 37 months., Results: Among 1899 patients (aged 49 ± 15 years, 85% were male), 83% and 84% had class I indication according to the American Heart Association and European Society of Cardiology, respectively, and most were offered repair surgery (92%). Twelve patients (0.6%) died after surgery, and 68 patients died within 10 years after the procedure. Heart failure symptoms (hazard ratio, 2.60 [1.20-5.66], P = .016) and either left ventricular end-systolic diameter greater than 50 mm or left ventricular end-systolic diameter index greater than 25 mm/m 2 (hazard ratio, 1.64 [1.05-2.55], P = .030) predicted survival independently over and above age, gender, and bicuspid phenotype. Therefore, patients who underwent surgery based on any class I trigger had worse adjusted survival. However, patients who underwent surgery while meeting early imaging triggers (left ventricular end-systolic diameter index 20-25 mm/m 2 or left ventricular ejection fraction 50% to 55%) had no significant outcome penalty., Conclusions: In this international registry of severe aortic regurgitation, surgery when meeting class I triggers led to postoperative outcome penalty compared with earlier triggers (left ventricular end-systolic diameter index 20-25 mm/m 2 or ventricular ejection fraction 50%-55%). This observation, which applies to expert centers where aortic valve repair is feasible, should encourage the global use of repair techniques and the conduction of randomized trials., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: In remodeling valve-sparing root replacement with external annuloplasty, we compare long-term outcomes of a calibrated expansible extra-aortic ring with a homemade Dacron ring., Methods: All patients (2003-2020) operated for root aneurysm with/without aortic insufficiency (AI) were included. The standardized CAVIAAR (conservation aortique valvulaire dans les insuffisances aortiques et anévrysmes de la racine-Valve sparing in aortic insufficiencies and root aneurysms) technique was root remodeling and external annuloplasty ring with the EXTRA-AORTIC Ring or homemade Dacron ring., Results: Among 486 patients (age 52.3 ± 14.0 years) operated for root aneurysm, 375 (repair rate: 77.1%) underwent root remodeling with annuloplasty (extra-aortic ring, n = 289, vs Dacron ring, n = 86). At 10 years (median follow-up, 4.08 years' interquartile range, 1.95-7.61), unmatched and matched analysis showed that patients with extra-aortic ring had greater survival, similar to the general population (93.3% vs 79.9%, P = .097), lower reoperation incidence (2.0% vs 9.7%, P = .0098), and lower AI grade >2 recurrence (1.9% vs 11.2%, P = .0042), compared with patients with a Dacron ring. Mixed-effect model showed that with extra-aortic ring annuloplasty, annular dilation over time (P = .0033) was prevented and, compared with the homemade Dacron ring, root expansibility was better preserved (3.22% vs 2.12%, P = .002) and mean transvalvular gradient was lower (6.58 mm Hg vs 7.94 mm Hg, P = .001). Tricuspid and bicuspid valves with extra-aortic ring had similar reoperation (4.3% vs 0.85%, P = .65) and AI grade >2 incidence (2.7% vs 1.2%, P = .61), expansibility (P = .29), and diameter (P = .47), whereas mean transvalvular gradient was lower for tricuspid valves (5.58 mm Hg vs 7.60 mm Hg, P = .004)., Conclusions: Valve-sparing root remodeling with calibrated expansible extra-aortic ring annuloplasty improves the outcomes of reoperation and recurrent AI compared with a homemade Dacron ring. It prevents dilation and maintains physiological root dynamics for durable valve repair., Competing Interests: Conflict of Interest Statement Dr Lansac discloses a financial relationship with CORONEO, Inc. All other authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques., Methods: All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups)., Results: During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension., Conclusions: Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2023. Published by Elsevier Inc.)

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Objectives: We investigated the predictive value of aortic valve cusp retraction, calcification, and fenestration for aortic valvuloplasty feasibility., Methods: Multicenter data were collected for 2082 patients who underwent surgical aortic valvuloplasty or aortic valve replacement. The study population had retraction, calcification, or fenestration in at least one aortic valve cusp. Controls had normal or prolapsed cusps., Results: All cusp characteristics demonstrated significantly increased odds ratios [ORs] for switch to valve replacement. This effect was strongest for cusp retraction, followed by calcification and fenestration (OR, 25.14; P ≤ .001; OR, 13.50, P ≤ .001; OR, 12.32, P ≤ .001). Calcification and retraction displayed increased odds for developing grade 4 aortic regurgitation compared with grade 0 or 1 combined on average over time (OR, 6.67; P ≤ .001; OR, 4.13; P = .038). Patients with cusp retraction showed increased risk for reintervention at 1- and 2-year follow-up after aortic valvuloplasty (hazard ratio, 5.66; P ≤ .001; hazard ratio, 3.22, P = .007). Cusp fenestration was the only group showing neither an increased risk of postoperative severe aortic regurgitation (P = .57) or early reintervention (P = .88) compared with the control group., Conclusions: Aortic valve cusp retraction, calcification, and fenestration were all related to increased rates of switch to valve replacement. Calcification and retraction were associated with recurrence of severe aortic regurgitation. Retraction was related to early reintervention. Fenestration was neither associated with recurrence of severe aortic regurgitation or reintervention. This indicates that surgeons are well able to distinguish aortic valve repair candidates in patients with cusp fenestration., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Although, valve sparing is commonly performed in patients with Marfan syndrome, feasibility and results of cusp repair for aortic insufficiency have not been studied., Aim: To report on the outcomes and durability of aortic cusp repair in valve sparing in patients with Marfan syndrome., Methods: All consecutive adult patients with Marfan syndrome who underwent remodelling and annuloplasty with aortic valve repair for aortic insufficiency between May 2005 and December 2020 were included. Patients with Marfan syndrome treated for aortic aneurysm, but without aortic insufficiency, were excluded. Data were collected prospectively and reviewed retrospectively from the Aorticvalve repair International Registry (AVIATOR)., Results: During the study period, 71 patients with Marfan syndrome were referred to surgery. Fifty-five patients with connective tissue disease and aortic insufficiency with aorta aneurysm were treated: 46 underwent aortic valve repair and nine underwent aortic valve replacement (five mechanical aortic valve replacements and four biological aortic valve replacements). The mean age was 42.9±15.4 years, and the mean EuroScore II was 2.5±2.2. No patient died, and no patient had significant aortic insufficiency (grade≥II) at discharge. The 5-year survival rate estimate was 94.4%, which seems statistically similar to that of the age- and sex-matched general population. At 5 years, freedom from reoperation was 94.6%, and the incidence of infective endocarditis was 2.6%. No valve thrombosis, aortic dissection, major bleeding events, thromboembolic events (stroke) or myocardial infarctions were noted during follow-up., Conclusion: Remodelling and aortic valve repair showed excellent durability at 5 years, even in connective tissue disorders., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Objectives: Differences in indication and technique make a randomised comparison between valve-sparing root replacement (VSRR) and personalised external aortic root support (PEARS) challenging. We performed a propensity score (PS)-matched comparison of PEARS and VSRR for syndromic root aneurysm., Methods: Patients in the PEARS 200 Database and Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (undergoing VSRR) with connective tissue disease operated electively for root aneurysm <60 mm with aortic regurgitation (AR) <1/4 were included. Using a PS analysis, 80 patients in each cohort were matched. Survival, freedom from reintervention and from AR ≥2/4 were estimated using a Kaplan-Meier analysis., Results: Median follow-up was 25 and 55 months for 159 PEARS and 142 VSRR patients. Seven (4.4%) patients undergoing PEARS required an intervention for coronary injury or impingement, resulting in one death (0.6%). After VSRR, there were no early deaths, 10 (7%) reinterventions for bleeding and 1 coronary intervention. Survival for matched cohorts at 5 years was similar (PEARS 98% vs VSRR 99%, p=0.99). There was no difference in freedom from valve or ascending aortic/arch reintervention between matched groups. Freedom from AR ≥2/4 at 5 years in the matched cohorts was 97% for PEARS vs 92% for VSRR (p=0.55). There were no type A dissections., Conclusions: VSRR and PEARS offer favourable mid-term survival, freedom from reintervention and preservation of valve function. Both treatments deserve their place in the surgical repertoire, depending on a patient's disease stage. This study is limited by its retrospective nature and different follow-ups in both cohorts., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY. Published by BMJ.)

Objectives: Our goal was to evaluate the outcome of valve-sparing root replacement (VSRR) and to compare the outcomes to those of patients having composite valve-graft conduit aortic root replacement (CVG-ARR) in a cohort of patients with aortic root aneurysm ± valve insufficiency, without valvular stenosis. Although valve-sparing procedures are preferable in young patients, there is a lack of comparative data in comparable patients., Methods: The VSRR procedures were performed in 2005 patients, and 218 patients underwent a CVG-ARR procedure. Exclusion criteria included aortic dissection, endocarditis and valvular stenosis. Propensity score matching (3:1 ratio) was applied to compare VSRR (reimplantation 33% and remodelling 67%) and CVG-ARR., Results: We matched 218 patients with CVG-ARR to 654 patients with VSRR (median age, 56.0; median follow-up was 4 years in both groups; interquartile range 1-5 years). Early mortality was 1.1% of those who had VSRR versus 2.3% in those who had CVG-ARR. Survival was 95.4% [95% confidence interval (CI) 94-97%] at 5 years in patients who had VSRR versus 85.4% (95% CI 82-92%) in those who had CVG-ARR; P = 0.002. Freedom from reintervention at 5 years was 96.8% (95% CI 95-98%) with VSRR and 95.4% (95% CI 91-99%) with CVG-ARR (P = 0.98). Additionally, there were more thromboembolic, endocarditis and bleeding events in the patients who had CVG-ARR (P = 0.02)., Conclusions: This multicentre study shows excellent results after valve-sparing root replacement in patients with an ascending aortic aneurysm with or without valve insufficiency. Compared to composite valve-graft aortic root replacement, survival is better and valve-related events are fewer. Consequently, valve-sparing procedures should be considered whenever a durable repair is feasible. We advocate a valve-sparing strategy even in more complex cases when performed in experienced centres., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Results and durability of aortic valve replacement in aortic stenosis are well known, but no study has focused on the results of aortic valve replacement in aortic insufficiency., Aim: The aim of this retrospective study was to describe our mid-term outcomes after aortic valve replacement for aortic insufficiency., Methods: All consecutive adult patients who underwent bioprosthetic aortic valve replacement for aortic insufficiency at two European centres (in France and Germany) between May 2005 and December 2020 were analysed., Results: During the study period, 289 patients were included. Mean age was 56.9±12.5 years. Overall operative mortality was 1.5%, and the 10-year survival estimate rate was 75.0%, which was significantly lower than in the age- and sex-matched general population, with a standardized mortality ratio of 2.88 (95% confidence interval 1.96-4.08; P<0001). Freedom from aortic valve-related death was 87.6%, and from aortic valve-related reoperation was 87.4%. No patient aged>60 years was reoperated on during follow-up. Freedom from severe structural valve deterioration at 10 years was 73.3%, and freedom from moderate structural valve deterioration at 10 years was 50.3%. Freedom from major adverse valve-related events at 10 years was 69.7%., Conclusions: Although bioprosthetic aortic valve replacement for aortic insufficiency shows good early results, 10-year mortality and major adverse valve-related event rates in young patients may be a concern, with a reduction in life expectancy compared with the general population., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Unicuspid aortic valve (UAV) is the second most common underlying cause of aortic valve dysfunction in young adults after the bicuspid valve. The valve may be replaced (for example by pulmonary autograft) or repaired using the bicuspidization technique. The aim of our study was to compare short- and mid-term outcomes of Ross procedure with bicuspidization in patients with severe UAV dysfunction., Methods: This was a multi-center retrospective observational cohort study comparing data from two dedicated Ross centers in the Czech Republic with bicuspidization outcomes provided by AVIATOR registry. As for the Ross group, only the patients with UAV were included. Primary endpoint was mid-term freedom from reintervention. Secondary endpoints were mid-term freedom from major adverse events, endocarditis and pacemaker implantation., Results: Throughout the study period, 114 patients underwent the Ross procedure (years 2009-2020) and 126 patients underwent bicuspidization (years 2006-2019). The bicuspidization group was significantly younger and presented with a higher degree of dyspnea, a lower degree of aortic valve stenosis and more often with pure regurgitation. The primary endpoint occurred more frequently in the bicuspidization group than in the Ross group - 77.9 vs. 97.9 % at 5 years and 68.4 vs. 75.2 % at 10 years ( p < 0.001). There was no difference in secondary endpoints., Conclusion: Ross procedure might offer a significantly lower mid-term risk of reintervention than bicuspidization in patients with UAV. Both procedures have comparable survival and risk of other short- and mid-term complications postoperatively., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Gofus, Karalko, Fila, Ondrášek, Schäfers, Kolesár, Lansac, El-Hamamsy, de Kerchove, Dinges, Hlubocký, Němec, Tuna and Vojáček.)

Objectives: This study was undertaken to analyse outcomes of aortic valve repair using additional material and compare the results to those of cusp repair without the use of the pericardial patch., Methods: All consecutive patients aged over 16 who underwent aortic valve repair with external ring annuloplasty for isolated aortic insufficiency, aortic insufficiency and tubular aortic aneurysm or aortic root aneurysm between May 2003 and November 2019 were included in a cohort study. Data were collected and analysed from the AVIATOR registry (AorticValve repair InternATiOnal Registry). Propensity score framework analysis (inverse probability of treatment weighting) was used to compare outcomes of the groups while controlling for confounders., Results: During the 16-year study period, 618 patients underwent aortic valve repair. Eight-year survival rate was 92% in the patch group and 90.2% in the no patch group without significant differences [P = 0.957 inverse probability of treatment weighting (IPTW) weighted]. Early valve-related reoperation was more frequent in the patch group as compared to the no patch group (6% vs 1%, P < 0.001 IPTW weighted), the freedom from aortic valve-related reintervention and from structural valve deterioration at 8 years was not significantly different between the patch and no patch groups (93.7% vs 94%, P = 0.968 IPTW weighted; and 99.3% vs 96.7%, P = 0.964 IPTW weighted)., Conclusions: Although a higher rate of early reintervention was observed, aortic valve repair using the pericardial patch, in a standardized approach using external annuloplasty, with effective coaptation height of at least 9 mm, was not associated with an increase in mid-term aortic valve-related reoperation or structural valve deterioration as compared to valve repair without the pericardial patch., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Unicuspid aortic valve repair relies on the principles of bicuspidization by creating a neo-commissure at 180° from the existing commissure, with pericardial patch interposition. We report a case of a 26-year-old patient with cor triatriatum and a severely regurgitating unicuspid valve. The left atrium membrane was resected. Aortic valve repair was performed creating a neo-commissure using a sliding plasty of the rudimentary right coronary cusp and patch reconstruction of the anterior part of the non-coronary cusp, protected by external subvalvular annuloplasty and hemi-root remodelling. We detail a repair technique of a partial autologous reconstruction approach for bicuspidization., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)