The bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. Though most often isolated, BAV may be associated with other cardiovascular malformations. BAV-related aortopathy is the most common, sharing genetic alterations and phenotypic heterogeneity characteristics. Sometimes silent for a lifetime, BAV may manifest as aortic valve dysfunction, aortic aneurysm, or more emergent situations, such as endocarditis or aortic dissection. Its embryological origin and the characterization of the genes involved, as well as the histopathological and hemodynamic aspects of its natural history, are becoming increasingly clear. In addition, emerging evidence of rhythm disorders associated with BAV has been identified. A new international nomenclature and classification has been introduced to interpret all the advances made in recent years for the comprehension of this condition. In the guidelines, more attention has been paid to the diagnosis of BAV and related aortopathy, together with surveillance, and family screening. Surgical treatment remains the gold standard, especially in young low-risk patients, and valve repair techniques have been shown to be effective and durable. Finally, the new era of transcatheter techniques is also being applied to dysfunctional BAV, allowing the treatment of patients at high surgical risk, with increasingly promising results, and the possibility of expanding indications through the introduction of more advanced devices. This review aims to comprehensively describe the BAV conundrum, focusing on anatomy, pathophysiology, genetics, diagnosis of BAV-related disorders, and the different treatment options available in the transcatheter era.

OBJECTIVE: Valve-sparing aortic root replacement for proximal aortic dilation with aortic regurgitation is associated with excellent outcomes. Modified aortic reimplantation entails reducing the anulus size to the expected size for sex and body surface area and creating neosinuses to preserve the aortic valve. We present our mid- and late-term outcomes with the modified technique, including a single-surgeon\'s experience over the past 2 decades.
METHODS: From January 2002 to January 2024, 528 patients underwent modified aortic reimplantation for aortic aneurysm or dilation; 491 were included in this study. Endpoints included time-related mortality and postoperative morbidities, including aortic valve reintervention and longitudinal aortic regurgitation grade.
RESULTS: There were no operative deaths. Survival at 30 days, 1 year, and 15 years were 100%, 99.6%, and 87%, respectively. Postoperative stroke occurred in 4 patients (0.81%) and reoperation for bleeding in 7 (1.4%). Moderate or severe aortic valve regurgitation was seen in 6.2% and 10% of patients at 1 and 10 years, respectively. Aortic valve mean gradients were 7.0 mmHg and 7.5 mmHg at 1 and 10 years, respectively. Freedom from reintervention on the aortic valve was 99.9%, 99%, and 95% at 30 days, 1 year, and 15 years, respectively.
CONCLUSIONS: Modified aortic reimplantation technique is a reliable and reproducible technique with excellent mid- and long-term outcomes in survival and freedom from reintervention. The results advocate for modified reimplantation in patients with enlarged aortic roots, especially in younger patients with connective tissue disorder.

BACKGROUND: Congenital aortic and truncal valve disease is challenging in infants and children given the lack of available prostheses in very small sizes and the limited durability of homograft aortic valve replacement.
METHODS: A comprehensive literature search was performed using the PubMed database. Studies were included either if the report included patients less than 1 year of age or if the technique was tailored to accommodate for somatic growth.
RESULTS: Techniques for aortic and truncal valve repair addressing each aspect of the aortic valve complex - the aorta, aortic annulus, commissures, and cusps - were reviewed. The incidence of reoperation following aortic or truncal valve repair is significant at 10-years ranging from 30% to 70% depending on the underlying diagnosis and the repair technique utilized. A significant challenge in interpreting the published literature relates to the lack of anatomic data available in the publications limiting the ability to make direct comparisons between operative techniques and also limits the ability to draw conclusions regarding these techniques as applied to varied etiologies.
CONCLUSIONS: A comprehensive understanding of the aortic valve complex is necessary to achieve adequate results in pediatric aortic valve repair given the high variability in these valves.

OBJECTIVE: Valve-sparing root replacement (VSRR) is attractive for aortic root dilation as it preserves the native aortic valve (AoV). Low effective height (eH) after reconstruction is a risk factor for repair failure and reoperation. We developed and validated a quantitative AoV repair strategy to reliably restore normal valve proportions to promote long-term function.
METHODS: Normal AoV proportions were used to derive geometric relationships for sinotubular junction diameter (DSTJ), free edge length (FEL), free edge angle, and commissure height. These relationships informed two models for predicting eH following VSRR: (1) assuming valve symmetry and (2) accounting for valve asymmetry. Porcine heart (n = 6) ex vivo validation was performed under 4 VSRR scenarios: \"Ideal\" (tube graft size targeting FEL/DSTJ = 1.28), \"Oversized\" (one graft size larger than Ideal), \"Undersized\" (two sizes smaller), and \"Undersized + Plicated\" (FEL/DSTJ = 1.28 restored with leaflet plication).
RESULTS: Our analytical models predicted eH using preoperative measurements and estimated reconstructed dimensions. The Oversized graft exhibited similar eH to Ideal but higher regurgitation in the ex vivo model, whereas the Undersized graft demonstrated lower eH and regurgitation. Plication in the Undersized graft restored valve function (regurgitation & eH) similar to Ideal in the ex vivo model and above Ideal in the analytical models. Both analytical models predicted ex vivo eH well except in the Oversized and Undersized + Plicated conditions.
CONCLUSIONS: Utilizing measurements from preoperative imaging and simple mathematical models, patient-specific operative plans for VSRR can be created by estimating valve dimensions necessary to achieve favorable valve features post-repair. Clinical application of this approach promises to improve consistency in achieving optimal long-term dimensions and durability.

OBJECTIVE: Both aortic root remodelling and aortic valve (AV) reimplantation have been used for valve-sparing root replacement in patients with aortic root aneurysm with or without aortic regurgitation. There is no clear evidence to support one technique over the another. This study aimed to compare remodelling with basal ring annuloplasty versus reimplantation on a multicentre level with the use of propensity-score matching.
METHODS: This was a retrospective international multicentre study of patients undergoing remodelling or reimplantation between 2010 and 2021. Twenty-three preoperative covariates (including root dimensions and valve characteristics) were used for propensity-score matching. Perioperative outcomes were analysed along with longer-term freedom from AV reoperation/reintervention and other major valve-related events.
RESULTS: Throughout the study period, 297 patients underwent remodelling and 281 had reimplantation. Using propensity-score matching, 112 pairs were selected and further compared. We did not find a statistically significant difference in perioperative outcomes between the matched groups. Patients after remodelling had significantly higher reintervention risk than after reimplantation over the median follow-up of 6 years (P = 0.016). The remodelling technique (P = 0.02), need for decalcification (P = 0.03) and degree of immediate postoperative AV regurgitation (P < 0.001) were defined as independent risk factors for later AV reintervention. After exclusion of patients with worse than mild AV regurgitation immediately after repair, both techniques functioned comparably (P = 0.089).
CONCLUSIONS: AV reimplantation was associated with better valve function in longer-term postoperatively than remodelling. If optimal immediate repair outcome was achieved, both techniques provided comparable AV function.

UNASSIGNED: The ideal position of suture annuloplasty relative to the aortic annulus (internal or external) remains unclear. This study aimed to investigate the effectiveness of internal and external suture annuloplasty for isolated type 1 bicuspid aortic valve (BAV) repair. Electrocardiogram (ECG)-gated computed tomography (CT) was used to compare the two techniques and analyze their impact on the aortic annulus.
UNASSIGNED: We retrospectively analyzed 20 patients who underwent isolated type 1 BAV repair with either internal or external suture annuloplasty. Each group included 10 patients with comparable clinical features. Preoperative and postoperative ECG-gated CT scans were performed to assess the anatomical relationship between the ventricular-aortic junction (VAJ) and virtual basal ring (VBR), and to measure the height of annuloplasty from the VBR at predefined landmarks in both groups. Perioperative annular geometries, including annular area and perimeter, were measured to quantify the impact of annuloplasty on annular expansibility. The discrepancy between the postoperative annular dimension and size of the Hegar dilator were compared between groups to evaluate the effectiveness of annuloplasty.
UNASSIGNED: In both groups, VAJ was higher than VBR at the right coronary (RC) ostium (7.7 ± 3.3 mm) and the raphe (7.9 ± 1.5 mm). The height from the VBR to the external suture annuloplasty shared a similar pattern at the RC ostium and raphe (5.3 ± 1.1 mm and 4.8 ± 1.0 mm, respectively). In contrast, the height differences were minimal for these landmarks in the internal group. Postoperative annular area expansibility decreased in the internal group compared to preoperative levels (4.9 ± 2.3% vs. 8.9 ± 5.5%, p = 0.038), while no significant change was found in the external group (7.6 ± 4.1% vs. 6.5 ± 2.8%, p = 0.473). The internal group showed less area discrepancy between the VBR and the Hegar dilator both at systole (10.1 ± 3.7% vs. 30.1 ± 16.6%, p = 0.004) and diastole (5.7 ± 4.9% vs. 20.9 ± 14.5%, p = 0.009) compared to the external group.
UNASSIGNED: Internal suture annuloplasty results in better positioning relative to the VBR than external suture annuloplasty due to the absence of VAJ interference. While this results in more precise annular reduction and less expansibility in the short term, a long-term follow-up evaluation is necessary to assess its effectiveness.

Background Aortic valve (AV) repair is a challenging procedure due to its complexity, lower reproducibility, and steep learning curve. To examine its durability and validity, we investigated mid-term outcomes following AV repair without aortic root replacement. Methods Between March 2007 and May 2018, we retrospectively identified 14 patients who underwent AV repair without aortic root replacement at our institution. We investigated their baseline characteristics and postoperative outcomes, including the reoperation rate due to aortic regurgitation (AR) recurrence. Furthermore, we divided them into two groups: those who required reoperation due to AR recurrence (Group R) and those who did not require reoperation (Group F), and statistically compared them. Results The median age was 52.5 years (IQR: 42.0-60.8), with 11 male patients (78.6%). Eight patients (57.1%) had a bicuspid AV. Five cases (35.7%) underwent reoperation due to AR recurrence during a median follow-up period of 5.5 years. There were no significant differences in baseline characteristics between Group R (n=5, 35.7%) and Group F (n=9, 64.3%), including AR etiology, AV repair procedure, and intraoperative AR grade after the final declamp. All cases in Group R had at least mild to moderate AR on the echocardiogram before discharge. Regarding the AR grade before discharge, Group R had a significantly higher grade than Group F (p = 0.013). Conclusions The indication for AV repair for AR might need to be reassessed due to the considerable mid-term reoperation rate. Cases of AV repair with more than mild AR at discharge should be carefully monitored, as they are likely to require future reoperation for AR.

OBJECTIVE: The objective of the study was to quantify the differences in cusp size and shape in patients with normal and dilated trileaflet aortic roots and in dilated roots with or without aortic regurgitation.
METHODS: A retrospective analysis of computed tomography studies in patients with normal and dilated trileaflet aortic roots was performed measuring root and cusp dimensions. Normal root size was defined as sinuses of Valsalva diameter less than 40 mm, dilated as 45 mm or greater. Root measurements normalized to basal ring diameter and cusp measurements normalized to geometric height were analyzed to assess the shape. Additionally, comparison of dilated roots with or without aortic regurgitation was made.
RESULTS: We analyzed 146 normal and 104 dilated aortic roots and 73 propensity-matched pairs. Dilated roots were larger in all dimensions and had increased ratio between commissural and basal ring diameter (1.58 ± 0.23 vs 1.11 ± 0.10, P < .001). Cusps in dilated roots were larger in all measured dimensions and were elongated with increased normalized cusp insertion length (3.64 ± 0.39 vs 3.26 ± 0.20, P < .001) and normalized free margin length (2.53 ± 0.30 vs 2.16 ± 0.19, P < .001). In patients with dilated root and no cusp prolapse (n = 83), those with moderate or severe aortic regurgitation had larger commissural diameter but similar cusp dimensions compared with those with no or mild aortic regurgitation.
CONCLUSIONS: The cusps in dilated roots elongate transversely and to a lesser degree radially. Functional aortic regurgitation is caused by extensive commissural dilatation and not by inadequate cusp adaptation.

UNASSIGNED: Bicuspid aortic valve (AV) patients with aortic regurgitation (AR) differ from tricuspid AV patients given younger age, greater left ventricle (LV) compliance, and more prevalent aortic stenosis (AS). Bicuspid AV-specific data to guide timing of AV replacement or repair are lacking.
UNASSIGNED: Adults with bicuspid AV and moderate or greater AR who underwent aortic valve replacement or repair at our center were studied. The presurgical echocardiogram, and echocardiograms within 3 years postoperatively were evaluated for LV geometry/function, and AV function. Semiquantitative AS/AR assessment was performed in all patients with adequate imaging.
UNASSIGNED: One hundred thirty-five patients (85% men, aged 44.5 ± 15.9 years) were studied (63% pure AR, 37% mixed AS/AR). Following aortic valve replacement or repair, change in LV end-diastolic dimension and change in LV end-diastolic volume were associated with preoperative LV end-diastolic dimension (β = 0.62 Δcm/cm; 95% CI, 0.43-0.73 Δcm/cm; P < .001), and LV end-diastolic volume (β = 0.6 ΔmL/mL; 95% CI, 0.4-0.7 ΔmL/mL; P < .001), respectively, each independent of AR/AS severity (P = not significant). Baseline LV size predicted postoperative normalization (LV end-diastolic dimension: odds ratio, 3.75/cm; 95% CI, 1.61-8.75/cm, LV end-diastolic volume: odds ratio, 1.01/mL; 95% CI, 1.004-1.019/mL, both P values < .01) whereas AR/AS severity did not (P = not significant). Indexed LV end diastolic volume outperformed LV end-diastolic dimension in predicting postoperative LV normalization (area under the curve = 0.74 vs 0.61) with optimal diagnostic cutoffs of 99 mL/m2 and 6.1 cm, respectively. Postoperative indexed LV end diastolic volume dilatation was associated with increased risk of death, transplant/ventricular assist device, ventricular arrhythmia, and reoperation (hazard ratio, 6.1; 95% CI, 1.7-21.5; P < .01).
UNASSIGNED: Remodeling extent following surgery in patients with bicuspid AV and AR relates to preoperative LV size independent of valve disease phenotype or severity. Many patients with LV end-diastolic dimension below current surgical thresholds did not normalize LV size. LV volumetric assessment offered superior diagnostic performance for predicting residual LV dilatation, and postoperative indexed LV end diastolic volume dilatation was associated with adverse prognosis.

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