Quadricuspid Aortic Valve: Interesting Images.

ABSTRACT: The quadricuspid aortic valve is a rare congenital anomaly, usually associated with aortic regurgitation requiring surgical intervention. It may be associated with other congenital anomalies such as coronary anomalies, patent ductus arteriosus, ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The diagnosis is generally established by either transthoracic or transesophageal echocardiography. Herein, we report a case of a 52-year-old woman who was diagnosed to have quadricuspid aortic valve by intraoperative transesophageal echocardiography.

Uncommon presentations of type A quadricuspid aortic valve in the Septuagenarian.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital anomaly characterized by the presence of four cusps instead of the usual three. It is estimated to occur in less than 0.05% of the population, with Type A (four equal-sized leaflets) accounting for roughly 30% of QAV subtypes. Based on limited clinical series, the usual presentation is progressive aortic valve regurgitation (AR) with symptoms occurring in the fourth to sixth decade of life. Severe aortic valve stenosis (AS) and acute AR are very uncommon. CASE PRESENTATION: We describe two cases of Type A QAV in patients who remained asymptomatic until their seventies with very uncommon presentations: one with severe AS and one with acute, severe AR and flail leaflet. In Case A, a 72-year-old patient with history of moderate AS presents to clinic with progressive exertional dyspnea. During work-up for transcatheter vs. surgical replacement pre-operative computed tomography angiogram (CTA) reveals a quadricuspid aortic valve with severe AS, and the patient undergoes surgical aortic valve replacement. Pre-discharge transthoracic echocardiography (TTE) shows good prosthetic valve function with no gradient or regurgitation. In Case B, a 76-year-old patient is intubated upon arrival to the hospital for acute desaturation, found to have wide open AR on catheterization, and transferred for emergent intervention. Intraoperative TEE reveals QAV with flail leaflet and severe AR. Repair is considered but deferred ultimately due to emergent nature. Post-operative TTE demonstrates good prosthetic valve function with no regurgitation and normal biventricular function. CONCLUSIONS: QAV can present as progressive severe AS and acute AR, with symptoms first occurring in the seventh decade of life. The optimal treatment for QAV remains uncertain. Although aortic valve repair or transcatheter option may be feasible in some patients, aortic valve replacement remains a tenable option.

Intraoperative Transesophageal Echocardiographic Assessment of Aortic Valve Repair in a Child - What to Look for?

ABSTRACT: Aortic valve (AV) repair is the desired surgical treatment option for young patients with aortic regurgitation (AR). It is considered as a class I indication for the surgical treatment of severeAR. The success of an AV repair depends on the detailed intraoperative transesophageal echocardiographic (TEE) examination which should fulfil the information required by the surgeon. The objective of this echo round is to describe the role of intraoperative TEE in systematic evaluation of the AV, before and after repair.

Comparisons of noncoronary sinus pivot implantation (NCPI) and conventional method for transcatheter aortic valve replacement with self-expanding valve in pure aortic regurgitation (PAR).

BACKGROUND: As compared to treatment of aortic stenosis (AS), transcatheter aortic valve replacement (TAVR) using the commercially available valves to treat pure aortic regurgitation (PAR) has a lower device success rate and higher complication rates. AIMS: The study compared the acute results between TAVR using a novel noncoronary sinus pivot implantation (NCPI) method and that using the conventional method, aiming to explore a more optimized and effective operation method for TAVR in PAR. METHODS: PAR patients who underwent TAVR with self-expanding valves in our center from September 2021 to September 2023 were enrolled were divided into the NCPI (group A, N = 16) and conventional method (group B, N = 39) groups. We analyzed the pre-operative evaluation parameters and procedural and postoperative data of the two subgroups. RESULTS: The total patients' mean age was 71.2 ± 8.7 years and most were male (61.8%), with a mean Society of Thoracic Surgeons score of 3.4 ± 1.9%. The device success rate of groups A and B was 100% and 71.8%, respectively. In group B, 48.7% had major adverse cardiac events (MACE); 46.2% patients had permanent pacemaker implantation or valve in valve implantation. None had MACE in group A. The noncoronary sinus implantation depth in NCPI was -1.1 + 1.0 and 4.2 + 3.7 mm in groups A and B (p < 0.001), respectively. CONCLUSIONS: TAVR with a self-expanding valve using the NCPI method had a higher procedure success rate and dramatically low complications than that using the conventional method in PAR patients.

Transcatheter aortic valve implantation for pure aortic insufficiency in a patient following David-type aortic valve-sparing root replacement after aortic dissection-A successful off-label procedure.

Transcatheter aortic valve implantation (TAVI) has traditionally been indicated for the treatment of aortic stenosis. However, in this case report, we describe a successful TAVI procedure in a 46-year-old male patient who had previously undergone David aortic valve-sparing aortic root replacement for type 1 aortic dissection. The patient presented with aortic valve insufficiency 4 years after the initial surgery and was subsequently treated with a 34 mm Medtronic CoreValve Evolut R prosthesis via TAVI. This case highlights the feasibility of TAVI as a viable treatment option for postoperative aortic valve insufficiency in patients with prior ascending aortic or aortic arch surgery.

[Long-term result of aortic root remodeling using the Florida sleeve technique in a patient with connective tissue dysplasia and aortic root aneurysm without aortic insufficiency]. / Otdalennyi rezul'tat ekzoprotezirovaniya kornya aorty po metodike Florida Sleeve u bol'nogo s soedinitel'notkannoi displaziei i anevrizmoi kornya aorty bez aortal'noi nedostatochnosti.

Aortic root remodeling using the Florida sleeve technique was proposed as a simpler alternative to classical reconstructive interventions in patients with aortic root aneurysm without aortic insufficiency. We present a 10-year result of valve-sparing surgical treatment using the Florida sleeve technique in a patient with connective tissue dysplasia and aortic root aneurysm.

Trends and Disparities in Valvular Heart Disease Mortality in the United States From 1999 to 2020.

BACKGROUND: Percutaneous heart valve procedures have been increasingly performed over the past decade, yet real-world mortality data on valvular heart disease (VHD) in the United States remain limited. METHODS AND RESULTS: We queried the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research database among patients ≥15 years old from 1999 to 2020. VHD and its subtypes were listed as the underlying cause of death. We calculated age-adjusted mortality rate (AAMR) per 100 000 individuals and determined overall trends by estimating the average annual percent change using the Joinpoint regression program. Subgroup analyses were performed based on demographic and geographic factors. In the 22-year study, there were 446 096 VHD deaths, accounting for 0.80% of all-cause mortality (56 014 102 people) and 2.38% of the total cardiovascular mortality (18 759 451 people). Aortic stenosis recorded the highest mortality of VHD-related death in both male (109 529, 61.74%) and female (166 930, 62.13%) populations. The AAMR of VHD has declined from 8.4 (95% CI, 8.2-8.5) to 6.6 (95% CI, 6.5-6.7) per 100 000 population. Similar decreasing AAMR trends were also seen for the VHD subtypes. Men recorded higher AAMR for aortic stenosis and aortic regurgitation, whereas women had higher AAMR for mitral stenosis and mitral regurgitation. Mitral regurgitation had the highest change in average annual percent change in AAMR. CONCLUSIONS: The mortality rate of VHD among the US population has declined over the past 2 decades. This highlights the likely efficacy of increasing surveillance and advancement in the management of VHD, resulting in improved outcomes.

Successful Implantation of Rapid Deployment Aortic Valve after TAVR Explantation.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) has become widely used in recent years, However, there is also an increasing need for removal of TAVR valves due to prosthetic valve dysfunction (PVD) and the development of infective endocarditis. Surgical aortic valve replacement (AVR) for these patients is risky due to the original patient background and anatomic conditions. Intuity rapid deployment aortic valve (Edwards Lifesciences, Irvine, CA) replacement would be useful for such high risk patients to prevent longer cardiac arrest time and obtain good hemodynamic results. However, there are few reports which present Intuity valve replacement after TAVR explantation. Herein, We report two cases in which we have achieved good hemodynamics with shorter cardiac arrest times by using a rapid deployment valve after TAVR explantation. CASE PRESENTATION: We present 2 cases of successful implantation of the Intuity rapid deployment valve after TAVR explantation. The 84- and 88-year-old female patients had previously received TAVR for severe aortic stenosis with SAPIEN XT (Edwards Lifesciences, Irvine, CA) and developed PVD during follow-up. The TAVR valve was removed carefully, then an Intuity valve was implanted with cardiac arrest times of 69 and 41 min. Both patients had good echocardiographic results with effective orifice area of 2.0 cm2 and 1.2 cm2 and mean trans-aortic plessure gradient of 9 mmHg and 15 mmHg respectively without aortic regurgitation. They were discharged without major complications. CONCLUSIONS: Surgical AVR using a rapid deployment valve is a useful alternative to sutured AVR after TAVR valve explantation. It allows for shorter cardiac arrest times and better postoperative hemodynamics without major complication.

Twenty-five years' experience with isolated bicuspid aortic valve repair: impact of commissural orientation.

OBJECTIVES: Repair of the bicuspid aortic valve (BAV) has evolved in the past 25 years. The aim of this study was to review and analyse the long-term durability of isolated BAV repair with particular focus on commissural orientation (CO). METHODS: All patients who underwent BAV repair for severe aortic regurgitation between October 1998 and December 2022 were included. The study group consists of all patients operated after 2009, i.e. since CO modification. The control group includes patients who were operated before 2009. CO was classified as symmetric, asymmetric and very asymmetric. RESULTS: Overall, 594 adult patients (93% male; mean age 42 years) were included. At 15 years, survival was 94.8% [standard deviation (SD): 2.2]; freedom from reoperation was 86.8% (SD: 2.3). Freedom from aortic insufficiency ≥II was 70.8% (SD: 4.7) at 15 years. Modification of CO by sinus plication was performed in 200 (33.7%) instances. Using competing risks analysis, the absence of effective height measurement (P = 0.018), very asymmetric CO (P = 0.028), the presence of calcification (P < 0.001), the use of pericardial patch (P < 0.001), the use of subcommissural sutures (P < 0.001) and preoperative endocarditis (P = 0.005) were identified as independent predictors for reoperation. Follow-up was 97% complete (4228 patient-years); mean follow-up was 7 years (SD: 5). CONCLUSIONS: Isolated BAV repair leads to good survival and durability in all morphologic types if cusp repair is guided by effective height, suture annuloplasty is performed, and CO is modified using sinus plication in asymmetrical valves. Very asymmetrical valves may should be treated with a lower threshold for replacement.

Clinical features of quadricuspid aortic valve in middle-aged and elderly patients: Insights from a regional study.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital disease. The clinical characteristics of this disease remain unclear except for those in relatively young patients reported from tertiary referral hospitals. The aim of this study was to determine the clinical features of QAV in a regional population. METHODS AND RESULTS: We retrospectively investigated 25 340 consecutive patients over middle age (median age, 73 (IQR 65-80) years; range, 45-102 years) who underwent transthoracic echocardiography (TTE) at our institute during the period from April 2008 to December 2023. Eight (0.032%) of the patients (median age, 65 years; range, 47-91 years) were diagnosed with QAV. Six patients suffered from aortic regurgitation (AR), and one patient had mild aortic stenosis at the time of QAV diagnosis. Two patients who had severe AR at referral underwent aortic valve surgery. The severity of AR in the other patients was moderate or less. During a median follow-up period of 27 months (range, 1-171 months), none of the patients other than above two patients had cardiac events. One patient died from a non-cardiac cause at 94 years of age. CONCLUSIONS: Patients diagnosed with QAV after middle age, who do not exhibit severe valve insufficiency at the time of diagnosis, may not experience worse clinical outcomes. However, further research is required for a better understanding of the long-term outcomes.

Real-time regurgitation estimation in percutaneous left ventricular assist device fully supported condition using an unscented Kalman filter.

Objective.Significant aortic regurgitation is a common complication following left ventricular assist device (LVAD) intervention, and existing studies have not attempted to monitor regurgitation signals and undertake preventive measures during full support. Regurgitation is an adverse event that can lead to inadequate left ventricular unloading, insufficient peripheral perfusion, and repeated episodes of heart failure. Moreover, regurgitation occurring during full support due to pump position offset cannot be directly controlled through control algorithms. Therefore, accurate estimation of regurgitation during percutaneous left ventricular assist device (PLVAD) full support is critical for clinical management and patient safety.Approach.An estimation system based on the regurgitation model is built in this paper, and the unscented Kalman filter estimator (UKF) is introduced as an estimation approach. Three offset degrees and three heart failure states are considered in the investigation. Using the mock circulatory loop experimental platform, compare the regurgitation estimated by the UKF algorithm with the actual measured regurgitation; the errors are analyzed using standard confidence intervals of ±2 SDs, and the effectiveness of the mentioned algorithms is thus assessed. The generalization ability of the proposed algorithm is verified by setting different heart failure conditions and different rotational speeds. The root mean square error and correlation coefficient between the estimated and actual values are quantified and the statistical significance of accuracy differences in estimation is illustrated using one-way analysis of variance (One-Way ANOVA), which in turn assessed the accuracy and stability of the UKF algorithm.Main results.The research findings demonstrate that the regurgitation estimation system based on the regurgitation model and UKF can relatively accurately estimate the regurgitation status of patients during PLVAD full support, but the effect of myocardial contractility on the estimation accuracy still needs to be taken into account.Significance.The proposed estimation method in this study provides essential reference information for clinical practitioners, enabling them to promptly manage potential complications arising from regurgitation. By sensitively detecting LVAD adverse events, valuable insights into the performance and reliability of the LVAD device can be obtained, offering crucial feedback and data support for device improvement and optimization.

Contemporary Valvular Mechanisms of Aortic Regurgitation in Tricuspid Aortic Valves: Importance in Repair Versus Replacement Strategy.

BACKGROUND: This study was performed to determine cusp causes of aortic regurgitation in patients with tricuspid aortic valves without significant aortic dilatation and define cusp pathologies amenable to surgical repair (aortic valve repair [AVr]) versus aortic valve replacement. METHODS AND RESULTS: We retrospectively reviewed surgical reports of consecutive adults with tricuspid aortic valves undergoing surgery for clinically significant aortic regurgitation within a prospective registry from January 2005 to September 2019. Valvular mechanisms were determined by systematic in vivo intraoperative quantification methods. Of 516 patients, 287 (56%) underwent repair (AVr; mean±SD age, 59.9±12.4 years; 81% men) and 229 (44%) underwent replacement (aortic valve replacement; mean±SD age, 62.8±13.8 years [P=0.01 compared to AVr]; 67% men). A single valvular mechanism was present in 454 patients (88%), with cusp prolapse (46%), retraction (24%), and perforation (18%) being the most common. Prolapse involved the right cusp in 86% of cases and was more frequent in men (P<0.001). Two-dimensional transesophageal echocardiography accuracy for predicting mechanisms was 73% to 82% for the right cusp, 55% to 61% for the noncoronary cusp, and 0% for the left-coronary cusp. Cusp prolapse, younger age, and larger patient size were associated with successful AVr (all P<0.03), whereas retraction, perforation, older age, and concomitant mitral repair were associated with aortic valve replacement (all P<0.03). CONCLUSIONS: Right cusp prolapse is the most frequent single valvular mechanism in patients with tricuspid aortic valve aortic regurgitation, followed by cusp retraction and perforation. The accuracy of 2-dimensional transesophageal echocardiography is limited for left and noncoronary cusp mechanistic assessment. Prolapse is associated with successful AVr, whereas retraction and perforation are associated with aortic valve replacement. With systematic intraoperative quantification methods and current surgical techniques, more than half of tricuspid aortic valve aortic regurgitation cases may be successfully repaired.

Behçet's syndrome masquerading as infective endocarditis: A diagnostic conundrum and therapeutic challenge.

This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's complex clinical presentation, including chest pain, aortic dilation, severe aortic regurgitation, and aortic root abscess, posed significant diagnostic and therapeutic challenges. Despite initial misdiagnosis and treatment difficulties, the patient's condition significantly improved with appropriate immunosuppressive therapy, underscoring the potential for successful management of this complex condition. This case serves as a valuable reminder of the diagnostic challenges posed by Behçet's syndrome and the importance of considering this condition in patients presenting with symptoms suggestive of infective endocarditis.

Quantifying aortic valve regurgitation in patients with congenital aortic valve disease by 2D and 4D flow magnetic resonance analysis.

BACKGROUND: In congenital aortic valve disease, quantifying aortic regurgitation (AR) varies by the measurement site. Our study aimed to identify the optimal site for AR assessment using 2D and 4D MR flow measurements, with a focus on vortices. METHODS: We retrospectively analysed 31 patients with congenital aortic valve disease, performing 2D and 4D MR flow measurements at the aortic valve, sinotubular junction (STJ), ascending aorta (AAo), and using midpulmonary artery measurements as a reference. We assessed percentage AR and net forward volumes, calculated linear correlations, and plotted Bland-Altman plots. Net forward flow at all aortic sites were correlated with the main pulmonary artery. Differences in AR between 2D and 4D flows were linked to vortices detected by 4D streamlines. RESULTS: The best agreement in % AR between 2D and 4D flows was at the aortic valve (mean difference 4D2D -2.9%, limits of agreement 8.7% to -14.3%; r2 = 0.7). Correlations weakened at STJ and AAo. Vortices in the ascending aorta led to AR overestimation in 2D measurements. Net forward flow at the aortic valve by 4D flow correlated closer with main pulmonary artery than did 2D flow. (Mean difference for 2D and 4D MR flow 7.5 ml and 4.2 ml, respectively). CONCLUSIONS: For congenital aortic valve disease, the most accurate AR quantification occurs at the aortic valve using 2D and 4D MR flow. Notably, vortices in the ascending aorta can result in AR overestimation with 2D MR flow.

[Catheter-based and surgical treatment for aortic valve diseases]. / Katheterbasierte und operative Therapie bei Aortenvitien.

The pathophysiology of aortic valve diseases is of predominantly degenerative nature, characterized by calcific aortic valve stenosis, which is associated with a reduction in prognosis. The prevalence of aortic valve insufficiency also increases with advancing age. Timely causal treatment is crucial in the management of aortic valve diseases. Following the indication for intervention, the heart team plays a central role in evaluating the results and making therapeutic decisions that consider the patient's preferences. In the assessment of treatment options, considerations regarding the long-term perspective are particularly crucial, especially in younger patients. The most common therapeutic approach for aortic valve diseases is the introduction of a new valve prosthesis. In the majority of cases, this is now achieved through catheter-based implantation of a bioprosthetic heart valve, known as transcatheter aortic valve implantation (TAVI). Open surgical aortic valve replacement (AVR) is favored in younger patients with low surgical risk or in the case that TAVI is not feasible. In AVR, both biological and the longest-lasting mechanical prosthesis types are used. Surgical repair techniques are primarily applied in cases of aortic valve regurgitation. Notably, TAVI, as well as surgical procedures for the treatment of aortic valve diseases, have undergone significant advancements in recent years, including expanded indications for TAVI and, on the surgical side, in particular the development of minimally invasive surgical techniques.

Aortic Regurgitation, Time to Aortic Valve Reintervention, and Mortality in Degenerated Trifecta Versus Non-Trifecta Bioprosthesis.

On July 31, 2023, the Trifecta valve was withdrawn from the market after concerns regarding early (≤5 years) structural valve deterioration (SVD), mainly as aortic regurgitation (AR). Our aim was to determine the timing, mechanism, and impact of bioprosthetic SVD in patients who underwent redo aortic valve replacement (redo-AVR) with either redo-SAVR or valve-in-valve transcatheter aortic valve replacement (TAVR) using Trifecta versus other bioprosthetic valves. Patients who underwent redo-AVR for SVD at our institution were categorized into 2 groups based on the valve type: Trifecta versus non-Trifecta. Multivariate Cox proportional hazard model and Kaplan-Meier curves were used to compare mortality. A total of 171 patients were included; 58 (34%) had previous SAVR with a Trifecta valve and 113 (66%) with non-Trifecta valve. A total of 103 patients (60%) underwent valve-in-valve TAVR and 68 redo-SAVR (40%). The age, gender, and Society of Thoracic Surgeons score were similar between Trifecta and non-Trifecta groups. In patients with bioprosthetic valves requiring redo-AVR, Trifecta valves had an earlier onset of greater than moderate AR (4.5 vs 11.9 years, p <0.001) and earlier time to redo-AVR (5.5 vs 12 years, p <0.001). AR was more common as the mechanism of SVD in Trifecta versus non-Trifecta valves (55.2% vs 30.1%, p = 0.006). All-cause adjusted mortality from index SAVR was higher in the Trifecta than in non-Trifecta group (hazard ratio 4.1, 95% confidence interval 1.5 to 11.5, p = 0.007). In conclusion, compared with non-Trifecta valves, Trifecta valves exhibit early SVD primarily as AR and progress rapidly to significant SVD requiring redo-AVR. Mortality is significantly higher with Trifecta than in non-Trifecta valves, potentially impacting the results of SAVR versus TAVR studies.

Prosthesis Infolding Incidence and Short-Term Outcomes in Transcatheter Aortic Valve Implantation Using Evolut Self-Expandable Device: A Multicenter Study.

Transcatheter aortic valve implantation (TAVI) is an established treatment strategy in aortic valve disease. Infolding, as a nonuniform expansion of the prosthesis leading to introflection of part of the device circumference, is a complication specific to self-expandable prostheses. The aim of the study is to determine incidence, predictors, treatment strategy, and outcomes of infolding during Medtronic Evolut TAVI (Minneapolis, MN, US). Between January 2018 and March 2022, all patients treated with Evolut TAVI were included in a multicenter observational retrospective study. According to the occurrence of infolding, the enrolled cohort was divided into 2 groups; periprocedural characteristics and 30-day outcomes were compared. A total of 1,470 patients were included; 23 infolding cases (1.6%) were detected. Preprocedural imaging showed larger aortic anatomy and greater calcium burden in the infolding group. Infolding occurred mostly with Evolut Pro+ and size 34 mm and was diagnosed before full prosthesis release in 78.3%. The rate of moderate-to-severe paravalvular regurgitation was higher in the infolding group (21.7% vs 1.9%, p <0.001). Short-term follow-up showed greater all-cause and cardiovascular mortality (respectively, 4.3% vs 0.7% and 4.3% vs 0.6%, p <0.05) and higher rate of pacemaker implantation (33.3% vs 15.7%, p = 0.042) in case of infolding. High right cusp calcium score and resheathing maneuvers were independent predictors of infolding. In conclusion, prosthesis infolding is a TAVI complication burdened by worse cardiovascular outcomes. Prompt intraprocedural infolding diagnosis is pivotal, especially in case of great native valve calcium burden and resheathing maneuvers, to safely overcome this complication by prosthesis recapture or postdilation.

Transcatheter Aortic Valve Replacement and Coronary Protection Guided by Deep Learning and 3-Dimensional Printing.

OBJECTIVE: In this case report, the auxiliary role of deep learning and 3-dimensional printing technology in the perioperative period was discussed to guide transcatheter aortic valve replacement and coronary stent implantation simultaneously. CASE PRESENTATION: A 68-year-old man had shortness of breath and chest tightness, accompanied by paroxysmal nocturnal dyspnea, 2 weeks before presenting at our hospital. Echocardiography results obtained in the outpatient department showed severe aortic stenosis combined with regurgitation and pleural effusion. The patient was first treated with closed thoracic drainage. After 800 mL of pleural effusion was collected, the patient's symptoms were relieved and he was admitted to the hospital. Preoperative transthoracic echocardiography showed severe bicuspid aortic valve stenosis combined with calcification and aortic regurgitation (mean pressure gradient, 42 mmHg). Preoperative computed tomography results showed a type I bicuspid aortic valve with severe eccentric calcification. The leaflet could be seen from the left coronary artery plane, which indicated an extremely high possibility of coronary obstruction. After preoperative imaging assessment, deep learning and 3-dimensional printing technology were used for evaluation and simulation. Guided transcatheter aortic valve replacement and a coronary stent implant were completed successfully. Postoperative digital subtraction angiography showed that the bioprosthesis and the chimney coronary stent were in ideal positions. Transesophageal echocardiography showed normal morphology without paravalvular regurgitation. CONCLUSION: The perioperative guidance of deep learning and 3-dimensional printing are of great help for surgical strategy formulation in patients with severe bicuspid aortic valve stenosis with calcification and high-risk coronary obstruction.