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.

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.

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.

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.

[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.

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.

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.

Experience with the ACURATE neo and neo2 transcatheter aortic valves in Spain. The PRECISA (PRospective Evaluation Complementing Investigation with ACURATE devices) registry.

BACKGROUND: Previous studies have documented a high rate of implantation success with the ACURATE neo2 valve, as well as a reduction in paravalvular leak (PVL) compared to its predecessor, the ACURATE neo. However, there are no studies that have reviewed and compared the long-term clinical and hemodynamic outcomes of these patients. AIMS: This study aimed to evaluate the results of the ACURATE neo transcatheter aortic valve in a real-world context, and to compare the results of the outcomes of both generations of this device (ACURATE neo and ACURATE neo2), with a specific focus on procedural success, safety, and long-term effectiveness. METHODS: A prospective study including all consecutive patients treated with the ACURATE neo device in seven hospitals was conducted (Clinical Trials Identification Number: NCT03846557). The primary endpoint consisted of a composite of adverse events, including mortality, aortic insufficiency, and other procedural complications. As the second-generation device (ACURATE neo2) replaced the ACURATE neo during the study period, hemodynamic and clinical results before admission, at 30 days, and at 1 year of follow-up were compared between the two generations. RESULTS: A total of 296 patients underwent transcatheter aortic valve implantation with the ACURATE device, with 178 patients receiving the ACURATE neo and 118 patients receiving the ACURATE neo2. In the overall population, the absence of device success occurred in 14.5%. The primary reason for the absence of device success was the presence of para-valvular regurgitation ≥ 2. There were no instances of coronary occlusions, valve embolization, annulus rupture, or procedural deaths. ACURATE neo2 was associated with a significantly higher device success rate (91.7% vs. 82%, p = 0.04), primarily due to a significantly lower rate of para-valvular regurgitation, which remained significant at 1 year. CONCLUSION: The use of ACURATE neo and neo2 transcatheter aortic valves is associated with satisfactory clinical results and an extremely low rate of complications. The ACURATE neo2 enables a significantly higher device success rate, primarily attributed to a significant reduction in the rate of PVL.

[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.

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.

Quadricuspid Aortic Valve: Imaging, Diagnosis, and Prognosis.

Quadricuspid aortic valve is a rare congenital cardiac anomaly with an incidence of 0.008% to 0.043%. Its clinical course varies depending on cusp anatomy, function, and associated cardiac malformations. It frequently progresses to aortic valve regurgitation that may require surgical valve replacement. Detection has shifted from incidental discovery during autopsies or cardiac surgeries in the early 20th century to various cardiac imaging methods in recent decades. In addition to contributing to the literature, this report supports the use of transesophageal echocardiography more liberally to detect aortic valve abnormalities. The case presents a 48-year-old female patient with an incidentally discovered quadricuspid aortic valve.

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.

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.

Prognostic Implications of Left Atrial Strain in Bicuspid Aortic Valve With Chronic Aortic Regurgitation.

BACKGROUND: Left atrial reservoir strain (LARS) is a novel imaging biomarker of left ventricular diastolic dysfunction. This study aimed to examine the prognostic implications of LARS in patients with bicuspid aortic valve and significant (moderate-severe to severe) aortic regurgitation. METHODS AND RESULTS: A total of 220 patients with bicuspid aortic valve and significant aortic regurgitation were prospectively enrolled in our study. LARS and left ventricular global longitudinal strain were derived from speckle-tracking echocardiography. The end point was a composite of all-cause mortality, heart failure hospitalization, and aortic valve repair or replacement. The threshold value of LARS <24% was used to identify impaired left atrial mechanics based on prior results. During a median follow-up of 364 (interquartile range, 294-752) days, 46 patients (20.9%) reached the composite end points. On multivariable Cox analysis, impaired LARS (adjusted hazard ratio, 2.08 [95% CI, 1.05-4.11]; P=0.036) was a statistically significant predictor of composite end points after adjustment for other statistically significant predictors. Finally, adding impaired LARS to other statistically significant predictors (New York Heart Association functional class and left ventricular global longitudinal strain) significantly improved the global χ2 (from 32.19 to 36.56; P=0.037) and reclassification (continuous net reclassification index=0.55; P<0.001) of the prediction model. CONCLUSIONS: In patients with bicuspid aortic valve and significant aortic regurgitation, the impairment of LARS is a strong independent prognostic predictor and confers incremental prognostic utility over clinical and other echocardiographic parameters. These findings suggest that LARS could be considered in risk stratification for such populations.

Surgical Heritage: You Had to Be There, Ross: The Comeback Kid.

Half a century after the first pulmonary autograft operation (Ross operation), performed in 1967 by Donald Ross in central London, there is a very strong conviction that the Ross operation is the best available valve substitute today, not only for children, but also for younger and older adults. The Ross operation has stimulated a lot of science to do with tissue-engineering and biology of heart valves, which is a promising avenue for the future. For one of us (M.Y.), it has certainly been a privilege to be associated with the comeback of the Ross operation.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Tailored Approach.

While it is the main viable option in the growing child and young adult, the Ross procedure has expanded its applicability to older patients, for whom long-term results are equivalent, if not superior, to prosthetic aortic valve replacement. Strategies aiming at mitigating long-term autograft failure from root enlargement and valve regurgitation have led some to advocate for root reinforcement with prosthetic graft material. On the contrary, we will discuss herein the rationale for a tailored approach to the Ross procedure; this strategy is aimed at maintaining the natural physiology and interplay between the various autograft components. Several technical maneuvers, including careful matching of aortic and autograft annuli and sino-tubular junction as well as external support by autologous aortic tissue maintain these physiologic relationships and the viability of the autograft, and could translate in a lower need for late reintervention because of dilation and/or valve regurgitation.

Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Supported Ross.

The Ross procedure is an excellent option for aortic valve replacement resulting in outstanding hemodynamic performance and the ability to avoid systemic anticoagulation. The long-term durability of the autograft is generally good but concerns for later aortic root dilation with ensuing neoaortic insufficiency have prompted efforts to stabilize the autograft, root, sinuses and Sino-tubular junction in order to delay or entirely avoid late reinterventions on the neoaortic root. We have employed an inclusion technique, supporting the Auto-graft in a Terumo Gelweave™ Valsalva graft. We performed a retrospective study of all 129 patients undergoing the Ross procedure from 1992 to 2019 at Children's Wisconsin. Fifty-one underwent the supported Ross (SR) and 78 underwent unsupported Ross (UR). Structured clinical data was collected and echocardiograms were reviewed. Median follow-up was 4.9 years (up to 22.6 years) for UR patients and 3.6 years (up to 11.4 years) for SR patients. In order to provide a fair comparison, we sub -analyzed patients aged 10 to 18 years who underwent the Ross procedure, 16 who underwent the UR and 18 patients who underwent the SR. Change in aortic annulus diameter (P = 0.002), aortic sinus diameter (P = 0.001) change in left ventricular function (P = 0.039) and change in aortic insufficiency (P = 0.008) were all worse in UR. The SR is simple, reproducible, and predictable. It seems to prevent change in annulus diameter, sinus diameter and to reduce late neoaortic insufficiency. Longer follow-up with a larger group of patients is required to draw definitive conclusions.