A clinical case of Takayasu disease: emphasis on cardiac manifestations.

Takayasu arteriitis (TA) is a rare systemic vasculitis, affecting large vessels, cardiac valves and myocardium. Cardiac involvement is a major cause of morbidity and mortality in such patients. This publication presents a clinical case of a patient with severe aortic regurgitation combined with restrictive cardiomyopathy. It is emphasized that surgical treatment is associated with potential difficulties in patients with TA due to its inflammatory nature, disease activity and multiorgan involvement.

[The Long-term Results of Valve Sparing Aortic Root Replacement and Aortic Cusp Repair for Aortic Regurgitation].

We reported our long-term results of valve sparing aortic root replacement (VSRR) and aortic cusp repair for aortic regurgitation( AR) were satisfactory. Three hundred twenty-seven patients had VSRR, and 164 patients of them had aortic cusp repair for prolapse. At 10 years after the operation, the overall survival was 91.5%, the freedom from more than mild recurrent AR was 71.2%, and the freedom from aortic valve reoperation was 82.0%. As for the aortic cusp repair technique, there was no significant difference in the mid-term results of the recurrent AR and reoperation for the aortic valve between the central plication technique and the resuspension technique (two layers of continuous mattress sutures placed the entire length of the free margin of the aortic cusp). The resuspension technique might be useful for repairing the aortic cusp with prolapse. Furthermore, among the patients with acute aortic dissection, connective tissue disease, or aortitis, the long-term results of VSRR and aortic cusp repair were also satisfactory.

Performance of Purpose-Built vs Off-Label Transcatheter Devices for Aortic Regurgitation: The PURPOSE Study.

BACKGROUND: Severe pure aortic regurgitation (AR) carries a high mortality and morbidity risk, and it is often undertreated because of the inherent surgical risk. Transcatheter heart valves (THVs) have been used off-label in this setting with overall suboptimal results. The dedicated "purpose-built" Jena Valve Trilogy (JVT, JenaValve Technology) showed an encouraging performance, although it has never been compared to other THVs. OBJECTIVES: The aim of our study was to assess the performance of the latest iteration of THVs used off-label in comparison to the purpose-built JVT in inoperable patients with severe AR. METHODS: We performed a multicenter, retrospective registry with 18 participating centers worldwide collecting data on inoperable patients with severe AR of the native valve. A bicuspid aortic valve was the main exclusion criterion. The primary endpoints were technical and device success, 1-year all-cause mortality, and the composite of 1-year mortality and the heart failure rehospitalization rate. RESULTS: Overall, 256 patients were enrolled. THVs used off-label were used in 168 cases (66%), whereas JVT was used in 88 (34%). JVT had higher technical (81% vs 98%; P < 0.001) and device success rates (73% vs 95%; P < 0.001), primarily driven by significantly lower incidences of THV embolization (15% vs 1.1%; P < 0.001), the need for a second valve (11% vs 1.1%; P = 0.004), and moderate residual AR (10% vs 1.1%; P = 0.007). The permanent pacemaker implantation rate was comparable and elevated for both groups (22% vs 24%; P = 0.70). Finally, no significant difference was observed at the 1-year follow-up in terms of mortality (HR: 0.99; P = 0.980) and the composite endpoint (HR: 1.5; P = 0.355). CONCLUSIONS: The JVT platform has a better acute performance than other THVs when used off-label for inoperable patients with severe AR. A longer follow-up is conceivably needed to detect a possible impact on prognosis.

Precise reconstruction of the aortic valve using glutaraldehyde-treated autologous pericardium in new formula: a case report.

BACKGROUND: The current study presents a novel and precise surgical technique for complete reconstruction of the aortic valve using glutaraldehyde-treated autologous pericardium in a patient with aortic valve disease and endocarditis. The technique aims to provide a more effective and reproducible method for aortic valve repair, with the goal of improving outcomes and quality of life for patients with aortic valve disease. CASE PRESENTATION: A 35-year-old Iranian male with aortic valve disease and endocarditis underwent aortic valve reconstruction surgery. Preoperative echocardiography showed a degenerative aortic valve with severe regurgitation, reduced left ventricular ejection fraction, and specific aortic root dimensions. The surgical technique involved precise measurements and calculations to design the size and shape of the new aortic valve cusps using autologous pericardium, with the goal of optimizing coaptation and function. The surgeon calculated the intercommissural distance based on the aortic annulus diameter to determine cusp size and shape. He tailored the pericardial cusps to have a height equal to 80% of the coaptation margin length. Detailed suturing techniques were used to ensure proper alignment and coaptation of the new cusps. Intraoperative evaluation of the valve function using suction and transesophageal echocardiography showed good coaptation and minimal residual regurgitation. At the 3-year follow-up, the patient had a well-functioning aortic valve with only trivial leak and was in satisfactory clinical condition. CONCLUSIONS: Glutaraldehyde-treated autologous pericardium is a validated leaflet alternative, and the causes of its failure are late annular dilatation and other technique breakdowns. Current evidence reveals that aortic valve reconstruction with glutaraldehyde-treated autologous pericardium is associated with many advantages with the potential to improve patient outcomes and quality of life. Further clinical studies are warranted to evaluate the long-term durability and efficacy of this approach.

Rapid deployment aortic valve implantation in complex patients with infective endocarditis or aortic valve insufficiency.

BACKGROUND: New prosthetic valves and surgical approaches that shorten operation time and improve the outcome of patients with aortic valve (AV) infective endocarditis (IE) and AV insufficiency (AVI) are crucial. The aim of this study was to evaluate the outcome of patients with AV IE or AVI treated with the EDWARDS INTUITY Rapid-Deployment AV prosthesis for this off-label indication. METHODS: This single-centre retrospective study analyzed data from patients who underwent AV replacement with the EDWARDS INTUITY Rapid-Deployment AV prosthesis for AV IE or regurgitation. (n = 8 for IE and n = 6 for AVI). RESULTS: Heart-lung machine times were significantly shorter in the AVI group (111.3 ± 20.7 min) compared to the IE group (171.9 ± 52.4 min) (p = 0.02). Aortic cross-clamp followed a similar trend (73.7 ± 9.9 min for AVI vs. 113.4 ± 35.6 min for IE) (p = 0.02). The length of ICU stay was also shorter in the AVI group (3.8 ± 2.6 days) compared to the IE group (16.9 ± 8.9 days) (p = 0.005). Postoperative echocardiography revealed no paravalvular leakage or significant valvular dysfunction in any patient. One patient died postoperatively from aspiration pneumonia. CONCLUSION: The INTUITY valve demonstrates as a safe option for complex AV IE and AVI surgery. Further prospective studies with larger patient cohorts are necessary to confirm these findings and explore the long-term benefits of this approach.

Iatrogenic non-coronary leaflet perforation as a complication after robotic mitral valve repair.

Iatrogenic aortic regurgitation secondary to leaflet injury is a rare complication of mitral valve surgery. For the first time, we report a patient who had progressive aortic regurgitation due to non-coronary leaflet perforation after robotic mitral valve repair and required aortic valve repair 18 months after this initial surgery. As in our case, aortic regurgitation after mitral valve surgery may remain undiagnosed on intraoperative transesophageal echocardiography or undetected until the patient's discharge due to gradual enlargement of very small perforations over the postoperative course.

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.

Commissural alignment and the ACURATE neo2 transcatheter aortic valve: Impact on valve performance.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is increasingly being used to treat severe aortic stenosis in younger patients. Accordingly, lifetime management regarding future reintervention and coronary access is a concern. AIMS: To assess the impact of commissural alignment on ACURATE neo2 transcatheter aortic valve (TAV) performance. METHODS: COMALIGN-neo2 was an observational, retrospective study enrolling consecutive TAVR patients treated with the ACURATE neo2 (October 2021 to October 2022). The degree of commissural (mis)-alignment (CMA) with the native aortic valve commissures was determined and transvalvular gradient, effective orifice area, patient-prosthesis mismatch (PPM), and aortic regurgitation (AR) were assessed. RESULTS: Among 825 patients, the mean age was 80.7 years and 42% were female. Commissural alignment was achieved in 60% of cases; mild (26%), moderate (9%), and severe misalignment (5%) were found less often. Severe PPM occurred more frequently in patients with severe CMA (14.7%) compared to aligned valves (p = 0.034). By multivariate analysis, severe CMA (odds ratio [OR]: 3.12, 95% confidence interval [CI] [1.09-8.90]; p = 0.033) and lack of postdilatation (OR: 3.85, [1.33-11.1]; p = 0.012) were associated with severe PPM. Higher rates of ≥mild AR (51.4%) were found in TAVs implanted with severe CMA compared to aligned (34.3%), mildly (38.1%) or moderately (36.0%) misaligned TAVs (p = 0.030). Multivariate analysis identified severe CMA (OR: 2.05, [1.05-4.02]; p = 0.037) to be an independent predictor of ≥mild AR. CONCLUSIONS: COMALIGN-neo2 is the largest study to date assessing the impact of commissural alignment on acute TAV performance. Severe CMA with the ACURATE neo2 platform was associated with worse valve hemodynamics and increased risk for mild AR.

Clinical journey for patients with aortic regurgitation: A retrospective observational study from a multicenter database.

BACKGROUND: Data using real-world assessments of aortic regurgitation (AR) severity to identify rates of Heart Valve Team evaluation and aortic valve replacement (AVR), as well as mortality among untreated patients, are lacking. The present study assessed these trends in care and outcomes for real-world patients with documented AR. METHODS: Using a deidentified data set (January 2018-March 2023) representing 1,002,853 patients >18 years of age from 25 US institutions participating in the egnite Database (egnite, Inc.) with appropriate permissions, patients were classified by AR severity in echocardiographic reports. Rates of evaluation by the Heart Valve Team, AVR, and all-cause mortality without AVR were examined using Kaplan-Meier estimates and compared using the log-rank test. RESULTS: Within the data set, 845,113 patients had AR severity documented. For moderate-to-severe or severe AR, respectively, 2-year rates (95% confidence interval) of evaluation by the Heart Valve Team (43.5% [41.7%-45.3%] and 65.4% [63.3%-67.4%]) and AVR (19.4% [17.6%-21.1%] and 46.5% [44.2%-48.8%]) were low. Mortality at 2 years without AVR increased with greater AR severity, up to 20.7% for severe AR (p < 0.001). In exploratory analyses, 2-year mortality for untreated patients with left ventricular end-systolic dimension index > 25 mm/m2 was similar for moderate (34.3% [29.2%-39.1%]) and severe (37.2% [24.9%-47.5%]) AR. CONCLUSIONS: Moderate or greater AR is associated with poor clinical outcomes among untreated patients at 2 years. Rates of Heart Valve Team evaluation and AVR were low for those with moderate or greater AR, suggesting that earlier referral to the Heart Valve Team could be beneficial.

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.

Short-Term Outcomes of Transcatheter Aortic Valve Replacement in Low-Risk Patients With Pure Severe Aortic Regurgitation.

Transcatheter aortic valve replacement (TAVR) has emerged as an alternative treatment for patients with pure severe aortic regurgitation (PSAR) who are contraindicated for surgery or have a high surgical risk. However, the therapeutic efficacy and safety of TAVR in low Society of Thoracic Surgeons (STS) score risk patients remain to be clarified. This study aimed to explore the feasibility of TAVR treatment in different STS-risk patients and to compare the adverse events between the groups. In this study, patients with PSAR who underwent TAVR at Zhongshan Hospital, Fudan University, China, during the inclusion period were included and categorized into 3 groups based on STS scores. The baseline data, imaging results, and follow-up data of the patients were documented. Therefore, of 75 TAVR patients, 38 (50.7%) were categorized as low risk (STS <4), and 37 (49.3%) patients were categorized as intermediate and high risk (STS ≥4). Compared with patients at intermediate and high risk, those in the low-risk group were younger, had a lower body mass index, had a lower prevalence of hypertension, chronic obstructive pulmonary disease, and previous percutaneous coronary intervention, and had better cardiac function (p all <0.05). In the hospital and at the 1-month follow-up, the degree of aortic regurgitation and cardiac function were significantly improved. No significant difference was found between the 2 groups in the hospital or during the 30-day follow-up. In conclusion, TAVR for PSAR in low-STS-risk patients is safe and efficient during 30 days of follow-up compared with intermediate- and high-STS-risk groups. TAVR for PSAR should not be limited to inoperable or STS-defined high-risk patients. Long-term follow-up is needed for further investigation.

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.

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.

Off-Label Use of Balloon-Expandable Transcatheter Valves to Treat Pure Aortic Regurgitation.

Transcatheter aortic valve implantation (TAVI) in native pure aortic regurgitation (AR) with off-label use of balloon-expandable valves (BEV) has been reported. However, there are scant data regarding optimal oversizing and its safety, and our study assessed BEV oversizing and outcomes of TAVI. Thirteen consecutive tricuspid aortic valve patients who underwent transfemoral TAVIs for pure AR with Sapien BEV at our center between 2019 and 2023 (69.2% males, mean age 80.8 years, Society of Thoracic Surgeons 4.0%) were divided into small annulus (SA) group (≤618 mm2) where ≥20% oversizing is achievable based on published data on BEV overexpansion, and larger annulus (LA) group (>618 mm2). Overexpansion and actual oversizing were measured on postprocedural computed tomography scan. Technical success was 92.3% with 1 valve embolization in the LA group. The postprocedural computed tomography showed a mean 28.3% oversizing, significantly higher in SA (31.2%) than in LA group (19.4%), p = 0.0092. Oversizing ≥20% was achieved in 100% SA versus 33.3% LA patients (p = 0.046). In conclusion, TAVI in pure AR with oversized Sapien BEV showed good procedural and short-term outcomes when ≥20% oversizing was predictably achievable.

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.

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.

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.