Multimodality Imaging of Intimal Sarcoma Causing Both Severe Mitral Stenosis and Mitral Regurgitation.

Intimal sarcomas (IS) are rare, malignant, rapidly progressive mesenchymal tumors that typically occur in the tunica intima of larger vessels, and they rarely involve the heart. IS are frequently misdiagnosed during the initial clinical presentation. This case report describes an uncommonly located IS, highlighting specific findings obtained through multimodality imaging.

Contemporary 1-Year Outcomes of Mitral Valve-in-Ring With Balloon-Expandable Aortic Transcatheter Valves in the U.S.

BACKGROUND: Adequate valve performance after surgical mitral valve repair with an annuloplasty ring is not always sustained over time. The risk of repeat mitral valve surgery may be high in these patients. Transcatheter mitral valve-in-ring (MViR) is emerging as an alternative for high-risk patients. OBJECTIVES: The authors sought to assess contemporary outcomes of MViR using third-generation balloon-expandable aortic transcatheter heart valves. METHODS: Patients who underwent MViR and were enrolled in the STDS/ACC TVT (Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy) Registry between August 2015 and December 2022 were analyzed. RESULTS: A total of 820 patients underwent MViR at 236 sites, mean age was 72.2 ± 10.4 years, 50.9% were female, mean STS score was 8.2% ± 6.9%, and most (78%) were in NYHA functional class III to IV. Mean left ventricular ejection fraction was 47.8% ± 14.2%, mean mitral gradient was 8.9 ± 7.0 mm Hg, and 75.5% had ≥ moderate mitral regurgitation. Access was transseptal in 93.9% with 88% technical success. All-cause mortality at 30 days was 8.3%, and at 1 year, 22.4%, with a reintervention rate of 9.1%. At 1-year follow-up, 75.6% were NYHA functional class I to II, Kansas City Cardiomyopathy Questionnaire score increased by 25.9 ± 29.1 points, mean mitral valve gradient was 8.4 ± 3.4 mm Hg, and 91.7% had ≤ mild mitral regurgitation. CONCLUSIONS: MViR with third-generation balloon-expandable aortic transcatheter heart valves is associated with a significant reduction in mitral regurgitation and improvement in symptoms at 1 year, but with elevated valvular gradients and a high reintervention rate. MViR is a reasonable alternative for high-risk patients unable undergo surgery who have appropriate anatomy for the procedure. (STS/ACC TVT Registry Mitral Module [TMVR]; NCT02245763).

Mitral Valve Transcatheter Edge-to-Edge Repair: 1-Year Outcomes From the MiCLASP Study.

BACKGROUND: Mitral transcatheter edge-to-edge repair (M-TEER) is a guideline-recommended treatment option for patients with severe symptomatic mitral regurgitation (MR). Outcomes with the PASCAL system in a post-market setting have not been established. OBJECTIVES: The authors report 30-day and 1-year outcomes from the MiCLASP (Transcatheter Repair of Mitral Regurgitation with Edwards PASCAL Transcatheter Valve Repair System) European post-market clinical follow-up study. METHODS: Patients with symptomatic, clinically significant MR were prospectively enrolled. The primary safety endpoint was clinical events committee-adjudicated 30-day composite major adverse event rate and the primary effectiveness endpoint was echocardiographic core laboratory-assessed MR severity at discharge compared with baseline. Clinical, echocardiographic, functional, and quality-of-life outcomes were assessed at 1 year. RESULTS: A total of 544 patients were enrolled (59% functional MR, 30% degenerative MR). The 30-day composite major adverse event rate was 6.8%. MR reduction was significant from baseline to discharge and sustained at 1 year with 98% of patients achieving MR ≤2+ and 82.6% MR ≤1+ (all P < 0.001 vs baseline). One-year Kaplan-Meier estimate for survival was 87.3%, and freedom from heart failure hospitalization was 84.3%. Significant functional and quality-of-life improvements were observed at 1 year, including 71.6% in NYHA functional class I/II, 14.4-point increase in Kansas City Cardiomyopathy Questionnaire score, and 24.2-m improvement in 6-minute walk distance (all P < 0.001 vs baseline). CONCLUSIONS: One-year outcomes of this large cohort from the MiCLASP study demonstrate continued safety and effectiveness of M-TEER with the PASCAL system in a post-market setting. Results demonstrate high survival and freedom from heart failure hospitalization, significant and sustained MR reduction, and improvements in symptoms, functional capacity, and quality of life.

Clinical and echocardiographic results of the MEMO 4D semi-rigid annuloplasty ring.

BACKGROUND: Mitral regurgitation is a frequent valvular disease, with an increasing prevalence. We analyzed the short-term outcomes of mitral valve repair procedures conducted in our clinic using a new semirigid annuloplasty ring featuring a gradual saddle shape design. METHODS: We retrospectively analyzed mitral valve repair surgeries performed at our Institution between December 2019 and November 2021 with the MEMO 4D semirigid annuloplasty ring. RESULTS: In total, 53 patients were included in the study. Mean patient age was 63.6 ± 11.7 years. Most patients presented with degenerative mitral valve regurgitation (N = 44; 83%). The grade of mitral regurgitation was equal or more than 3 + in 98.1% of the patients (N = 52). The most used ring size was size 34 mm (N = 30, 56.6%). There was no intraoperative or hospital mortality. No cases of stroke, bleeding, endocarditis or other major complications occurred. At discharge, most patients were in NYHA class I. Postoperative echocardiographic results showed no (90.6%) or 1+ (5.7%) mitral valve regurgitation. Only 1 patient (1.9%) presented with mitral valve regurgitation grade 2+. Mean postoperative transvalvular gradient was low (mean = 3.3 ± 1.2 mmHg). No cases of LVOT obstruction or systolic anterior motion occurred. CONCLUSIONS: Our series showed excellent mitral valve competency and very satisfactory early clinical outcomes. The transesophageal echocardiographic follow-up, despite obtained in a limited number of patients, further confirmed the effectiveness of findings of this preliminary experience.

Assessing emerging causes of mitral regurgitation: atrial functional mitral regurgitation.

Functional or secondary mitral regurgitation is linked to increased cardiovascular morbidity and mortality. From a mechanical perspective, secondary mitral regurgitation occurs due to an imbalance between the forces that tether the mitral leaflets and those that close them. This results in incomplete coaptation. Most commonly, functional mitral regurgitation, which occurs in both ischaemic and non-ischaemic disease states, is usually caused by dysfunction and changes in the left ventricle. Atrial functional mitral regurgitation (AFMR) is a disease state that has been more recently recognized. It occurs when mitral annular enlargement is associated with left atrial dilatation, preserving left ventricular geometry and function. AFMR is typically seen in patients with chronic atrial fibrillation or heart failure who have a conserved ejection fraction. Published reports and ongoing investigations vary in how they define AFMR. This publication examines the pathophysiology of AFMR and highlights the importance of having a common working standard for the definition of AFMR to ensure consistency in the data reported and to drive forward the much needed research into the outcomes and treatment strategies in this area. Several studies have reported that restrictive annuloplasty and transcatheter edge-to-edge repair can reduce mitral regurgitation and improve symptoms. This narrative review will explore the pathophysiology, echocardiographic diagnosis and treatment of AFMR.

Large Animal Translational Validation of 3 Mitral Valve Repair Operations for Mitral Regurgitation Using a Mitral Valve Prolapse Model: A Comprehensive In Vivo Biomechanical Engineering Analysis.

BACKGROUND: Various mitral repair techniques have been described. Though these repair techniques can be highly effective when performed correctly in suitable patients, limited quantitative biomechanical data are available. Validation and thorough biomechanical evaluation of these repair techniques from translational large animal in vivo studies in a standardized, translatable fashion are lacking. We sought to evaluate and validate biomechanical differences among different mitral repair techniques and further optimize repair operations using a large animal mitral valve prolapse model. METHODS: Male Dorset sheep (n=20) had P2 chordae severed to create the mitral valve prolapse model. Fiber Bragg grating force sensors were implanted to measure chordal forces. Ten sheep underwent 3 randomized, paired mitral valve repair operations: neochord repair, nonresectional leaflet remodeling, and triangular resection. The other 10 sheep underwent neochord repair with 2, 4, and 6 neochordae. Data were collected at baseline, mitral valve prolapse, and after each repair. RESULTS: All mitral repair techniques successfully eliminated regurgitation. Compared with mitral valve prolapse (0.54±0.18 N), repair using neochord (0.37±0.20 N; P=0.02) and remodeling techniques (0.30±0.15 N; P=0.001) reduced secondary chordae peak force. Neochord repair further decreased primary chordae peak force (0.21±0.14 N) to baseline levels (0.20±0.17 N; P=0.83), and was associated with lower primary chordae peak force compared with the remodeling (0.34±0.18 N; P=0.02) and triangular resectional techniques (0.36±0.27 N; P=0.03). Specifically, repair using 2 neochordae resulted in higher peak primary chordal forces (0.28±0.21 N) compared with those using 4 (0.22±0.16 N; P=0.02) or 6 neochordae (0.19±0.16 N; P=0.002). No difference in peak primary chordal forces was observed between 4 and 6 neochordae (P=0.05). Peak forces on the neochordae were the lowest using 6 neochordae (0.09±0.11 N) compared with those of 4 neochordae (0.15±0.14 N; P=0.01) and 2 neochordae (0.29±0.18 N; P=0.001). CONCLUSIONS: Significant biomechanical differences were observed underlying different mitral repair techniques in a translational large animal model. Neochord repair was associated with the lowest primary chordae peak force compared to the remodeling and triangular resectional techniques. Additionally, neochord repair using at least 4 neochordae was associated with lower chordal forces on the primary chordae and the neochordae. This study provided key insights about mitral valve repair optimization and may further improve repair durability.

Evaluation of left ventricular function in patients with mitral annular disjunction using speckle tracking echocardiography.

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality characterized by the systolic detachment of the posterior mitral annulus and the ventricular myocardium. It is usually observed coexistent with mitral valve prolapse (MVP) and associated with a mechanical dysfunction despite preserved electrical isolation function of the mitral annulus. This study aimed to evaluate left ventricular (LV) function using speckle tracking echocardiography in MVP patients with MAD. METHODS: This study was designed as a prospective, single-center study including 103 patients with MVP and 40 age- and sex-matched control subjects. Transthoracic echocardiography and cardiac magnetic resonance imaging were performed to assess LV function and MAD presence. RESULTS: MAD (+) MVP (n = 34), MAD (-) MVP (n = 69), and control (n = 40) groups were enrolled in the study. Among the MVP patients, 34 (33%) had MAD. T-negativity in the inferior leads on electrocardiography was more frequent in the MAD (+) group than in the MAD (-) patients (4.3% vs. 20.6%, p = .014). Mitral regurgitation degree, Pickelhaube sign (17.6% vs. 1.4%, p = .005), and late gadolinium enhancement frequency (35.3% vs. 10.6%, p = .002) were significantly higher in MAD (+) patients. MAD (+) patients had significantly impaired global longitudinal strain (-23.1 ±  2.1 vs. -23.5 ± 2.3, p < .001), basal longitudinal strain (BLS) (-19.6 ±  1.5 vs. -20.5 ± 1.9, p < .001), Mid-Ventricular Longitudinal Strain (-22.2 ± 1.7 vs. -23.2 ± 2.2, p < .001) and LA strain (-24.5 ± 3.9 vs. -27.2 ± 3.6, p < .001) when compared to MAD (-) MVP patients, despite similar LV ejection fraction. All these values of MVP patients were also significantly lower than the control group. The mean MAD distance was 7.8 ± 3.2 mm in MAD (+) patients. Patients with two or more symptoms were higher in the MAD (+) group than in the MAD (-) group (4.3% vs. 44.1%, p < .001). CONCLUSION: This study demonstrated a significant decrease in longitudinal strain in MVP patients with MAD, indicating myocardial dysfunction. These findings suggest that MAD may contribute to LV dysfunction and highlight the importance of early detection in younger patients. Further research is needed to explore the functional implications and long-term outcomes of MAD.

Interpapillary muscle distance independently predicts recurrent mitral regurgitation.

OBJECTIVE: Ischaemic secondary mitral regurgitation (ISMR) after surgery is due to the displacement of papillary muscles resulting from progressive enlargement of the left ventricle end-diastolic diameter (LVEDD). Our aim was to prove that if the interpapillary muscle distance (IPMD) is surgically stabilized, an increase in LVEDD will not lead to a recurrence of ischaemic mitral regurgitation (MR). METHODS: Ninety-six patients with ISMR, who underwent surgical revascularisation and annuloplasty, were randomly assigned in a 1:1 ratio to undergo papillary muscle approximation (PMA). At the 5-year follow-up, we assessed the correlation between PMA and echocardiographic improvements, the effect size of PMA on echocardiographic improvements, and a prediction model for recurrent MR using inferential tree analysis. RESULTS: There was a significant correlation between PMA and enhancements in both the α and ß angles (Spearman's rho > 0.7, p < 0.01). The α angle represents the angle between the annular plane and either the A2 annular-coaptation line or the P2 annular-coaptation line. The ß angle indicates the angle between the annular plane and either the A2 annular-leaflet tip line or the P2 annular-leaflet tip line. PMA led to substantial improvements in LVEDD, tenting area, α and ß angles, with a large effect size (Hedge's g ≥ 8, 95% CI ORs ≠ 1). The most reliable predictor of recurrent MR grade was the interpapillary distance, as only patients with an interpapillary distance greater than 40 mm developed ≥ 3 + grade MR. For patients with an IPMD of 40 mm or less, the best predictor of recurrent MR grade was LVEDD. Among the patients, only those with LVEDD greater than 62 mm showed moderate (2+) MR, while only those with LVEDD less than or equal to 62 mm had absent to mild (1+) MR. CONCLUSION: Prediction of recurrent ischaemic MR is not independent of progressive LVEDD increase. PMA-based surgical procedure stabilises IPMD.

Isolated mitral valve aneurysm in a 9-year old boy.

BACKGROUND: Isolated mitral valve aneurysm is rarely reported in children. In most cases it is associated with an underlying disease such as infective endocarditis. MVA can lead to severe complications that needs surgical intervention. CASE: In this report, we present a 9-year old asymptomatic male patient with anterior mitral valve aneurysm and rhythm disturbance diagnosed incidentally during pre-operative evaluation. CONCLUSIONS: Being rare in children, isolated MVA should be kept in mind in the differential diagnosis of mass lesions seen on the atrial side of the mitral valve. A 24-hour electrocardiogram may define subtle rhythm disturbances in these patients.

Prognostic impact of combined non-severe aortic stenosis and mitral regurgitation on clinical outcomes: a single-centre retrospective study.

OBJECTIVES: Though the concomitant occurrence of non-severe aortic stenosis (AS) and mitral regurgitation (MR) is highly prevalent, there are limited data to guide clinical decision-making in this condition. Here, we attempt to determine an aortic valve area (AVA) cut-off value associated with worse clinical outcomes in patients with combined non-severe AS and MR. METHODS: Single-centre, retrospective analysis of consecutive patients who underwent echocardiography examination between 2010 and 2021 with evidence of combined non-severe AS and MR. We excluded patients with ≥moderate aortic valve regurgitation or mitral stenosis, as well as patients who underwent any aortic or mitral intervention either prior or following our assessment (n=372). RESULTS: The final cohort consisted of 2933 patients with non-severe AS, 506 of them with >mild MR. Patients with both pathologies had lower cardiac output and worse diastolic function.Patients with an AVA ≤1.35 cm² in the presence of >mild MR had the highest rates of heart failure (HF) hospitalisations (HR 3.1, IQR 2.4-4, p<0.001) or mortality (HR 2, IQR 1.8-2.4, p<0.001), which remained significant after adjusting for clinical and echocardiographic parameters. CONCLUSION: Patients with combined non-severe AS and MR have a higher rate of HF hospitalisations and mortality. An AVA≤1.35 cm² in the presence of >mild MR is associated with worse clinical outcomes.

Volumetric quantification identifies some left atrial dilations undetected by left atrium:aorta ratio measurements: A prospective echocardiographic study in 155 Cavalier King Charles Spaniels with and without degenerative mitral valve disease.

INTRODUCTION: Degenerative mitral valve disease (DMVD) is the most common canine heart disease with a high predisposition in Cavalier King Charles Spaniels (CKCSs). Mitral regurgitation related to DMVD can lead to left atrial (LA) dilation, which is associated with survival time. Left-atrial-to-aortic (LA:Ao) ratio assessed by two-dimensional echocardiography is commonly used to evaluate LA size. The objectives of this prospective observational study were therefore 1) to compare different echocardiographic methods (i.e., monoplane and biplane Simpson's methods of discs (SMOD) and area-length methods (ALM)) in evaluating LA volume (LAvol) in CKCSs, 2) to assess LA volumes according to DMVD severity and, 3) compare the ability of LAvol and LA:Ao ratio to identify LA enlargement in CKCSs with subclinical DMVD (i.e., American College of Veterinary Internal Medicine (ACVIM) stage B). MATERIALS AND METHODS: 155 CKCSs, either healthy or affected by DMVD, were recruited. Variability and concordance between volumetric methods were evaluated. Values were analyzed according to 2019 ACVIM stages. RESULTS: All Lin's concordance correlation coefficients regarding intra- and inter-observer variability were considered as very good to excellent. Monoplane methods and ALM produced higher values of LAvol than biplane methods and SMOD, respectively. The upper limit of normal end-systolic LAvol/body weight (LASvol/BW) was defined as 0.90 mL/kg. Left atrial volumes significantly increased with ACVIM stages. Additionally, 37% of stage B1 CKCSs demonstrated LA enlargement using LASvol/BW assessment, with significantly lower LASvol/BW values in dogs with regurgitation fraction ≤30% than in others (p<0.01). CONCLUSION: In CKCSs, LAvol methods are not interchangeable. In ACVIM stage B CKCSs, LAvol quantification is more effective to detect LA enlargement than LA linear measurements.

Comparison of Transcatheter Edge-to-Edge Mitral Valve Repair for Primary Mitral Regurgitation Outcomes to Hospital Volumes of Surgical Mitral Valve Repair.

BACKGROUND: Transcatheter edge-to-edge mitral valve (MV) repair (TEER) is an effective treatment for patients with primary mitral regurgitation at prohibitive risk for surgical MV repair (MVr). High-volume MVr centers and high-volume TEER centers have better outcomes than low-volume centers, respectively. However, whether MVr volume predicts TEER outcomes remains unknown. We hypothesized that high-volume MV surgical centers would have superior risk-adjusted outcomes for TEER than low-volume centers. METHODS: We combined data from the American College of Cardiology/Society of Thoracic Surgeons Transcatheter Valve Therapy registry and the Society of Thoracic Surgeons adult cardiac surgery database. MVr was defined as leaflet resection or artificial chords with or without annuloplasty and was evaluated as a continuous variable and as predefined categories (<25, 25-49, and ≥50 MV repairs/year). A generalized linear mixed model was used to evaluate risk-adjusted in-hospital/30-day mortality, 30-day heart failure readmission, and TEER success (mitral regurgitation ≤2+ and gradient <5 mm Hg). RESULTS: The study comprised 41 834 patients from 500 sites of which 332 (66.4%) were low, 102 (20.4%) intermediate, and 66 (13.2%) high-volume surgical centers (P<0.001). TEER success was 54.6% and was not statistically significantly different across MV surgical site volumes (P=0.4271). TEER mortality at 30 days was 3.5% with no significant difference across MVr volume on unadjusted (P=0.141) or adjusted (P=0.071) analysis of volume as a continuous variable. One-year mortality was 15.0% and was lower for higher MVr volume centers when adjusted for clinical and demographic variables (P=0.027). Heart failure readmission at 1 year was 9.4% and was statistically significantly lower in high-volume centers on both unadjusted (P=0.017) or adjusted (P=0.015) analysis. CONCLUSIONS: TEER can be safely performed in centers with low volumes of MV repair. However, 1-year mortality and heart failure readmission are superior at centers with higher MVr volume.

Contemporary experience of mitral transcatheter edge-to-edge repair technology in patients with mitral annular calcification.

BACKGROUND: Mitral annular calcification (MAC) has been an exclusion for many of the earlier pivotal trials that were instrumental in gaining device approval and indications for mitral transcatheter edge-to-edge repair (M-TEER). AIMS: To evaluate the impact of MAC on the procedural durability and success of newer generation MitraClip® systems (G3 and G4 systems). METHODS: Data were collected from Northwell TEER registry. Patients that underwent M-TEER with third or fourth generation MitraClip device were included. Patients were divided into -MAC (none-mild) and +MAC (moderate-severe) groups. Procedural success was defined as ≤ grade 2 + mitral regurgitation (MR) postprocedure, and durability was defined as ≤ grade 2 + MR retention at 1 month and 1 year. Univariate analysis compared outcomes between groups. RESULTS: Of 260 M-TEER patients, 160 were -MAC and 100 were +MAC. Procedural success was comparable; however, there were three patients who required conversion to cardiac surgery during the index hospitalization in the +MAC group versus none in the -MAC group (though this was not statistically significant). At 1-month follow-up, there were no significant differences in MR severity. At 1-year follow-up, +MAC had higher moderate-severe MR (22.1% vs. 7.5%; p = 0.002) and higher mean transmitral gradients (5.3 vs. 4.0 mmHg; p = 0.001) with no differences in mortality, New York Heart Association functional class or ejection fraction. CONCLUSION: In selective patients with high burden of MAC, contemporary M-TEER is safe, and procedural success is similar to patients with none-mild MAC. However, a loss of procedural durability was seen in +MAC group at 1-year follow-up. Further studies with longer follow-ups are required to assess newer mTEER devices and their potential clinical implications in patients with a high burden of MAC.