The Anterior Mitral Valve Leaflet Prolapse as a Key to Diagnosis of ALCAPA Syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital coronary abnormality carrying a poor prognosis. We present a clinical case series of ALCAPA-related anterior mitral valve leaflet prolapse and discuss its mechanism as a key to suspicion of ALCAPA syndrome.

Three-dimensional analysis of the tricuspid annular geometry in healthy subjects and in patients with different grades of functional tricuspid regurgitation.

BACKGROUND: Accurate sizing of the tricuspid valve annulus is essential for determining the optimal timing of tricuspid valve (TV) intervention. Two-dimensional (2D) echocardiography has limitations for comprehensive TV analysis. Three-dimensional (3D) imaging of the valve provides a better understanding of its spatial anatomy and enables more accurate measurements of TV structures. OBJECTIVES: The study aimed to analyze tricuspid annulus (TA) parameters in normal heart and in different grades of functional tricuspid regurgitation (TR); to compare TA measurements obtained by 2D and 3D echocardiography. METHODS: One hundred fifty-five patients (median age 65 years, 57% women) with normal TV and different functional TR grades underwent 2D and 3D transthoracic echocardiography. The severity of TR was estimated using multiparametric assessment according to the guidelines. Mid-systolic 3D TA parameters were calculated using TV dedicated software. The conventional 2D systolic TA measurements in a standard four-chamber view were performed. RESULTS: In mid-systole, the normal TA area was 9.2 ± 2.0 cm2 for men and 7.4 ± 1.6 cm2 for women. When indexed to body surface area (BSA), there were no significant differences in the 3D parameters between genders. The 2D TA diameters were smaller than those measured in 3D. The ROC curve analysis identified that all 3D TA parameters can accurately differentiate between different functional TR grades. Additionally, the optimal cut-off values were identified for each TA parameter. CONCLUSIONS: Gender, body size, and age have an impact on the TA parameters in healthy subjects. 2D measurements are smaller than 3D parameters. The reference values for 3D metrics according to TR severity can help in identifying TA dilation and distinguishing between different functional TR grades.

The Early Effects on Tricuspid Annulus and Right Chambers Dimensions in Successful Tricuspid Valve Bicuspidization.

BACKGROUND: It is unclear to what degree of tricuspid annulus (TA) reduction is necessary to achieve good postoperative results in surgical bicuspidization. The study aimed to evaluate TA and right heart chamber's dimensions before and after heart surgery; and to compare TA parameters assessed by different modalities. METHODS: Forty patients underwent mitral valve surgery with or without concomitant tricuspid valve (TV) bicuspidization. Preoperative and postoperative measurements of TA dimensions were performed prospectively using two-dimensional (2D) and three-dimensional (3D) transthoracic echocardiography (TTE). Additionally, preoperative transesophageal echocardiography (TOE) was performed in the operating room prior to surgery. RESULTS: All patients had no or mild TR immediately after surgery. There was a significant reduction in 2D and 3D parameters of the TV and right chambers in the TV bicuspidization group. However, TV leaflets' tethering parameters did not change significantly. Preoperative 3D TTE measurements were smaller than those obtained through 3D TOE in the operation room, before surgery under general anesthesia. The 2D systolic apical 4Ch diameter and the parasternal short axis diameter mainly represent the 3D minor axis of the TA and are smaller than its 3D major axis. CONCLUSIONS: Although bicuspidization results in a one-third reduction of the TV area, tethering of the TV leaflets remains unchanged. Moreover, 3D TOE parameters of the TV under general anesthesia are larger than preoperative 3D TTE measurements. Conventional 2D measurements are insufficient for evaluating the maximum diameter of the TA.

Safety and effectiveness of standardized exercise training in patients with pulmonary hypertension associated with heart failure with preserved ejection fraction (TRAIN-HFpEF-PH): study protocol for a randomized controlled multicenter trial.

BACKGROUND: Left heart failure (HF) is characterized by an elevation in left-sided filling pressures, causing symptoms of dyspnea, impairing exercise capacity, and leading to pulmonary venous congestion and secondary pulmonary hypertension (PH). There is an increased incidence of PH associated with left heart disease, particularly with heart failure with preserved ejection fraction (HFpEF-PH). Treatment possibilities in HFpEF-PH are non-specific and very limited, thus additional pharmacological and non-pharmacological therapeutic strategies are needed. Various types of exercise-based rehabilitation programs have been shown to improve exercise capacity and quality of life (QoL) of HF and PH patients. However, no study focused on exercise training in the population of HFpEF-PH. This study is designed to investigate whether a standardized low-intensity exercise and respiratory training program is safe and may improve exercise capacity, QoL, hemodynamics, diastolic function, and biomarkers in patients with HFpEF-PH. METHODS: A total of 90 stable patients with HFpEF-PH (World Health Organization functional class II-IV) will be randomized (1:1) to receive a 15-week specialized low-intensity rehabilitation program, including exercise and respiratory therapy and mental gait training, with an in-hospital start, or standard care alone. The primary endpoint of the study is a change in 6-min walk test distance; secondary endpoints are changes in peak exercise oxygen uptake, QoL, echocardiographic parameters, prognostic biomarkers, and safety parameters. DISCUSSION: To date, no study has investigated the safety and efficacy of exercising specifically in the HFpEF-PH population. We believe that a randomized controlled multicenter trial, which protocol we are sharing in this article, will add important knowledge about the potential utility of a specialized low-intensity exercise and respiratory training program for HFpEF-PH and will be valuable in finding optimal treatment strategies for these patients. TRIAL REGISTRATION: ClinicalTrials.gov NCT05464238. July 19, 2022.

A Comparison of the Catheter-Based Transapical and Surgical Treatment Modalities for Mitral Paravalvular Leak.

BACKGROUND: There is a lack of studies where the outcomes of mitral paravalvular leak treatment were compared between surgery and catheter-based closure. The aim of this study was to compare the outcomes of re-do surgery with transapical catheter-based paravalvular leak closure. METHODS: This is a retrospective observational study at a single institution; 76 patients were included. According to the treatment, two groups were formed: the "Surgical" group (49 patients after re-do surgery) and the "Catheter" group (27 patients after transapical catheter-based treatment). RESULTS: In-hospital myocardial infarction occurred in 9 (18%) cases in the "Surgical" group and none in the "Catheter" group, p = 0.018. Procedure-related life-threatening bleeding occurred in 9 (18%) patients in the "Surgical" group and none in the "Catheter" group, p = 0.018. Nine (18%) patients died in 30 days in the "Surgical" group, and none died in the "Catheter" group, p = 0.039. A mean follow-up was 3.3 years. No difference was found between the groups by the degree of residual paravalvular regurgitation either at discharge or at follow-up. During the follow-up, 19 (39%) patients died in the "Surgical" group and 2 (7%) among the "Catheter" patients. CONCLUSIONS: Transapical catheter-based closure of mitral paravalvular leak seems to be a safer treatment procedure than conventional re-do surgery, and the effectiveness of these procedures does not differ.

Safety, Efficacy and Long-Term Outcomes of Patients Treated with the Occlutech Paravalvular Leak Device for Significant Paravalvular Regurgitation.

Between December 2014 and March 2021, 144 patients with aortic (Ao) or mitral (Mi) paravalvular leaks (PVLs) were enrolled at 21 sites in 10 countries. Safety data were available for 137 patients, who were included in the safety analysis fraction (SAF), 93 patients with Mi PVLs and 44 patients with Ao PVLs. The full analysis set (FAS) comprised 112 patients with available stratum (aortic/mitral leak) as well as baseline (BL), 180-day or later assessments (2 years). Procedural success (implantation of the device with a proper closure of the PVL, defined as reduction in paravalvular regurgitation of ≥one grade as assessed by echocardiography post implantation) was achieved in 91.3% of FAS patients with Mi PVLs and in 90.0% of those with Ao PVLs. The proportion of patients suffering from significant or severe heart failure (HF), classified as New York Heart Association (NYHA) class III/IV, decreased from 80% at baseline to 14.1% at 2-year follow-up (FAS). The proportion of FAS patients needing hemolysis-related blood transfusion decreased from 35.5% to 3.8% and from 8.1% to 0% in Mi patients and Ao patients, respectively. In total, 35 serious adverse events (SAEs) were reported in 27 patients (19.7%) of the SAF population. The SAEs considered possibly or probably related to the device included device embolization (three patients), residual leak (two patients) and vascular complication (one patient). During follow-up, 12/137 (8.8%) patients died, but none of the deaths was considered to be device-related. Patients implanted with the Occlutech Paravalvular Leak Device (PLD) showed long-lasting improvements in clinical parameters, including NYHA class and a reduced dependency on hemolysis-related blood transfusions.

Technical Recommendations for Real-Time Echocardiography and Fluoroscopy Imaging Fusion in Catheter-Based Mitral Valve Paravalvular Leak and Other Procedures.

Widespread catheter-based interventions for structural heart disease have overtaken the treatment of paravalvular leaks (PVL). Multimodality imaging techniques play a crucial role in accurate diagnosis, procedure planning and performance. However, PVL closure is often technically challenging due to the complex anatomy of the defects and their relation to surrounding anatomical structures. The application of echocardiography and fluoroscopy imaging fusion (EFF) may simplify challenging imaginative three-dimensional reconstruction of the intracardiac anatomy and facilitate the procedure. To master new technology, personnel must make cognitive changes, overcome a learning curve, and obtain adequate theoretical knowledge. Main aim of this manuscript is to present basic recommendations for EFF application in practice, alongside, each scenario is supported by technically challenging clinical examples. We may conclude that our manuscript may provide useful information for physicians on EEF application in clinical practice.

Midterm results of transventricular mitral valve repair: Single-center experience.

OBJECTIVE: The study objective was to evaluate the midterm outcomes of transventricular mitral valve repair and its association with the initial anatomy of the mitral valve. METHODS: This nonrandomized observational study included 88 patients (mean age, 60 years; 69% were men) who underwent transventricular mitral valve repair for severe degenerative mitral regurgitation between 2011 and 2017. Mitral valve function was assessed by echocardiography at 1 and 6 months and annually after the procedure. According to the location of mitral valve pathology, all patients were stratified into 4 anatomic types (A, B, C, and D). Results were assessed using Kaplan-Meier method, mixed-effects continuation ratio model, and multivariable Cox regression. RESULTS: Median follow-up of 42 months (interquartile range, 27-55) was complete for 83 patients (94.3%). There were 3 late deaths: 2 cardiac and 1 noncardiac. Recurrent mitral regurgitation greater than 2+ was observed in 29 patients (33%), and 18 patients (20.5%) underwent repeat surgery. Device success was 82% in type A at 6 months and thereafter; 87%, 85%, and 75% at 6, 12, and 36 months in type B, respectively; and 53% at 1 month and 20% at 24 months in type C. Probability of postoperative mitral regurgitation progression was higher in patients with greater preoperative left ventricular end-diastolic diameter, type B pathology, and type C pathology (P < .05). Risk factors of mitral regurgitation recurrence included increased left ventricle size (hazard ratio, 1.11; 95% confidence interval, 1.04-1.20; P = .001) and type C pathology (hazard ratio, 5.99; 95% confidence interval, 1.87-19.21; P = .003). CONCLUSIONS: Initial acceptable mitral regurgitation reduction after transventricular mitral valve repair of isolated P2 prolapse was possible but found durable in only 82% at 3 years. Higher risk of mitral regurgitation recurrence occurred with complex degenerative pathology.

First in human experience with an epicardial beating heart device for secondary mitral regurgitation.

OBJECTIVE: We describe a novel, off-pump, epicardial implant that is intended to reshape both the mitral valve annulus and the left ventricle (LV) in those with secondary mitral regurgitation (MR). METHODS: Five patients underwent an epicardial implant with the Mitral Touch device (Mitre Medical Corp, Morgan Hill, Calif), during concomitant off-pump coronary artery bypass for secondary MR. The median age was 71.2 years; 4 patients had severe MR and 1 moderate. Patients were followed for 1 year with transthoracic echocardiography and computed tomography. Safety, cardiac remodeling, and MR were assessed by an independent core laboratory. RESULTS: One patient died within 30 days from nondevice-related organ failure and the remaining 4 survived through 1-year follow-up. Implant technical success was 100% and took an average of 52 minutes. Paired computed tomography showed mean left ventricular end-systolic volume remodeling at 1 and 12 months of -35% and -31%, respectively. They averaged left atrial end-systolic volume remodeling of -12% and -15% at 1 and 12 months. Right ventricular end-systolic volume changes of -19% and -8% and right atrial end-systolic volume remodeling of -5% and 1%, at the 1- and 12-month time points were noted. Regurgitant volume by transthoracic echocardiography decreased by 46% and 44% and the ejection fraction from 34.6% to 32.1% and 39.5%, at 1 and 12 months, respectively. There were no device-related complications reported to 1 year. CONCLUSIONS: The Epicardial Mitral Touch System for Mitral Regurgitation (ENRAPT-MR) study demonstrates a first-in-man, off-pump, epicardial repair of secondary MR. Procedural safety and geometric correction of the mitral valve apparatus and LV was achieved. Further studies in the United States are underway.

Mitral valve repair with a device for artificial chordal implantation at 2 years.

Objectives: This study examines the early and midterm safety, efficacy, and durability of mitral valve repair for primary mitral regurgitation (MR) using the ChordArt device (CoreMedic) for chordal replacement. Methods: Five patients with symptomatic severe primary degenerative MR due to isolated central posterior leaflet prolapse/flail were treated with the ChordArt device in a transseptal surgical approach and followed prospectively with periodical clinical and echocardiographic assessments for 2 years. Results: Reduction of MR immediately after the implantation of artificial chords was achieved in all patients showing no or trace MR (<1+/4+). In all patients, MR <1+ was maintained during 24 months of follow-up. No dehiscence, detachment, or dislocation of the implanted ChordArt devices was observed. Transthoracic echocardiography showed that left ventricle end diastolic diameter significantly decreased during the whole follow-up period in comparison to baseline condition, especially at discharge and 1-month follow-up. Left ventricle end systolic diameter also significantly decreased during the whole follow-up period in comparison to baseline condition. Left atrial volume significantly decreased during the follow-up period in comparison to discharge. No major adverse events, as defined per protocol, were observed during the intervention or during the follow-up period. Conclusions: The ChordArt device allows successful treatment of primary degenerative MR due to posterior mitral leaflet prolapse or flail, with a good safety profile and promising immediate clinical and echocardiographic benefits that are confirmed up to 24 months.

Rescue transventricular off-pump mitral valve repair with artificial neochords for acute mitral regurgitation due to postinfarction papillary muscle rupture.

BACKGROUND: We report 3 cases of rescue transventricular off-pump mitral valve (MV) repair in high-risk patients with acute mitral regurgitation (MR) due to post-myocardial infarction (MI) papillary muscle rupture (PMR). METHODS: The 3 patients presented with acute inferior ST elevation myocardial infarction, cardiogenic shock, and pulmonary edema. Their preoperative peak troponin I levels were 1909 ng/L, 16,963 ng/L, and 8299 ng/L. All 3 patients underwent successful percutaneous intervention to the culprit coronary artery, and antiplatelet therapy was initiated. All patients required inotropic support and had an intra-aortic balloon pump inserted preoperatively. Transesophageal echocardiography (TEE) demonstrated severe eccentric MR due to the leaflet prolapse secondary to PMR. The patients' estimated EuroSCORE II scores were 16.03%, 16.68%, and 7.81%, and their Society of Thoracic Surgeons scores were 14.77%, 18.24%, and 9.8%, respectively. All 3 patients underwent urgent transventricular off-pump MV repair using artificial chords, with 2 or 3 three neochords implanted. The duration of operation was <2 hours, and intraoperative and postoperative drainage was minimal in all cases. MV function was assessed by qualitative and semiquantitative TEE. RESULTS: Intraoperative MR reduction to a mild level was achieved in all 3 patients. All patients had moderate MR at discharge, likely due to left ventricular remodeling. Severe MR recurred in all patients, at 5, 4, and 2 months of follow-up, respectively. All 3 patients underwent an elective MV reoperation via conventional approach. CONCLUSIONS: Off-pump transventricular MV repair may offer a safe and feasible alternative to stabilize high-risk patients with acute MR due to post-MI PMR. Although early MR recurrence is concerning, urgent transventricular MV repair may serve as a bridge to conventional surgery in such unstable patients.

Automated Implantation of Artificial Mitral Chords: Preliminary Results From the Feasibility Trial.

PURPOSE: A novel chordal system enables automated implantation of artificial mitral chords to treat mitral regurgitation (MR). This article summarizes the first-in-man initial clinical results. DESCRIPTION: The CHAGALL (CHordArt system study for the treatment of mitral ReGurgitAtion due to leaflet proLapse or fLail) trial is a single arm, multicenter, prospective study to test the feasibility of this system for mitral repair. The interim clinical and echocardiographic results of the first 5 patients with a 12-month follow-up are presented. EVALUATION: Five patients (mean age, 64 years) with severe MR received implantation of neochords with this device under cardiopulmonary bypass. Technical success was obtained in all patients. Transesophageal echocardiogram showed either no or trace residual MR (<1+/4+) after repair. Survival at 30 days was 100%, and no device-related complication occurred. Reduction of MR was sustained up to 12 months. CONCLUSIONS: This novel chordal system is promising because it greatly facilitates the deployment of neochords to repair the mitral valve. Results at 12 months are encouraging. The device is currently under development for transcatheter approach.

Midterm procedural and clinical outcomes of percutaneous paravalvular leak closure with the Occlutech Paravalvular Leak Device.

AIMS: The aim of this study was to assess the efficacy and safety of the Occlutech Paravalvular Leak Device (PLD) for the percutaneous closure of paravalvular leaks (PVL). METHODS AND RESULTS: Patients with PVL were enrolled at 21 sites from nine countries. Indications for PVL closure were heart failure and/or haemolytic anaemia. Endpoint measures were changes in PV regurgitation grade, NYHA class and requirement for haemolysis-related transfusion. One-hundred and thirty-six patients with mitral (67.6%) or aortic (32.4%) leaks were included (mean age 66.7 years, 58% male); 31% had multiple PVLs. The proportion of patients with NYHA Class III/IV decreased from 77.3% at baseline to 16.9% at latest follow-up. The proportion of patients with need for haemolysis-related blood transfusion decreased from 36.8% to 5.9% and from 8.3% to 0% for ML patients and AL patients, respectively. All-cause mortality was 7.4%. Complications included interference with valve leaflets (0.7%), transient device embolisation (percutaneously solved) (0.7%), late device embolisation (0.7%), recurrent haemolytic anaemia (2.2%), new-onset haemolytic anaemia (0.7%), valve surgery (2.2%), need for repeat closure (0.7%), complications at femoral puncture site (0.7%) and arrhythmias requiring treatment (4.4%). CONCLUSIONS: PVL closure with the Occlutech PLD demonstrated a high success rate associated with significant clinical improvement and a relatively low rate of serious complications. Visual summary. PVL characteristics, leak approach, device types and midterm procedural and clinical outcomes of percutaneous paravalvular leak closure with the Occlutech® PLD. Transcatheter PVL closure with the specifically designed PLD was demonstrated to be effective with a relatively low rate of major complications. Procedural success for ML and AL closure was high with a low rate of residual or recurrent leaks. Significant improvement of NYHA class, and reduction of haemolytic anaemia and transfusion dependency were achieved.

Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review.

BACKGROUND: We performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease. METHODS: A PubMed literature search for 'cor triatriatum sinistrum' published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared. RESULTS: A total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30-60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28-52) vs 50 years (IQR, 32-64), p=0.003). Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004).A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001). CONCLUSION: The natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane.

Comparison of early postoperative results between conventional and transapical mitral valve repair.

INTRODUCTION: Conventional mitral valve repair (CMVR) is well-established, safe and effective treatment for degenerative mitral regurgitation (MR). Transapical off-pump implantation of artificial chordae (TA) has been introduced into practice and gained interest among surgeons. However, there are no publications comparing the results between TA and CMVR. AIM: To compare early postoperative outcomes of CMVR with TA in patients with degenerative MR. MATERIAL AND METHODS: This was a retrospective cohort study. A total of 169 patients who underwent mitral valve repair between 2011 and 2018 were included in this analysis. Patients were divided into two groups: the TA group, n = 78 and CMVR group, n = 91. The groups were compared for early postoperative outcomes. RESULTS: Patients in the TA group were younger, 54.2 ±11.1 vs. 59.5 ±12.8 years (p = 0.005). Patients in the CMVR group had more complicated postoperative course with higher incidence of blood transfusion (42.9% vs. 7.8%, p = 0.001), atrial fibrillation (25.3% vs. 11.7%, p = 0.031), renal insufficiency (15.4% vs. 2.6%, p = 0.007) and stroke (2.1% vs. 0%). In the early postoperative period, one patient died in the TA group, and there were no deaths in the CMVR group (p = 0.277). Residual moderate to severe mitral regurgitation was present in nine (11.5%) TA patients, while none of the patients in the CMVR group had moderate or a higher degree of residual regurgitation (p = 0.001). CONCLUSIONS: Off-pump transapical MV repair is a feasible and safe procedure with low postoperative morbidity rates. Higher rates of mitral regurgitation reoccurrence would require a careful and thorough selection of the patients suitable for the TA approach.

Retrospective analysis of single-center early and midterm results of transapical catheter-based mitral paravalvular leak closure with a purpose-specific device.

INTRODUCTION: Due to the recent lack of definitions to establish the severity of paravalvular leak (PVL) and endpoints for its treatment, the effectiveness and safety of a new device for PVL closure have not been comprehensively analyzed. AIM: To analyze a single center's experience of mitral PVL closure in a surgical transapical catheter-based fashion with a purpose-specific device. MATERIAL AND METHODS: This is a retrospective cohort study of patients following transapical catheter-based mitral PVL closure with a purpose-specific device. Data were analyzed at baseline, perioperatively, at discharge, at six months and annually after the procedure. RESULTS: Nineteen patients underwent surgical transapical catheter-based mitral PVL closure with the Occlutech PLD Occluder. Mean follow-up time was 20 ±7 (range: 9-33) months. The patients' mean age was 64 ±7 years, and 11 (58%) were male. Technical, device and individual patient success at follow-up was achieved in 18 (95%), 16 (84%) and 16 (84%) patients respectively. Median intensive therapy unit stay was one day (1-4) and mean hospital stay was 11 ±4 days. A reduction of paravalvular regurgitation to a mild or lesser degree was achieved in 18 (95%) patients. There were no strokes or myocardial infarctions at follow-up. There were no deaths at 30 days after the procedure. One (5%) patient expired due to progression of heart failure 12 months after surgery. None of the patients required immediate conversion to full sternotomy. CONCLUSIONS: Surgical transapical catheter-based mitral PVL closure with the Occlutech PLD Occluder is a safe and clinically effective treatment.