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The ValveClamp system is a novel edge-to-edge mitral valve repair system designed for the ease of operation. We report the outcomes of our initial experience of treating functional mitral regurgitation (MR) with the ValveClamp system.The subjects of this study were patients with symptomatic functional MR despite standard medical therapy, who were treated with transapical ValveClamp implantation. The patients were divided into an atrial functional mitral regurgitation (AFMR) group and a ventricular functional mitral regurgitation (VFMR) group. Clinical and echocardiographic outcomes were evaluated at baseline and then at the 3-month follow up.Twelve patients, with a median age of 71 years (range 65-78 years), were assigned to the AFMR group (n = 5) or the VFMR group (n = 7). The device implantation rate was 100%, and 10 (83.3%) patients required implantation of only one clamp. The catheter time was less than 10 min in half of the patients, the fastest time being 5 min. There were no procedure-related complications. At the 3-month follow up, all patients were free from all-cause mortality, surgery, and rehospitalization. MR improved to ≤ 2 + in all 12 patients with MR grade 3 + or 4 + at baseline, (100%) and to ≤ 1 + in 9 of these patients (75%), with a low-pressure gradient. The left atrial diameter and the left ventricular end diastolic diameter decreased significantly in both the AFMR and VFMR groups. The left ventricular eject fraction at the 3-month follow up showed a rising trend in both the AFMR and VFMR groups, whereas PASP decreased remarkably. All 12 patients with baseline NYHA functional class III/IV (100%) showed improvement of at least 1 class, and 2 of these patients (16.7%) showed improvement of at least 2 classes.The ValveClamp system is simple and effective for transapical transcatheter edge to edge repair in patients with functional MR.

Gradually, more attention has been paid to tricuspid regurgitation. Formerly the "forgotten valvular heart disease," it has been recognized as having high incidence and adverse prognosis if untreated. However, isolated tricuspid valve surgery carries substantial risk and is thus not recommended. The K-Clip transcatheter tricuspid annuloplasty system has recently emerged as a minimally invasive therapeutic option that may help address these unmet clinical needs. The K-Clip technology simulates the Kay's procedure, which reduces the dimensions of the tricuspid annulus by folding and clamping the tricuspid annulus issue with a rigid clamping device. The feasibility of the K-Clip technology was investigated in this study, and annulus area reduction and declining tricuspid regurgitation grades were noted following the procedure. The findings of this study suggest that the K-Clip system may potentially be applicable as a novel transcatheter tricuspid annuloplasty device for the treatment of severe tricuspid in humans.

Background The purpose of the study was to determine the association between vena contracta area (VCA) and secondary leaflet tethering among mitral valve prolapse (MVP) patients, and thus to further identify and characterize an MVP with pathological leaflet tethering (MVPt+) phenotype. Methods We prospectively evaluated 94 consecutive MVP patients with significant mitral regurgitation (MR) and 20 healthy controls. MVPt+ group was defined as tenting volume index (TVi) > 0.7 ml/m . The three-dimensional (3D) geometry of mitral valve apparatus and VCA was measured with dedicated quantification software. Results Of the 94 patients with MVP and significant MR, 31 patients showed a TVi > 0.7 ml/m and entered the MVP with leaflet tethering (MVPt+) group. In stepwise multivariate analysis, only prolapse volume index and TVi was independently associated with 3D VCA. Apart from marked left ventricular and annular enlargement, MVPt+ group presented significantly higher frequency of leaflet flail, greater VCA, elevated plasma levels of NT-proBNP and sPAP. ROC curve revealed that occurrence of leaflet tethering is associated with a VCA ≥0.55 cm in MVP patients. Conclusions Secondary leaflet tethering is a significant mechanism behind severe degenerative mitral regurgitation, resulting an MVPt+ phenotype featuring more advanced morphological and hemodynamical characteristics .

BACKGROUND At present, little research concerning the assessment of left atrial (LA) dysfunction in patients with obstructive sleep apnea (OSA) using a combined assessment by speckle tracking (STE) and real-time three-dimensional echocardiography (RT3DE) is available. The objective of this study was to evaluate the LA volume and function by STE and RT3DE in patients with OSA. METHODS In our cohort study, ninety-two OSA patients and 50 healthy individuals were enrolled. According to the apnea hypopnea index (AHI), patients (AHI >15/h) classified as having moderate and severe OSA were included. The patients were divided into 2 subgroups according to the left ventricular mass index (LVMI): the left ventricular hypertrophy (LVH) group in which patients had LVH (n=30), and the nonLVH group in which patients did not have LVH (n=62). All subjects underwent LA function assessment by conventional techniques and the combination of STE and RT3DE. RESULTS OSA patients showed impaired LA global longitudinal strain during early diastole (LA S-E) and systole (LA S-S) but increased LA global longitudinal strain during late diastole (LA S-A) compared with controls (all P

Objectives To evaluate intraprocedural assessments using transesophageal echocardiography (TEE) and fluoroscopy during left atrial appendage occlusion (LAAO) with the WATCHMAN device. Method A total of 208 patients with non-valvular atrial fibrillation (AF) undergoing LAAO were included in this study[101 standard procedures (retrospective cohort) and 107 with fluoroscopy-alone approach (prospective cohort). Individual device position, anchoring, compression and peri-device leak (PDL) were successively analyzed based on TEE and fluoroscopy in the retrospective cohort to summarize detailed fluoroscopic assessments for prospective application. Clinical outcomes were assessed between two cohorts. Results For retrospective cohort, TEE and fluoroscopy agreed on device position and anchoring. Compression upon fluoroscopy was well correlated with 2D-TEE (r = 0.908) and the difference in overall detection of PDL was not statistically significant between two imaging modalities (p = 0.304). For the prospective cohort with fluoroscopy-guidance alone, implantation success was similar to that of the retrospective cohort (98.13% vs 100%, p = 0.498). The incidence rate of major clinical adverse events was relatively higher in prospective cohort during hospitalization and follow-up but did not reach significant difference (5.61% vs 1.98%; 0.99% vs 0.93%, p > 0.05). Moreover, the prospective group presented with shorter procedural duration, shorter in-hospital stay and lower total hospitalization cost than retrospective group. Conclusion LAAO performed by experienced operators in large volume centers is feasible under fluoroscopy guidance. However, there is still a trend in favor of TEE for greater procedural safety and more complete LAA seal. We suggest that this minimalist approach could be proposed in cases with contraindication to general anesthesia and/or TEE.

Objectives: This study aimed to diversify the spectrum of PRKAG2 variants and explore its clinical features in a Chinese Han population with hypertrophic cardiomyopathy (HCM). Methods: Whole-exome sequencing was performed on 200 patients diagnosed with HCM, and four causative PRKAG2 variants were identified in the probands and their relatives using Sanger sequencing. Their clinical manifestations, laboratory examinations, therapeutic methods, and outcomes were documented and analyzed. Results: Four variants were identified in six probands and seven of their relatives. Left ventricular hypertrophy was present in all probands. Five probands had sinus bradycardia, three had implanted pacemakers (PM), one developed heart failure, two had ventricular preexcitation, and one had atrial fibrillation. Conclusions: PRKAG2 cardiac syndrome (PCS) is a rare autosomal dominant disease characterized by ventricular hypertrophy, preexcitation, and progressive conduction defects, resulting in a high incidence of PM implantation. Genetic testing provides robust information for distinguishing PCS from sarcomeric HCM, which will be beneficial in guiding therapy and improving prognosis.

The aim of this study was to investigate the utility of perimeter-derived diameter (PDD) measured by three-dimensional (3D) transesophageal echocardiography (TEE) in predicting the size of left atrial appendage (LAA) occluder.Left atrial appendage landing zone diameter (LZD) was measured by two-dimensional (2D) TEE, 3DTEE, and digital subtraction angiography (DSA) as LZD-2Dmax, LZD-2Dmean, LZD-3Dmax, LZD-3Dmean, LZD-PDD, LZD-DSAmax, respectively, before and during transcatheter LAA closure with Watchman devices in 100 patients. A difference of one or more device size intervals between the predicted size and the size actually implanted was defined as mismatching. Seventy-eight patients were followed up by TEE to obtain occluder compression ratio. The correlation between LZD and the final implanted occluder size was 0.559, 0.641, 0.754, 0.760, 0.782, and 0.848 for LZD-2Dmax, LZD-2Dmean, LZD-3Dmax, LZD-3Dmean, LZD-PDD and LZD-DSAmax, respectively (P .001). Matching ratio between the size predicted by retrospective measurements of LZD and the device size actually implanted was 65%, 57%, 66%, 63%, 70%, and 83% for LZD-2Dmax, LZD-2Dmean, LZD-3Dmax, LZD-3Dmean, LZD-PDD and LZD-DSAmax, respectively. There was no significant difference in LZD value, matching ratio, and compression ratio between the patients with eccentric and noneccentric LAA landing zone (P .05). Compression ratio of the mismatching subjects was higher than that in the matching subjects when evaluated by LZD-2Dmean, LZD-3Dmean, and LZD-PDD (P .05).Landing zone diameter derived from LAA perimeter measured by preprocedure 3DTEE showed reference value for LAA occluder size selection, providing superior correlation and matching ratio with the final implanted size and indicating the adjustment of oversizing.

Background To evaluate the detailed dynamic change of left ventricular diastolic function (LVDF) by echocardiography in aortic stenosis (AS) patients receiving transcatheter aortic valve implantation (TAVI) and compare LVDF classification according to 2009 ASE/EAE and 2016 ASE/EACVI recommendations. Methods Thirty-five AS patients receiving TAVI underwent echocardiography the day before operation (PRE), on the third day (3D), in the first-month (1 M) and the six-month (6 M) after TAVI. LVDF was analyzed using 2D and doppler imaging to get parameters including E/A, E/e’, isovolumic relaxation time (IVRT), deceleration time, LA area, LA volume index (LAVI) and systolic tricuspid regurgitation velocity (TR). LVDF classification was evaluated four times for each patient according to 2009 and 2016 recommendations respectively and the results were compared. Results The decrease of IVRT and TR occurred immediately post surgery up to 1-month. Improvement of E/e’ occurred late from 3-day to 1-month. LA area and LAVI decreased continuously shortly after operation till 6-month. Forty-four percent (62/140) by 2009 recommendations were reclassified with different grades when using 2016 guidelines. Comparing PRE and 6 M, with 2009 guidelines, 19 patients improved 1 grade, 8 patients improved 2 grades; with 2016 guidelines, 9 patients improved 1 grade, 13 patients improved 2 grades, 1 patient improved 3 grades. Conclusions The conventional 2D echocardiography could effectively reflect variation process of LVDF in AS patients after TAVI. For LVDD classification, obvious differences resulted by the 2009 and updated recommendations were found, and more patients can be regarded as benefiting from TAVI by 2016. recommendations.

The ValveClamp system is a novel edge-to-edge mitral valve repair system that is designed for ease of operation. We aimed to report the 1-year outcomes of the early feasibility study of this system.Patients with severe degenerative mitral regurgitation (MR) at higher surgical risk and who received transapical ValveClamp implantation were followed for 1 year for clinical and echocardiographic outcomes.Twelve patients (mean age, 76.5 ± 6.3 years; mean Society of Thoracic Surgery score, 6.9 ± 1.9%) were enrolled at three sites in China. At 1 year, no patient died, received reoperation, or had long-term complications. Of the 12 patients with MR of 3+ or 4+ at baseline, 11 patients (91.67%) remained with MR ≤ 2+ at 1 year, and no patient had mitral stenosis. Significant reductions in maximum MR area (from 15.1 ± 6.51 cm

Previous studies reported a controversial left ventricular (LV) function impairment and pathophysiology in patients with coronary slow flow (CSF). Greater arterial load has been shown to increase aortic impedance and endothelial shear stress, potentially affecting coronary anatomy and function. We investigated LV systolic function by a new layer-specific strain technology and assessed the association between pulsatile arterial load and contractility.A total of 70 patients with CSF and 50 controls with normal coronary angiography were included in the study. Layer-specific longitudinal and circumferential strains were assessed from endocardium, mid-myocardium, and epicardium (global longitudinal strain (GLS)-endo, GLS-mid, GLS-epi and GCS-endo, GCS-mid, GCS-epi) by two-dimensional speckle tracking imaging (2D-STI). Pulsatile arterial load was estimated by indexed arterial compliance (ACI). Layer-specific GLS showed a decreasing gradient from the endocardium to the epicardium in both the controls and CSF group. GLS-endo and GLS-mid in the CSF group were significantly lower than the control group (all P < 0.05). Layer-specific longitudinal strain showed a good correlation with the number of affected coronary arteries (all P < 0.05) and the mean thrombolysis in the myocardial infarction frame count (TFC) (all P < 0.05). ACI was lower in patients with CSF (P = 0.005), and ACI was correlated negatively with layer-specific GLS (all P < 0.05).Layer-specific evaluation of the LV provides an understanding of the layer-specific properties of the LV wall and the possible process of the LV impairment in patients with CSF. Greater pulsatile arterial load, as manifested by a lower ACI, is coupled with worse LV longitudinal function in patients with CSF.

Objectives: The aim of this study was to investigate the influence of right ventricular (RV) dysfunction on the response to cardiac resynchronization therapy (CRT) and the impact of CRT on RV function in a beagle model of heart failure (HF). Methods: Twenty-one beagles were implanted with transvenous cardiac pacemakers and underwent rapid RV pacing for 2 weeks at 260 bpm to induce HF. Dogs were subsequently divided into three groups that were either treated with bi-ventricular pacing (CRT group) or untreated (control group and HF group). Echocardiographic images were acquired at baseline, before CRT, and 4 weeks after CRT. Results: Left ventricular systolic function and synchrony (left ventricular internal dimensions, left ventricular end systolic volume [LVESV], left ventricular ejection fraction [LVEF], septal-to-posterior wall motion delay [SPWMD], and aorta pre-ejection interval and the pulmonary artery pre-ejection interval [|APEI-PPEI|]) were significantly improved in the CRT group compared with the HF group. RV myocardial performance index (MPI) and pulmonary artery systolic pressure deteriorated with left ventricular dysfunction and improved after CRT. RV outflow ESV, standard deviation of time to minimum systolic volume (Tmsv), and Tmsv% decreased in the CRT group compared with the HF group. LVESV, LVEF, SPWMD, and |APEI-PPEI| were significantly different between responders and nonresponders, while there was no difference about RV functional parameters. Conclusions: RV function deteriorated with left ventricular dysfunction and improved after CRT. RV function did not significantly influence the response to CRT.

Objectives The present study was performed to evaluate the feasibility and safety of the ValveClamp system for transcatheter edge-to-edge mitral valve repair in a porcine model. Background The ValveClamp system is a novel, transapically delivered edge-to-edge mitral valve repair system designed for ease of operation. Methods Thirteen young adult swine were enrolled in the study. The procedure was performed via the transapical approach under epicardial echocardiography guidance. Results The acute procedure success rate was 92.3% (12/13). The catheter manipulation time was just 18.5 ± 8.2 min. Four pigs were explanted on the 14th day after the procedure. One pig died at the 20th day because of pneumonia. The other eight pigs lived to the study endpoint (140 days). No pig had severe mitral valve dysfunction. The gross observation showed that the clamp device was securely attached and created a double-orifice mitral valve in all pigs except the failed one. In the eight pigs living to the endpoint, the clamp device was noted to be completely endothelialized. None of the 13 pigs developed infective endocarditis, thrombosis, thromboembolism, or valve impairment. In two pigs for which mitral regurgitation (MR) was created, the degree of MR was reduced from severe to trace after the device implantation. Conclusions It was feasible and safe to use ValveClamp to perform a catheter-mediated edge-to-edge mitral valve repair in a porcine model. This system is potentially applicable as a novel user-friendly system for the treatment of MR.

Background Prior studies have suggested that patients with atrial fibrillation (AF) referred for left atrial appendage occlusion (LAAO) are confronted with considerable risk of periprocedural thromboembolism and hemmorhagic events. The purpose of this study was to evaluate the safety and feasibility of bivalirudin during LAAO. Methods This retrospective, observational study included 420 AF patients who were evaluated as being at high risk of stroke or bleeding and indicated for LAAO at our center between June 2018 and June 2019 (158 with bivalirudin and 262 with heparin). The primary outcome was the incidence of any bleeding within 48 hours of LAAO. Secondary outcomes were major adverse cardiac events (MACE) between 48 hours and 60 days post-procedure and overall bleeding events during follow up. Results No significant difference was observed between bivalirudin and heparin for major periprocedural bleeding (1.27% for bivalirudin vs. 2.29% for heparin, P=0.716) or minor bleeding (1.27% vs. 1.15%, P>0.9). At 48 hours post-procedure, strokes occurred at a rate of 0.63% in the bivalirudin group and 1.15% in the heparin group (P>0.9), and one case treated with bivalirudin developed systemic embolization. At 60 days, the rates of MACE (1.90% vs. 2.29%, P>0.9), a device-related thrombus (DRT) (1.27% vs. 1.52%, P>0.9), and overall bleeding events (5.06% vs. 4.96%, P=0.963) were comparable between the 2 cohorts. Upon Kaplan-Meier survival analysis, early safety during the 60-day follow-up was 93.67% in the bivalirudin group and 91.60% in the heparin group (P=0.570). Conclusions Bivalirudin has a comparable safety and efficacy profile to heparin as an intraprocedural anticoagulant, but currently, it should still be reserved for patients in which heparin is contraindicated.

MitraClip edge-to-edge (E2E) repair system is the only transcatheter device recommended in the current guidelines for treating mitral regurgitation (MR). The percutaneous femoral venous transseptal access of MitraClip requires a complex steerable delivery system and may thus be technically complex to optimally position and deploy the clip onto the mitral valve. A transapical approach for E2E repair has been devised to treat MR for the ease of operation (ValveClamp system, Hanyu Medical Technology, Shanghai). The first-in-human study of ValveClamp has demonstrated its early feasibility and effectiveness for the treatment of patients with degenerative MR. Transesophageal echocardiography (TEE) is the only imaging modality required for intraoperative guidance of ValveClamp implantation. Successful implantation depends on accurate localization and orientation of the clamp and efficient intraoperative communication between the echocardiographer and the intervention team. Thus, the focus of this review is on elaborating how two-dimensional (2D) and three-dimensional (3D) TEE are used in clinical practice to guide ValveClamp implantation and it may facilitate the understanding of simplicity and safety of this novel procedure. We also describe the implementation of several novel advancements in 3D TEE imaging, which improve the confidence of image interpretation for intraoperative guidance and expedite implantation times.

Background: Grade 2+ residual mitral regurgitation (MR 2+) is associated with the recurrence of MR and a lower survival rate in interventional mitral valve (MV) edge-to-edge (EE) repair. We sought to determine the MV anatomic factors affecting residual MR 2+ during interventional EE repair with the ValveClamp system in patients with degenerative MR (DMR).Methods: In this multicenter study, 62 patients with significant (grade 3+ to 4+) DMR underwent ValveClamp implantation across eight centers from July 2018 to December 2019. Patient clinical, anatomical, and procedural characteristics were prospectively collected and retrospectively analyzed.Results: A single clamp was implanted in 59 patients, and two clamps were implanted in three patients. Residual MR 2+ was found in 14 patients (22.6%) immediately after the ValveClamp procedure. Patients with residual MR 2+ showed significantly larger preoperative tenting sizes and annular dimensions than the residual MR ≤1+ group. Multivariate analysis identified tenting volume as the major determinant of residual MR 2+ after ValveClamp procedures (odds ratio, 1.410 per 0.1-mL/m2 increase; 95% confidence interval, 1.167–1.705; P < 0.001). Receiver operating characteristic curves identified a tenting volume index ≥0.82 mL/m2 as the optimal cutoff point to predict residual MR 2+ (area under curve, 0.84). Patients with a tenting volume index ≥0.82 mL/m2 were more likely to develop recurrent 3+ MR or undergo MV surgery during short-term follow-up (P < 0.001).Conclusions: Preoperative assessment of the tenting volume index will help to predict intraoperative residual MR 2+ in patients with DMR receiving EE-based interventional repair. Improvements in the interventional strategy are warranted for sustained MR reduction in patients with DMR with unfavorable anatomy.

BACKGROUND: A new technique has been developed for treating mitral regurgitation (MR) via a transapical approach, which encompasses an easy-to-use leaflet clamp and a smaller-sized delivery system (14F-16F). OBJECTIVES: We aimed to evaluate the effectiveness of this device in a porcine model of acute MR. METHODS: Acute MR was induced in 36 anesthetized porcine subjects by severing the chordae supporting the corresponding segment of the leaflet. The ValveClamp system was then transapically implanted on the prolapsing segment under epicardial echocardiographic guidance. Echocardiographic assessments were performed before and after the transapical interventions. All of the animals were killed 30 days after the procedure to verify the proper location of the implanted devices. RESULTS: Epicardial echocardiography revealed severe MR (n = 26) or moderate to severe MR (n = 10) in the pig model of acute MR. Overt MR reduction was observed following the procedure through echocardiography; residual MR was absent in 10 cases, mild in 17 cases, and moderate in 9 cases. There was no evidence of mitral stenosis at the end of the procedure in terms of mitral valve area and mitral valve pressure gradient. Autopsy demonstrated that all ValveClamp devices were precisely placed to clamp the prolapsing segment of the mitral valve. CONCLUSIONS: Transapical implantation of the ValveClamp device under epicardial echocardiographic guidance was effective and safe in reducing acutely induced MR in this pig model. It is potentially applicable as a novel user-friendly transcatheter edge-to-edge mitral valve repair device for the treatment of MR in humans.

Background Gene mutations have been implicated in DCM. However, due to the difficulty of clinical genetic diagnosis, more causal genes potentially related to DCM remain to be discovered. Methods We screened for gene mutations in more than 400 cases from families with hereditary cardiovascular disease using whole-exome sequencing. Then we validated biological functions of CHMP4C mutations in zebrafish models. To further assess the mechanism of CHMP4C mutations, we evaluated the potential signaling pathway in the cells. Results We identification of CHMP4C variants that segregated with DCM variants in four families from a total of 411 families via whole-exome sequencing. We further validate the function of CHMP4C in heart function in zebrafish models and found that over-expression of CHMP4C variants in zebrafish resulted in cardiac malformation, pericardial edema and increased heart rate, consistent with CHMP4C mutation-associated findings in DCM patients. Furthermore, we found that mutations in CHMP4C impaired autophagy and activated apoptosis in HEK293T cells, suggesting that the molecular mechanism of CHMP4C is involved in heart development. Conclusions CHMP4C is a novel candidate gene for DCM and may play a critical role in cardiac development by regulating autophagy. Funding Acknowledgement Type of funding source: None

Background The quantification of tricuspid regurgitation(TR) using three-dimensional(3D) proximal isovelocity surface area (PISA) derived effective regurgitant orifice area (EROA) is feasible in functional TR. The aim of our study was to explore the diagnostic accuracy and utility of 3D PISA EROA in a larger population of different etiologies. Methods One hundred and seven patients with confirmed TR underwent 2D and 3D transthoracic echocardiography (TTE). 3D PISA EROA was calculated and EROA derived from 3D regurgitant volume (Rvol) was used as the reference. Results 3D PISA EROA showed better correlation in primary TR than in functional TR(r = 0.897, P 2 vs. 0.41 cm2). The chi-square value for incorporated clinical symptoms, positive echocardiographic results and 3D PISA EROA to grade severe TR was higher than only included clinical symptoms or incorporated clinical symptoms and positive echocardiographic results (chi-square value 137.233, P Conclusion TR quantification using 3D PISA EROA is feasible and accurate under different etiologies. It has incremental diagnostic value for evaluating severe TR.

OBJECTIVES We sought to assess the acute intraprocedural effects of the ValveClamp system in DMR patients on the mitral valve (MV) three-dimensional (3D) geometry and the association of these effects with mitral regurgitation (MR) reduction. BACKGROUND Few data are available about the specific impact of transcatheter edge-to-edge repair in patients with degenerative mitral regurgitation (DMR). METHODS Thirty-five symptomatic patients (age 74.26 ± 6.61 years) with Grade 3 to 4+ degenerative MR underwent 3D transoesophageal echocardiography (TEE) during ValveClamp implantation. Volumetric data sets were retrospectively analyzed using mitral valve quantitative 3D modeling software. RESULTS Mitral valve annular anterior-posterior (AP) diameter decreased from 33.24 ± 4.03 to 31.12 ± 3.66 mm (p

Background Previous studies found different cutoffs of vena contracta area (VCA) to define severe tricuspid regurgitation (TR). The aim of this study was to investigate the factors associated with such variability by comparing technical variables and vendors. Methods Sixty-nine patients with scheduled tricuspid surgery were included in this prospective study. For each patient, TR data sets were obtained on three-dimensional color Doppler transthoracic echocardiography on at least two of three systems: GE Vivid E95 (n = 39), Siemens SC2000 Prime (n = 64), and Philips EPIQ 7C (n = 60). VCA was measured using default settings or with color baseline shifted on all three platforms and with minimal color gain (10%–20%) on the GE platform. Results Color gain reduction and baseline shift caused significant change sin VCA measurement (−46% and 10%, respectively). Intervendor comparison exhibited wide limits of agreement (narrowest range, −74% to 167%), with either default or optimized settings. Different technical settings, platforms, and reference methods all produced different VCA cutoffs for severe TR. Conclusions VCA measurement in TR is sensitive to technical factors and demonstrates intervendor variability. Technical variables in VCA measurement should be reported in detail to allow comparison among research studies. The same vendor and settings should be used for longitudinal analysis of TR VCA in the same patient in multivendor echocardiography laboratories.

Background: Gene mutations have been implicated in DCM. However, due to the difficulty of clinical genetic diagnosis, additional causal genes potentially related to DCM remain to be discovered. Methods: We screened for gene mutations in more than 400 cases from families with hereditary cardiovascular disease using whole-exome sequencing and then validated the biological functions of CHMP4C mutations in zebrafish models. To further assess the mechanism of CHMP4C mutations, we determined the potential signaling pathway in a cell line. Results: We identified via whole-exome sequencing CHMP4C variants that segregated with DCM in four families among a total of 411 families. We further validated the function of CHMP4C in heart function in zebrafish models and found that overexpression of CHMP4C variants resulted in cardiac malformation, pericardial edema and an increased heart rate, consistent with CHMP4C mutation-associated findings in DCM patients. Furthermore, mutations in CHMP4C impaired autophagy and activated apoptosis in HEK293T cells, suggesting that the molecular mechanism of CHMP4C is involved in heart development. Conclusions: CHMP4C is a novel candidate gene causing DCM and may play a critical role in cardiac development by regulating autophagy. Funding Statement: This work was supported by NSFC Grants (# 81671685, #31625013, and #91732302); Shanghai Science and Technology Committee Foundation (#17411954400); Shanghai Brain-Intelligence Project from STCSM (16JC1420501); Strategic Priority Research Program of the Chinese Academy of Sciences (XDBS01060200); Program of Shanghai Academic Research Leader, the Open Large Infrastructure Research of Chinese Academy of Sciences; and the Shanghai Municipal Science and Technology Major Project (#2018SHZDZX05). Declaration of Interests: None. Ethics Approval Statement: The study was approved by the Ethics Committee of Zhongshan Hospital, Fudan University (No. B2016-016(2)R).

Background Diastolic mitral regurgitation (DMR) is a type of functional mitral regurgitation. Its occurrence in the diastolic phase of cardiac cycle renders DMR an easily ignored entity. Confusing it with systolic mitral regurgitation occasionally happens. The reversal of left atrioventricular pressure gradient during diastole and the incomplete closure of mitral valve are the essential conditions for DMR. Diastolic mitral regurgitation develops under various situations, where the mechanisms of diastolic reversal of left atrioventricular pressure gradient differ. Case summary Patient 1 was a 50-year-old man diagnosed with 2:1 second-degree atrioventricular block (AVB). Patient 2 was a 70-year-old man diagnosed with first-degree AVB. Patient 3 was a 66-year-old man diagnosed with atrial fibrillation with long intermission and occasional atrial flutter with unequal conduction. Patient 4 was a 54-year-old woman diagnosed with dilated cardiomyopathy with complete left bundle branch block. Patient 5 was a 36-year-old man diagnosed with severe acute aortic regurgitation secondary to subacute bacterial endocarditis. Discussion Although the degree of DMR is relatively mild, its appearance generally prompts further clinical considerations. The appreciation of DMR has an incremental value for diagnosing and evaluating the underlying cardiovascular disease.

Aims: This study aims to investigate the application of three-dimensional transesophageal echocardiography in aortic valve stenosis for the assessment of aortic valve ring size, to monitor the procedure of transcatheter aortic valve implantation (TAVI), and perform postoperative follow-up. Methods: Eighteen patients with bicuspid valve malformation and severe aortic stenosis bicuspid aortic valve (Group BAV-AS) and 23 patients with a tricuspid valve and severe aortic stenosis trileaflet aortic valve (Group TAV-AS) were enrolled in this study. Preoperative routine transthoracic echocardiographic (TTE) examination and two- and three-dimensional transesophageal echocardiography (2D and 3DTEE) were performed, followed by perioperative 2D and 3D TEE monitoring and postoperative routine TTE at 6-month follow-up. Results: Both BAV-AS and TAV-AS patient groups were successfully implanted with bioprosthetic valves under 3DTEE guidance. Parameters at 6-month postoperatively, including prosthetic valve orifice area, mean aortic transvalvular pressure gradient, and left ventricular ejection fraction, showed significant improvement compared with baseline measures (P < 0.0001) in both the groups. No differences were observed between the groups. The maximum diameter of the aortic annulus and eccentricity index were larger in the BAV-AS group than in the TAV-AS group, whereas the minimum diameter of the aortic annulus was larger in the latter (both P < 0.0001) after TAVI. Moreover, the values of maximum and minimum diameters on 3DTEE were strongly correlated with those on multidetector computed tomography. Conclusions: TEE is capable of clearly displaying the morphology of aortic valves and valve rings and precisely quantifying the size of the aortic annulus, thereby playing an essential role during preoperative and perioperative periods. The postoperative shape of the prosthetic valve ring was more oval (larger than normal eccentricity index) in the BAV-AS group and more circular (smaller than normal eccentricity index) in the TAV-AS group.

Objective: The objective of this study is to evaluate novel variations in a Chinese family of Han ethnicity, with arrhythmogenic right ventricular cardiomyopathy (ARVC) by the powerful technology of whole-exome sequencing (WES). Methods: Genomic DNA from representative family members was extracted and processed for WES via standardized methods. The exome sequence data were analyzed by Genome Analysis Toolkit software. Sanger sequencing was performed on DNA from the proband for genotype confirmation and from the other family members for familial co-segregation analysis. Results: A rare single nucleotide variant c.1592T>G (p.Phe531Cys) in exon 10 of desmoglein-2 (DSG2) was identified by WES in the affected individuals but not in the healthy control, which was confirmed by Sanger sequencing, suggesting that the mutation probably was a causal mutation for the familial disorder of ARVC. Conclusion: We identified a rare disease-causing mutation of the DSG2 gene associated with familial ARVC. The results might contribute to the early genetic analysis in inherited cardiomyopathies.

To evaluate the effect of hyperuricemia (HU) on subclinical changes of left ventricle (LV) function and structure in patients with hypertension (HT) using three-dimensional speckle-tracking echocardiography (3DSTE) and to explore the relationships between serum uric acid (SUA) levels and three-dimensional speckle tracking echocardiography (3DSTE) parameters in hypertensive and nonhypertensive patients with HU.Four age- and sex-matched groups were studied: I: healthy controls, HT- HU- (n = 40); II: HT- HU+ (n = 40); III: HT+ HU- (n = 40); IV: HT+ HU+ (n = 44). Conventional echocardiography and 3DSTE were recorded. Relative wall thickness (RWT) and left ventricular mass index assessed by M-mode echocardiography (LVMi-M) were calculated. 3DSTE parameters including LV volumes and ejection fraction (EF), LVMi-3D, global longitudinal strain (GLS), and global circumferential strain (GCS) were compared. The relationships between SUA levels and 3DSTE parameters were investigated.Despite LV diameters, LV volumes and EF were similar among groups (all p0.05), GLS decreased and LVMi-3D increased from controls (group I) to patients with HU or HT alone (group II or III), and patients with both HU and HT (group IV) (all p0.05). SUA levels were significantly correlated with the absolute value of GLS (r = -0.461, p0.05) and LVMi-3D (r = 0.504, p0.05) in hypertensive and nonhypertensive patients with HU.HU may exacerbate LV systolic dysfunction and remodeling in hypertensive patients, which can be detected by 3DSTE. Early uric acid lowing treatment may be beneficial for hypertensive patients with HU.

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva has received increasing attention due to its association with malignant cardiac events in otherwise healthy and asymptomatic individuals. Despite this, we still face tremendous challenges in detecting the anomalies in a timely fashion to prevent sudden cardiac death. Here, we present two cases of anomalous left coronary artery with single ostium (shared with right coronary artery); in patients with normal cardiac function. The diagnosis of AAOCA in both cases was first suspected on transthoracic echocardiography and was later established using either coronary computed tomography angiography or invasive coronary angiography. Although angiography is the gold standard for diagnosing AAOCA, echocardiography is a more frequently used imaging modality in the workup of cardiac patients.