Long-term outcomes of a novel fully magnetically levitated ventricular assist device for the treatment of advanced heart failure in China.

PURPOSE: Left ventricular assist devices (LVADs) are well-established for treating end-stage heart failure, but this therapy is only available to Chinese patients in recent years. The CH-VAD is the first used fully magnetically levitated pump and the most widely used device in China. This study reports the long-term outcomes of a cohort supported by the CH-VAD for the first time. METHODS: From June 2017 to August 2023, 50 consecutive patients received CH-VAD implantation in Fuwai Hospital. Clinical data were collected during follow-up and retrospectively analyzed. RESULTS: Baseline characteristics included a mean age of 47.9±13.9 years, 90% male, and 26% ischemic etiology. The INTERMACS profile revealed 12% Profile 1, 56% Profile 2, 26% Profile 3 and 6% Profile 4. Mean support duration was 868 ± 630 days (range 33 days-6.4 years). Kaplan-Meier survival rate was 96% (95% confidence interval [CI], 85 to 99) at 6 months, 93% (95% CI, 79-98) at 1 year, 93% (95% CI, 79-98) at 2 years and 89% (95% CI, 71-96) at 3 years. 40 patients (80%) currently remain on support, 3 were bridged to recovery, 2 received transplant, and 5 expired during support. Major adverse events included right heart failure (10%), surgical related bleeding (8%), arrhythmia (8%) and driveline infection (16%). Major hemocompatibility-related adverse events were limited to 3 non-disabling strokes and 1 gastrointestinal bleeding. There was no major device malfunction during the follow-up period. CONCLUSIONS: The largest single-center experience with the leading LVAD in China shows high survival with low complication rates, demonstrating the CH-VAD is safe and efficient in providing long-term support for end-stage heart failure patients.

A complexity evaluation system for mitral valve repair based on preoperative echocardiographic and machine learning.

BACKGROUND: To develop a novel complexity evaluation system for mitral valve repair based on preoperative echocardiographic data and multiple machine learning algorithms. METHODS: From March 2021 to March 2023, 231 consecutive patients underwent mitral valve repair. Clinical and echocardiographic data were included in the analysis. The end points included immediate mitral valve repair failure (mitral replacement secondary to mitral repair failure) and recurrence regurgitation (moderate or greater mitral regurgitation [MR] before discharge). Various machine learning algorithms were used to establish the complexity evaluation system. RESULTS: A total of 231 patients were included in this study; the median ejection fraction was 66% (63-70%), and 159 (68.8%) patients were men. Mitral repair was successful in 90.9% (210 of 231) of patients. The linear support vector classification model has the best prediction results in training and test cohorts and the variables of age, A2 lesions, leaflet height, MR grades, and so on were risk factors for failure of mitral valve repair. CONCLUSION: The linear support vector classification prediction model may allow the evaluation of the complexity of mitral valve repair. Age, A2 lesions, leaflet height, MR grades, and so on may be associated with mitral repair failure.

Transcatheter Edge-to-Edge Repair for Severe Mitral Regurgitation With Double-Orifice Mitral Valve.

Double-orifice mitral valve (DOMV), a rare congenital heart disease, is the inspiration behind the transcatheter edge-to-edge repair (TEER). Here we report a successful TEER case in a patient with DOMV with severe regurgitation. The patient's symptoms were greatly improved after the procedure.

Novel sizing role of 3D transesophageal echocardiography in a novel left atrial appendage clip device for patients undergoing video-assisted atrial fibrillation ablation: a cohort study.

Background: Three-dimensional (3D) transesophageal echocardiography (TEE) has been successfully used in the sizing of left atrial appendage (LAA) occlusion devices, but its use has not yet been studied in LAA clip devices. We sought to develop and validate the novel use of 3D-TEE sizing in a novel LAA clip device for atrial fibrillation (AF) patients undergoing video-assisted thoracic surgery (VATS) ablation. Methods: Consecutive patients with isolated AF undergoing LAA clipping or excision during VATS ablation were included in the study between June 2021 and September 2022 at Fuwai Hospital. The patients underwent 3D-TEE examinations preoperatively and postoperatively. The VATS length, LAA clip effective length, and LAA excision margin length were recorded. A correlation analysis, intraclass correlation coefficient (ICC) analysis, and Bland-Altman plot analysis were conducted to examine the TEE parameters, VATS length, LAA clip effective length, and LAA excision margin length. Results: In total, 26 AF patients undergoing LAA clipping and 15 undergoing LAA excision were included in the study. In the LAA clipping group, in which the Atriclip size served as the control, the 3D-TEE with volumetric measurement (the perimeter-derived maximum orifice diameter) (R=0.938; ICC =0.934; Bland-Altman plot variability, 3.85%) showed the best sizing efficacy for the LAA clip device among the 3D-TEE with multiplanar reformatting sizing (the perimeter-derived maximum orifice diameter) (R=0.808; ICC =0.772; Bland-Altman plot variability, 3.85%), VATS sizing (R=0.851; ICC =0.756; Bland-Altman plot variability, 11.54%), and VATS plus 0.5-cm sizing (R=0.851; ICC =0.775; Bland-Altman plot variability, 11.54%) measurements (all P<0.001). In addition, for the distribution of matched sizing in the LAA clip group, 3D-TEE with volumetric measurement sizing (20/26) had a higher proportion than 3D-TEE with multiplanar reformatting sizing (11/26, P=0.011), VATS sizing (9/26, P=0.002), and VATS plus 0.5-cm sizing (14/26, P=0.08). Using the LAA excision margin length as the control, the mean difference in the LAA diameter was 1.17 cm [95% confidence interval (CI): 0.71-1.62 cm , P<0.001] in the maximum orifice diameter of two-dimensional-TEE, 0.15 cm (95% CI: -0.32 to 0.61 cm , P=0.523) in the perimeter-derived 3D multiplanar reformatting (the maximum orifice diameter), and 0.03 cm (95% CI: -0.47 to 0.53, P=0.901) in the perimeter-derived 3D volumetric (3DV) measurement (the maximum orifice diameter), and the related Pearson correlation coefficients for these modalities were 0.760 (P=0.001), 0.843 (P<0.001), and 0.963 (P<0.001), respectively. Conclusions: Our study showed that 3D-TEE might be employed in the sizing of a novel LAA clip device using the VATS approach in patients with AF. The 3DV measurement (the perimeter-derived maximum orifice diameter) was superior to the VATS measurement. These findings might also apply to LAA VATS excision patients with AF.

Morphology of hypertrophied basal septum contributes to left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: a retrospective case-control study.

Background: Detailed assessment of basal septal morphology is essential for understanding the morphological mechanism of left ventricular outflow tract (LVOT) obstruction. We aimed to analyze the morphological alterations of the basal septum (BS) and its surrounding structures and explore their role in LVOT obstruction (LVOTO) in patients with hypertrophic cardiomyopathy (HCM). Methods: During January 2019 and December 2019, 239 patients were diagnosed with HCM at Fuwai Hospital. We retrospectively reviewed echocardiographic data sets from 105 consecutive patients with HCM [64 with hypertrophic obstructive cardiomyopathy (HOCM) and 41 with hypertrophic non-obstructive cardiomyopathy (HNOCM)] and 28 healthy controls. For quantitatively assessing the basal septal morphology, a novel measurement method was used to obtain the IVSa (the area of the BS protruding into the LVOT), LA (the largest distance of the BS protruding into the LVOT), LB (IVSa length in the direction perpendicular to the LA), and S-IVSa (IVSa divided by LB). Echocardiographic parameters associated with LVOTO were analyzed using multivariable logistic regression analyses. Results: There was no significant difference in the maximal basal septal thickness between the HOCM and HNOCM patients (P>0.99). Among the three groups, there were significant differences in the length of the anterior and posterior mitral leaflets (AML and PML), the angle between the mitral valve orifice and ascending aorta (MV-AO) angle, IVSa, LA, LB, and S-IVSa (all P<0.001). Compared with HNOCM patients, HOCM patients had significantly longer AML and PML, as well as larger MV-AO angle, IVSa, LA, and S-IVSa (P<0.001, P<0.001, P<0.001, P=0.002, P<0.001, and P=0.03, respectively). In the multivariate analysis, AML, MV-AO angle, IVSa, and S-IVSa were associated with LVOTO {odds ratio (OR) [95% confidence interval (CI)]: 0.649 (0.462-0.911), P=0.01; 0.842 (0.768-0.923), P<0.001; 1.025 (1.001-1.049), P=0.04; and 0.276 (0.101-0.754), P=0.01, respectively}. Conclusions: Morphological alterations of the BS relative to the LVOT may provide additional value for estimating the extent of LVOTO. The length of AML, MV-AO angle, IVSa, and S-IVSa were associated with LVOTO.

Association between left ventricular reverse remodeling and long-term outcomes after alcohol septal ablation for hypertrophic obstructive cardiomyopathy.

There is a paucity of data regarding the effect of left ventricular (LV) reverse remodeling (r-LVR) on diastolic function and outcomes after alcohol septal ablation (ASA) in patients with hypertrophic obstructive cardiomyopathy (HOCM). The aim of this study was to identify the impact of r-LVR on the outcome and the predictors of such changes after ASA. Eighty-seven patients (57.5% men) were enrolled and underwent both echocardiography and cardiovascular magnetic resonance (CMR) imaging at baseline and 27 months after the procedure. The study population was divided into two groups by the degree of r-LVR. Compared to the greater r-LVR group, the lesser r-LVR group had a significantly larger LV mass (LVM) and lower diastolic function parameters at baseline. The greater r-LVR group had significantly greater LVM regression and improvement of diastolic function after ASA. Kaplan‒Meier analysis showed significantly worse composite events in the lesser r-LVR group after ASA (P = 0.016). After adjusting for multiple clinical variables, r-LVR was associated with an improved E/e' (ß = 0.390, p < 0.001) and reduced events (hazard ratio: 0.795; 95% confidence interval (CI), 0.644-0.983; p = 0.034). Preablation LVM was associated with a decreased probability of r-LVR (ß = -0.228, p = 0.021) and diastolic function improvement (ß= -0.245, p = 0.006). r-LVR was associated with long-term outcome benefit in patients with HOCM. Preablation LVM prevented LV from favoring reverse remodeling and thus may be a potential parameter for risk stratification and prognosis after ASA treatment.

The Role of the Submitral Apparatus in Hypertrophic Obstructive Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, is characterized by unexplained hypertrophy of any myocardial segment, and has a prevalence of 0.2% to 0.5% among the general population. As one of the phenotypes of hypertrophic cardiomyopathy, left ventricular outflow tract obstruction (LVOTO) is associated with high morbidity and mortality, including cardiac death. The integration of various factors, including septal hypertrophy, malformation of the mitral valve apparatus, and an anomalous mitral subvalvular apparatus, may contribute to the occurrence of LVOTO. Previous studies have thoroughly discussed the role of the mitral valve in the mechanisms of systolic anterior motion and LVOTO. Recent studies have shown the importance of determining the potential mechanism of the submitral apparatus in inducing systolic anterior motion and LVOTO. The authors review recent advances in knowledge regarding the submitral apparatus of patients with hypertrophic cardiomyopathy.

Mitral Annulus Geometry and Dynamic Motion Changes in Patients With Aortic Regurgitation: A Three-Dimensional Transesophageal Echocardiographic Study.

OBJECTIVE: The aim of the present study was to investigate the mitral annulus (MA) geometry and dynamic motion changes in patients with aortic regurgitation (AR) before and after aortic valve replacement (AVR). Moreover, the difference in the effect of the type of prosthetic aortic valve on MA was compared. DESIGN: Prospective observational study. SETTING: Cardiac operating room at a single hospital. PARTICIPANTS: Eighty-two patients with isolated moderate-to-severe AR who underwent AVR. Forty patients with normal valves were enrolled as controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The MA geometry and dynamic motion throughout the cardiac cycle were evaluated semiautomatically by three-dimensional transesophageal echocardiography. The severity of functional mitral regurgitation was intraoperatively evaluated. All patients were divided into 2 groups depending on the type of prosthetic valve (mechanical valve and bioprosthetic valve groups). Before AVR, compared with the control group without AR, the AR group demonstrated larger MA dimensions and the MA geometry was flatter. The contraction fraction of the MA area, perimeter, and height during the whole cardiac cycle were larger in the AR group (p < 0.05 for all). After AVR, most MA geometric and dynamic parameters decreased and functional mitral regurgitation also improved. In the postoperative subset analyses, the mechanical valve group showed a larger contraction fraction of the MA area and perimeter than the bioprosthetic valve group (p < 0.05 for both). CONCLUSIONS: The MA geometry and dynamic motion changed markedly in patients with AR. These spatial and dynamic changes were restored to a certain extent after surgical correction of the aortic valve. However, the effects produced by mechanical and bioprosthetic valves on MA were different.

Incidence, risk factors and prognostic effect of imaging left ventricular diastolic dysfunction in patients with COVID-19: protocol for a systematic review.

INTRODUCTION: Recent reports linked acute COVID-19 infection in critical patients to cardiac structure and function abnormalities. The left ventricular (LV) diastolic dysfunction could result in obvious adverse prognostic impacts. The aim of this meta-analysis is to summarise the incidence, risk factors and the prognostic effect of imaging LV diastolic dysfunction in adult patients with COVID-19. METHODS: Databases to be used for the pertinent literature are PubMed, EMBase, ISI Knowledge via Web of Science, and preprint databases (MedRxiv and BioRxiv) (until May 2023) to identify all cohort studies in adult patients with COVID-19. The primary outcome will be the incidence of LV diastolic dysfunction assessed by echocardiography or cardiac MRI. Secondary outcomes will include the risk factors for LV diastolic dysfunction and the association with all-cause mortality during hospitalisation. Additional outcomes will be septal or lateral é, average E/é, E/A, peak tricuspid regurgitation velocity, left atrial volume index and LV wall thickness. Univariable or multivariable meta-regression and subgroup analyses will be conducted for related risk factors and the association of LV diastolic dysfunction with all-cause mortality. Sensitivity analyses will be used to assess the robustness of our results by removing each included study at one time to obtain and evaluate the remaining overall estimates of LV diastolic dysfunction incidence and related risk factors, association with all-cause mortality and other LV diastolic dysfunction parameters. ETHICS AND DISSEMINATION: There was no need for ethics approval for the systematic review protocol according to the Institutional Review Board/Independent Ethics Committee of Fuwai Hospital. This meta-analysis will be disseminated through a peer-reviewed journal for publication. PROSPERO REGISTRATION NUMBER: CRD42021256666; URL: https://www.crd.york.ac.uk/prospero/.

Simultaneous hybrid maze procedure for long-standing persistent atrial fibrillation with dilated atrium.

BACKGROUND: The efficacy of catheter-based or thoracoscopic ablation for treating long-standing persistent atrial fibrillation (AF) with a dilated atrium remains suboptimal. This study aimed to assess the feasibility and initial results of simultaneous hybrid ablation with a new biatrial lesion set in these patients. METHODS: Twenty-seven consecutive patients with long-standing persistent AF and dilated atrium underwent simultaneous hybrid ablation with a new biatrial lesion set consistent with the principle of the Cox maze procedure at our institution. Patients were followed up at 3, 6, and 12 months after the procedure and annually thereafter. RESULTS: After thoracoscopic epicardial ablation, endocardial mapping confirmed complete bilateral pulmonary vein isolation in 25 patients (96.2%). All patients were followed for a mean of 18.7 ± 8.9 months. Freedom from atrial tachyarrhythmia at 1 year after the single hybrid procedure was 64% with antiarrhythmic drugs and 60% without antiarrhythmic drugs. Freedom from atrial tachyarrhythmia at latest follow-up after the hybrid procedure and redo catheter ablation was 77.8% with antiarrhythmic drugs and 74.1% without antiarrhythmic drugs. In patients with sinus rhythm restoration, mean left atrial diameter decreased from 54.4 ± 4.3 mm to 45.2 ± 4.1 mm (P < .001), and mean right atrial diameter decreased from 59.4 ± 3.9 mm to 54.9 ± 4.4 mm (P < .001). Postoperative pleural effusion was observed in 3 patients (11.1%), and no adverse events occurred during follow-up. CONCLUSIONS: In patients with long-standing persistent AF and a dilated atrium, simultaneous hybrid ablation with the new biatrial lesion set, in combination with touch-up catheter ablation when necessary, can achieve promising results. More studies are needed to confirm these findings.

Incidence, risk factors and prognostic effect of imaging right ventricular involvement in patients with COVID-19: a dose-response analysis protocol for systematic review.

INTRODUCTION: Emerging evidence has shown that COVID-19 infection may result in right ventricular (RV) disturbance and be associated with adverse clinical outcomes. The aim of this meta-analysis is to summarise the incidence, risk factors and the prognostic effect of imaging RV involvement in adult patients with COVID-19. METHODS: A systematical search will be performed in PubMed, EMBase, ISI Knowledge via Web of Science and preprint databases (MedRxiv and BioRxiv) (until October 2021) to identify all cohort studies in adult patients with COVID-19. The primary outcome will be the incidence of RV involvement (dysfunction and/or dilation) assessed by echocardiography, CT or MRI. Secondary outcomes will include the risk factors for RV involvement and their association with all-cause mortality during hospitalisation. Additional outcomes will include the RV global or free wall longitudinal strain (RV-GLS or RV-FWLS), tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC) and RV diameter. Univariable or multivariable meta-regression and subgroup analyses will be performed for the study design and patient characteristics (especially acute or chronic pulmonary embolism and pulmonary hypertension). Sensitivity analyses will be used to assess the robustness of our results by removing each included study at one time to obtain and evaluate the remaining overall estimates of RV involvement incidence and related risk factors, association with all-cause mortality, and other RV parameters (RV-GLS or RV-FWLS, TAPSE, S', FAC and RV diameter). Both linear and cubic spline regression models will be used to explore the dose-response relationship between different categories (>2) of RV involvement and the risk of mortality (OR or HR). ETHICS AND DISSEMINATION: There was no need for ethics approval for the systematic review protocol according to the Institutional Review Board/Independent Ethics Committee of Fuwai Hospital. This meta-analysis will be disseminated through a peer-reviewed journal for publication. PROSPERO REGISTRATION NUMBER: CRD42021231689.

Efficacy and Safety of Tranexamic Acid in Pediatric Patients Undergoing Cardiac Surgery: A Single-Center Experience.

Aims: This study evaluated the efficacy and safety of tranexamic acid (TXA) undergoing cardiac surgery. Methods: Using a retrospective cohort study design, 2,026 consecutive pediatric patients who underwent surgical repair of atrial or ventricular septal defect or complete repair of Tetralogy of Fallot were included, and divided into a control group and a TXA group. Results: Compared with that in the control group, there were statistically significant reduction of both the 12-h and total postoperative blood loss in the TXA group [6.573 ± 0.144 vs. 5.499 ± 0.133 ml kg-1, mean difference (MD) 1.074 ml kg-1, p < 0.001; 12.183 ± 0.298 vs. 9.973 ± 0.276 ml kg-1, MD, 2.210 ml kg-1, p < 0.001]. There was a statistically significant reduction of the MD of 12-h postoperative blood loss due to TXA in patients aged < 1 year compared with that in patients aged ≥1 year (MD, 1.544 vs. 0.681 ml kg-1, P = 0.007). There were statistically significant reduction of the MD of both the 12-h and total postoperative blood loss due to TXA in patients weighing < 10 kg compared with that in patients weighing ≥10 kg (MD, 1.542 vs. 0.456 ml kg-1, P < 0.001, and MD, 2.195 vs. 0.929 ml kg-1, P = 0.036, respectively). There was a statistically significant reduction of the MD of total postoperative blood loss due to TXA in cyanotic patients compared with that in acyanotic patients (MD, 3.381 vs. 1.038 ml kg-1, P = 0.002). There was no significant difference in the postoperative volume or exposure of allogeneic transfusion, in-hospital morbidity or mortality between the groups. Conclusions: TXA took effects in reduction of postoperative blood loss but not the allogeneic transfusion requirement in pediatric patients undergoing cardiac surgery, particularly in infants weighing < 10 kg and cyanotic children. Moreover, the study suggested the use of TXA was safe in pediatric cardiac surgery.

Phenotypes of aortic valve disease according to detailed anatomical classification of patients who underwent aortic valve replacement surgery.

BACKGROUND: The anatomy of the aortic valves plays an important role in the progression of aortic valve disease (AVD) and determination of the appropriate treatment strategy. We described and compared AVD phenotypes according to detailed anatomical classifications and assessed the accuracy of imaging diagnostics using surgical diagnosis as the gold standard. METHODS AND RESULTS: The study enrolled 602 patients (mean age 55.5±12.9 years; 74.1% male) who underwent their first aortic valve replacement surgery between June 2017 and March 2018 at Fuwai Hospital in Beijing, China. Clinical characteristics, histopathological diagnoses, preoperative transthoracic echocardiography (TTE), and other available imaging data were collected retrospectively from medical records. During surgery, 370 patients were diagnosed with a tricuspid aortic valve (TAV), 228 with a bicuspid aortic valve (BAV), and 4 with a quadricuspid aortic valve (QAV). The primary histopathological finding was myxomatous degeneration (48.4%) for those with TAV and fibrocalcification (57.9%) for those with BAV. Nearly all TAV patients (96.2%) had aortic regurgitation, while the majority of BAV patients had aortic stenosis (73.7%); the severity of stenosis and regurgitation varied across the subtypes of BAV. The overall diagnostic accuracy of preoperative TTE for aortic valve anatomy was 85.5%; accuracy was higher for TAV versus BAV (96.8% vs. 68.0%, P<.001). CONCLUSIONS: We identified different phenotypes of AVD among patients with different detailed anatomical classifications. In addition, the diagnostic accuracy of preoperative TTE for BAV was suboptimal, and more attention is needed to ensure an accurate anatomic diagnosis prior to surgical intervention.

Effects of bone marrow mononuclear cells delivered through a graft vessel in patients with previous myocardial infarction and chronic heart failure: An echocardiographic study of left ventricular dyssynchrony.

OBJECTIVES: Several trials are investigating the delivery of stem cells to treat ischemic cardiomyopathy. The aim of this study was the echocardiographic evaluation of the effectiveness of isolated coronary artery bypass graft (CABG) combined with bone marrow mononuclear cells (BMMNC) delivered through the graft vessels to improve left ventricular dyssynchrony in patients with previous myocardial infarction and chronic heart failure. METHODS: 42 patients with previous myocardial infarction and chronic heart failure were randomly allocated to either the CABG only group (n = 18) or the CABG with BMMNC graft group (n = 24group). We used 2D strain imaging to measure the absolute difference in time-to-peak radial strain between the earliest and the latest activated segments on LV short-axis images at the apical (RSTa), at the mitral annulus (RSTb), and at the papillary muscle (RSTm) level. RESULTS: The effective rate of LV dyssynchrony improvement was significantly higher in the CABG + BMMNC than in the CABG only group (RSTb: 91.7% vs 50%, P < .05; RSTm: 78.6% vs 35.7%, P < .05; RSTa: 92.3% vs 50%, P < .05). The deterioration rate of LV synchrony was significantly lower in the CABG + BMMNC than in the CABG only group for RSTb (8.3% vs 70%, P < .05;) and RSTm (0 vs 50%, P < .05), but not for RSTa (18.2% vs 37.5%, P > .05). CONCLUSIONS: Combining CABG with BMMNC delivering provided a better improvement of left ventricular dyssynchrony than CABG only.

Extended myectomy for hypertrophic obstructive cardiomyopathy patients with midventricular obstruction.

OBJECTIVES: Surgical strategies for patients with midventricular obstruction remain underappreciated. We sought to assess clinical and haemodynamic results, summarize the surgical technique of extended myectomy and provide reliable pre- and intraoperative methods of evaluating patients with midventricular obstruction. METHODS: The preoperative evaluation process, intraoperative surgical strategy and early outcomes were thoroughly reviewed in 40 patients with midventricular obstruction. RESULTS: Isolated transaortic myectomy was conducted in 38 (95.0%) patients, and 2 (5.0%) other patients with an apical aneurysm were treated with a combined transaortic and transapical myectomy. The median resection length of the removed muscle was 50 mm (45-55 mm), approximately 5 mm more than the obstruction length measured using preoperative transthoracic echocardiography. There were no early or late deaths, complete heart blocks or iatrogenic septal perforations in our study series with a median follow-up time of 19 months (13-54 months). Instantaneous pressure gradients at the subaortic level decreased from 70.5 mmHg (51-89.5 mmHg) preoperatively to 7.7 mmHg (6-11 mmHg) (P < 0.001) at the most recent evaluation and at the midventricular level from 61.0 mmHg (42.8-85.5 mmHg) to 8.5 mmHg (6.3-11.8 mmHg) (P < 0.001). In all patients, the New York Heart Association functional classifications improved, with a better haemodynamic status. CONCLUSIONS: Transaortic myectomy can be extended to the midventricular level, improving haemodynamic status and yielding satisfactory early outcomes in selected patients. Additional transapical myectomy should be considered in patients with a long obstruction, limited exposure of the midventricular area or a concomitant apical aneurysm.

Numerical simulation study on systolic anterior motion of the mitral valve in hypertrophic obstructive cardiomyopathy.

BACKGROUND: The hydrodynamic mechanisms of systolic anterior motion (SAM) of the mitral valve in hypertrophic obstructive cardiomyopathy (HOCM) remain unclear. METHODS: Based on computed tomography (CT) images and clinical data, pre- and post-operative computational models of the left ventricle were constructed for 6 HOCM patients receiving septal myectomy. SAM was abolished in 5 patients and persisted in one after septal myectomy surgery. The obtained simulation results including flow field of the left ventricle and mechanical behaviors of the mitral valve (MV) between pre- and post-operative FSI models were compared. RESULTS: The pressure difference and shear stress on the mitral valve leaflets (MVL) were relatively high pre-operatively, and decreased significantly after satisfactory surgery, but remained high following failed surgery. The significant increase in coaptation-to-septal distance was found when SAM was abolished. CONCLUSIONS: Our results indicated that high pressure difference and shear stress on the MVL might directly initiate SAM in HOCM. Successful septal myectomy enlarged the coaptation-to-septal distance sufficiently to keep the MVL away from the ejection flow, thereby eliminating SAM.

Mid-term outcomes of biventricular obstruction and left ventricular outflow tract obstruction after surgery correction in child and adolescent patients with hypertrophic cardiomyopathy.

BACKGROUND: Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited. OBJECTIVE: Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection. METHODS: This retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution. The primary endpoint was all-cause death, and secondary endpoints were cardiovascular events. RESULTS: More BVOTO patients (61.9%) than LVOTO (19.2%) and NO-HCM patients (25%) exhibited New York Heart Association (NYHA) III/IV status (p < 0.01). Fourteen BVOTO and 16 LVOTO patients obtained a significant reduction of outflow tract pressure gradients after surgery (vs. preoperative baseline, p < 0.001). One of the 14 BVOTO patients died, whereas no deaths occurred among LVOTO patients. Three of 14 BVOTO surgery patients had complete heart block (CHB) and 4 had new right bundle branch block (RBBB), while no CHB or RBBB occurred in the LVOTO surgery patients. The BVOTO patients had a longer duration of aortic cross-clamping and postoperative hospital days than the LVOTO patients (p < 0.05). During a median 42-month follow-up, no deaths occurred among the remaining patients. The primary and secondary endpoint-free survival rates of the BVOTO group were comparable to those of the LVOTO and NO-HCM groups. CONCLUSIONS: In children and adolescents, BVOTO patients were associated with more severe symptoms than LVOTO and NO-HCM patients; however, good mid-term outcomes similar to those of the LVOTO and NO-HCM groups can be achieved with the application of contemporary cardiovascular treatment strategies. Notably, BVOTO surgery was associated with an increased risk of CHB and RBBB compared to LVOTO surgery.

Genetic anticipation in a special form of hypertrophic cardiomyopathy with sudden cardiac death in a family with 74 members across 5 generations.

Hypertrophic cardiomyopathy (HCM) is the most common heritable heart disease. The genetic anticipation of HCM and its associated etiology, sudden cardiac death (SCD), remains unclear. The aim of this study was to investigate the mechanism underlying the genetic anticipation of HCM and associated SCD.An HCM family including 5 generations and 74 members was studied. Two-dimensional echocardiography was performed to diagnose HCM. The age of onset of HCM was defined as the age at first diagnosis according to hospital records. The information on SCD was confirmed by verification by ≥2 family members and a review of hospital records. Whole-genome sequencing was performed on 4 HCM subjects and 1 healthy control in the family. The identified mutations were screened in all available family members and 216 unrelated healthy controls by Sanger sequencing.The median ages of onset of HCM were 63.5, 38.5, and 18.0 years in members of the second, third, and fourth generations of the family, respectively, and the differences between the generations were significant (P < 0.001). The age at SCD also decreased with each subsequent generation (P < 0.05). In particular, among the third-generation family members, SCD occurred between 30 and 40 years of age at approximately 8 AM, whereas among the fourth-generation family members, all 5 males who experienced SCD were 16 years of age and died at approximately 8 AM. The sarcomere gene mutations MYH7-A719H and MYOZ2-L169G were detected in the HCM individuals in this pedigree. Increases in the number of mutations and the frequency of multiple gene mutations were observed in the younger generations. Moreover, a structural variant was present in the HCM phenotype-positive subjects but was absent in the HCM phenotype-negative subjects.HCM may exhibit genetic anticipation, with a decreased age of onset and increased severity in successive generations. Multiple gene mutations may contribute to genetic anticipation in HCM and thus may be of prognostic value.

The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy.

INTRODUCTION: Severe right ventricular hypertrophy (SRVH) is a rare phenotype in hypertrophic cardiomyopathy (HCM) for which limited information is available. This study was undertaken to investigate the clinical, prognostic and genetic characteristics of HCM patients with SRVH. METHODS: HCM with SRVH was defined as HCM with a maximum right ventricular wall thickness ≥10 mm. Whole-genome sequencing (WGS) was performed in HCM patients with SRVH. Multivariate Cox proportional hazards regression models were used to identify risk factors for cardiac death and events in HCM with SRVH. Patients with apical hypertrophic cardiomyopathy (ApHCM) were selected as a comparison group. The clinical features and outcomes of 34 HCM patients with SRVH and 273 ApHCM patients were compared. RESULTS: Compared with the ApHCM group, the HCM with SRVH group included younger patients and a higher proportion of female patients and also displayed higher cardiovascular morbidity and mortality. The multivariate Cox proportional hazards regression models identified 2 independent predictors of cardiovascular death in HCM patients with SRVH, a New York Heart Association class ≥III (hazard ratio [HR] = 8.7, 95% confidence interval (CI): 1.43-52.87, p = 0.019) and an age at the time of HCM diagnosis ≤18 (HR = 5.5, 95% CI: 1.24-28.36, p = 0.026). Among the 11 HCM patients with SRVH who underwent WGS, 10 (90.9%) were identified as carriers of at least one specific sarcomere gene mutation. MYH7 and TTN mutations were the most common sarcomere mutations noted in this study. Two or more HCM-related gene mutations were observed in 9 (82%) patients, and mutations in either other cardiomyopathy-related genes or ion-channel disease-related genes were found in 8 (73%) patients. CONCLUSIONS: HCM patients with SRVH were characterized by poor clinical outcomes and the presentation of multiple gene mutations.