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Mitral valve (MV) leaflet elongation is recognized as a primary phenotypic expression of hypertrophic cardiomyopathy (HCM) that contributes to obstruction. This study investigates the correlation between MV length and genotype mutations in the two predominant genes, myosin-binding protein C (MYBPC3), and the ß-myosin heavy chain (MYH7) in patients with obstructive HCM (OHCM). Among the 402 OHCM patients, there were likely pathogenic or pathogenic variations in MYH7 (n = 94) and MYBPC3 (n = 76), along with a mutation-negative group (n = 212). Compared to genotype-negative patients, genotype-positive individuals exhibited elongated MV length, thicker interventricular septum, and increased instances of late gadolinium enhancement. Notably, MYH7 mutations were associated with a more severe disease trajectory than MYBPC3 mutations. After adjusting for potential confounders, multivariate linear regression analysis revealed that MYH7 gene mutations and left ventricular volume were independently associated with MV leaflet elongation. The study indicates that mutations in MYH7 and hemodynamics factors are significant risk factors for elongated MV leaflet. Consequently, regular assessment of MV length, especially in patients with MYH7 mutation and enlarged LV volume, is crucial for timely preoperative strategic planning and improved prognosis.
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Objective To analyze the research progress and hot topics in hypertrophic cardiomyopathy from 2018 to 2022.Methods The publications in the field of hypertrophic cardiomyopathy from January 1,2018 to December 31,2022 were retrieved from Web of Science core collection database and included for a bibliometric analysis.Results A total of 6355 publications were included,with an average citation frequency of 7 times.The year 2021 witnessed the most publications (1406).The analysis with VOSviewer showed that the research on sudden death related to hypertrophic cardiomyopathy,especially the predictive value of late gadolinium-enhanced cardiac MRI in sudden death,was a hot topic.In addition,gene detection and the new drug mavacamten became hot research topics.The United States was the country with the largest number of publications and the highest citation frequency in this field.Chinese scholars produced the second largest number of publications,which,however,included few high-quality research results.Conclusions Risk stratification and prevention of sudden death is still an important and hot research content in the field of hypertrophic cardiomyopathy.Chinese scholars should carry out multi-center cooperation in the future to improve the research results.
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Bibliometrics , Cardiomyopathy, Hypertrophic , Cardiomyopathy, Hypertrophic/epidemiology , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/diagnosis , Humans , Death, Sudden, Cardiac/epidemiology , Publications/statistics & numerical data , China/epidemiologyABSTRACT
Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and is characterized by primary left ventricular hypertrophy usually caused by mutations in sarcomere genes. The mechanism underlying cardiac remodeling in HCM remains incompletely understood. An investigation of HCM through integrative analysis at multi-omics levels will be helpful for treating HCM. DNA methylation and chromatin accessibility, as well as gene expression, were assessed by nucleosome occupancy and methylome sequencing (NOMe-seq) and RNA-seq, respectively, using the cardiac tissues of HCM patients. Compared with those of the controls, the transcriptome, DNA methylome and chromatin accessibility of the HCM myocardium showed multifaceted differences. At the transcriptome level, HCM hearts returned to the fetal gene program through decreased sarcomeric and metabolic gene expression and increased extracellular matrix gene expression. In the DNA methylome, hypermethylated and hypomethylated differentially methylated regions (DMRs) were identified in HCM. At the chromatin accessibility level, HCM hearts showed changes in different genome elements. Several transcription factors (TFs), including SP1 and EGR1, exhibited a fetal-like pattern of binding motifs in nucleosome-depleted regions (NDRs) in HCM. In particular, the inhibition of SP1 or EGR1 in an HCM mouse model harboring sarcomere mutations markedly alleviated the HCM phenotype of the mutant mice and reversed fetal gene reprogramming. Overall, this study not only provides a high precision multi-omics map of HCM heart tissue but also sheds light on the therapeutic strategy by intervening the fetal gene reprogramming in HCM.
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AIMS: Type 2 diabetes (T2D), a prevalent cardiovascular disease, is linked with cardiac arrhythmias such as atrial fibrillation (AF) and ventricular arrhythmia. This study evaluated T2D's impact on these arrhythmias in patients with obstructive hypertrophic cardiomyopathy (OHCM). METHODS AND MATERIALS: We retrospectively analyzed the data of 75 patients with OHCM and T2D from two medical centers in China from 2011 to 2020. A propensity score-matched cohort of 150 patients without T2D was also analyzed. RESULTS: Altogether, 225 patients were included. The prevalence of supraventricular tachycardia (SVT), AF, and non-sustained ventricular tachycardia (NSVT) was higher in patients with HCM and T2D than in those without T2D. Multivariate logistic regression showed T2D as an independent risk factor for SVT (odds ratio [OR] = 1.90, 95% confidence interval [CI] = 1.01-3.58, P = 0.04), AF (OR = 2.68, 95% CI = 1.27-5.67, P = 0.01), and NSVT (OR = 2.18, 95% CI = 1.04-4.57, P = 0.04). Further analysis identified fasting glucose and glycosylated hemoglobin levels as independent risk factors for AF and NSVT in patients with T2D. CONCLUSIONS: T2D independently increases the risk of cardiac arrhythmias (SVT, AF, NSVT) in OHCM patients. Furthermore, fasting glucose and glycosylated hemoglobin levels independently heighten AF and NSVT risk in OHCM patients with T2D.
Subject(s)
Arrhythmias, Cardiac , Cardiomyopathy, Hypertrophic , Diabetes Mellitus, Type 2 , Humans , Diabetes Mellitus, Type 2/complications , Male , Female , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/epidemiology , Middle Aged , Retrospective Studies , Arrhythmias, Cardiac/etiology , Arrhythmias, Cardiac/epidemiology , Follow-Up Studies , Risk Factors , Prognosis , China/epidemiology , Aged , AdultSubject(s)
Atrial Function, Left , Cardiac Surgical Procedures , Cardiomyopathy, Hypertrophic , Predictive Value of Tests , Humans , Cardiomyopathy, Hypertrophic/physiopathology , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/complications , Treatment Outcome , Cardiac Surgical Procedures/adverse effects , Female , Male , Middle Aged , Recovery of Function , Time Factors , Risk Factors , Aged , AdultABSTRACT
BACKGROUND: Myocardial bridging (MB) is common in patients with hypertrophic cardiomyopathy (HCM). There are sparse data on the impact of MB on myocardial fibrosis in HCM. This study was designed to evaluate the relationship between MB and myocardial fibrosis in patients with obstructive HCM. METHODS: In this cohort study, retrospective data were collected from a high-volume HCM center. Patients with obstructive HCM who underwent septal myectomy and preoperative cardiac magnetic resonance (CMR) were screened from 2011 to 2018. RESULTS: Finally, 492 patients were included in this study, with an average age of 45.7 years. Of these patients, 76 patients had MB. MB occurred mostly in the left anterior descending artery (73/76). The global extent of late gadolinium enhancement (LGE) was correlated with the degree of systolic compression (r = 0.33, p = 0.003). Multivariable linear regression analysis revealed that the degree of systolic compression was an independent risk factor for LGE (ß = 0.292, p = 0.007). The LGE fraction of basal and mid anteroseptal segments in patients with severe MB (compression ratio ≥ 80%) was significantly greater than that in patients with mild to moderate MB (compression ratio < 80%). During a median follow-up of 28 (IQR: 15-52) months, 15 patients died. Kaplan-Meier analysis did not identify differences in all-cause death (log-rank p = 0.63) or cardiovascular death (log-rank p = 0.72) between patients undergoing MB-related surgery and those without MB. CONCLUSIONS: MB with severe systolic compression was significantly associated with a high extent of fibrosis in patients with obstructive HCM. Concomitant myotomy or coronary artery bypass grafting might provide excellent survival similar to that of patients without MB. Identification of patients with severe MB and providing comprehensive management might help improve the prognosis of patients with HCM.
Subject(s)
Cardiomyopathy, Hypertrophic , Myocardial Bridging , Humans , Middle Aged , Myocardium/pathology , Contrast Media , Retrospective Studies , Cohort Studies , Myocardial Bridging/complications , Myocardial Bridging/diagnostic imaging , Myocardial Bridging/pathology , Gadolinium , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/surgery , Fibrosis , Risk FactorsABSTRACT
With sodium tripolyphosphate (STPP) as cross-linker, Persimmon tannin-chitosan microspheres (PT-CS) were synthesized by hydrothermal for removing U(VI) from acidic effluent. The batch experiments indicated that PT-CS adsorbed U(VI) most effectively at pH 1.5, the maximum adsorption capacity for PT-CS was 245 mg/g. Compared to pure CS dissolved at pH 3, PT-CS still maintain high stability at pH 1. Moreover, single system of common metal ions in rare earth wastewater only slightly affected the adsorption of uranium at pH 1.5, but this process was inhibited about 30% at pH 5. Those results indicated that the selectivity of PT-CS for uranium removal could be controlled by regulating the pH and there are excellent potentials for PT-CS using in acid metal water treatment. Its adsorption selectivity and ability to adapt different condition was demonstrated with uraniferous rare earth wastewater treatment. The adsorption for PT-CS to U(VI) were well fitted for both Langmuir isothern and pseudo-secondary kinetic model equations, and that meant chemisorption dominated the removal process. Spectroscopic analyses confirmed that the adsorption of U(VI) occurred via surface complexation by -OH and ion exchange with Na+. Therefore, this study provides a high-efficiency, low-cost, valuable and highly adaptable method for the treatment of acidic uranium-containing effluents.
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Hypertrophic cardiomyopathy (HCM) is a common inherited cardiovascular disease, which can cause heart failure and lead to death. In this study, we performed high-resolution single-cell RNA-sequencing of 2115 individual cardiomyocytes obtained from HCM patients and normal controls. Signature up- and down-regulated genes in HCM were identified by integrative analysis across 37 patients and 41 controls from our data and published human single-cell and single-nucleus RNA-seq datasets, which were further classified into gene modules by single-cell co-expression analysis. Using our high-resolution dataset, we also investigated the heterogeneity among individual cardiomyocytes and revealed five distinct clusters within HCM cardiomyocytes. Interestingly, we showed that some extracellular matrix (ECM) genes were up-regulated in the HCM cardiomyocytes, suggesting that they play a role in cardiac remodelling. Taken together, our study comprehensively profiled the transcriptomic programs of HCM cardiomyocytes and provided insights into molecular mechanisms underlying the pathogenesis of HCM.
Subject(s)
Cardiomyopathy, Hypertrophic , Myocytes, Cardiac , Humans , Gene Expression Profiling , Transcriptome/genetics , Cardiomyopathy, Hypertrophic/genetics , RNA-SeqABSTRACT
OBJECTIVES: This study aimed to assess the effectiveness of three-dimensional printing (3DP) in patients with complex hypertrophic cardiomyopathy requiring combined transaortic and transapical septal myectomy. METHODS: We created 3DP models for 7 patients undergoing this surgery approach between June and October 2022 using silicone-like resin and conducted mock operations. The models were compared with echocardiography to identify abnormal muscle bundles and heart structures. These patients were then compared with a 1:2 matched group without 3DP, considering age, sex and additional operations. RESULTS: The models mostly presenting with midventricular obstruction showed high consistency with original computed tomography data (r = 0.978, P < 0.001). 3DP identified more abnormal muscle bundles than echocardiography, primarily between the interventricular septum and apex. Excised specimens in mock operations mirrored those in actual myectomies. While cardiopulmonary bypass time was not significantly different, a near-20-min decrease was observed in the 3DP group (135.5 ± 31.1 vs 154.4 ± 36.6 min, P = 0.054). CONCLUSIONS: While no significant differences in surgical outcomes were observed, 3DP appeared to enhance the visualization and understanding of spatial structures (average Likert scale score 4.0), potentially contributing to surgical proficiency (overall rating score 3.9). The use of 3DP may offer additional value in the preparation and execution of operations for complex hypertrophic cardiomyopathy cases.
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Cardiac Surgical Procedures , Cardiomyopathy, Hypertrophic , Ventricular Septum , Humans , Cardiac Surgical Procedures/methods , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/surgery , Ventricular Septum/surgery , Coronary Artery Bypass , Printing, Three-Dimensional , Treatment OutcomeABSTRACT
Danon disease is a rare X-linked disorder caused by deficiency of the lysosome-associated membrane protein-2. We report a case of hypertrophic obstructive cardiomyopathy secondary to a novel mutation in the lysosome-associated membrane protein-2 gene in a 10-year-old male adolescent. We performed a modified extended Morrow procedure to minimise the risk of death and improve the patient's quality of life. The patient did not have exertional dyspnoea, and auscultation did not reveal a cardiac murmur at 1-year follow-up.
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Cardiomyopathy, Hypertrophic , Glycogen Storage Disease Type IIb , Male , Adolescent , Humans , Child , Glycogen Storage Disease Type IIb/complications , Glycogen Storage Disease Type IIb/diagnosis , Glycogen Storage Disease Type IIb/genetics , Quality of Life , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/geneticsABSTRACT
Obstructive hypertrophic cardiomyopathy (oHCM) and mitral valve (MV) prolapse (MVP) are the 2 conditions which could cause symptomatic heart failure and sudden cardiac death. The clinical characteristics and surgical outcomes of patients with oHCM and MVP have not been well reported. From April 2012 to February 2018, 84 patients with oHCM (28 patients with MVP and 56 gender- and age-matched patients without MVP) who underwent septal myectomy at our institution were enrolled in this study. Information on clinical characteristics and outcomes was obtained from electronic medical records and follow-up surveys. Compared with those without MVP, patients with MVP were more symptomatic (New York Heart Association class III to IV; 96% vs 77%), more often moderate-to-severe mitral regurgitation (86% vs 48%), atrial fibrillation (39% vs 11%) and higher incidence of nonsustained ventricular tachycardia (44% vs 15%). Twenty (71%) had MV repair and 8 (29%) had MV replacement. Compared with patients without MVP, those with MVP had a longer postoperative hospital stay (10.9 ± 6.4 vs 7.8 ± 2.8 days). None of the 84 study patients died during hospital or follow-up. At the most recent echocardiographic evaluation, left ventricular outflow tract gradient significantly decreased from 69.7 ± 35.4 millimeters of mercury to 7.3 ± 5.1 millimeters of mercury and the degree of mitral valve regurgitation improved from grade 2.43 ± 0.69 to grade 0.5 ± 0.69. In conclusion, MVP occurs rarely in oHCM, and was related to atrial fibrillation, ventricular arrhythmia and mitral regurgitation. Mitral valve surgery in combination with myectomy is effective and safe for patients with oHCM and MVP, relieving substantially left ventricular outflow tract gradients and mitral regurgitation.
Subject(s)
Atrial Fibrillation , Cardiomyopathy, Hypertrophic , Mercury , Mitral Valve Insufficiency , Mitral Valve Prolapse , Humans , Mitral Valve Prolapse/complications , Mitral Valve Prolapse/surgery , Mitral Valve Insufficiency/diagnosis , Mitral Valve Insufficiency/surgery , Treatment Outcome , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/surgeryABSTRACT
BACKGROUND: Mitral annular calcification (MAC) is a risk factor for cardiac surgery, but there is limited study on the prognosis value and the impact for valve function of MAC based on computed tomography (CT) diagnosis after myectomy for hypertrophic obstructive cardiomyopathy (OHCM). METHODS: Consecutive OHCM patients underwent septal myectomy were compared according to the existence of MAC and its severity in preoperative CT scans. The survival data were evaluated and compared by Kaplan Meier analysis and log rank test. Cox regression analysis was used to evaluate the impact of MAC on endpoint events. RESULTS: From the entire cohort of 1035 patients, 10.8% had MAC. In multivariate regression, female (OR = 2.23), age (OR = 1.07), aortic annular calcification (OR = 2.52), aortic calcification (OR = 2.56), systolic anterior motion of the mitral valve (SAM) (OR = 0.42), mitral valve thickening (OR = 2.13), and tricuspid regurgitation (OR = 3.12) were independent predictors of MAC. All-cause mortality (3.57% vs. 1.08%, p = 0.031), major adverse cardiovascular and cerebrovascular events (MACCE) (23.32% vs. 13.65%, p = 0.014), recurrent MR > 2+ (8.04% vs. 2.49%, p = 0.001) and NYHA III-IV (11.61% vs. 5.53%, p = 0.012) were more frequent in OHCM patients with MAC after myectomy. MAC was discovered to be an independent predictor of postoperative recurrent MR > 2+ after other risk factors were taken into account (HR 2.47, 95% CI 1.08-5.67, p = 0.0329). Moderate-to-severe MAC was an independent risk factor (HR 2.03, 95% CI 1.09-3.75, p = 0.0244) for long-term major adverse cardiovascular and cerebrovascular events (MACCE). CONCLUSION: MAC was detected in one-tenth of OHCM patients in preoperative CT scanning and is mainly associated with aging and atherosclerosis. OHCM patients with MAC had a worse prognosis and more recurrent mitral valve regurgitation than those without MAC after septal myectomy.
Subject(s)
Cardiomyopathy, Hypertrophic , Mitral Valve Insufficiency , Humans , Female , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Prognosis , Treatment Outcome , Incidence , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/epidemiology , Cardiomyopathy, Hypertrophic/surgery , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/epidemiology , Mitral Valve Insufficiency/surgeryABSTRACT
Although increased premature atrial contractions (PACs) reportedly predict atrial fibrillation (AF) in both general and specific (e.g., patients with stroke) populations, early postoperative AF (POAF) risk in patients with increased PAC burden who require cardiac surgery remains unclear. We examined the correlation between different preoperative PAC burdens and POAF in patients with obstructive hypertrophic cardiomyopathy (OHCM) who underwent surgical treatment. We analyzed 304 consecutively admitted patients with OHCM without previous AF who underwent isolated septal myectomy between January 2015 and December 2018. All patients underwent preoperative 24-hour Holter electrocardiogram monitoring. PACs were present in 259 patients (85.20%) and absent in 45 patients (14.80%). According to the cut-off PAC number of 100 beats/24 hours, there were 211 patients (69.41%) with low-burden PACs and 48 patients (15.79%) with high-burden PACs. AF after septal myectomy occurred in 73 patients, which consisted of 3/45 in the non-PAC group (6.67%), 47/211 in the low-PAC-burden group (22.27%), and 23/48 in the high PAC burden group (47.92%). POAF incidence was higher in both low- and high-burden patients than in patients without PAC (p <0.01). Multivariate logistic regression analyses demonstrated that high-burden PACs (p = 0.02) and age (p <0.01) but not low-burden PACs (p = 0.22) independently predicted POAF in patients with OHCM. The area under the receiver operating characteristic curve for preoperative PACs was 0.72 (95% confidence interval 0.66 to 0.79, p <0.01, sensitivity: 68.49%, specificity: 69.26%). In conclusion, POAF incidence was significantly higher in patients with preoperative high-burden PACs and can predict POAF in patients with OHCM.
Subject(s)
Atrial Fibrillation , Atrial Premature Complexes , Cardiac Surgical Procedures , Cardiomyopathy, Hypertrophic , Humans , Atrial Fibrillation/diagnosis , Atrial Fibrillation/epidemiology , Atrial Fibrillation/etiology , Atrial Premature Complexes/diagnosis , Atrial Premature Complexes/epidemiology , Atrial Premature Complexes/complications , Coronary Artery Bypass/adverse effects , Cardiac Surgical Procedures/adverse effects , Electrocardiography, Ambulatory , Cardiomyopathy, Hypertrophic/diagnosis , Cardiomyopathy, Hypertrophic/epidemiology , Cardiomyopathy, Hypertrophic/surgery , Risk FactorsABSTRACT
Background Sarcomere gene mutation and myocardial fibrosis are both associated with poorer clinical outcomes in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to determine the relationship between sarcomere gene mutation and myocardial fibrosis measured by both histopathology and cardiac magnetic resonance (CMR). Methods and Results Two hundred twenty-seven patients with HCM who underwent surgical treatment, genetic testing, and CMR were enrolled. We retrospectively analyzed basic characteristics, sarcomere gene mutation, and myocardial fibrosis measured by CMR and histopathology. In our study, the mean age was 43 years, and 152 patients (67.0%) were men. A total of 107 patients (47.1%) carried a positive sarcomere gene mutation. The myocardial fibrosis ratio was significantly higher in the late gadolinium enhancement (LGE)+ group (LGE+ 14.3±7.5% versus LGE- 9.0±4.3%; P=0.001). Patients with HCM with SARC+ showed a high probability of fibrosis both in histopathology (myocardial fibrosis ratio 15.3±8.0% versus 12.4±6.5%; P=0.003) and CMR examination (LGE+ 98.1% versus 84.2%; P<0.001; LGE quantification 8.3% versus 5.8%; P<0.001). Linear regression analysis showed that sarcomere gene mutation (B=2.661; P=0.005) and left atrial diameter (B=0.240; P=0.001) were related factors for histopathological myocardial fibrosis. Also, the myocardial fibrosis ratio was significantly higher in the MYH7 (myosin heavy chain) group (MYH7 18.1±9.6% versus MYBPC3 [myosin binding protein C] 13.1±5.2%; P=0.019). Conclusions Patients with HCM with positive sarcomere gene mutation had a higher myocardial fibrosis extent than patients without mutation, and a significant difference in myocardial fibrosis was also observed between the MYBPC3 and MYH7 groups. In addition, a high consistency was found between CMR-LGE and histopathological myocardial fibrosis in patients with HCM.
Subject(s)
Cardiomyopathy, Hypertrophic , Contrast Media , Male , Humans , Adult , Female , Retrospective Studies , Gadolinium , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/genetics , Cardiomyopathy, Hypertrophic/complications , Fibrosis , Magnetic Resonance Spectroscopy , MutationABSTRACT
BACKGROUND: Assessing the structure and function of left atrium (LA) is crucial in hypertrophic obstructive cardiomyopathy (HOCM) because LA remodeling correlates with atrial fibrillation. However, few studies have investigated the potential effect of myomectomy on LA phasic remodeling in HOCM after myectomy using cardiovascular magnetic resonance (CMR) feature tracking (FT). This study aims to evaluate the LA structural and functional remodeling with HOCM after myectomy by CMR-FT and to further investigate the determinants of LA reverse remodeling. METHODS: In this single-center study, we retrospectively studied 88 patients with HOCM who received CMR before and after myectomy between January 2011 and June 2021. Preoperative and postoperative LA parameters derived from CMR-FT were compared, including LA reservoir function (total ejection fraction [EF], total strain [εs], peak positive strain rate [SRs]), conduit function (passive EF, passive strain [εe], peak early negative strain rate [SRe]) and booster function (booster EF, active strain [εa], late peak negative strain rate [SRa]). Eighty-six healthy participants were collected for comparison. Univariate and multivariate linear regression identified variables associated with the rate of change of εa. RESULTS: Compared with preoperative parameters, LA reservoir function (total EF, εs, SRs), booster function (booster EF, εa, SRa), and SRe were significantly improved after myectomy (all P < 0.05), while no significant differences were observed in passive EF and εe. Postoperative patients with HOCM still had larger LA and worse LA function than healthy controls (all P < 0.05). After analyzing the rates of change in LA parameters, LA boost function, especially εa, showed the most dramatic improvement beyond the improvements in reservoir function, conduit function, and volume. In multivariable regression analysis, minimum LA volume index (adjusted ß = - 0.39, P < 0.001) and Δleft ventricular outflow tract (LVOT) pressure gradient (adjusted ß = - 0.29, P = 0.003) were significantly related to the rate of change of εa. CONCLUSIONS: Patients with HOCM after septal myectomy showed LA reverse remodeling with a reduction in LA size and restoration in LA reservoir and booster function but unchanged LA conduit function. Among volumetric and functional changes, booster function had the greatest improvement postoperatively. Besides, preoperative LAVmin index and ΔLVOT might be potential factors associated with the degree of improvement in εa.
Subject(s)
Cardiomyopathy, Hypertrophic , Heart Atria , Humans , Retrospective Studies , Predictive Value of Tests , Heart Atria/diagnostic imaging , Heart Atria/surgery , Cardiomyopathy, Hypertrophic/diagnostic imaging , Cardiomyopathy, Hypertrophic/surgery , Cardiomyopathy, Hypertrophic/complications , Magnetic Resonance SpectroscopyABSTRACT
Hypertrophic cardiomyopathy (HCM) is the most common cardiac genetic disorder characterized by cardiomyocyte hypertrophy and cardiac fibrosis. Pathological cardiac remodeling in the myocardium of HCM patients may progress to heart failure. An in-depth elucidation of the lineage-specific changes in pathological cardiac remodeling of HCM is pivotal for the development of therapies to mitigate the progression. Here, we performed single-nucleus RNA-seq of the cardiac tissues from HCM patients or healthy donors and conducted spatial transcriptomic assays on tissue sections from patients. Unbiased clustering of 55,122 nuclei from HCM and healthy conditions revealed 9 cell lineages and 28 clusters. Lineage-specific changes in gene expression, subpopulation composition, and intercellular communication in HCM were discovered through comparative analyses. According to the results of pseudotime ordering, differential expression analysis, and differential regulatory network analysis, potential key genes during the transition towards a failing state of cardiomyocytes such as FGF12, IL31RA, and CREB5 were identified. Transcriptomic dynamics underlying cardiac fibroblast activation were also uncovered, and potential key genes involved in cardiac fibrosis were obtained such as AEBP1, RUNX1, MEOX1, LEF1, and NRXN3. Using the spatial transcriptomic data, spatial activity patterns of the candidate genes, pathways, and subpopulations were confirmed on patient tissue sections. Moreover, we showed experimental evidence that in vitro knockdown of AEBP1 could promote the activation of human cardiac fibroblasts, and overexpression of AEBP1 could attenuate the TGFß-induced activation. Our study provided a comprehensive analysis of the lineage-specific regulatory changes in HCM, which laid the foundation for targeted drug development in HCM.
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INTRODUCTION: The necessity of complete bi-atrial lesion created by radiofrequency clamp and pen for nonparoxysmal atrial fibrillation (AF) in patients with rheumatic mitral valve disease (RMVD) remains unclear. METHODS: From January 2014 to December 2018, patients with RMVD concomitant with nonparoxysmal AF who underwent mitral valve surgery concomitant surgical ablation were retrospectively enrolled. We divided patients into Group A (complete bi-atrial lesion set created by radiofrequency clamp and pen) and Group B (simplified lesion sets created by radiofrequency clamp alone including bi-atrial ablation with incomplete mitral isthmus line and stand-alone left atrial ablation) according to the surgical ablation lesion sets. Propensity score matching was applied to analyze freedom from atrial tachyarrhythmias between the two groups. RESULTS: Two hundred eight (38.5%) and 332 (61.5%) patients were divided into Group A and Group B, respectively. In Group B, the proportion of patients with recurrent atrial flutter in the subgroup of bi-atrial ablation with incomplete mitral isthmus line was higher than that in Group A (p = .044). After propensity score matching, there were 203 patients in each group. Better freedom from atrial tachyarrhythmias without antiarrhythmic drugs was obtained in Group A (83.1%, 79.6%, and 65.4%) than Group B (73.1%, 68.4%, and 52.7%) at 12, 36, and 60 months after operation (p = .012). CONCLUSION: The application of radiofrequency clamp and pen to create complete bi-atrial lesion set in surgical ablation for nonparoxysmal AF in RMVD was associated with superior long-term efficacy.
Subject(s)
Atrial Fibrillation , Catheter Ablation , Heart Valve Diseases , Humans , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Atrial Fibrillation/diagnosis , Atrial Fibrillation/surgery , Atrial Fibrillation/complications , Retrospective Studies , Follow-Up Studies , Heart Valve Diseases/diagnosis , Heart Valve Diseases/diagnostic imaging , Catheter Ablation/adverse effects , Catheter Ablation/methods , Treatment OutcomeABSTRACT
BACKGROUND: Atrial high-rate episode (AHRE) and stroke are related; however, the magnitude of the correlations between different AHRE burdens and stroke remains unknown. We analysed studies that evaluated this relationship. METHODS: We searched for observational controlled studies that reported the associations of different AHRE burdens with stroke in populations with cardiac implantable electronic devices (CIEDs). The primary endpoint was stroke or thrombosis during follow-up. We performed subgroup analyses according to the classification criteria and research design of the included studies. RESULTS: Of the 5985 studies identified, 9 met the eligibility criteria and included 42,958 patients. Patients with low and high AHRE burdens had a 1.2-fold risk of stroke (no heterogeneity) and a 2.52-fold risk of stroke (moderate heterogeneity), respectively. After excluding studies analysing the atrial fibrillation history, no significant difference in progressive stroke risk was observed for patients with a low AHRE burden (without significant heterogeneity). An increased likelihood of stroke was observed for patients with a high AHRE burden (decreased heterogeneity). Four of the nine studies classified high and low AHRE burdens using the longest AHRE time. Five studies classified high and low AHRE burdens based on the median of the total AHRE time as the cutoff value. Low and high AHRE burdens were more closely related to stroke when classified by the total AHRE duration than when classified by the single longest AHRE duration. CONCLUSIONS: For populations with CIEDs without an atrial fibrillation history, a high AHRE burden was significantly associated with stroke.
Subject(s)
Atrial Fibrillation , Defibrillators, Implantable , Stroke , Humans , Atrial Fibrillation/diagnosis , Atrial Fibrillation/epidemiology , Atrial Fibrillation/complications , Heart Atria/diagnostic imaging , Stroke/diagnosis , Stroke/epidemiology , Stroke/etiology , Risk Factors , Defibrillators, Implantable/adverse effectsABSTRACT
Surgical myectomy remains the time-honored primary treatment for hypertrophic cardiomyopathy patients with drug refractory limiting symptoms due to LV outflow obstruction. Based on >50 years experience, surgery reliably reverses disabling heart failure by permanently abolishing mechanical outflow impedance and mitral regurgitation, with normalization of LV pressures and preserved systolic function. A consortium of 10 international currently active myectomy centers report about 11,000 operations, increasing significantly in number over the most recent 15 years. Performed in experienced multidisciplinary institutions, perioperative mortality for myectomy has declined to 0.6%, becoming one of the safest currently performed open-heart procedures. Extended myectomy relieves symptoms in >90% of patients by ≥ 1 NYHA functional class, returning most to normal daily activity, and also with a long-term survival benefit; concomitant Cox-Maze procedure can reduce the number of atrial fibrillation episodes. Surgery, preferably performed in high volume clinical environments, continues to flourish as a guideline-based and preferred high benefit: low treatment risk option for adults and children with drug refractory disabling symptoms from obstruction, despite prior challenges: higher operative mortality/skepticism in 1960s/1970s; dual-chamber pacing in 1990s, alcohol ablation in 2000s, and now introduction of novel negative inotropic drugs potentially useful for symptom management.
Subject(s)
Atrial Fibrillation , Cardiac Surgical Procedures , Cardiomyopathy, Hypertrophic , Ventricular Outflow Obstruction , Adult , Atrial Fibrillation/complications , Cardiac Surgical Procedures/methods , Cardiomyopathy, Hypertrophic/complications , Child , Humans , Treatment Outcome , Ventricular Outflow Obstruction/complications , Ventricular Outflow Obstruction/surgeryABSTRACT
Purpose: To investigate myocardial remodeling using cardiac MRI (CMR) feature tracking (FT) and to explore the relationship between CMR parameters with outcomes in hypertrophic obstructive cardiomyopathy (HOCM) after myectomy. Materials and Methods: In this single-center retrospective study, patients with HOCM undergoing myectomy between 2011 and 2019 were included. Pre- and postmyectomy global and regional strains were compared. Healthy participants were included for comparison. Composite events were recorded at follow-up performed after a minimum of 12 months. The paired-samples t test was utilized to compare pre- and postmyectomy variables. Results: A total of 73 patients (44 years ± 14 [SD]; 45 men) were evaluated. Compared with preoperative parameters, global circumferential strain (CS) (-17.6% ± 4.4 vs -16.7% ± 3.9, P = .02) was impaired, but global longitudinal strain (LS) was improved (-9.3% ± 2.8 vs -10.8% ± 3.3, P < .001). Septal CS (-14.2% ± 4.0 vs -11.0% ± 4.4, P < .001) and septal radial strain (RS) (16.4% ± 10.6 vs 13.7% ± 9.5, P = .007) worsened, while septal LS (-8.1% ± 3.5 vs -10.2% ± 3.4, P < .001), lateral RS (40.1% ± 16.6 vs 54.4% ± 22.6, P < .001), lateral CS (-20.2% ± 4.1 vs -23.1% ± 4.8, P < .001), and lateral LS (-5.6% ± 5.6 vs -8.4% ± 5.2, P = .001) were improved. Sixteen of 73 patients (22%) experienced composite events after median follow-up of 39.1 months. Postoperative global CS provided the highest discrimination for composite event occurrence (area under the receiver operating characteristic curve, 0.73; 95% CI: 0.61, 0.83) with a cutoff of -16.7%. Patients with postoperative global CS greater than -16.7% had reduced event-free survival compared with those with postoperative global CS less than or equal to -16.7% (log-rank P = .002). Conclusion: CMR-FT analysis demonstrated longitudinal and lateral restorations, but impaired global CS, after myectomy in patients with HOCM; furthermore, increased global CS was associated with poorer outcomes.Keywords: MR Imaging, Cardiac, Outcomes Analysis, Comparative Studies, Surgery© RSNA, 2022 Supplemental material is available for this article.