Myocardial bridging in obstructive hypertrophic cardiomyopathy: a risk factor for myocardial fibrosis.

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.

Paediatric hypertrophic cardiomyopathy secondary to Danon disease.

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.

[Bibliometrics Analysis of Studies on Hypertrophic Cardiomyopathy From 2018 to 2022].

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.

Superiority of complete bi-atrial ablation procedure for atrial fibrillation in rheumatic mitral valve disease.

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.

Reverse remodeling of left atrium assessed by cardiovascular magnetic resonance feature tracking in hypertrophic obstructive cardiomyopathy after septal myectomy.

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.

Myocardial bridging of the left anterior descending coronary artery as a risk factor for atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy: a matched case-control study.

BACKGROUND: Myocardial bridging (MB) is associated with various forms of arrhythmia. However, whether MB is a risk factor for atrial fibrillation (AF) in patients with hypertrophic obstructive cardiomyopathy (HOCM) remains unknown. This study aimed to identify the relationship between myocardial bridging of the left anterior descending coronary artery (MB-LAD) and AF in patients with HOCM. METHODS: We reviewed the medical records of 1925 patients diagnosed with HOCM at Fuwai Hospital from January 2012 to March 2019. Patients with coronary artery disease, a history of heart surgery, and those who had not been subjected to angiography were excluded. Finally, 105 patients with AF were included in this study. The control group was matched in a ratio of 3:1 based on age and gender. RESULTS: Forty-three patients were diagnosed with MB-LAD in this study. The presence of MB was significantly higher in patients with AF than in those without AF (19.0% vs. 7.3%; p = 0.001), although MB compression and MB length did not differ between the two groups. In conditional multivariate logistic analysis, MB (odds ratio [OR] 2.33; 95% confidence interval [CI] 1.08-5.01; p = 0.03), pulmonary arterial hypertension (OR 2.63; 95% CI 1.26-5.47; p = 0.01), hyperlipidemia (OR 1.83; 95% CI 1.12-3.00; p = 0.016), left atrial diameter (OR 1.09; 95% CI 1.05-1.13; p < 0.001), and interventricular septal thickness (OR 1.06; 95% CI 1.003-1.12; p = 0.037) were independent risk factors for AF in patients with HOCM. CONCLUSIONS: The presence of MB is an independent risk factor for AF in patients with HOCM. The potential mechanistic link between MB and the development of AF warrants further investigation.

Midterm results of different treatment methods for myocardial bridging in patients after septal myectomy.

BACKGROUND: Myocardial bridging (MB) is commonly treated in patients with hypertrophic cardiomyopathy. However, whether and how MB should be treated in patients with hypertrophic obstructive cardiomyopathy (HOCM) who underwent septal myectomy remain unclear. METHODS: A total of 823 adults with HOCM who underwent septal myectomy at the Fuwai Hospital from 2011 to 2017 were retrospectively studied. RESULTS: Overall, 31 events occurred: 24 patients died and 7 had nonfatal myocardial infarction (MI). The 3-year cumulative event-free survival of all-cause death (97.9% vs. 100% vs. 100% vs. 98.4%, p = .89) and cardiovascular death (98.3% vs. 100% vs. 100% vs. 98.4%, p = .63) were similar among the four groups (non-MB, coronary artery bypass grafting [CABG], unroofing, untreated, respectively). However, the 3-year cumulative event-free survival of nonfatal MI (100% vs. 97.5% vs. 98.0% vs. 89.9%, p < .001) and combined endpoints (97.9% vs. 97.5% vs. 98.0% vs. 88.4%, p = .02) were significantly lowest in untreated MB (non-MB, CABG, unroofing, untreated, respectively). Cox regression analysis indicated that untreated MB was a significant independent predictor of combined endpoints (hazard ratio: 4.06, 95% confidence interval: 1.60-10.32, p < .001). Moreover, 49 patients underwent coronary artery computed tomography 1 year after surgery. The patency rate of the saphenous vein graft was significantly higher than that of the left internal mammary artery (13.3% vs. 84.2%, p < .001). No MB was detected in the unroofing group. CONCLUSIONS: Surgical MB treatment could be beneficial and performed safely during septal myectomy. Myocardial unroofing is the recommended treatment for MB, and unroofing when technically possible may be preferable for long-term outcomes.

Impact of type 2 diabetes mellitus on mid-term mortality for hypertrophic cardiomyopathy patients who underwent septal myectomy.

BACKGROUND: Type 2 diabetes mellitus is common in cardiovascular disease. It is associated with adverse clinical outcomes for patients who had undergone coronary artery bypass and valve operations. The aim of this study was to evaluate the impact of type 2 diabetes mellitus on the midterm outcomes of patients with hypertrophic cardiomyopathy who underwent septal myectomy. METHODS: We retrospectively analyzed the data of 67 hypertrophic cardiomyopathy patients with type 2 diabetes mellitus who underwent septal myectomy from two medical centers in China from 2011 to 2018. A propensity score-matched cohort of 134 patients without type 2 diabetes mellitus was also analyzed. RESULTS: During a median follow-up of 28.0 (interquartile range: 13.0-3.0) months, 9 patients died. The cause of death of all of these patients was cardiovascular, particularly sudden cardiac death in 3 patients. Patients with type 2 diabetes mellitus had a higher rate of sudden cardiac death (4.5% vs. 0.0%, p = 0.04). The Kaplan-Meier survival analysis revealed that the rates of predicted 3-year survival free from cardiovascular death (98.1% vs. 95.1%, p = 0.14) were similar between the two groups. However, the rates of predicted 3-year survival free from sudden cardiac death (100% vs. 96.7%, p = 0.01) were significantly higher in hypertrophic cardiomyopathy patients without type 2 diabetes mellitus than in those with type 2 diabetes mellitus. Furthermore, after adjustment for age and sex, only N-terminal pro-brain natriuretic peptide (hazards ratio: 1.002, 95% confidence interval: 1.000-1.005, p = 0.02) and glomerular filtration rate ≤ 80 ml/min (hazards ratio: 3.23, 95% confidence interval: 1.34-7.24, p = 0.047) were independent risk factors for hypertrophic cardiomyopathy patients with type 2 diabetes mellitus. CONCLUSIONS: Hypertrophic cardiomyopathy patients with and without type 2 diabetes mellitus have similar 3-year cardiovascular mortality after septal myectomy. However, type 2 diabetes mellitus is associated with higher sudden cardiac death rate in these patients. In addition, N-terminal pro-brain natriuretic peptide and glomerular filtration rate ≤ 80 ml/min were independent risk factors among hypertrophic cardiomyopathy patients with type 2 diabetes mellitus.

Effect of Septal Myectomy on Obstructive Sleep Apnoea Syndrome in Patients With Hypertrophic Obstructive Cardiomyopathy.

BACKGROUND: The prevalence of obstructive sleep apnoea (OSA) is high in patients with hypertrophic cardiomyopathy. The effect of septal myectomy on OSA is not clear. This study aimed to examine the association between hypertrophic obstructive cardiomyopathy and OSA before and after septal myectomy. METHOD: We included 85 consecutive patents with a confirmed diagnosis of hypertrophic obstructive cardiomyopathy who underwent septal myectomy. Polysomnography was performed in all patients before and 3 months after the surgery. RESULTS: Of the 85 patients, 49 (58%) were diagnosed with OSA. Patients with OSA were significantly older than those without OSA. The incidence of atrial fibrillation significantly increased during the perioperative period in patients with OSA (p = 0.03). The severity of OSA significantly increased 3 months after surgery, as determined by the apnoea-hypopnoea index (AHI; p < 0.001), obstructive apnoea index (p = 0.024), and hypopnoea index (p = 0.003), whereas central apnoea index was decreased (p = 0.008). In the multivariate linear regression analysis, mean oxygen desaturation and time% with SpO2 <90% during sleep before surgery were significantly associated with increased AHI, independently of body mass index and sex (p = 0.026 and p = 0.007, respectively; adjusted R2 = 0.365). CONCLUSIONS: The severity of OSA significantly increased 3 months after septal myectomy as determined by AHI, obstructive apnoea index, and hypopnoea index. Mean oxygen saturation and time% with SpO2 <90% during sleep before surgery were independently associated with the increase of AHI. However, the specific mechanism of such deterioration of OSA after septal myectomy needs to be determined in detail.

The co-segregation of the MYL2 R58Q mutation in Chinese hypertrophic cardiomyopathy family and its pathological effect on cardiomyopathy disarray.

Hypertrophic cardiomyopathy (HCM), a major cause of sudden death in youth, is largely affected by genetic factors. The R58Q mutation in the MYL2 gene was identified in some HCM patients and was considered as a deleterious HCM mutation. However, the passing of R58Q between generations along with HCM symptoms was observed only in small families with only two or three members; thus, whether R58Q is as deleterious as previously claimed remains questionable. Here, we reported a large four-generation Chinese family, and found that R58Q existed in all six members with HCM and two healthy juveniles who had not yet developed HCM yet, and presumably in three deceased members who suffered from sudden death. In addition, we also found that compared with other mutations, R58Q had a more severe effect on the cellular level. Therefore, we confirmed that R58Q could be passed from generation to generation along with HCM symptoms and that it was indeed a deleterious mutation for HCM. However, further study is needed to identify additional factors that may determine the various symptoms shown in different family members within the same family.

Plasma big endothelin-1 predicts new-onset atrial fibrillation after surgical septal myectomy in patients with hypertrophic cardiomyopathy.

BACKGROUND: Postoperative atrial fibrillation (POAF) is a common complication in patients with obstructive hypertrophic cardiomyopathy (HOCM) who undergo surgical myectomy. POAF is associated with poor outcome. The role of plasma big endothelin-1 level in predicting atrial fibrillation after surgical septal myectomy in HOCM patients has not well been studied. METHODS: A total of 118 patients with HOCM who underwent surgical septal myectomy were recruited in this study. Plasma big endothelin-1 level was measured. The heart rhythm was continuously monitored during hospital stay. Preoperative, intraoperative, and postoperative variables were collected. RESULTS: POAF developed among 26 of the 118 patients (22%) in this study. Compared with those without POAF, patients with POAF were significantly older (53.5 ± 10.6 vs. 47.3 ± 13.6 years, P = 0.033), more likely to undergo mitral valve surgery (38.5% vs. 18.5%, P = 0.032), and had higher plasma big endothelin-1 levels (0.41 ± 0.19 vs. 0.27 ± 0.14 pmol/l, P = 0.001), longer hospital stay (9.1 ± 3.7 vs. 7.5 ± 2.8 days, P = 0.022), larger preoperative left atria (48.0 ± 5.2 vs. 44.1 ± 5.9 mm; P = 0.003). In the receiver operating characteristic curve analysis, the area under the curve for big endothelin-1 was 0.734 (95% CI, 0.634 to 0.834, P<0.001). In multivariate logistic regression analysis, preoperative big endothelin-1 level (OR 100.7, 95%CI: 5.0-2020.0, P = 0.003) and left atrial diameter (OR 1.106, 95%CI: 1.015-1.205, P = 0.022) were independent predictors of POAF. CONCLUSION: Elevated preoperative plasma big endothelin-1 level is an independent predictor of POAF in HOCM patients undergoing surgical septal myectomy.

Mid-term outcomes of simultaneous coronary artery bypass graft surgery and septal myectomy in patients with hypertrophic obstructive cardiomyopathy: A case-controlled study.

BACKGROUND: The mid-term outcome of patients with hypertrophic obstructive cardiomyopathy (HOCM) undergoing coronary artery bypass graft (CABG) is unclear. MATERIALS AND METHODS: We studied 44 patients with HOCM and coronary artery disease (CAD) who underwent septal myectomy and CABG) between 2011 and 2017. The control group was matched in a ratio of 4:1 based on age, sex, body mass index, hypertension, and chest pain. RESULTS: Compared to patients without CAD, patients with CAD had a higher long-term cardiovascular mortality rate (0.6% vs 6.8%, P = 0.03; hazard ratio [HR] = 8.16, 95% confidence interval [CI]: 1.27-74.48, P = 0.03). In addition, 10 out of 176 (5.7%) patients without CAD and nine out of 44 (20.5%) patients with CAD achieved the secondary endpoints (progressive heart failure, unexplained syncope, stroke, atrial fibrillation, and myocardial infarction) (HR = 2.89, 95%CI: 1.03-8.12, P = 0.04). The 5-year survival rate and cardiovascular event-free survival rate were significantly higher in patients without CAD than in those with CAD (97.4% vs 93.9%, P = 0.03; 89.2% vs 80.1%; P = 0.04). In the multivariate analysis, presence of CAD, New York Heart Association class, and left atrial diameter were predictors of combined cardiovascular events when adjusted for age and male sex. CONCLUSIONS: The cardiovascular death and cardiovascular events are significantly increased in patients with HOCM and CAD who underwent CABG at the time of septal myectomy.

Predictors of long-term outcome after septal myectomy in symptomatic hypertrophic obstructive cardiomyopathy patients with previous alcohol septal ablation and residual obstruction.

BACKGROUND AND AIM: Recently alcohol septal ablation (ASA) has emerged as an alternative treatment for drug-refractory hypertrophic obstructive cardiomyopathy (HOCM) and a subgroup of HOCM patients with previous ASA may need myectomy. However, subsequent outcome and mechanism of residual obstruction has not been determined. This study aims to determine outcome after myectomy and mechanism of residual obstruction in HOCM patients with previous ASA. METHODS: From February 2009 to June 2017, 38 HOCM patients with previous ASA underwent surgical septal myectomy at our institution. Seventy-six patients who underwent surgical septal myectomy initially were included as the comparison group through one-to-two propensity score matching method. RESULTS: Fourteen available cardiac magnetic resonance images revealed inferior location and small area of infarcted myocardium induced by ASA in 12 patients and outside targeted location in two patients. During follow-up (median, 2.4; maximum, 7.8 years), event-free survival at 7 years was 83.2% in the previous ASA group and 94.6% in the comparison group, respectively (P = 0.0378). Multivariable analysis indicated previous ASA (hazard ratio, 4.28; 95% confidence intervals [CI], 1.20-15.26; P = 0.025) and postoperative left ventricular end-diastolic diameter (hazard ratio, 1.14; 95% CI, 1.05-1.23; P = 0.002) were independent predictors of adverse events. CONCLUSIONS: This study demonstrated that uncontrollable extent and location of infarcted myocardium induced by ASA may attribute to residual obstruction after previous ASA, and the long-term event-free survival after myectomy was inferior. It may provide special precaution to patient selection and the increased number of ASA practiced worldwide.

Comparison of Outcomes of Mitral Valve Repair for Leaflet Prolapse with Advanced versus Mild/Moderate Myxomatous Degeneration.

There is limited information on long-term outcomes of mitral valve repair for mitral regurgitation (MR) caused by different degrees of myxomatous degeneration. The aim of this study was to compare the surgical results of patients with advanced and mild/moderate myxomatous mitral valve degeneration (MVD). We identified 130 patients (25 advanced and 105 mild/moderate MVD patients) who underwent mitral valve repair for MR and were pathologically diagnosed as myxomatous degeneration. Follow-up was 100% complete (mean length, 5.1 ± 1.8 years). Survival differed significantly between the advanced and mild/moderate MVD groups (76.0 ± 9.7% versus 95.0 ± 5.4% at 8 years, P < 0.001). The univariate predictors of mortality were advanced myxomatous degeneration, recurrent MR, and early series (surgeries before 2011). The mild/moderate MVD group had higher freedom from a moderate or severe MR rate compared with the advanced MVD group (77.4 ± 4.5% versus 50.5 ± 10.2% at 7 years, P = 0.003). Multivariable Cox analysis revealed advanced myxomatous degeneration and residual MR as independent predictors of recurrent moderate or severe MR. A total of 25 patients (19.2%) had persistent atrial fibrillation (AF) after repair. In multivariate analysis, advanced myxomatous degeneration was found to be an independent predictor of postoperative persistent AF.In conclusion, the long-term outcomes of mitral valve repair in patients with advanced MVD are poorer than in those with mild/moderate MVD. Advanced myxomatous degeneration is an independent predictor of recurrent moderate or severe MR and postoperative persistent AF in MVD patients performing repair, which deserves more attention before and after surgery.

Time and age dependent decrease of NT-proBNP after septal myectomy in hypertrophic obstructive cardiomyopathy.

BACKGROUND: NT-proBNP level is a predictive factor in hypertrophic cardiomyopathy patients, in which left ventricular outflow tract obstruction contributes to an increased NT-proBNP level. However, studies regarding the influence of septal myectomy on NT-proBNP level in hypertrophic obstructive cardiomyopathy are lacking. METHODS: One hundred and eighty-two patients who underwent septal myectomy in Fuwai Hospital from May 2011 to March 2016 and were included in the present study. Preoperative and follow-up NT-proBNP was retrospectively collected. Correlation analysis was performed to determine the factors affecting preoperative NT-proBNP and postoperative decrease. RESULTS: The cohort had a median age of 46.2 [IQR: 36.5-53.1] years, and 106 (58.2%) patients were male. NT-proBNP decreased to 816.5 [IQR: 400.3-1661.8] pg/mL from preoperative 1732.4 [IQR: 819-3296.5] pg/mL (p<0.001). Baseline NT-proBNP was correlated with gender (p<0.001), maximum septal thickness (p<0.001), and resting pressure gradient (p=0.006). The extent of NT-proBNP decrease was positively correlated with age (p<0.001), baseline NT-proBNP (p<0.001), follow-up time (p=0.020), and enlargement of the ascending aorta (p=0.019). NT-proBNP exhibited a persistent decrease after myectomy. CONCLUSIONS: Myectomy significantly reduced NT-proBNP level in hypertrophic obstructive cardiomyopathy patients, in which a time-dependent manner of decrease indicated myocardial remodeling of the heart after myectomy.

Effectiveness of Alcohol Septal Ablation Versus Transaortic Extended Myectomy in Hypertrophic Cardiomyopathy with Midventricular Obstruction.

OBJECTIVES: Investigate the effectiveness of alcohol septal ablation (ASA) and transaortic extended myectomy (TEM) in hypertrophic cardiomyopathy (HCM) with midventricular obstruction (MVO). BACKGROUND: MVO is less common than subaortic obstruction. Data on the effectiveness of ASA and TEM in MVO are lacking. METHODS: The clinical profiles of 22 patients undergoing ASA and 37 patients undergoing TEM were compared. No patient had apical aneurysm, abnormal chordae, mitral valve replacement or repair. RESULTS: Baseline midventricular pressure gradient and symptoms were comparable between the ASA and TEM groups. During follow-up, both groups demonstrated substantial reduction in pressure gradient (the ASA group: 79.7 ± 21.2 mm Hg to 43.7 ± 28.9 mm Hg, P < 0.001; the TEM group: 69.0 ± 23.9 mm Hg to 15.0 ± 16.9 mm Hg, P < 0.001). The reduction in pressure gradient was greater (78.9 ± 18.6% vs. 46.4 ± 33.4%, P < 0.001) and the residual pressure gradient was lower after TEM versus ASA (P < 0.001). Patients with New York Heart Association class III/IV dyspnea decreased from 59.1 to 18.2% (P = 0.022) in the ASA group and from 56.8 to 5.6% (P < 0.001) in the TEM group. Patients with Canadian Cardiovascular Society class III/IV angina decreased from 40.9 to 9.1% (P = 0.016) in the ASA group and from 32.4 to 0% (P < 0.001) in the TEM group. CONCLUSIONS: While ASA and TEM both improve gradients and symptoms, TEM may provide a more reliable reduction in gradients compared to ASA.

[Predictive value of qualitative assessment of general movements for adverse outcomes at 24 months of age in infants with asphyxia].

OJBECTIVE: To investigate the predictive value of the qualitative assessment of general movements (GMs) for adverse outcomes at 24 months of age in full-term infants with asphyxia. METHODS: A total of 114 full-term asphyxiated infants, who were admitted to the neonatal intensive care unit between 2009 and 2012 and took part in follow-ups after discharge were included in the study. All of them received the qualitative assessment of GMs within 3 months after birth. The development quotient was determined with the Bayley Scales of Infant Development at 24 months of age. RESULTS: The results of the qualitative assessment of GMs within 3 months after birth showed that among 114 infants, 20 (17.5%) had poor repertoire movements and 7 (6.1%) had cramped-synchronized movements during the writhing movements period; 8 infants (7.0%) had the absence of fidgety movements during the fidgety movements period. The results of development quotient at 24 months of age showed that 7 infants (6.1%) had adverse developmental outcomes: 6 cases of cerebral palsy and mental retardation and 1 case of mental retardation. There was a poor consistency between poor repertoire movements during the writhing movements period and the developmental outcomes at 24 months of age (Kappa=-0.019; P>0.05). There was a high consistency between cramped-synchronized movements during the writhing movements period and the developmental outcomes at 24 months of age (Kappa=0.848; P<0.05), and the results of predictive values of cramped-synchronized movements were shown as follows: predictive validity 98.2%, sensitivity 85.7%, specificity 99.1%, positive predictive value 85.7%, and negative predictive value 99.1%. There was a high consistency between the absence of fidgety movements during the fidgety movements period and the developmental outcomes at 24 months of age (Kappa=0.786; P<0.05), and its predictive values were expressed as follows: predictive validity 97.4%, sensitivity 85.7%, specificity 98.1%, positive predictive value 75.0%, and negative predictive value 99.1%. CONCLUSIONS: Cramped-synchronized movements and absence of fidgety movements can predict adverse developmental outcomes at 24 months of age in full-term infants with asphyxia.

MiR-451 is decreased in hypertrophic cardiomyopathy and regulates autophagy by targeting TSC1.

The molecular mechanisms that drive the development of cardiac hypertrophy in hypertrophic cardiomyopathy (HCM) remain elusive. Accumulated evidence suggests that microRNAs are essential regulators of cardiac remodelling. We have been suggested that microRNAs could play a role in the process of HCM. To uncover which microRNAs were changed in their expression, microRNA microarrays were performed on heart tissue from HCM patients (n = 7) and from healthy donors (n = 5). Among the 13 microRNAs that were differentially expressed in HCM, miR-451 was the most down-regulated. Ectopic overexpression of miR-451 in neonatal rat cardiomyocytes (NRCM) decreased the cell size, whereas knockdown of endogenous miR-451 increased the cell surface area. Luciferase reporter assay analyses demonstrated that tuberous sclerosis complex 1 (TSC1) was a direct target of miR-451. Overexpression of miR-451 in both HeLa cells and NRCM suppressed the expression of TSC1. Furthermore, TSC1 was significantly up-regulated in HCM myocardia, which correlated with the decreased levels of miR-451. As TSC1 is a known positive regulator of autophagy, we examined the role of miR-451 in the regulation of autophagy. Overexpression of miR-451 in vitro inhibited the formation of the autophagosome. Conversely, miR-451 knockdown accelerated autophagosome formation. Consistently, an increased number of autophagosomes was observed in HCM myocardia, accompanied by up-regulated autophagy markers, and the lipidated form of LC3 and Beclin-1. Taken together, our findings indicate that miR-451 regulates cardiac hypertrophy and cardiac autophagy by targeting TSC1. The down-regulation of miR-451 may contribute to the development of HCM and may be a potential therapeutic target for this disease.