[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.

Analysis and evaluation of patient-specific three-dimensional printing in complex septal myectomy.

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.

[Interpretation of new concepts and approaches in the ESICM guidelines on acute respiratory distress syndrome: definition, phenotyping and respiratory support strategies].

Acute respiratory distress syndrome (ARDS) continues to be one of the most life-threatening conditions for patients in the intensive care unit (ICU). The 2023 European Society of Intensive Care Medicine guidelines on ARDS: definition, phenotyping and respiratory support strategies (2023 Guideline) update the 2017 An Official American Thoracic Society/European Society of Intensive Care Medicine/Society of Critical Care Medicine clinical practice guideline: mechanical ventilation in adult patients with ARDS (2017 Guideline), including 7 aspects of 3 topics of definitions, phenotyping, and respiratory support strategies [including high flow nasal cannula oxygen (HFNO), non-invasive ventilation (NIV), neuromuscular blocking agents (NMBA), extracorporeal life support (ECLS), positive end-expiratory pressure (PEEP) with recruitment maneuvers (RM), tidal volume (VT), and prone positioning]. 2023 Guideline review and summarize the literature since the publication of the 2017 Guideline, covering ARDS and acute hypoxemic respiratory failure, as well as ARDS caused by novel coronavirus infection. Based on the most recent medical evidence, the 2023 Guideline provide clinicians with new ideas and approaches for nonpharmacologic respiratory support strategies for adults with ARDS. This article provides interpretation of the new concepts, the new approaches, the new recommended grading and new levels of evidence for ARDS in the 2023 Guideline.

Mitral annular calcification in obstructive hypertrophic cardiomyopathy: Incidence, risk factors, and prognostic value after myectomy.

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.

High-Burden Premature Atrial Contractions Predict New-Onset Atrial Fibrillation After Surgical Septal Myectomy.

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.

Atrial high-rate episode burden and stroke risks for patients with device-detected subclinical atrial fibrillation: A systematic review and meta-analysis.

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.

Clinical Efficacy and Safety of Cox-Maze IV Procedure for Atrial Fibrillation in Patients With Hypertrophic Obstructive Cardiomyopathy.

Objective: Atrial fibrillation is the most prevalent persistent arrhythmia in patients with hypertrophic obstructive cardiomyopathy. Comparative analyses of the safety and effectiveness of septal myectomy with and without surgical ablation are limited. This study aimed to compare the outcomes of septal myectomy with and without the Cox-maze IV procedure in patients with hypertrophic obstructive cardiomyopathy and atrial fibrillation. Methods: Ninety-four patients with hypertrophic obstructive cardiomyopathy and atrial fibrillation who underwent septal myectomy were analyzed, we divided it into concomitant Cox maze surgery (Cox-maze group) and no concomitant Cox maze operation (no Cox-maze group). Freedom from atrial fibrillation recurrence and all-cause mortality after surgery were assessed. Results: Freedom from all-cause mortality after septal myectomy at 1, 3, and 5 years was 98.5 ± 1.5% each in the Cox-maze group and 90.8 ± 6.3%, 85.1 ± 8.1%, and 85.1 ± 8.1%, respectively, in the no Cox-maze group. Patients in the no Cox-maze group had lower survival, freedom from atrial fibrillation recurrence off antiarrhythmic drugs, and arrhythmia control rate (including patients with successful antiarrhythmic drug conversion) than those in the Cox-maze group (P = 0.046, P = 0.040, and P = 0.012, respectively). Patients who underwent the Cox-maze IV procedure had lower atrial fibrillation recurrence rate than those who did not (hazard ratio, 0.141; 95% confidence interval, 0.042-0.479; P = 0.002). Post-operative increases in left atrial diameters (hazard ratio, 1.099; 95% confidence interval, 1.024-1.179; P = 0.009) were associated with atrial fibrillation recurrence. Conclusions: The Cox-maze IV procedure combined with septal myectomy improved mid-term survival and reduced mid-term atrial fibrillation recurrence in patients with hypertrophic obstructive cardiomyopathy and atrial fibrillation. The concomitant Cox-maze IV procedure was associated with a lower atrial fibrillation recurrence in patients with surgical hypertrophic obstructive cardiomyopathy and atrial fibrillation.

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.

Risk Factors of Pulmonary Arterial Hypertension and Its Relationship With Atrial Fibrillation in Patients With Obstructive Hypertrophic Cardiomyopathy.

Background: Pulmonary arterial hypertension (PH) is a common complication in patients with obstructive hypertrophic cardiomyopathy (OHCM). The risk factor of PH in patients with OHCM has not been fully elucidated, and even atrial fibrillation (AF) was considered a risk factor of PH. Thus, our study aimed to investigate risk factors of PH and the relationship between PH and AF in patients with OHCM. Methods: We retrospectively enrolled 483 consecutive patients diagnosed with OHCM at Fuwai Hospital (Beijing, China) from January 2015 to December 2017. Clinical and echocardiographic parameters were compared between patients with and without PH. Results: Eighty-two (17.0%) patients were diagnosed with PH in this study. Compared to patients without PH, those with PH were significantly older, had a lower body mass index (BMI), were more likely to be female and more symptomatic [New York Heart Association Class 3 or 4 symptoms], and had a higher AF prevalence. A multivariate analysis indicated that AF was an independent risk factor of PH (odds ratio [OR] 2.31, 95% confidence interval [CI] 1.03-5.20, p = 0.042). Moreover, PH was independently associated with a higher AF incidence after adjusting for age and left atrial diameter (OR 2.24, 95% CI 1.07-4.72, p = 0.034). Conclusion: AF was independently associated with PH in patients with OHCM. Further, PH was significantly associated with an increased risk of AF, which suggested that AF could aggravate PH and that PH may promote AF processes, forming a vicious circle.

Proliferative Vascular Smooth Muscle Cells Stimulate Extracellular Matrix Production via Osteopontin/p38 MAPK Signaling Pathway.

INTRODUCTION: Extracellular matrix disorder and cellular phenotype transformation are the major histopathological features associated with ascending aortic aneurysms. Rare studies have investigated the relationship between cellular phenotype transformation and the abnormalities of the matrix constituents. In this study, we investigated whether the cellular phenotype transformation resulted in the extracellular matrix disorder. METHODS: Aortic samples were obtained from 20 patients undergoing operations for ascending aortic aneurysms. Control aortic samples were obtained from 15 patients who underwent coronary artery bypass graft. The protein levels of osteopontin (OPN), collagen, and elastin were examined using Western blot, and quantitative reverse transcriptase-PCR was used to analyze the mRNA expression of collagen and elastin. In vitro experiment, vascular smooth muscle cells (VSMCs) were treated with recombinant human OPN (rh-OPN) or p38 MAPK inhibitor (SB203580) to investigate whether OPN and p38 MAPK regulated the expression of collagen and elastin. RESULTS: The protein level of OPN and collagen III increased in ascending aortic aneurysm samples, compared with controls (p < 0.05). There was no difference in the protein level of elastin between aneurysm tissues and the controls. VSMCs treated with rh-OPN increased the collagen III and elastin protein level and mRNA expression (p < 0.05). Cells treated with SB203580 decreased the collagen III and elastin protein level and mRNA expression (p < 0.05). Furthermore, VSMCs incubated with SB203580 reduced the rh-OPN-induced production of collagen III and elastin (p < 0.05). CONCLUSION: OPN, the proliferative VSMCs maker, increased the expression of extracellular matrix. OPN/p38 MAPK signaling pathways may protect against ascending aortic aneurysm progression.

The preoperative glomerular filtration rate predicts new-onset postoperative atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy who undergo isolated septal myectomy.

BACKGROUND: Few studies have focused on new-onset postoperative atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy who have undergone septal myectomy. Therefore, we investigated the incidence and prognosis effects of postoperative atrial fibrillation following septal myectomy in patients with hypertensive obstructive cardiomyopathy. Additionally, we investigated the relationship of estimated glomerular filtration rate and postoperative atrial fibrillation. METHODS: Data from 300 patients with hypertrophic obstructive cardiomyopathy who underwent isolated surgical septal myectomy were collected from January 2012 to March 2018. RESULTS: The overall incidence of postoperative atrial fibrillation during hospitalization was 22.67% (68 of 300 patients). Patients with postoperative atrial fibrillation were older (P<0.001), had lower preoperative estimated glomerular filtration rate (P<0.001), and a larger preoperative left atrial diameter (P=0.038) compared to patients without. The preoperative estimated glomerular filtration rate predicted postoperative atrial fibrillation with sensitivity and specificity of 0.824 and 0.578 (P<0.001), respectively. Multivariate regression analyses showed that age [odds ratio (OR) =1.090, 95% confidence interval (CI): 1.034-1.110], an New York Heart Association functional class ≥ III (OR =2.985, 95% CI: 1.349-6.604), hypertension (OR =2.212, 95% CI: 1.062-4.608), a history of syncope (OR =3.890, 95% CI: 1.741-8.692), and the preoperative estimated glomerular filtration rate (OR =0.981, 95% CI: 0.965-0.996) were independent risk factors associated in the development of postoperative atrial fibrillation. Survival analysis showed that the incidence of long-term cardiovascular events was higher in the patients with postoperative atrial fibrillation than that in the patients without the condition (P<0.001). CONCLUSIONS: The preoperative estimated glomerular filtration rate was a moderate predictor of postoperative atrial fibrillation after septal myectomy. Postoperative atrial fibrillation affected the early recovery and the long-term prognoses of patients with hypertrophic obstructive cardiomyopathy who underwent septal myectomy.

Mid-ventricular obstruction is associated with non-sustained ventricular tachycardia in patients with hypertrophic obstructive cardiomyopathy.

BACKGROUND: Mid-ventricular obstruction (MVO) is a rare subtype of hypertrophic cardiomyopathy (HCM) but it is associated with ventricular arrhythmia. The relationship between MVO and non-sustained ventricular tachycardia (NSVT) in HCM patients is unknown. HYPOTHESIS: The severity of MVO increases the incidence of NSVT in patients with hypertrophic obstructive cardiomyopathy (HOCM). METHODS: Five hundred and seventy-two consecutive patients diagnosed with HOCM in Fuwai Hospital between January 2015 and December 2017 were enrolled in this study. Holter electrocardiographic and clinical parameters were compared between HOCM patients with and without MVO. RESULTS: Seventy-six (13.3%) of 572 patients were diagnosed with MVO. Compared to patients without MVO, those with MVO were much younger, and had a higher incidence of syncope, greater left ventricular (LV) posterior wall thickness, a higher percentage of LV late gadolinium enhancement, and higher prevalence of NSVT. Furthermore, the prevalence of NSVT increased with the severity of MVO (without, mild, moderate or severe: 11.1%, 18.2%, 25.6%, respectively, p for trend < .01). Similarly, the prevalence of NSVT differed among patients with isolated LV outflow tract (LVOTO), both MVO and LVOTO, and isolated MVO (11.1%, 21.3%, 26.6%, respectively, p for trend = .018). In addition to age, diabetes, left atrial diameter, and maximal wall thickness, multivariate analysis revealed the presence of MVO as an independent risk factor for NSVT (Odds ratio 2.69; 95% confidence interval 1.41 to 5.13, p = .003). CONCLUSIONS: The presence and severity of MVO was associated with higher incidence of NSVT in HOCM patients.

Prevalence and characteristics of intramural coronary artery in hypertrophic obstructive cardiomyopathy: a coronary computed tomography and invasive angiography study.

BACKGROUND: The prevalence and morphologic characteristics of intramural coronary artery (ICA) in patients with hypertrophic obstructive cardiomyopathy (HOCM) have yet to be fully illuminated. Our study aimed to investigate the prevalence and morphologic characteristics of ICA in patients with HOCM using coronary computed tomography (CT) angiography and invasive coronary angiography. METHODS: Patients with a diagnosis of HOCM who were admitted for selective myectomy in Fuwai Hospital were prospectively enrolled between September 2015 and June 2019. Both preoperative coronary CT and invasive angiography were scheduled for all participants. RESULTS: Coronary CT angiography detected ICA in 106 (23.3%) out of 455 patients. Dynamic compression of coronary arteries was observed in 87 patients (19.1%) by invasive coronary angiography. We found ICA covered with complete myocardial encasement in 98 patients (92.5%), with deep myocardial bridging (MB) observed most frequently (P=0.005). All patients with dynamic compression of coronary arteries had ICA. Dynamic luminal reduction ≥50% was present in 77 (16.9%) of the study participants. Pearson's correlation analysis revealed that the length and degree of dynamic compression were significantly related with MB length and depth (Pearson's correlation r=0.241, 0.581, 0.316, and 0.209; P=0.014, <0.001, 0.002, and 0.032, respectively). CONCLUSIONS: Patients with HOCM commonly present with ICA and it can be visualized well by coronary CT angiography. Deep or extensive MB is more likely to produce coronary artery dynamic compression. Preoperative identification of this congenital coronary artery variant may be helpful for surgical planning.

Childhood Hypertrophic Obstructive Cardiomyopathy and Its Relevant Surgical Outcome.

BACKGROUND: Contemporary experiences regarding childhood hypertrophic obstructive cardiomyopathy are limited. This study aimed to describe the clinical presentation of childhood hypertrophic obstructive cardiomyopathy and its relevant surgical outcome. METHODS: In all, 117 consecutive children with hypertrophic obstructive cardiomyopathy aged 0.6 to 17.5 years who underwent septal myectomy at our institution between February 2009 and December 2018 were included. Medical records and other patient-related data were reviewed. RESULTS: In the present study, the anatomic and physiologic characteristics of childhood hypertrophic obstructive cardiomyopathy were highly heterogeneous, with simultaneous right ventricular outflow tract obstruction in 22 patients (18.8%), coronary myocardial bridging in 25 patients (21.4%), and intraventricular anatomic abnormalities in 61 patients (52.1%). The mean peak left or right ventricular outflow tract gradient, interventricular septal thickness, and degree of mitral regurgitation significantly decreased after surgery. One early death was noted in the study. During follow-up, three sudden cardiac deaths were noted. The overall survival rates at follow-up were 100% at 1 year and 96.5% at 3 years. The overall survival rates free from reoperation were 99.1% at 1 year and 98.0% at 3 years. CONCLUSIONS: In our cohort of children with hypertrophic obstructive cardiomyopathy undergoing septal myectomy, biventricular obstruction, myocardial bridging, and intraventricular anatomic abnormalities are frequent phenotypic components. Despite the complexity of childhood hypertrophic obstructive cardiomyopathy, surgical treatment results in a favorable outcome in carefully selected patients.

Stop codons and the +4 nucleotide may influence the efficiency of G418 in rescuing nonsense mutations of the HERG gene.

The importance of the local sequence context in determining how efficiently aminoglycosides rescue nonsense mutations has been established previously in disease models. Different stop codons appear to facilitate the termination process with differing efficiencies. Furthermore, the efficiency with which termination is suppressed may also be influenced by the local sequence context surrounding the stop codon. The strongest bias has usually been identified with the nucleotide base that immediately follows the stop codon in the majority of experiments. However, how the sequence context influences the efficiency of aminoglycosides in rescuing the human ether­a­go­go­related (HERG) protein in mammalian cells remains to be fully elucidated. Therefore, the present study was devised to examine the susceptibility of different termination codons on the HERG gene and the +4 nucleotide immediately following them to be suppressed by aminoglycosides in 293 cells. The 293 cells were transiently transfected with the wild­type or mutant genes. The read­through effect was subsequently examined by adding aminoglycoside G418 into the culture medium, followed by incubation of the cells for 24 h. An immunofluorescence method was then used to observe the protein expression of HERG prior to and following drug treatment. Patch clamping was performed to evaluate the function of the HERG protein. These experiments revealed that stop codons TGA and TAA in the R1014X mutant were more susceptible to treatment with the drug G418. Similar results were observed with the W927X­TGA and W927X­TAA mutants. Subsequently, R1014X­TGAC, R1014X­TGAG and R1014X­TGAA mutants were constructed based on the R1014X­TGAT mutant. The level of red fluorescence was observed prior to and following the administration of G418 using antibodies targeting the N­ or C­terminus of the HERG protein. However, the tail current density was found only to increase with the R1014X­TGAT mutant following G418 treatment. Taken together, the results of the present study suggest that the type of premature stop codon and the context of the nucleotide immediately following at the +4 position, may determine the pharmacological rescue efficiency of the HERG gene.

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.

Result of surgical treatment of hypertrophic obstructive cardiomyopathy with coronary heart disease / 中国胸心血管外科临床杂志

@#Objective To summarize the perioperative management strategies and early results of modified Morrow expanded operation and coronary artery bypass grafting (CABG) in patients with hypertrophic obstructive cardiomyopathy (HOCM) and coronary atherosclerotic heart disease. Methods Between January 2012 and December 2017, in the Second Inpatient Department of Fuwai Hospital, 32 patients (20 females and 12 males) underwent modified expanded Morrow operation and CABG. The median age was 53.7±8.7 years (interquartile range 37 to 67 years). Preoperative chest distress symptom was found in 24 patients, chest pain symptom was found in 14 patients, history of syncope in 6 patients. Cardiac echocardiography, electrocardiogram, chest X-ray, magnectic resonance imaging (MRI) were performed routinely after operation and follow-up to analyze structure and function of heart and mitral valve. Results All patients underwent modified and expanded Morrow combined with CABG. The preoperative left ventricular outflow tract peak pressure difference (LVOTG) was 40 to 152 (79.6±28.7) mm Hg. Four patients underwent myocardial bridge releasing in the same period, mitral valve replacement in 2 patients, mitral valve angioplasty in 3 patients, Maze operation in 2 patients and tricuspid valveoplasty in 3 patients. There was no hospital mortality. CABG surgery in patients with branches included anterior descending artery in 26 patients, diagonal branch in 16 patients, left circumflex in 8 patients, right coronary artery in 11 patients. There were 15 patients with one coronary artery (CA) bypass graft, 5 patients with two CA bypass grafts, and 12 patients with 3 CA bypass grafts. The average of CA bypass grafts was 1.9±0.6. The postoperative ICU time ranged from 1–13 (4.1±2.8) days and postoperative hospital stay ranged from 7 to 30 (12.6±5.5) days. No severe postoperative complications were found and 1 patient had postoperative incision healing. The postoperative new arrhythmia included left bundle branch block in 6 patients. Compared with the preoperative values, postoperative left ventricular outflow tract peak pressure (79.6±28.7 mm Hg vs. 10.8±5.9 mm Hg, P<0.001), interventricular septum thickness (1.9±0.4 cmvs. 1.3±0.5 cm, P<0.001) were decreased obviously. Mitral valve closure is good or only mild reflux, mitral valve forward movement (SAM sign) disappeared. The patients were followed up for 6-68 months, with an average of 38.8±20.6 months. All patients were followed up with symptoms disappeared or only mild symptoms. NYHA classification decreased Ⅰ to Ⅱ grade after surgery, without long-term mortality, complications or reoperation. Conclusion For patients with hypertrophic obstructive cardiomyopathy with coronary atherosclerotic heart disease, the application of improved expand morrow operation at the same time undergoing coronary artery bypass grafting is safe. It can significantly improve patients' survival and reduce symptoms, play a synergistic effect, and do not increase the patient's surgical complications.

Risk factors of prolonged ventilation in adults after atrioventricular septal defect operation / 中国胸心血管外科临床杂志

@#Objective To investigate the risk factors of prolonged postoperative mechanical ventilation for adult patients with atrioventricular septal defect (AVSD). Methods We retrospectively analyzed the clinical data of 76 patients with AVSD aged more than 18 years in our hospital from January 1, 2011 to December 31, 2017. The patients ventilated longer than 24 hours were described as a prolonged ventilation group (n=27) and the others as a normal group (n=49). There were 9 males and 18 females aged 32.22±9.64 years in the prolonged ventilation group, and 16 males and 33 females aged 35.98±11.34 years in the normal group. Perioperative variables between the two groups were compared and selected, and then analyzed by logistic regression analysis. Results The result of univariate analysis showed that there was a statistical difference in weight, preoperative pulmonary artery systolic pressure, duration of cardiopulmonary bypass, the level of postoperative platelet, hemoglobin, blood glucose, lactic acid and serum creatinine, postoperative maximum heart rate and postoperative infection rate between the prolonged ventilated group and the normal group. Multivarable logistic regression showed that preoperative pulmonary artery hypertension (OR=1.056, 95%CI 1.005 to 1.110, P=0.030), prolonged duration of cardiopulmonary bypass (OR=1.036, 95%CI 1.007 to 1.066, P=0.016) and the low postoperative hemoglobin level (OR=0.874, 95%CI 0.786 to 0.973, P=0.014) were the risk factors of prolonged postoperative mechanical ventilation. Conclusion Preoperative pulmonary artery hypertension, long duration of cardiopulmonary bypass and postoperative anaemia are the risk factors associated with prolonged postoperative mechanical ventilation.

Clinical effects of pulmonary valve replacement after tetralogy of Fallot repair: A systematic review and meta-analysis / 中国胸心血管外科临床杂志

@#Objective    To evaluate the clinical outcomes of  pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF) after re-PVR surgery. Methods    PubMed, EMbase, the Cochrane Controlled Trials Register databases, CNKI, CBM disc and VIP datebases were searched, and study eligibility and data abstraction were determined independently and in duplicate. Literature searches from database establishment to December 2018. The heterogeneity and data were analyzed by the software of Stata 11.0. Results    Of 4 831 studies identified, 26 studies met eligibility criteria, and invovled with a total of 3 613 patients. The combined 30-day mortality for PVR was 2.2% (95% CI 1.5%-3.1%) and follow-up mortality was 3.4% (95% CI 2.4%-4.9%), re-PVR rate was 6.8% (95% CI 5.1%-9.2%), and the rate of intervention was 11.4% (95% CI 8.0%-16.4%). Subgroup analysis showed that the patient's age range may be a heterogeneous source of mortality during the follow-up period, and there was no statistical heterogeneity for adult patients (P=0.63, I2=0%), with a lower incidence than those including adolescents patients. The type of valve was likely to be a source of retrospective PVR. There was no statistical heterogeneity in bioprosthetic valves and allograft lobes (P=0.24, I2=25%). And the incidence of re-PVR was lower than that of the mechanical valve patients. Heart function classification (NYHA) of patients with TOF after PVR was statistically improved (P<0.05). Electrocardiogram QRS change was not statistically differently (P>0.05). Postoperative MRI findings showed a decrease in RVEDV, an increase in RVEF, a decrease in RV/LV ratio, and a decrease in pulmonary valve (all P<0.05). Funnel map monitoring, Begg test and Egger's test both indicated that there was no publication bias. Conclusions    According to the results of the analysis, PVR after TOF surgery is a more mature surgery, the clinical effect was significant, with lower early and long-term mortality. The long-term mortality rate of adolescent patients undergoing PVR is higher than that of adult patients. Long-term outocme of re-PVR or re-intervention is still the main problem affecting the effect of the operation. Indications for surgery and choice of valve need further investigation.