Objective To explore the application value of echocardiography in the differential diagnosis of Fabry disease and hypertrophic cardiomyopathy (HCM). Methods Baseline data and echocardiographic parameters of Fabry disease patients and HCM patients admitted to the First Affiliated Hospital of Xi’an Jiaotong University between January 2022 and January 2024 were selected and compared between groups. The diagnostic ability for Fabry disease and HCM was analyzed using receiver operating characteristic curves and area under the curve (AUC). Results A total of 16 Fabry disease patients and 41 HCM patients were included. The Fabry disease group had lower age, body mass index, proportion of electrocardiogram abnormalities, and smoking history than the HCM group (P<0.05); the Fabry disease group had a longer medical history than the HCM group (P<0.05). The maximum thickness of the left ventricular myocardium and the ascending aortic diameter in the Fabry group were both smaller than those in the HCM group (P<0.05). The e-peak velocity in the Fabry group was greater than that in the HCM group (P<0.05). For the differential diagnosis of Fabry disease and HCM, the AUC for the e-peak velocity was 0.698 [95% confidence interval (0.502, 0.894), P<0.05], sensitivity was 41.7%, specificity was 100%, and Youden index was 41.7%. When the three factors were combined, both sensitivity and accuracy were significantly higher than the e-peak. The AUC was 0.773 [95% confidence interval (0.585, 0.961), P<0.05], with a sensitivity of 100% and specificity of 45.5%. There were no statistically significant differences in the 2D-speckle tracking imaging echocardiography parameters between the two groups, including global longitudinal strain of the left ventricle, strain of the apical segment, strain of the basal segment, and so on (P>0.05). Conclusion Echocardiography may have certain significance in the diagnosis of Fabry disease and HCM.
ObjectiveTo compare clinical outcomes between modified and traditional Morrow procedures for the treatment of hypertrophic obstructive cardiomyopathy (HOCM). MethodsForty-two HOCM patients undergoing surgical correction in Beijing Anzhen Hospital between January 2005 and July 2011 were recruited in this study. According to different surgical techniques, all the patients were divided into 2 groups. In traditional Morrow procedure group, there were 16 patients including 13 males and 3 females with their age of 49±15 years. In modified Morrow procedure group, there were 26 patients including 14 males and 12 females with their age of 40±18 years. Preoperative and postoperative echocardiography were performed to compare ventricular septal thickness (VST), left ventricular outflow tract velocity (LVOTV)and left ventricular outflow gradient (LVOG)between the 2 groups. ResultsVST, LVOTV and LVOG of HOCM patients were significantly reduced after both traditional and modified Morrow procedure. There was statistical difference in preoperative and postoperative VST (23.10±3.64 mm vs. 17.38±4.39 mm), LVOTV (433.08±101.68 mm/s vs. 248.46±101.88 mm/s)and LVOG (78.57±40.16 mm Hg vs. 4.29±21.52 mm Hg)in traditional Morrow procedure group (P < 0.05). There was statistical difference in preoperative and postoperative VST (25.04±47.05 mm vs. 18.38±6.55 mm, P < 0.05), LVOTV (414.83±83.33 mm/s vs. 159.72±60.84 mm/s, P < 0.05)and LVOG (77.94±29.16 mm Hg vs. 17.56±9.39 mm Hg, P < 0.05)in modified Morrow procedure group (P < 0.05). Preoperative and postoperative difference in LVOG of modified Morrow procedure group was more significant than that of traditional Morrow procedure group (74.25±27.91 mm Hg vs. 34.63±30.66 mm Hg, P < 0.05). ConclusionModified Morrow procedure is superior to traditional Morrow procedure in reducing postoperative LVOG for HOCM patients.
Objective To analyze the long-term outcome of modified Morrow surgery (interventricular septal cardiomyectomy) in the treatment of hypertrophic obstructive cardiomyopathy (HOCM) in children. Methods The clinical data of the children with HOCM (aged≤14 years) who underwent modified Morrow surgery from January 2010 to August 2022 in Guangdong Provincial People's Hospital were retrospectively analyzed, including changes in hospitalization status, perioperative period, and long-term 15-lead electrocardiogram and echocardiography. Results A total of 29 patients were collected, including 22 males and 7 females, aged 10.00 (5.00, 12.00) years. Five (17.9%) patients had New York Heart Association (NYHA) heart function grade Ⅲ or Ⅳ. Ventricular septal cardiomyectomy was performed in all patients. All 29 patients survived and their cardiac function recovered after operation. Before discharge, right bundle branch block was observed in 2 patients and left bundle branch block in 6 patients. After surgery, in the left ventricular septal cardiomyectomy, the left atrial diameter decreased (P＜0.001), left ventricular end-systolic diameter increased (P=0.009), the peak pressure gradient of left ventricular outflow tract decreased (P＜0.001), and the thickness of ventricular septum decreased (P＜0.001). The systolic anterior motion of mitral valve disappeared and mitral regurgitent jet area decreased (P＜0.001). The flow velocity and peak pressure gradient of right ventricular outflow tract also decreased in the patients who underwent right ventricular septal cardiomyectomy. The average follow-up of the patients was 69.03±10.60 months. All the patients survived with their NYHA cardiac function grading Ⅰ or Ⅱ. No new-onset arrythmia event was found. Echocardiography indicated that the peak pressure gradient of the left ventricular outflow tract remained low (P<0.001). Moderate mitral regurgitation occurred in 2 patients, and left ventricular outflow tract obstruction with moderate mitral regurgitation occurred in 1 patient after simple right ventricular septal cardiomyectomy. Conclusion Right ventricular or biventricular obstruction is frequent in the children with HOCM and they usually have more symptoms before surgery. Modified Morrow surgery can effectively relieve outflow tract obstruction and improve their cardiac function. The long-term outcome is satisfactory. However, the posterior wall of the left ventricle remains hypertrophic. Also, there is an increased risk of a conduction block.
ObjectiveTo systematically review clinical efficacy and safety of bone marrow stem cells transplantation in treating primary dilated cardiomyopathy (DCM). MethodsSuch databases as PubMed, CENTRAL, EMbase, Web of Knowledge, VIP, CNKI, CBM and WanFang Data were searched from inception to March 2014 for the randomized controlled trials (RCTs) about bone marrow stem cells transplantation for DCM. According to the inclusion and exclusion criteria, two reviewers independently screened literature, extracted data, and assessed methodological quality of included studies. Then meta-analysis was performed using RevMan 5.2.0 software. ResultsA total of ten RCTs involving 374 patients were included. The results of meta-analysis showed that, a) for safety, after 3 months there was no significant difference in the incidence of malignant arrhythmia events between bone marrow stem cell transplantation group and routine treatment group (RR=0.81, 95%CI 0.38 to 1.72, P=0.58); and b) for efficacy, compared with the control group, left ventricular ejection fraction (LVEF) increased in the bone marrow stem cell transplantation group after 3 months (WMD=3.86, 95% CI 2.53 to 5.20, P<0.000 01) and after 6 months (WMD=5.54, 95%CI 3.02 to 8.06, P<0.000 1). The bone marrow stem cell transplantation group were better in increased 6-minute walking distance after 3 months (WMD=22.12, 95%CI 7.78 to 36.46, P=0.003), increased 6-minute walking distance after 6 months (WMD=102.79, 95%CI 50.16 to 155.41, P=0.000 1), decreased perfusion defect of myocardium percentage after 3 months (WMD=-4.00, 95%CI -5.87 to -2.13, P<0.000 1). However, there was no significant difference in left ventricular end-diastolic diameter (LVEDD) between two groups after 3 months (WMD=-0.37, 95%CI -1.67 to 0.93, P=0.57) and after 6 months (WMD=-0.70, 95%CI -2.76 to 1.36, P=0.51). ConclusionBone marrow stem cells transplantation for dilated cardiomyopathy is effective in improve patients' heart function with good safety, with significant difference. Due to limited quantity and quality of the included studies, more high quality and large-scale RCTs are needed to verify the above conclusion.
【摘要】 目的 观察长期大量酒精摄入对大鼠心肌结构及心肌组织中丙二醛(MDA)、超氧化物歧化酶(SOD)和金属硫蛋白(MT)含量的影响,探讨氧化应激在酒精性心肌病大鼠中的作用。 方法 雄性健康SD大鼠45只,随机分为2组,即对照组20只和模型组25只。模型组酒精浓度从5%、10%、20%和30%依次各自由饮1周,然后递增至36%后以该浓度维持饲喂。对照组每日饮用与模型组酒精同等热量的葡萄糖水。6个月后,观察大鼠心肌组织的形态学改变及超微结构的变化,测定心肌组织中MDA、SOD及MT的含量。结果 模型组大鼠心肌细胞排列紊乱、间质充血、炎细胞浸润、线粒体肿胀、空泡形成、肌丝溶解、核膜不规则和核仁裂解。心肌组织中MDA含量明显升高(Plt;0.01),SOD活力含量明显降低(Plt;0.01),MT含量明显降低(Plt;0.01)。 结论 长期摄入大量酒精可使氧自由基代谢失衡,导致心肌损伤。氧化应激在酒精性心肌病发病机制中发挥着重要的作用。【Abstract】 Objective To observe the effect of longterm and large quantities of alcohol intake on myocardial structure of rats and the content of malondialdehyde (MDA), superoxide dismutase (SOD) and metallothionein (MT) in myocardium tissue. To study the effect of oxidative stress on the rats with alcoholic cardiomyopathy. Methods Fortyfive male and healthy SD rats were randomly divided into the control group (20 rats) and model group (25 rats).The alcoholic concentrate in model group was increased from 5%,10%,20% to 30% every week, and maintain free drinking mass concentration of 36% alcohol. The control group drink the same calories of glucose water. Six months later, the myocardial tissues were observed both in light microscope and electron microscope .The level of MDA、SOD and MT were tested in myocardium tissue. Results In the model rats, the cells of myocardial disarray, interstitial congestion, inflammatory cell infiltration, mitochondrial swelling, vacuole formation, melt filaments, irregular nuclear membrane and nucleolus cracking. The content of MDA incresed(Plt;0.01)and the activities of SOD decreased(Plt;001),levels of MT decreased (Plt;0.01) in the cardiac muscular tissues in the model group compared with the control group. Conclusion Longterm intake of large amounts of alcohol can break the balance of oxygen free radicals, which leading to the damage of myocardial. Oxidative stress plays an important role in the etiopathogenesis of alcoholic cardiomyopathy.
ObjectiveTo analyze the changes in myocardial injury markers and cardiac function in patients with hypertrophic obstructive cardiomyopathy (HOCM) after Liwen surgery. MethodsA retrospective analysis was conducted on the clinical data of HOCM patients who underwent Liwen surgery at the Department of Cardiac Surgery, Wuhan Asia Heart Hospital from December 2019 to April 2023, mainly including preoperative and postoperative dynamic follow-up laboratory test results and echocardiograms. ResultsA total of 42 patients were included, with 25 males and 17 females, aged (44.76±17.72) years, and a postoperative follow-up time of (15.02±6.97) months. The myocardial troponin level of the patients decreased from preoperative 0.03 (0.02, 0.06) ng/mL to postoperative 0.02 (0.01, 0.05) ng/mL (P=0.006), and the N-terminal pro-brain natriuretic peptide level decreased from preoperative 748.95 (337.40, 1600.75) ng/L to postoperative 367.15 (126.93, 1030.25) ng/L (P<0.001). After surgery, the left atrial diameter of the patients decreased from preoperative (4.18±0.57) cm to postoperative (3.93±0.55) cm (P=0.004), the end-diastolic interventricular septum thickness decreased from preoperative 2.25 (1.90, 2.75) cm to postoperative 1.70 (1.50, 1.90) cm (P<0.001), the left ventricular mass index decreased from preoperative 211.73 (172.28, 261.54) g/m2 to postoperative 156.78 (132.34, 191.36) g/m2 (P<0.001), the left ventricular weight decreased from preoperative 368.89 (292.34, 477.72) g to postoperative 266.62 (224.57, 326.04) g (P<0.001), the end-diastolic posterior wall thickness of the left ventricle decreased from preoperative 1.30 (1.20, 1.60) cm to postoperative 1.20 (1.18, 1.40) cm (P<0.001), the relative wall thickness decreased from preoperative 0.78 (0.78, 1.02) to postoperative 0.63 (0.56, 0.72) (P<0.001), the end-systolic inner diameter of the left ventricle increased from preoperative (2.91±0.50) cm to postoperative (3.19±0.53) cm (P=0.001), and the end-diastolic inner diameter of the left ventricle increased from preoperative (4.41±0.48) cm to postoperative (4.66±0.52) cm (P=0.005). The left ventricular outflow diameter increased from preoperative (1.28±0.46) cm to postoperative (1.57±0.32) cm (P=0.001), the left ventricular outflow pressure gradient decreased from preoperative 58.50 (40.75, 92.50) mm Hg to postoperative 11.50 (7.75, 20.50) mm Hg (P<0.001), the left ventricular ejection fraction increased from preoperative 60.00% (56.75%, 65.00%) to postoperative 63.00% (62.00%, 66.00%) (P=0.024), and the degree of systolic anterior motion of the mitral valve leaflets decreased (P＜0.001). ConclusionThe cardiac function of patients with HOCM is improved after Liwen surgery, myocardial injury marker levels are decreased, cardiac reverse remodeling occurres, and the surgical outcome is good.
Sepsis is a common complication after severe trauma, infection, shock and major surgery. It has the characteristics of high morbidity, high mortality, and high hospitalization costs. Septic cardiomyopathy is one of the main causes of death in patients with sepsis. This article reviews the pathogenesis and treatment of septic cardiomyopathy. The pathogenesis includes hemodynamics and myocardial changes, mitochondrial fission, cardiomyocyte apoptosis and autophagy, calcium ion imbalance, inflammation mechanism and immune regulation mechanism. The treatment includes conventional treatment, β1 receptor blocker treatment, melatonin, serotonin 3 receptor antagonist, dexmedetomidine and traditional Chinese medicine treatment, etc., aiming to provide a reference for the diagnosis and treatment of septic cardiomyopathy.
ObjectiveTo 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.MethodsBetween 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.ResultsAll 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 cm vs. 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.ConclusionFor 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.
We tried to explore the value of contrast echocardiography (CEcho) on evaluating hypertrophic cardiomyopathy (HCM) with the inferior wall hypertrophy. A total of 114 patients with HCM were investigated. All the patients received CEcho and routine echocardiography (Echo), and 45 of them received cardiac magnetic resonance (CMR) and 47 of them received Holter. The frequency and percentage of inferior wall hypertrophy were analyzed in HCM patients, as well as the structure and function. The results showed that: (1) Inferior wall hypertrophy was detected in 55 patients (48%) by Echo, while 68 patients (60%) by CEcho. (2) There was no significant difference between CMR and CEcho in the measurement of inferior wall at end-diastole and end-systole. Thickness of inferior wall by CEcho tended to be higher than CMR. However, the inferior wall thickness measured by Echo was obviously lower than that by CMR (P < 0.05) and CEcho ( P < 0.05). (3) Bland-Altman plot suggested good consistency between CEcho and CMR in measuring inferior wall thickness. 95% CI of mean differences in inferior wall thickness between CEcho and CMR were smaller in HCM patients as compared with that between Echo and CMR. Unary linear regression analysis showed good degree of fitting between CEcho and CMR. (4) Holter showed that HCM patients with inferior wall hypertrophy were likely to have higher incidence of premature ventricular complexes (PVC) ≥ 500/24 h. We demonstrate that CEcho is rather sensitive in detecting inferior wall hypertrophy. Echo may underestimate the inferior wall thickness. The risk of ventricular premature beats may increase in HCM patients with inferior hypertrophy.
We reported a 26-year-old male who was diagnosed with apical hypertrophic cardiomyopathy with left ventricular aneurysm. The location of the hypertrophic myocardium and the extent of resection were accurately assessed preoperatively using 3D modeling and printing technology. Myectomy was performed via transapical approach, and the intraoperative exploration was consistent with the description of the preoperative 3D modeling. The patient underwent the surgery successfully without any complications during the hospitalization, and the cardiopulmonary bypass time was 117 min, the aortic cross-clamping time was 57 min, and the hospital stay time was 7 d. The postoperative echocardiography demonstrated left ventricular cavity flow patency. This case provides a reference for the management of patients with apical hypertrophic cardiomyopathy.