Background Mutations in the lamin A/C gene （LMNA） may cause familial dilated cardiomyopathy （dilated cardiomyopathy） characterized by early onset atrio-ventricular block （A-V block） before the manifestation of d...Background Mutations in the lamin A/C gene （LMNA） may cause familial dilated cardiomyopathy （dilated cardiomyopathy） characterized by early onset atrio-ventricular block （A-V block） before the manifestation of dilated cardiomyopathy and high risk of sudden death due to ventricular arrhythmia, which is very similar to the phenotype of gap junction related heart disease. This study aimed to determine the expression and localization of connexins in neonatal myocytes transfected with wild-type （WT） or mutant LMNA to elucidate how these mutations cause heart diseases. Methods We studied the connexin 43 （Cx43） and connexin 40 （Cx40） expression in cultured neonatal myocytes transfected with wild-type （WT） or mutant LMNA （Glu82Lys （E82K） and Arg644Cys （R644C）） using confocal imaging and Western blotting analysis. Results Cx43 protein expression was reduced by 40% in cells transfected with LMNA E82K than that in cells transfected with WT LMNA cDNA. Confocal imaging showed that the Cx43 located inside the cells by LMNA E82K. By contrast, LMNA E82K mutation had no effect on expression and localization of Cx40. LMNA R644C transfection did not show any significant effects on gap junctions at all. Conclusions Our findings suggest that LMNA E82K significantly reduced the Cx43 expression and altered its localization which may be one of the pathological mechanisms underlying LMNA-related heart disease.展开更多
AIM: To compare the atrio-ventricular(AV/PV) delay optimization by echocardiography and intra-cardiac electrocardiogram(IEGM) based Quick Opt algorithm in complete heart block(CHB) patients, implanted with a dual cham...AIM: To compare the atrio-ventricular(AV/PV) delay optimization by echocardiography and intra-cardiac electrocardiogram(IEGM) based Quick Opt algorithm in complete heart block(CHB) patients, implanted with a dual chamber pacemaker. METHODS: We prospectively enrolled 20 patients(age 59.45 ± 18.1 years; male: 65%) with CHB, who were implanted with a dual chamber pacemaker. The left ventricular outflow tract velocity time-integral was measured after AV/PV delay optimization by both echocardiography and Quick Opt algorithm method. Bland-Altman analysis was used for agreement between the two techniques. RESULTS: The optimal AV and PV delay determined by echocardiography was 155.5 ± 14.68 ms and 122.5 ± 17.73 ms(P < 0.0001), respectively and by Quick Opt method was 167.5 ± 16.73 and 117.5 ms ± 9.10 ms(P < 0.0001), respectively. A good agreement was observed between optimal AV and PV delay as measured by two methods. However, the correlation of the optimal AV(r = 0.0689, P = 0.77) and PV(r = 0.2689, P = 0.25) intervals measured by the two techniques was poor. The time required for AV/PV optimization was 45.26 ± 1.73 min by echocardiography and 0.44 ± 0.08 min by Quick Opt method(P < 0.0001).CONCLUSION: The programmer based IEGM method is an automated, quick, easier and reliable alternative to echocardiography for the optimization of AV/PV delay in CHB patients, implanted with a dual chamber pacemaker.展开更多
Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay ...Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay （AVD）. Methods A total of 72 patients with congestive heart failure （CHF） were randomized to RAAVD LUV pacing versus standard biventricular （BiV） pacing in a 1 ： 1 ratio. Echocardiography was used to optimize AVD for both groups. The effects of sequential BiV pacing and LUV pacing with optimized A-V （right atrio-LV） delay using an RAAVD algorithm were compared. The standard deviation （SD） of the S/R ratio in lead VI at five heart rate （HR） segments （Rs/R-SD5）, defined as the ＂tracking index,＂ was used to evaluate the accuracy of the RAAVD algorithm for tracking physiological AVD. Results TheQRS complex duration （132 ± 9.8 vs. 138± 10ms, P 〈 0.05）, the time required for optimization （21 ±5 vs. 50±8min, P〈 0.001）, the mitral regurgitant area （1.9 ± 1.1 vs. 2.5 ± 1.3 em2, P 〈 0.05）, the interventricular mechanical delay time （60.7 ± 13.3 ms vs. 68.3 ± 14.2 ms, P 〈 0.05）, and the average annual cost （13,200 ± 1000 vs. 21,600 ± 2000 RMB, P 〈 0.001） in the RAAVD LUV pacing group were significantly less than those in the standard BiV pacing group. The aortic valve velocity-time integral in the RAAVD LUV pacing group was greater than that in the standard BiV pacing group （22.7 ± 2.2 vs. 21.4 ± 2.1 cm, P 〈 0.05）. The Rs/R-SD5 was 4.08 ± 1.91 in the RAAVD LUV pacing group, and was significantly negatively correlated with improved left ventricular ejection fraction （LVEF） （ALVEF, Pearson＇s r = -0.427, P = 0.009）, and positively correlated with New York Heart Association class （Spearman＇s r - 0.348, P 0.037）. Conclusions RAAVD LUV pacing is as effective as standard BiV pacing, can be more physiological than standard BiV pacing, and can de- crease the average annual cost of CRT.展开更多
TC-RF （transcatheter radiofrequency） ablation has an important role in the treatment ofSVA （supraventricular arrhythmias）. The indication to TC-RF ablation is usually made to improve the patient＇s quality of life...TC-RF （transcatheter radiofrequency） ablation has an important role in the treatment ofSVA （supraventricular arrhythmias）. The indication to TC-RF ablation is usually made to improve the patient＇s quality of life through the elimination of the arrhythmic substrate. The objective of this study is to make a brief review of the literature and to report the Electrophysiology Unit experience of the Cardiovascular Department of the University Hospital of Trieste. From the study, it can be found that about one thousand of patients are visited annually in the Cardiovascular Department of the University Hospital of Trieste （1,019 ± 71/year over the past five years）. The acute success rate of atrial fibrillation and flutter ablation procedures was respectively 93.9% and 97.9% with a relapse rate of 30% and 10% at 12 month. Accessory pathways and nodal atrio ventricular re-entry tachycardia ablation procedures had an acute success rate of respectively 91.1% and 96.6% with a relapse rate of 10% at 12 months. The overall complication rate was 5%. Catheter ablation is an effective therapy for SVA treatment. It can often be definitive, and it is generally superior to drug therapy, with a low complication rate. The improvement in patients＇ quality of life is associated with a reduced need for access to health services.展开更多
BACKGROUND Treatment of congenitally corrected transposition of great arteries(cc-TGA)with anatomic repair strategy has been considered superior due to restoration of the morphologic left ventricle in the systemic cir...BACKGROUND Treatment of congenitally corrected transposition of great arteries(cc-TGA)with anatomic repair strategy has been considered superior due to restoration of the morphologic left ventricle in the systemic circulation.However,data on long term outcomes are limited to single center reports and include small sample sizes.AIM To perform a systematic review and meta-analysis for observational studies reporting outcomes on anatomic repair for cc-TGA.METHODS MEDLINE and Scopus databases were queried using predefined criteria for reports published till December 31,2017.Studies reporting anatomic repair of minimum 5 cc-TGA patients with at least a 2 year follow up were included.Metaanalysis was performed using Comprehensive meta-analysis v3.0 software.RESULTS Eight hundred and ninety-five patients underwent anatomic repair with a pooled follow-up of 5457.2 patient-years(PY).Pooled estimate for operative mortality was 8.3%[95%confidence interval(CI):6.0%-11.4%].0.2%(CI:0.1%-0.4%)patients required mechanical circulatory support postoperatively and 1.7%(CI:1.1%-2.4%)developed post-operative atrioventricular block requiring a pacemaker.Patients surviving initial surgery had a transplant free survival of 92.5%(CI:89.5%-95.4%)per 100 PY and a low rate of need for pacemaker(0.3/100 PY;CI:0.1-0.4).84.7%patients(CI:79.6%-89.9%)were found to be in New York Heart Association(NYHA)functional class I or II after 100 PY follow up.Total re-intervention rate was 5.3 per 100 PY(CI:3.8-6.8).CONCLUSION Operative mortality with anatomic repair strategy for cc-TGA is high.Despite that,transplant free survival after anatomic repair for cc-TGA patients is highly favorable.Majority of patients maintain NYHA I/II functional class.However,monitoring for burden of re-interventions specific for operation type is very essential.展开更多
文摘Background Mutations in the lamin A/C gene （LMNA） may cause familial dilated cardiomyopathy （dilated cardiomyopathy） characterized by early onset atrio-ventricular block （A-V block） before the manifestation of dilated cardiomyopathy and high risk of sudden death due to ventricular arrhythmia, which is very similar to the phenotype of gap junction related heart disease. This study aimed to determine the expression and localization of connexins in neonatal myocytes transfected with wild-type （WT） or mutant LMNA to elucidate how these mutations cause heart diseases. Methods We studied the connexin 43 （Cx43） and connexin 40 （Cx40） expression in cultured neonatal myocytes transfected with wild-type （WT） or mutant LMNA （Glu82Lys （E82K） and Arg644Cys （R644C）） using confocal imaging and Western blotting analysis. Results Cx43 protein expression was reduced by 40% in cells transfected with LMNA E82K than that in cells transfected with WT LMNA cDNA. Confocal imaging showed that the Cx43 located inside the cells by LMNA E82K. By contrast, LMNA E82K mutation had no effect on expression and localization of Cx40. LMNA R644C transfection did not show any significant effects on gap junctions at all. Conclusions Our findings suggest that LMNA E82K significantly reduced the Cx43 expression and altered its localization which may be one of the pathological mechanisms underlying LMNA-related heart disease.
文摘AIM: To compare the atrio-ventricular(AV/PV) delay optimization by echocardiography and intra-cardiac electrocardiogram(IEGM) based Quick Opt algorithm in complete heart block(CHB) patients, implanted with a dual chamber pacemaker. METHODS: We prospectively enrolled 20 patients(age 59.45 ± 18.1 years; male: 65%) with CHB, who were implanted with a dual chamber pacemaker. The left ventricular outflow tract velocity time-integral was measured after AV/PV delay optimization by both echocardiography and Quick Opt algorithm method. Bland-Altman analysis was used for agreement between the two techniques. RESULTS: The optimal AV and PV delay determined by echocardiography was 155.5 ± 14.68 ms and 122.5 ± 17.73 ms(P < 0.0001), respectively and by Quick Opt method was 167.5 ± 16.73 and 117.5 ms ± 9.10 ms(P < 0.0001), respectively. A good agreement was observed between optimal AV and PV delay as measured by two methods. However, the correlation of the optimal AV(r = 0.0689, P = 0.77) and PV(r = 0.2689, P = 0.25) intervals measured by the two techniques was poor. The time required for AV/PV optimization was 45.26 ± 1.73 min by echocardiography and 0.44 ± 0.08 min by Quick Opt method(P < 0.0001).CONCLUSION: The programmer based IEGM method is an automated, quick, easier and reliable alternative to echocardiography for the optimization of AV/PV delay in CHB patients, implanted with a dual chamber pacemaker.
文摘Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay （AVD）. Methods A total of 72 patients with congestive heart failure （CHF） were randomized to RAAVD LUV pacing versus standard biventricular （BiV） pacing in a 1 ： 1 ratio. Echocardiography was used to optimize AVD for both groups. The effects of sequential BiV pacing and LUV pacing with optimized A-V （right atrio-LV） delay using an RAAVD algorithm were compared. The standard deviation （SD） of the S/R ratio in lead VI at five heart rate （HR） segments （Rs/R-SD5）, defined as the ＂tracking index,＂ was used to evaluate the accuracy of the RAAVD algorithm for tracking physiological AVD. Results TheQRS complex duration （132 ± 9.8 vs. 138± 10ms, P 〈 0.05）, the time required for optimization （21 ±5 vs. 50±8min, P〈 0.001）, the mitral regurgitant area （1.9 ± 1.1 vs. 2.5 ± 1.3 em2, P 〈 0.05）, the interventricular mechanical delay time （60.7 ± 13.3 ms vs. 68.3 ± 14.2 ms, P 〈 0.05）, and the average annual cost （13,200 ± 1000 vs. 21,600 ± 2000 RMB, P 〈 0.001） in the RAAVD LUV pacing group were significantly less than those in the standard BiV pacing group. The aortic valve velocity-time integral in the RAAVD LUV pacing group was greater than that in the standard BiV pacing group （22.7 ± 2.2 vs. 21.4 ± 2.1 cm, P 〈 0.05）. The Rs/R-SD5 was 4.08 ± 1.91 in the RAAVD LUV pacing group, and was significantly negatively correlated with improved left ventricular ejection fraction （LVEF） （ALVEF, Pearson＇s r = -0.427, P = 0.009）, and positively correlated with New York Heart Association class （Spearman＇s r - 0.348, P 0.037）. Conclusions RAAVD LUV pacing is as effective as standard BiV pacing, can be more physiological than standard BiV pacing, and can de- crease the average annual cost of CRT.
文摘TC-RF （transcatheter radiofrequency） ablation has an important role in the treatment ofSVA （supraventricular arrhythmias）. The indication to TC-RF ablation is usually made to improve the patient＇s quality of life through the elimination of the arrhythmic substrate. The objective of this study is to make a brief review of the literature and to report the Electrophysiology Unit experience of the Cardiovascular Department of the University Hospital of Trieste. From the study, it can be found that about one thousand of patients are visited annually in the Cardiovascular Department of the University Hospital of Trieste （1,019 ± 71/year over the past five years）. The acute success rate of atrial fibrillation and flutter ablation procedures was respectively 93.9% and 97.9% with a relapse rate of 30% and 10% at 12 month. Accessory pathways and nodal atrio ventricular re-entry tachycardia ablation procedures had an acute success rate of respectively 91.1% and 96.6% with a relapse rate of 10% at 12 months. The overall complication rate was 5%. Catheter ablation is an effective therapy for SVA treatment. It can often be definitive, and it is generally superior to drug therapy, with a low complication rate. The improvement in patients＇ quality of life is associated with a reduced need for access to health services.
文摘BACKGROUND Treatment of congenitally corrected transposition of great arteries(cc-TGA)with anatomic repair strategy has been considered superior due to restoration of the morphologic left ventricle in the systemic circulation.However,data on long term outcomes are limited to single center reports and include small sample sizes.AIM To perform a systematic review and meta-analysis for observational studies reporting outcomes on anatomic repair for cc-TGA.METHODS MEDLINE and Scopus databases were queried using predefined criteria for reports published till December 31,2017.Studies reporting anatomic repair of minimum 5 cc-TGA patients with at least a 2 year follow up were included.Metaanalysis was performed using Comprehensive meta-analysis v3.0 software.RESULTS Eight hundred and ninety-five patients underwent anatomic repair with a pooled follow-up of 5457.2 patient-years(PY).Pooled estimate for operative mortality was 8.3%[95%confidence interval(CI):6.0%-11.4%].0.2%(CI:0.1%-0.4%)patients required mechanical circulatory support postoperatively and 1.7%(CI:1.1%-2.4%)developed post-operative atrioventricular block requiring a pacemaker.Patients surviving initial surgery had a transplant free survival of 92.5%(CI:89.5%-95.4%)per 100 PY and a low rate of need for pacemaker(0.3/100 PY;CI:0.1-0.4).84.7%patients(CI:79.6%-89.9%)were found to be in New York Heart Association(NYHA)functional class I or II after 100 PY follow up.Total re-intervention rate was 5.3 per 100 PY(CI:3.8-6.8).CONCLUSION Operative mortality with anatomic repair strategy for cc-TGA is high.Despite that,transplant free survival after anatomic repair for cc-TGA patients is highly favorable.Majority of patients maintain NYHA I/II functional class.However,monitoring for burden of re-interventions specific for operation type is very essential.
