BACKGROUND His bundle pacing(HBP)and left bundle branch pacing(LBBP)both provide physiologic pacing which maintain left ventricular synchrony.They both improve heart failure(HF)symptoms in atrial fibrillation(AF)patie...BACKGROUND His bundle pacing(HBP)and left bundle branch pacing(LBBP)both provide physiologic pacing which maintain left ventricular synchrony.They both improve heart failure(HF)symptoms in atrial fibrillation(AF)patients.We aimed to assess the intra-patient comparison of ventricular function and remodeling as well as leads parameters corresponding to two pacing modalities in AF patients referred for pacing in intermediate term.METHODS Uncontrolled tachycardia AF patients with both leads implantation successfully were randomized to either modality.Echocardiographic measurements,New York Heart Association(NYHA)classification,quality-of-life assessments and leads parameters were obtained at baseline and at each 6-month follow up.Left ventricular function including the left ventricular endosystolic volume(LVESV),left ventricular ejection fraction(LVEF)and right ventricular(RV)function quantified by tricuspid annular plane systolic excursion(TAPSE)were all assessed.RESULTS Consecutively twenty-eight patients implanted with both HBP and LBBP leads successfully were enrolled(69.1±8.1 years,53.6% male,LVEF 59.2%±13.7%).The LVESV was improved by both pacing modalities in all patients(n=23)and the LVEF was improved in patients with baseline LVEF at less than 50%(n=6).The TAPSE was improved by HBP but not LBBP(n=23).CONCLUSION In this crossover comparison between HBP and LBBP,LBBP was found to have an equivalent effect on LV function and remodeling but better and more stable parameters in AF patients with uncontrolled ventricular rates referred for atrioventricular node(AVN)ablation.HBP could be preferred in patients with reduced TAPSE at baseline rather than LBBP.展开更多
BACKGROUND Fabry disease(FD)is a rare X-linked lysosomal storage disease caused by a deficiency of the enzymeα-galactosidase A.CASE SUMMARY Herein,we analyzed a four-generation Chinese family.The proband is a 57-year...BACKGROUND Fabry disease(FD)is a rare X-linked lysosomal storage disease caused by a deficiency of the enzymeα-galactosidase A.CASE SUMMARY Herein,we analyzed a four-generation Chinese family.The proband is a 57-yearold woman who was diagnosed with left ventricular hypertrophy and atrial fibrillation 7 years ago.Echocardiography showed an end-diastolic diameter of the interventricular septum of 19.9 mm,left ventricular end-diastolic diameter of 63.1 mm,and moderate-to-severe mitral regurgitation.Cardiac magnetic resonance indicated an enlarged left heart and right atrium,decreased left ventricular systolic and diastolic function,a left ventricular ejection fraction of 20%,and thickening of the left ventricular septum.In March 2019,gene and enzyme activity tests confirmed the diagnosis of FD.Her son was diagnosed with FD after gene and enzyme activity assay,and was prescribed agalsidase-βfor enzyme replacement therapy in July 2020.Two sisters of the proband were also diagnosed with FD by genetic testing.Both of them had a history of atrial fibrillation.CONCLUSION A novel mutation was identified in a Chinese family with FD,in which the male patient had a low level of enzyme activity,early-onset,and severe organ involvement.Comprehensive analysis of clinical phenotype genetic testing and enzyme activity testing helped in the diagnosis and treatment of this FD family.展开更多
基金supported by Medical Science and Technology Project of Zhejiang Province(Grant Number 2020KY220 and 2022506537)the funding from Clinical research project of Zhejiang Medical Association(No.2016ZYC-A28).
文摘BACKGROUND His bundle pacing(HBP)and left bundle branch pacing(LBBP)both provide physiologic pacing which maintain left ventricular synchrony.They both improve heart failure(HF)symptoms in atrial fibrillation(AF)patients.We aimed to assess the intra-patient comparison of ventricular function and remodeling as well as leads parameters corresponding to two pacing modalities in AF patients referred for pacing in intermediate term.METHODS Uncontrolled tachycardia AF patients with both leads implantation successfully were randomized to either modality.Echocardiographic measurements,New York Heart Association(NYHA)classification,quality-of-life assessments and leads parameters were obtained at baseline and at each 6-month follow up.Left ventricular function including the left ventricular endosystolic volume(LVESV),left ventricular ejection fraction(LVEF)and right ventricular(RV)function quantified by tricuspid annular plane systolic excursion(TAPSE)were all assessed.RESULTS Consecutively twenty-eight patients implanted with both HBP and LBBP leads successfully were enrolled(69.1±8.1 years,53.6% male,LVEF 59.2%±13.7%).The LVESV was improved by both pacing modalities in all patients(n=23)and the LVEF was improved in patients with baseline LVEF at less than 50%(n=6).The TAPSE was improved by HBP but not LBBP(n=23).CONCLUSION In this crossover comparison between HBP and LBBP,LBBP was found to have an equivalent effect on LV function and remodeling but better and more stable parameters in AF patients with uncontrolled ventricular rates referred for atrioventricular node(AVN)ablation.HBP could be preferred in patients with reduced TAPSE at baseline rather than LBBP.
基金Supported by Key Research and Development Program of Zhejiang Province,No.2019C03022.
文摘BACKGROUND Fabry disease(FD)is a rare X-linked lysosomal storage disease caused by a deficiency of the enzymeα-galactosidase A.CASE SUMMARY Herein,we analyzed a four-generation Chinese family.The proband is a 57-yearold woman who was diagnosed with left ventricular hypertrophy and atrial fibrillation 7 years ago.Echocardiography showed an end-diastolic diameter of the interventricular septum of 19.9 mm,left ventricular end-diastolic diameter of 63.1 mm,and moderate-to-severe mitral regurgitation.Cardiac magnetic resonance indicated an enlarged left heart and right atrium,decreased left ventricular systolic and diastolic function,a left ventricular ejection fraction of 20%,and thickening of the left ventricular septum.In March 2019,gene and enzyme activity tests confirmed the diagnosis of FD.Her son was diagnosed with FD after gene and enzyme activity assay,and was prescribed agalsidase-βfor enzyme replacement therapy in July 2020.Two sisters of the proband were also diagnosed with FD by genetic testing.Both of them had a history of atrial fibrillation.CONCLUSION A novel mutation was identified in a Chinese family with FD,in which the male patient had a low level of enzyme activity,early-onset,and severe organ involvement.Comprehensive analysis of clinical phenotype genetic testing and enzyme activity testing helped in the diagnosis and treatment of this FD family.
