目的通过对心肌肥厚兔模型进行电生理研究,探讨心肌肥厚对心传导系统的影响。方法雄性新西兰大耳兔24只,随机分为心肌肥厚组和对照组,每组各12只。经耳缘静脉注射异丙肾上腺素制备心肌肥厚模型。造模2周后行超声检查评价造模结果。对所...目的通过对心肌肥厚兔模型进行电生理研究,探讨心肌肥厚对心传导系统的影响。方法雄性新西兰大耳兔24只,随机分为心肌肥厚组和对照组,每组各12只。经耳缘静脉注射异丙肾上腺素制备心肌肥厚模型。造模2周后行超声检查评价造模结果。对所有动物行在体电生理检查,测量两组动物心房有效不应期(AERP)、房室结有效不应期(AVERP)、房室结反向传导有效不应期(VAERP)、窦房传导时间(SACT)、最大窦房恢复时间(SNRTMAX)、文氏周期长度(WCL),反文氏周期长度(RWCL)、房室2∶1传导频率等指标。结果心肌肥厚组AERP较对照组缩短(68.60±6.20 ms vs 77.30±12.30 ms,P<0.05),SACT、SNRTMAX、AVERP、WCL及房室2:1传导频率较对照组延长(分别为101.57±25.11 ms vs 78.33±25.17 ms,616.91±59.89 ms vs 540.64±83.18 ms,160.30±9.73 ms vs 133.80±3.89 ms,191.40±32.90 ms vs 151.10±24.30 ms,148.8±35.00 ms vs 137.30±23.40 ms,P均<0.05),而VAERP与RWCL较对照组则无明显变化(P>0.05)。结论心肌肥厚可导致心传导系统功能受损,为心肌肥厚时心律失常的发生提供条件。展开更多
经皮酒精间隔消融（ASA）是一种用于缓解梗阻性肥厚型心肌病患者难治性症状的已确立技术。多数患者在行ASA后出现右束支传导阻滞（RBBB），但是尚不清楚出现RBBB的患者是否具有与无RBBB患者类似的梗死特征，梗死特征可能会影响左心室（L...经皮酒精间隔消融（ASA）是一种用于缓解梗阻性肥厚型心肌病患者难治性症状的已确立技术。多数患者在行ASA后出现右束支传导阻滞（RBBB），但是尚不清楚出现RBBB的患者是否具有与无RBBB患者类似的梗死特征，梗死特征可能会影响左心室（LV）压力阶差降低和逆转重构。纳入连续27例患者（15例男性，12例女性，年龄62±16岁），分别在基线时和ASA后1个月、6个月（n=25）时进行心电图和心脏磁共振成像检查。1个月时根据延迟对比增强心脏磁共振成像确定梗死区大小和位置。与无RBBB的患者相比，17例出现RBBB的患者有梗死面积较大的倾向（CK-MB251±92U vs 148±97U，P=0．03；展开更多
1病例介绍患者,男性,22岁。既往无相关病史及体征,体检心电图提示:窦性心动缓、完全性右束支传导阻滞、右室高电压(提示右室大)。患者自诉既往无冠心病、先天性心脏病病史,家族病史不详。体格检查:血压124/63 mm Hg,颈静脉未见怒张,心...1病例介绍患者,男性,22岁。既往无相关病史及体征,体检心电图提示:窦性心动缓、完全性右束支传导阻滞、右室高电压(提示右室大)。患者自诉既往无冠心病、先天性心脏病病史,家族病史不详。体格检查:血压124/63 mm Hg,颈静脉未见怒张,心界不大,心率70次/min,律齐,未闻及病理性杂音。实验室检查:血常规、尿常规、肝功能二项正常。X线胸片示:两肺未见实质性病变,心脏横径不大,右心缘饱满,膈面光滑,展开更多
OBJECTIVES: The aim of this study was to investigate the clinical expression of adenosine monophosphate-activated protein kinase(AMPK) gene mutations(PRKAG2) in adenosine monophosphate(AMP) kinase disease based on 12 ...OBJECTIVES: The aim of this study was to investigate the clinical expression of adenosine monophosphate-activated protein kinase(AMPK) gene mutations(PRKAG2) in adenosine monophosphate(AMP) kinase disease based on 12 years follow-up of known mutation carriers and to define the prevalence of PRKAG2 mutations in hypertrophic cardiomyopathy(HCM). BACKGROUND:Adenosine mono-phosphate-activated protein kinase genemutations cause HCM withWolff-Parkinson-White syndrome and conduction disease. METHODS: Clinical evaluation of 44 patients with known AMP kinase disease was analyzed. Mutation analysis of PRKAG2 was performed by fluorescent single-strand confirmation polymorphism analysis and direct sequencing of abnormal conformers in 200 patients with HCM. RESULTS: Only one additional mutation was identified. The mean age at clinical diagnosis in the 45 gene carriers was 24 years(median 20 years, range 9 to 55 years). Symptoms of palpitation, dypspnea, chest pain, or syncope were present in 31(69%) gene carriers; 7(15%) complained of myalgia and had clinical evidence of proximal myopathy. Skeletal muscle biopsy showed excess mitochondria and ragged red fibers with minimal glycogen accumulation. Disease penetrance defined by typical electrocardiogram abnormalities was 100%by age 18 years. Thirty-two of 41 adults(78%) had left ventricular hypertrophy(LVH) on echocardiography, and progressive LVH was documented during follow-up. Survival was 91%at a mean follow-up of 12.2 years. Progressive conduction disease required pacemaker implantation in 17 of 45(38%) at a mean age of 38 years. CONCLUSIONS: The AMP kinase disease is uncommon in HCM and is characterized by progressive conduction disease and cardiac hypertrophy and includes extracardiac manifestations such as a skeletal myopathy, consistent with a systemic metabolic storage disease. Defects in adenosine triphosphate utilization or in specific cellular substrates, rather than mere passive deposition of amylopectin, may account for these clinical features.展开更多
文摘目的通过对心肌肥厚兔模型进行电生理研究,探讨心肌肥厚对心传导系统的影响。方法雄性新西兰大耳兔24只,随机分为心肌肥厚组和对照组,每组各12只。经耳缘静脉注射异丙肾上腺素制备心肌肥厚模型。造模2周后行超声检查评价造模结果。对所有动物行在体电生理检查,测量两组动物心房有效不应期(AERP)、房室结有效不应期(AVERP)、房室结反向传导有效不应期(VAERP)、窦房传导时间(SACT)、最大窦房恢复时间(SNRTMAX)、文氏周期长度(WCL),反文氏周期长度(RWCL)、房室2∶1传导频率等指标。结果心肌肥厚组AERP较对照组缩短(68.60±6.20 ms vs 77.30±12.30 ms,P<0.05),SACT、SNRTMAX、AVERP、WCL及房室2:1传导频率较对照组延长(分别为101.57±25.11 ms vs 78.33±25.17 ms,616.91±59.89 ms vs 540.64±83.18 ms,160.30±9.73 ms vs 133.80±3.89 ms,191.40±32.90 ms vs 151.10±24.30 ms,148.8±35.00 ms vs 137.30±23.40 ms,P均<0.05),而VAERP与RWCL较对照组则无明显变化(P>0.05)。结论心肌肥厚可导致心传导系统功能受损,为心肌肥厚时心律失常的发生提供条件。
文摘经皮酒精间隔消融（ASA）是一种用于缓解梗阻性肥厚型心肌病患者难治性症状的已确立技术。多数患者在行ASA后出现右束支传导阻滞（RBBB），但是尚不清楚出现RBBB的患者是否具有与无RBBB患者类似的梗死特征，梗死特征可能会影响左心室（LV）压力阶差降低和逆转重构。纳入连续27例患者（15例男性，12例女性，年龄62±16岁），分别在基线时和ASA后1个月、6个月（n=25）时进行心电图和心脏磁共振成像检查。1个月时根据延迟对比增强心脏磁共振成像确定梗死区大小和位置。与无RBBB的患者相比，17例出现RBBB的患者有梗死面积较大的倾向（CK-MB251±92U vs 148±97U，P=0．03；
文摘1病例介绍患者,男性,22岁。既往无相关病史及体征,体检心电图提示:窦性心动缓、完全性右束支传导阻滞、右室高电压(提示右室大)。患者自诉既往无冠心病、先天性心脏病病史,家族病史不详。体格检查:血压124/63 mm Hg,颈静脉未见怒张,心界不大,心率70次/min,律齐,未闻及病理性杂音。实验室检查:血常规、尿常规、肝功能二项正常。X线胸片示:两肺未见实质性病变,心脏横径不大,右心缘饱满,膈面光滑,
文摘OBJECTIVES: The aim of this study was to investigate the clinical expression of adenosine monophosphate-activated protein kinase(AMPK) gene mutations(PRKAG2) in adenosine monophosphate(AMP) kinase disease based on 12 years follow-up of known mutation carriers and to define the prevalence of PRKAG2 mutations in hypertrophic cardiomyopathy(HCM). BACKGROUND:Adenosine mono-phosphate-activated protein kinase genemutations cause HCM withWolff-Parkinson-White syndrome and conduction disease. METHODS: Clinical evaluation of 44 patients with known AMP kinase disease was analyzed. Mutation analysis of PRKAG2 was performed by fluorescent single-strand confirmation polymorphism analysis and direct sequencing of abnormal conformers in 200 patients with HCM. RESULTS: Only one additional mutation was identified. The mean age at clinical diagnosis in the 45 gene carriers was 24 years(median 20 years, range 9 to 55 years). Symptoms of palpitation, dypspnea, chest pain, or syncope were present in 31(69%) gene carriers; 7(15%) complained of myalgia and had clinical evidence of proximal myopathy. Skeletal muscle biopsy showed excess mitochondria and ragged red fibers with minimal glycogen accumulation. Disease penetrance defined by typical electrocardiogram abnormalities was 100%by age 18 years. Thirty-two of 41 adults(78%) had left ventricular hypertrophy(LVH) on echocardiography, and progressive LVH was documented during follow-up. Survival was 91%at a mean follow-up of 12.2 years. Progressive conduction disease required pacemaker implantation in 17 of 45(38%) at a mean age of 38 years. CONCLUSIONS: The AMP kinase disease is uncommon in HCM and is characterized by progressive conduction disease and cardiac hypertrophy and includes extracardiac manifestations such as a skeletal myopathy, consistent with a systemic metabolic storage disease. Defects in adenosine triphosphate utilization or in specific cellular substrates, rather than mere passive deposition of amylopectin, may account for these clinical features.
