BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis case...BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.展开更多
目的探讨缝隙连接开放剂抗心律失常肽(AAP10)对低钾、低镁条件下伊布利特致尖端扭转性室性心动过速(TdP)及心肌电不稳定性的影响。方法 54只日本长耳大白兔随机制备成左室楔形心肌块模型,随机分为对照组、低钾低镁组、伊布利特组、低钾...目的探讨缝隙连接开放剂抗心律失常肽(AAP10)对低钾、低镁条件下伊布利特致尖端扭转性室性心动过速(TdP)及心肌电不稳定性的影响。方法 54只日本长耳大白兔随机制备成左室楔形心肌块模型,随机分为对照组、低钾低镁组、伊布利特组、低钾低镁伊布利特组和AAP10干预组。对照组灌流正常台氏液,低钾低镁组灌流低钾低镁台氏液,伊布利特组灌流浓度2mg/l的伊布利特,低钾低镁伊布利特组灌流溶有同浓度伊布利特的低钾低镁台氏液,AAP10组给予AAP10干预的条件后灌流溶有伊布利特的低钾低镁台氏液。比较各组早期后除极(EAD)、R on T室性早搏和TdP的发生情况,以及QT间期、T波顶点到终点距离(Tp-e)和Tp-e/QT的变化。结果与对照组比较,低钾低镁伊布利特组QT间期、Tp-e和Tp-e/QT延长,EAD、R on T室性早搏、TdP的发生率明显增多。AAP10干预组,TdP、QT间期、Tp-e和Tp-e/QT比低钾、低镁伊布利特组显著减少。结论 AAP10可能通过开放缝隙连接减少跨壁复极离散度,从而起到预防伊布利特在低钾、低镁条件下的致TdP的作用。展开更多
文摘BACKGROUND Sarcoidosis is a rare multisystem disease characterized histologically by noncaseating granuloma formation in the affected organ.While cardiac sarcoidosis is found on autopsy in up to 25%of sarcoidosis cases,it is still underdiagnosed and is associated with a poor prognosis.Although the etiology of sarcoidosis remains unclear,an antigen triggered exaggerated immune response has been hypothesized.Early detection and prompt management of cardiac sarcoidosis remains pivotal.CASE SUMMARY A 60-year-old female,with pulmonary sarcoidosis in remission,presented to the cardiology outpatient clinic for evaluation of weeks-long dyspnea on moderate exertion(New York Heart Association class II)that was relieved by rest.Submaximal exercise stress test showed multifocal ventricular extrasystoles,followed by a self-limiting torsades de pointes.Cardiac magnetic resonance imaging showed nondilated and normotrophic left ventricle with basoseptal and mid-septal dyskinesis.The magnetic resonance imaging-derived left ventricular ejection fraction was 45%.Delayed enhancement showed patchy transmural fibrosis of the septum and hyperenhancement of the papillary muscles,all in favor of extensive cardiac involvement of sarcoidosis.A double-chamber implantable cardiac defibrillator was implanted,and methylprednisolone(12 mg/d)and methotrexate(12.5 mg/wk)treatment was initiated.Follow-up and implantable cardiac defibrillator interrogation showed episodes of asymptomatic nonsustained ventricular tachycardia and an asymptomatic episode of nonsustained ventricular tachycardia ending by the first antitachycardia pacing run.CONCLUSION Along an extensive review of the literature,this unusual case report highlights the importance of early detection of cardiac involvement of sarcoidosis,in order to avoid potential complications and increase survival.
文摘目的探讨缝隙连接开放剂抗心律失常肽(AAP10)对低钾、低镁条件下伊布利特致尖端扭转性室性心动过速(TdP)及心肌电不稳定性的影响。方法 54只日本长耳大白兔随机制备成左室楔形心肌块模型,随机分为对照组、低钾低镁组、伊布利特组、低钾低镁伊布利特组和AAP10干预组。对照组灌流正常台氏液,低钾低镁组灌流低钾低镁台氏液,伊布利特组灌流浓度2mg/l的伊布利特,低钾低镁伊布利特组灌流溶有同浓度伊布利特的低钾低镁台氏液,AAP10组给予AAP10干预的条件后灌流溶有伊布利特的低钾低镁台氏液。比较各组早期后除极(EAD)、R on T室性早搏和TdP的发生情况,以及QT间期、T波顶点到终点距离(Tp-e)和Tp-e/QT的变化。结果与对照组比较,低钾低镁伊布利特组QT间期、Tp-e和Tp-e/QT延长,EAD、R on T室性早搏、TdP的发生率明显增多。AAP10干预组,TdP、QT间期、Tp-e和Tp-e/QT比低钾、低镁伊布利特组显著减少。结论 AAP10可能通过开放缝隙连接减少跨壁复极离散度,从而起到预防伊布利特在低钾、低镁条件下的致TdP的作用。
