Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy?
Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy?
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致心律失常性右心室发育不良：全身性心肌病？
作者：
D. Manyari;G. Klein;S. Gulamhusein;D. Boughner;G. Guiraudon;G. Wyse;L. Mitchell;W. Kostuk
心律失常性右心室发育不良（ARVD）是最近被描述的以右心室肌病改变和右心室心动过速为特征的实体。arvd患者是否存在左心室功能障碍尚不清楚。我们通过超声心动图和放射性核素血管心动图评估了6例在运动中站立的arvd患者的右心室和左心室功能和大小。所有患者均有左束支阻滞形态的复发性室性心动过速，4例患者经心内膜标测证实室性心动过速起源于右心室。结果与10名正常受试者和5名服用胺碘- 1的沃尔夫-帕金森-怀特综合征患者进行了比较。后一组为对照组，因为我们没有对arvd患者进行胺碘酮治疗。arvd住院患者的右心室射血分数（EF）平均（+ SD）为25+ 11%，运动期间为26 + 12%。不正常组运动时右心室ef51 + 4%，运动时59.6% （p . 0.05）。异常受试者在休息时左心室ef61 + 4%，运动时为72.5% (p。05)正常受试者。我们得出结论，arvd住院患者以右室功能障碍为主，但潜伏性左室功能障碍比通常认为的更常见。这些发现可能具有重要的诊断和治疗意义。68年循环。2251 - 257年,1983年。
Arrhythmogenic right ventricular dysplasia (ARVD)isa recently described entity characterized byright ventricular myopathic changes andright ventricular tachycardia. Thepresenceor extentofleft ventricular dysfunction inARVD isnotknown. We assessed right ventricular andleft ventricular function andsize insixpatients withARVDbyechocardiography andradionuclide angio- cardiography doneinpatients atrestandduring exercise. Allpatients hadrecurrent ventricular tachycardia ofleft bundle branch blockmorphology, andright ventricular origin oftheventricular tachycardia was confirmed byendocardial mapping infourpatients. Theresults werecompared with those of10normal subjects andfive patients withWolff-Parkinson-White syndrome taking amiodar- one.Thelatter group was a control group,since we didnotwithhold amiodarone therapy infour patients withARVD.Mean(+ SD)right ventricular ejection fraction (EF)inpatients withARVD was 25+ 11%atrestand26 12%during exercise. Innormal subjects right ventricular EFwas 51+ 4% atrestand59 6% during exercise (p .05). Innormal subjects, left ventricular EFwas 61+ 4%atrest and72 5%during exercise (p .05) tothose of normal subjects. We conclude that right ventricular dysfunction predominates inpatients withARVD butlatent left ventricular dysfunction ispresent more often thaniscommonly recognized. These findings may haveimportant diagnostic andtherapeutic implications. Circulation 68,No.2,251-257, 1983.