RIGHT VENTRICULAR DYSPLASIA - A REPORT OF 24 ADULT CASES
RIGHT VENTRICULAR DYSPLASIA - A REPORT OF 24 ADULT CASES
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DOI：
10.1161/01.cir.65.2.384
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发表时间：
1982-01-01
期刊：
影响因子：
37.8
通讯作者：
GROSGOGEAT, Y
中科院分区：
文献类型：
--
作者：
MARCUS, FI;FONTAINE, GH;GROSGOGEAT, Y
右心室发育不良的特征是部分右心室肌肉组织发育异常。右心室发育不良患者可能会出现室性心动过速、室上性心律失常、右心衰竭或无症状心脏肥大。在 7 年期间观察到患有右心室发育不良且反复出现室性心动过速的成年患者 (22)。男女比例为2.7：1。住院时的平均年龄为 39 岁。除 1 名患者外，所有患者均出现左束支传导阻滞构型的室性心动过速。除少数例外，T 波在右心前导联上倒置。心脏通常增大，肺血管系统通常正常。 6 名接受二维超声心动图检查的患者均显示右心室舒张期尺寸增大。所有患者均进行右心室造影； 12 名患者的右心室发育不良诊断在手术期间得到证实，另一名患者则在尸检中得到证实。另外两名没有心律失常的患者通过右心和左心血管造影诊断为右心室发育不良。
Right ventricular dysplasia is characterized by an abnormality in the development of part of the right ventricular musculature. Patients with right ventricular dysplasia may present with ventricular tachycardia, supraventricular arrhythmias, right heart failure or asymptomatic cardiomegaly. Adult patients (22) with right ventricular dysplasia who had recurrent ventricular tachycardia were seen during a 7 yr period. The male/female ratio was 2.7:1. The mean age at the time of hospitalization was 39 yr. All but 1 of the patients had ventricular tachycardia of a left bundle branch block configuration. With few exceptions, the T waves were inverted over the right precordial leads. The heart was usually enlarged and the pulmonary vasculature was usually normal. In 6 patients who had 2-dimensional echocardiograms, all showed increased right ventricular diastolic dimensions. All patients had right ventricular angiography; the diagnosis of right ventricular dysplasia was substantiated during surgery in 12 patients and at autopsy in another. Two other patients who did not have arrhythmias had right ventricular dysplasia diagnosed by right and left heart angiography.