Sudden cardiac death in isolated right ventricular hypertrabeculation/noncompaction cardiomyopathy
Sudden cardiac death in isolated right ventricular hypertrabeculation/noncompaction cardiomyopathy
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DOI：
10.1111/anec.12487
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发表时间：
2018-07-01
影响因子：
1.9
通讯作者：
Lafferty, James
中科院分区：
文献类型：
--
作者：
Bekheit, Soad;Karam, Boutros;Lafferty, James
心肌小梁过度形成/致密化不全是一种罕见的疾病，最常见于心脏的左心室，世界卫生组织已将其视为一种独特的心肌病。然而，这种情况只涉及右心室是非常罕见的。我们报告三个病人谁提出了室性快速心律失常和心脏性猝死的情况。超声心动图发现他们有孤立的右心室小梁形成过度/致密化不全。这支持了这一假设，即这种疾病是高度致瘤性的，与高死亡率相关，类似于左心室小梁形成过度/致密化不全心肌病。
Hypertrabeculation/noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/noncompaction on echocardiography. This supports the hypothesis that this condition is highly arrhythmogenic and is associated with high mortality similarly to the left ventricular hypertrabeculation/noncompaction cardiomyopathy.