Bekheit Soad, Karam Boutros, Daneshvar Farshid, Zaidan Julie, Tabet Rabih, Spagnola Jonathan, Lafferty James
Department of Cardiovascular Medicine and Electrophysiology, Staten Island University Hospital, Northwell Health, Staten Island, NY, USA.
Department of Internal Medicine, Staten Island University Hospital, Northwell Health, Staten Island, NY, USA.
Ann Noninvasive Electrocardiol. 2018 Jul;23(4):e12487. doi: 10.1111/anec.12487. Epub 2017 Sep 12.
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.
心肌小梁增多/心肌致密化不全是一种罕见的疾病,最常累及心脏左心室,世界卫生组织已将其认定为一种独特的心肌病。然而,这种情况仅累及右心室极为罕见。我们报告了3例出现室性快速心律失常和心源性猝死的患者。经超声心动图检查发现,他们均存在孤立性右心室小梁增多/心肌致密化不全。这支持了以下假说:与左心室小梁增多/心肌致密化不全性心肌病一样,这种情况具有高度致心律失常性且与高死亡率相关。