Isolated non-compaction of the ventricular myocardium associated with long QT syndrome: a report of 2 cases.
Isolated non-compaction of the ventricular myocardium (INCVM) is a relatively newly defined clinicopathologic entity. INCVM is clinically accompanied by depressed ventricular function, arrhythmias, and systemic embolization. We report two cases of INCVM with long QT syndrome (LQTS). In both cases the QT interval was over 0.55 s with episodes of torsades de pointes, and prominent ventricular trabeculations and deep intertrabecular recesses were detected by 2-dimensional echocardiography. Both cases had the KCNH2 mutation. To the best of our knowledge, this is the first report investigating INCVM with LQTS.
Ogawa
Kiyoshi
K
Division of Cardiology, Saitama Children's Medical Center, Saitama, Japan.
Nakamura
Yoshihiro
Y
Terano
Kazuhiro
K
Ando
Tatsuya
T
Hishitani
Takashi
T
Hoshino
Kenji
K
D002675:Child, Preschool; D000072237:ERG1 Potassium Channel; D004452:Echocardiography; D004562:Electrocardiography; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D008133:Long QT Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D008297:Male; D009154:Mutation; D016171:Torsades de Pointes / Q000150:complications
D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C000606913:KCNH2 protein, human