Two pediatric cases of isolated non-compaction of the ventricular myocardium were found to be associated with long QT syndrome and KCNH2 mutations.
Case Report (n=2)
No
This report provides the first documented evidence of an association between isolated non-compaction of the ventricular myocardium and long QT syndrome linked to the KCNH2 mutation.
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 et al. (2009) conducted a case report in Isolated non-compaction of the ventricular myocardium (INCVM) with long QT syndrome (LQTS) (n=2). Isolated non-compaction of the ventricular myocardium with long QT syndrome was evaluated on Clinical presentation and genetic analysis. Two pediatric cases of isolated non-compaction of the ventricular myocardium were found to be associated with long QT syndrome and KCNH2 mutations.