Isolated Non-Compaction of the Ventricular Myocardium Associated With Long QT Syndrome A Report of 2 Cases

Kiyoshi Ogawa; Yoshihiro Nakamura; Kazuhiro Terano; Tatsuya Ando; Takashi Hishitani; Kenji Hoshino

doi:10.1253/circj.CJ-08-0339

This article has now been updated. Please use the final version.

Isolated Non-Compaction of the Ventricular Myocardium Associated With Long QT Syndrome

A Report of 2 Cases

Kiyoshi Ogawa, Yoshihiro Nakamura, Kazuhiro Terano, Tatsuya Ando, Takashi Hishitani, Kenji Hoshino

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Keywords: Cardiomyopathy, Long QT syndrome, Myocardium

JOURNAL FREE ACCESS Advance online publication

Article ID: CJ-08-0339

DOI https://doi.org/10.1253/circj.CJ-08-0339

The final version of this article is now available: Vol. 73 (2009) , No. 11 p.2169

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Abstract

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.

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