Clinical features of isolated noncompaction of the ventricular myocardium: Long-term clinical course, hemodynamic properties, and genetic background

Fukiko Ichida, Yuji Hamamichi, Toshio Miyawaki, Yasuo Ono, Tetsuro Kamiya, Teiji Akagi, Hiromichi Hamada, Osamu Hirose, Takeshi Isobe, Katsuhiko Yamada, Shunji Kurotobi, Hiroshi Mito, Toshiharu Miyake, Yasuo Murakami, Takeshi Nishi, Makoto Shinohara, Masashi Seguchi, Shinjiro Tashiro, Hirofumi Tomimatsu

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Abstract

OBJECTIVES: A nationwide survey was conducted to clarify the clinical features of isolated noncompaction of the ventricular myocardium (INVM) in Japanese children in comparison with features previously described in patients with INVM. BACKGROUND: Isolated noncompaction of the ventricular myocardium is a rare disorder characterized by an excessively prominent trabecular meshwork. It is accompanied by depressed ventricular function, systemic embolism and ventricular arrhythmia. METHODS: A questionnaire specifically designed for this study was sent to 150 hospitals in Japan where a pediatric cardiology division exists. RESULTS: Twenty-seven patients were diagnosed by two-dimensional echocardiography, their ages ranging from one week to 15 years at presentation, with follow-up lasting as long as 17 years. The gross anatomical appearance and the extension of noncompacted myocardium predominantly at the apex observed on two-dimensional echocardiograms were similar to observations reported previously. Dissimilarities included a greater number of asymptomatic patients at initial presentation, a longer clinical course with gradually depressed left ventricular function, no systemic embolism, and rare ventricular tachycardia in the Japanese children. Cardiac catheterization disclosed normal left ventricular end-diastolic volume and increased left ventricular end-diastolic pressure in most cases, consistent with restrictive hemodynamics. A higher incidence of Wolff- Parkinson-White syndrome was found in the children, whereas left bundle branch block was rarer than reported in adults. Familial recurrence was high (44%) and included many women. CONCLUSIONS: In Japanese children, INVM can be found by screening examinations at asymptomatic stage, and it might have a longer clinical course with gradually depressed left ventricular function and restrictive hemodynamics. The pattern of familial recurrence we observed implies that INVM is a distinctive clinical entity with a heterogeneous genetic background.

Original languageEnglish
Pages (from-to)233-240
Number of pages8
JournalJournal of the American College of Cardiology
Volume34
Issue number1
DOIs
Publication statusPublished - Jul 1 1999
Externally publishedYes

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ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Ichida, F., Hamamichi, Y., Miyawaki, T., Ono, Y., Kamiya, T., Akagi, T., Hamada, H., Hirose, O., Isobe, T., Yamada, K., Kurotobi, S., Mito, H., Miyake, T., Murakami, Y., Nishi, T., Shinohara, M., Seguchi, M., Tashiro, S., & Tomimatsu, H. (1999). Clinical features of isolated noncompaction of the ventricular myocardium: Long-term clinical course, hemodynamic properties, and genetic background. Journal of the American College of Cardiology, 34(1), 233-240. https://doi.org/10.1016/S0735-1097(99)00170-9