CLINICAL STUDIES
Clinical features of isolated noncompaction of the ventricular myocardium
Long-term clinical course, hemodynamic properties, and genetic background
Fukiko Ichida, MD*,
Yuji Hamamichi, MD*,
Toshio Miyawaki, MD*,
Yasuo Ono, MD ,
Tetsuro Kamiya, MD,
Teiji Akagi, MD ,
Hiromichi Hamada, MD ,
Osamu Hirose, MD||,
Takeshi Isobe, MD¶,
Katsuhiko Yamada, MD#,
Shunji Kurotobi, MD**,
Hiroshi Mito, MD,
Toshiharu Miyake, MD,
Yasuo Murakami, MD,
Takeshi Nishi, MD||||,
Makoto Shinohara, MD¶¶,
Masashi Seguchi, MD##,
Shinjiro Tashiro, MD*** and
Hirofumi Tomimatsu, MD
* Department of Pediatrics, Toyama Medical and Pharmaceutical University, Toyama, Japan
Department of Pediatrics, National Cardiovascular Center, Suita, Japan
Department of Pediatrics, Kurume University, Kurume, Japan
Department of Pediatrics, Chiba University, Chiba, Japan
|| Department of Pediatrics, Matsuyama Red Cross Hospital, Matsuyama, Japan
¶ Department of Pediatrics, Ibaraki Children’s Hospital, Mito, Japan
# Department of Pediatrics, Oita Medical Univesity, Oita, Japan
** Department of Pediatrics, Osaka University, Suita, Japan
Department of Pediatrics, Saiseikai Yamaguchi General Hospital, Yamaguchi, Japan
Department of Pediatrics, Kinki University, Sayama, Japan
Department of Pediatrics, Sakakibara Memorial Hospital, Tokyo, Japan
|||| Department of Pediatrics, Hiroshima Municipal General Hospital, Hiroshima, Japan
¶¶ Department of Pediatrics, Gunma Children’s Medical Center, Gunma, Japan
## Department of Pediatrics, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
*** Department of Pediatrics, Miyazaki Medical College, Miyazaki, Japan
Department of Pediatrics, Tokyo Women’s Medical College, Tokyo, Japan
Manuscript received June 19, 1998;
revised manuscript received February 17, 1999,
accepted March 24, 1999.
Reprint requests and correspondence: Dr. Fukiko Ichida, Department of Pediatrics, Toyama Medical and Pharmaceutical University, 2630 Sugitani, Toyama 930-0194 Japan.
fukiko{at}ms.toyama-mpu.ac.jp
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.
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Abbreviations and Acronyms
| | A-V block | = atrioventricular block | | DCM | = dilated cardiomyopathy | | ECG | = electrocardiogram | | INVM | = isolated noncompaction of the ventricular myocardium | | LBBB | = left bundle branch block | | PSVT | = paroxysmal supraventricular tachycardia | | PVC | = premature ventricular contraction | | RBBB | = right bundle branch block | | RCM | = restrictive cardiomyopathy | | WPW | = Wolff-Parkinson-White |
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