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European Heart Journal 1999 20(12):910-916; doi:10.1053/euhj.1998.1398
Copyright © 1999 by the European Society of Cardiology.
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Myocardial ischaemia in children with isolated ventricular non-compaction

G. Jungaa, S. Kneifelb, A.Von Smekalc, H. Steinertb and U. Bauersfelda,f1

a Division of Cardiology, Children’s University Hospital Zurich, Zurich, Switzerland
b Department of Radiology, University Hospital Zurich, Zurich, Switzerland
c Clinic of Nuclear Medicine, University Hospital Zurich, Zurich, Switzerland

accepted November 4, 1998 1998, Revision submitted 1998

Abstract

Aims Isolated ventricular non-compaction is a rare congenital cardiomyopathy with a high morbidity and mortality due to malignant arrhythmias and pump failure. Areas affected by non-compaction are characterized by increased trabecularization and deep inter-trabecular spaces. We hypothesized perfusion defects in these areas and performed positron emission tomography to evaluate the myocardial perfusion in non-compacted areas.

Methods and Results Five children (age 10–14 years) with isolated ventricular non-compaction underwent positron emission tomography using N-13-ammonia as flow marker and intravenous dipyridamole for stress testing. Myocardial blood flow was quantified using the positron emission tomography time–activity curves in non-compacted areas and normal myocardium, which were diagnosed by echocardiography, magnetic resonance imaging, and angiography. Coronary angiography, performed in two children with extensive forms of left ventricular non-compaction, demonstrated normal coronary arteries. Myocardial blood flow measurements at rest and after dipyridamole application demonstrated 16–33% and 32–57% perfusion impairment, respectively, in non-compacted areas compared to normal myocardium. Areas of restricted myocardial perfusion corresponded well to the non-compacted areas, defined echographically and by magnetic resonance imaging.

Conclusion Positron emission tomography demonstrates restricted myocardial perfusion and decreased flow reserve in areas of ventricular non-compaction in children. The myocardial perfusion defects in non-compacted areas may be the cause of myocardial damage and possibly form the basis of arrhythmias and pump failure.

Key Words: Non-compaction • ischaemia • cardiomyopathy • ventricles • imaging • electrophysiology

f1 Correspondence : Urs Bauersfeld, MD, Division of Cardiology, Children’s University Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

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