Myocardial ischaemia in children with isolated ventricular non-compaction.
OBJECTIVE
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.


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.


CONCLUSIONS
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.
D000293:Adolescent; D002648:Child; D003326:Coronary Circulation; D005260:Female; D006321:Heart / Q000000981:diagnostic imaging; D006331:Heart Diseases / Q000151:congenital* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006352:Heart Ventricles; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000473:pathology; D012039:Regional Blood Flow; D014055:Tomography, Emission-Computed; D014463:Ultrasonography; D018754:Ventricular Dysfunction / Q000209:etiology