Isolated noncompaction of the ventricular myocardium: Ultrafast computed tomography and magnetic resonance imaging

This study was undertaken to evaluate the feasibility of ultrafast computed tomography (CT) and magnetic resonance imaging (MRI) for anatomical and pa thophysiological diagnosis of isolated noncompaction of the left ventricula r myocardium (INVM) compared with other imaging modalities including thalli um myocardial imaging. Six patients, three sets of siblings, ranging in age from 13 to 18 years, were included in this study. Two-dimensional echocard iograms revealed numerous prominent trabeculations and deep intertrabecular recesses in one or more ventricular wall segments in all cases. Thallium-2 01 myocardial imaging disclosed a hypoperfusion area corresponding to the z ones where noncompacted ventricular myocardium was localized. Ultrafast CT showed early defects of varying degrees and rate enhancement of the noncomp acted ventricular myocardium, implying fibrosis in this area. MRI disclosed inner zones of noncompacted myocardium distinguishable from thin outer zon es of compacted myocardium. T2-weighted imaging revealed high intensity are as at the apex of the left ventricle, suggesting disturbed microcirculation due to fibrosis, thrombus formation, and hypokinesis. Cine MRI revealed hy pokinesis of the noncompacted ventricular wall during the cardiac cycle. In conclusion, ultrafast CT and MRI provide high-resolution imaging of noncom pacted myocardium, and also pathophysiological details regarding this rare disease.