Myocardial perfusion abnormality and necrosis in a patient with isolated noncompaction of the ventricular myocardium: evaluation by myocardial perfusion SPECT and magnetic resonance imaging.

A 39-year-old woman presented with left ventricular failure. Coronary angiography revealed normal coronary arteries and endomyocardial biopsy from the right ventricular apex was normal. Myocardial perfusion SPECT revealed no perfusion in the inferior wall segment and mild hypoperfusion in the apico-lateral segment. Cine magnetic resonance imaging showed double-layered appearance on 4-chamber view and marked trabeculations and intratrabecular recesses in the infero-lateral segments on short-axis view, findings consistent with isolated noncompaction of the ventricular myocardium (INVM). Contrast-enhanced imaging revealed hypoperfusion and delayed enhancement of the endomyocardium in the anterior and infero-lateral segments. Myocardial perfusion abnormality and necrosis may constitute the diagnostic criteria of INVM.