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A 66 year old man presented with a one year history of atypical chest pain. The physical examination was normal, the ECG showed sinus rhythm and left anterior fascicular block, and the exercise test was normal. Two echocardiograms were performed, demonstrating an apical obliteration, which led to suspicion of endomyocardial fibrosis or apical tumour. Magnetic resonance imaging (MRI) was undertaken, which showed a typical pattern of an apical inner zone of non-compacted myocardium (NCM) distinguished from the thin outer zones of compacted myocardium (panels A, B). Late enhancement with gadolinium was also normal. The echocardiogram with second harmonic imaging was repeated (panel C) with a more detailed evaluation of the left ventricular apex, revealing trabeculations with intertrabecular recesses perfused from the left ventricular cavity (panel D). The left ventricle presented with mild dilation with mild globally depressed systolic function, and mild central mitral regurgitation. The pulsed wave tissue Doppler of septal mitral annulus showed an E’ peak velocity of 5.9 cm/s, characterising impaired relaxation, and E/E’ of 6.6, demonstrating a normal left ventricular end diastolic pressure. Screening showed one sister with the same echocardiographic pattern and one brother with two parallel false tendons extended between the interventricular septum and lateral wall.
Although echocardiography is the most frequently used first line diagnostic tool for NCM, sometimes the diagnosis is uncertain. Thus, in patients with a ventricular mass, low scale colour flow mapping demonstrates blood flow through deep recesses in continuity with the ventricular cavity. In addition, other imaging modality such as MRI, transoesophageal echocardiography, or contrast enhanced echocardiography may be performed to confirm the diagnosis.
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