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A 65 year old man was admitted with syncope. An ECG in the emergency department revealed ventricular tachycardia with haemodynamic compromise necessitating electrical cardioversion. Serum electrolytes and post-cardioversion ECG were normal. Two dimensional transthoracic echocardiography showed moderate concentric left ventricular hypertrophy with normal systolic function (panels A, B). Cardiac catheterisation showed no evidence of obstructive coronary artery disease. The left ventriculogram was peculiar with a double wall appearance to the left ventricular free wall (panel C). A cardiac biopsy was performed at the same time, which showed normal histology. Having identified the abnormal left ventriculogram, the patient underwent real time three dimensional echocardiography (RT3DE) to study the left ventricle in detail (panels D, E). This revealed severe trabeculation of the left ventricular wall, particularly in the apical–lateral segments. Contrast enhanced RT3DE (panels F, G) demonstrated large amounts of filling defects, consistent with extensive trabeculation in the same area. These findings were consistent with non-compaction of the left ventricle (NCLV). The patient was commenced on a β blocker and an angiotensin converting enzyme inhibitor, and a cardiac defibrillator was implanted.
This is the first case to report the value of three dimensional echocardiography in the diagnosis of NCLV, an unusual cause of malignant ventricular tachycardia that may be underdiagnosed on two dimensional echocardiography. Moreover, RT3DE allows assessment of any left ventricular dyssynchrony causing heart failure, a recognised complication of NCLV, which helps in planning treatment such as biventricular pacing. These make RT3DE a single superior investigation modality for both the diagnosis and assessment of treatment of LVNC.
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