Increasing identification of isolated left ventricular non-compaction with cardiovascular magnetic resonance: a mini case series highlighting variable clinical presentation.

Background: Isolated left ventricular non-compaction (IVNC) was a previously rarely diagnosed condition with an apparent very poor prognosis. However, with high quality cardiac imaging with cardiac magnetic resonance (CMR) becoming increasingly available, it is being increasingly identified. However, asymptomatic patients with a serendipitous diagnosis of IVNC are being discovered, and their prognosis is less clear. We present our consecutive case series of four patients with this condition, and discuss the implications of their clinical presentation in context of the current literature.

Methods: Four patients in whom a CMR diagnosis of IVNC was established are presented. All CMR studies were performed on a Siemens Sonata 1.5T MRI scanner and phased array surface coil. ECG gated trueFISP (fast image with steady state precision) images were performed and analysed off-line with proprietary software. Where transthoracic echocardiography (TTE) had been performed it was retrospectively re-assessed for the diagnosis.

Results: In none of the four patients was a diagnosis of IVNC confirmed or even suspected until the CMR study. Two of the four patients were asymptomatic and identified serendipitously; aged 21 and 72 years, respectively. The other two presented for the investigation of ventricular dysrhythmias, and one of these had had a previous (incorrect) diagnosis of hypertrophic non-obstructive cardiomyopathy.

Conclusions: Isolated left ventricular non-compaction is becoming increasingly identified and the initial poor prognosis described may not represent the true natural history of this condition. The long-term outlook for this asymptomatic cohort of patients with IVNC is unknown, but it may not be as sinister as first thought.