641 Left ventricular non-compaction as a distinct cardiomyopathy or a phenotypic feature: a case report

Abstract

Abstract A 71-year-old male with no cardiovascular risk factors and a history of two uninvestigated episodes of loss of consciousness one year before, presented in our department to undergo a treadmill exercise stress test in relation to inverted T waves in V5-V6 on his basal ECG and frequent ventricular extrasystoles (PVCs). The stress test was positive for inducible ischemia, with a significant depression of the ST-segment in the lateral lead. A transthoracic echocardiography revealed normal thicknesses and volumes of the left ventricle (LV) with a dyskinesia of the lateral wall and a mildly reduced ejection fraction (EF) up to 46%. Epicardial surface was unremarkable. Next, a coronary angiography showed no critical obstruction in the epicardial coronary arteries. In order to further investigate the frequent PVCs and the reduction of the LV systolic function, a cardiac magnetic resonance (CMR) with paramagnetic contrast agent was performed. The images outlined the presence of non-compaction in the distal lateral wall and apex with a late gadolinium enhancement (LGE) in the epicardial portion of the lateral wall (Figure 1). Moreover, the CMR showed an adipose infiltration of the interventricular septum and thickened right ventricular trabeculae, suggesting an overlap with arrhythmogenic left ventricular dysplasia. During the subsequent follow-up, the patient was implanted with an implantable cardiac defibrillator (ICD), after two reported episodes of loss of consciousness and an electrophysiology study (EPS) resulting in the induction of a symptomatic ventricular tachycardia. Non-compaction cardiomyopathy is a rare and still relatively novel nosological entity, which can sometimes be observed in the same patient with other forms of cardiomyopathy. In the context of an unexplained reduction of LVEF and frequent PVCs, the accuracy of CMR in describing cardiac morphology and myocardial layer remains an indispensable tool to detect otherwise underdiagnosed potentially fatal cardiomyopathies.