Isolated left ventricular abnormal trabeculation in adults is associated with neuromuscular disorders

Background: Isolated left ventricular abnormal trabeculation (ILVAT) is def ined as > 3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. Hypothesis: The aims of the study were to assess by echocardiography the pr evalence of ILVAT, to confirm the diagnosis by cardiac magnetic resonance i maging (CMRI), to look for cardiac findings in ILVAT, and to determine whet her ILVAT is familial and associated with neurological disorders. Methods: During a 1-year period (July 1995 to July 1996) all patients in wh om transthoracic echocardiography visualized ILVAT, were included in the st udy. The examination and measurements were performed according to establish ed guidelines. Results: During 1 year, ILVAT was found in 6 of 3,397 patients (0.2%). When applied, CMRI confirmed ILVAT. Four patients had heart failure, all had el ectrocardiographic (ECG) abnormalities. None of the investigated relatives showed ILVAT. One patient had Becker's muscular dystrophy, three had mitoch ondrial myopathy, one had polyneuropathy, and one had muscle wasting of unk nown origin. Conclusions: Isolated left ventricular abnormal trabeculation is rare, visi ble on echocardiography and CMRI, associated with ECG abnormalities, someti mes with heart failure, and always with neuromuscular disorders. Thus, when ILVAT is found, the cardiologist should consider a neurology referral.