Isolated Left Ventricular Abnormal Trabeculation Is a Cardiac Manifestation of Neuromuscular Disorders

OBJECTIVES: Isolated left ventricular abnormal trabeculation (ILVAT) is defined as >3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. The aims of the study were to assess by echocardiography the prevalence of ILVAT, look for cardiac findings in ILVAT and find out if ILVAT is associated with neuromuscular disorders. DESIGN AND RESULTS: In the course of 3 years, ILVAT was found in 15/17, 648 patients (0.08%). Twelve patients had heart failure, all had ECG abnormalities. All patients had neuromuscular disorders. A definite diagnosis could be established in 10 patients (metabolic myopathy, n = 9; Becker's muscular dystrophy, n = 1). The remaining 5 patients refused further neurological investigations. CONCLUSIONS: ILVAT is a rare disorder associated with ECG abnormalities, heart failure and neuromuscular disorders, which is a reason why both cardiologists and neurologists should be involved.