Left ventricular hypertrabeculation/noncompaction in hereditary inclusion body myopathy

Abstract

Left ventricular hypertrabeculation (LVHT) also known as noncompaction has not been reported in association with hereditary inclusion body myopathy (IBM). In a 62 year old Caucasian male, with a history of muscle stiffness, myalgias, recurrent hyper-creatin-kinase(CK)-aemia, muscle cramps particularly during cold, polyarthralgias, a family history positive for muscle cramping and muscle disease, normal clinical neurologic examination, and myogenic needle EMG, muscle biopsy was indicative of hereditary IBM. Cardiologic investigations revealed arterial hypertension, left anterior hemiblock, slight myocardial thickening, and surprisingly lone LVHT. LVHT was not associated with arrhythmias, systolic dysfunction, or cardioembolic events, No neurological or cardiac therapy was necessary. During a follow-up of 18 months neither the neurological nor the cardiologic abnormalities progressed. LVHT may also occur in patients with IBM. Patients with hereditary IBM should be investigated for cardiac involvement, which may manifest not only as myocardial damage but also as impulse propagation abnormalities.