Left ventricular hypertrabeculation/noncompaction associated with coronary heart disease and myopathy

Abstract Objectives The association of left ventricular hypertrabeculation (LVHT), also known as noncompaction, coronary heart disease, and metabolic myopathy, as presented in the following report, is rare. Case report In a 77-yo male with a history of arterial hypertension, coronary heart disease, dilative cardiomyopathy, mitral and tricuspid insufficiency, AV-block III, implantation of a pacemaker, atrial fibrillation, and heart failure, LVHT was detected on transthoracic echocardiography during hospitalization for worsening heart failure. Clinical neurologic investigation, revealing bilateral ptosis, madarosis, absent eyelashes, bilateral hypacusis, sore neck muscles, generally absent deep tendon reflexes, weakness for foot extension, and ataxic stance, and recurrently elevated creatine-kinase with normal troponine, suggested a metabolic myopathy. Autopsy after death from intractable heart failure. 17 months later confirmed severe coronary heart disease and LVHT in the apex. Conclusions LVHT may be associated with coronary heart disease and myopathy and may be exclusively located in the left ventricular apex.