Acquired noncompaction associated with coronary heart disease and myopathy

In a 77-year-old man with a history of arterial hypertension, coronary heart disease, dilative cardiomyopathy, mitral and tricuspid insufficiency, arteriovenous block III, implantation of a pacemaker, atrial fibrillation, and heart failure, left ventricular hypertrabeculation (LVHT) was detected on transthoracic echocardiography during hospitalization for worsening heart failure. Revision of previous echocardiography did not show LVHT in any of the previous investigations why LVHT was interpreted as acquired. The additional presentation with bilateral ptosis, madarosis (absent eyelashes), bilateral hypoacusis, sore neck muscles, absent tendon reflexes, weakness for foot extension, ataxic stance, and recurrently elevated creatine kinase with normal troponin-T suggested a metabolic myopathy. Autopsy after death resulting from intractable heart failure, 17 months later, confirmed severe coronary heart disease and LVHT in the apex. The case confirms that LVHT may be acquired in single cases with neuromuscular disease and may represent an adaptive mechanism of an impaired myocardium. (Heart Lung (R) 201039:240-241.)