• noncompaction;
  • echocardiogram;
  • electrocardiogram;
  • pacing;
  • heart failure

Noncompaction of ventricular myocardium (NVM) is a rare cardiomyopathy, most probably caused by disturbances in embryonic morphogenesis of the myocytes, resulting in prominent trabeculations in the ventricular myocardium. Several diagnostic criteria have been proposed by echocardiography, and its association with other heart diseases is common. Its mortality is closely related to ventricular dysfunction, cardiac arrhythmias, and cardioembolic events.

We report here a case of occurrence of atrioventricular block with syncope as the clinical manifestation of NVM. A brief review of the literature on morphological features, diagnosis, prognosis, and treatment will also be discussed.