Late detection of noncompaction of the myocardium in an adult with complete interventricular septal defect

Noncompaction of the ventricular myocardium (NVM) is a morphogenetic anomaly that leads to the development of cardiomyopathy. It is often associated with other congenital cardiac malformations. Common clinical presentations of NVM involve heart failure symptoms, ventricular tachyarrhythmias, and thromboembolic events. Although the peculiar echocardiographic picture is characteristic for this entity, it may often be misdiagnosed. In this case report, we describe a 27-year-old man who had been followed-up since childhood, with the diagnosis of interventricular septal defect or enlarged “single ventricle” with a very small rudiment of the apical part of the interventricular septum. On his last echocardiographic examination, NVM was detected with heavy trabeculations and intertrabecular recesses. This case suggests that physicians should be more patient to detect other congenital abnormalities including NVM, which may influence the clinical and prognostic outcome of these patients.