Septal hypertrabeculation/noncompaction: cardiac and neurologic implications.

BACKGROUND

Left ventricular hypertrabeculation/noncompaction is mainly detected by echocardiography. Left ventricular hypertrabeculation/noncompaction is commonly associated with cardiac and extra-cardiac disorders, preferentially neuromuscular disorders. Left ventricular hypertrabeculation/noncompaction is mainly located within the left ventricular apex, lateral, posterior and anterior wall but only rarely in the medial and basal portions of the interventricular septum. Aim of the present review is to summarize the knowledge about septal affection in left ventricular hypertrabeculation/noncompaction.

RESULTS AND CONCLUSIONS

Septal affection in left ventricular hypertrabeculation/noncompaction is a finding in predominantly children and adolescents. Septal left ventricular hypertrabeculation/noncompaction occurs more in females than in males. Patients with septal left ventricular hypertrabeculation/noncompaction have a poor prognosis. Septal left ventricular hypertrabeculation/noncompaction is most likely congenital. The association of septal left ventricular hypertrabeculation/noncompaction with extracardiac abnormalities and neuromuscular disorders remains unclear. Presumably left ventricular hypertrabeculation/noncompaction affecting the septum does not represent a cardiac manifestation of a neuromuscular disorder.