Presentation of LVNC:
The classical clinical presentation is heart failure, arrhythmias, and embolic events.
However, LVNC may present in several subtypes, such as isolated LVNC with or without arrhythmias, dilated LVNC, hypertophic LVNC, restrictive LVNC, and in association with congenital defects, such as ASD, VSD, AS, and coarctation.
There is a bilayered LV wall consisting of a thick endocardial layer with prominent intertrabecular recesses with a thin, compact epicardial layer.
Trabeculations are typically most evident in the apical portion of the LV.
Diagnosis of LVNC:
Myocardial trabeculations even fulfilling the diagnostic criteria for non-compaction cardiomyopathy can be seen in athletes, patients with sickle cell anemia, and in pregnant women in whom they resolve within the next 2 years.
The ECG is typically abnormal and may show giant voltages.
Echocardiographic diagnosis is based on the presence of:
- At least three trabeculations, with a ratio of the distance from the epicardial surface to the trough of the trabecular areas divided by the distance from the epicardial surface to the trough of the trabecular areas ≤5
- A two-layer structure and a maximal end-systolic ratio of non-compacted to compacted layers of >2
- Colour Doppler evidence of deep perfused intertrabecular recesses.
Such findings, however, can also be found in up to 8% of normal subjects.
Cardiac MRI offers better spatial resolution than echocardiograpy.