Left ventricle remodeling in patients with β-thalassemia major. An emerging differential diagnosis with left ventricle noncompaction disease

To differentiate left ventricle non-compaction (LVNC) from hypertrabeculated myocardium due to LV remodeling in β-thalassemia major (β-TM) patients, cardiac magnetic resonance (CMR) images of 38 β-TM patients and 10 LVNC patients were compared using 3 diagnostic criteria: ratio of diastolic segmental non-compacted to compacted myocardium (NC/C ratio) > 2.5, percentage of non-compacted LV mass (NC-LVM%) > 20% and > 25% of global LV mass. Specificity of NC/C ratio of > 2.5 was the lowest (58%) and of NC-LVM% of > 25% the highest (93%). A NC-LVM% > 20% showed sensitivity 100% and specificity 87%. Disease differentiation depends on the selected CMR criterion and is better with NC-LVM%.