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

摘要

Abstract 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%. Highlights ? Differentiation of remodeled left ventricle myocardium of β-thalassemia major from left ventricle (LV) non-compaction disease using cardiac magnetic resonance (CMR) imaging is still a challenging task. ? Among the most used CMR criteria, those based on the percentage of non-compacted LV mass are better than the ratio of diastolic non-compacted to compacted myocardium at a segmental level.