Aim To investigate the left ventricular (LV) functional, morphological, and structural features revealed by cardiac magnetic resonance (CMR) in children/adolescents with isolated LV non-compaction (iLVNC), and to compare them with those observed in young adults with iLVNC and healthy controls. Methods 56 subjects were included: 12 children/adolescents (mean age 15 ± 3 years, 75% male) and 20 young adults (mean age 35 ± 7 years, 75% male) with first diagnosis of iLVNC, 12 healthy children/adolescents (mean age 15 ± 3 years, 75% male) and 12 healthy young adults (mean age 34 ± 8 years, 75% male). CMR with late gadolinium enhancement (LGE) imaging was performed to evaluate LV function, extent of LV trabeculation, and presence/extent of LV LGE, a surrogate of myocardial fibrosis. Tissue-tracking analysis was applied to assess LV global longitudinal (GLS), circumferential (GCS) and radial (GRS) strain. Results The extent of LVNC and the presence/extent of LV LGE in children/adolescents and young adults with iLVNC were similar. Compared to healthy subjects, young adults with iLVNC had significantly lower LVEF; conversely, no significant difference in this parameter was observed between children/adolescents with iLVNC and healthy subjects. However, compared to healthy subjects, LV strain parameters were lower in both children/adolescents and young adults with iLVNC. Conclusions Complete phenotypic expression, subclinical impairment of myocardial deformation properties, and cardiac injury occur early in iLVNC patients, being already noticeable in the pediatric age group. The application of CMR myocardial deformation imaging permits earlier detection of LV functional impairment in children/adolescents with iLVNC, which would otherwise be missed with standard CMR imaging.
- Cardiac magnetic resonance
- Isolated left ventricular non-compaction
- Late gadolinium enhancement
- Myocardial fibrosis