Abstract
BackgroundAlthough cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to CMR is conducted in children with LVNC. This study was performed to characterize and compare CMR features and clinical outcomes in children with LVNC with and without late gadolinium enhancement (LGE).MethodsA cohort of 40 consecutive children (age, 13.7 ± 3.3 years; 29 boys and 11 girls) with isolated LVNC underwent a baseline CMR scan with subsequent clinical follow-up. Short-axis cine images were used to calculate left ventricular (LV) ejection fraction (EF), end-diastolic volume (EDV), end-systolic volume (ESV), myocardial mass, ratio of non-compacted-to-compacted myocardial thickness (NC/C ratio), and number of non-compacted segments. The LGE images were analyzed to assess visually presence and patterns of LGE. The primary end point was a composite of cardiac death and heart transplantation.ResultsThe LGE was present in 10 (25 %) children, and 46 (27 %) segments were involved, including 23 non-compacted segments and 23 normal segments. Compared with LGE- cohort, LGE+ cohort had significantly lower LVEF (23.8 ± 10.7 % vs. 42.9 ± 16.7 %, p < 0.001) and greater LVEDV (169.2 ± 65.1 vs. 118.2 ± 48.9 mL/m2, p = 0.010), LVESV (131.3 ± 55.5 vs. 73.3 ± 46.7 mL/m2, p = 0.002), and sphericity indices (0.75 ± 0.19 vs. 0.60 ± 0.20, p = 0.045). There were no differences in terms of number and distribution of non-compacted segments, NC/C ratio, and myocardial mass index between LGE+ and LGE- cohort. In the LGE+ cohort, adverse events occurred in 6 patients compared to 2 events in the LGE- cohort. Kaplan-Meier analysis showed a significant difference in outcome between LGE+ and LGE- cohort for cardiac death and heart transplantation (p = 0.011).ConclusionsThe LGE was present in up to one-fourth of children with LVNC, and the LGE+ children exhibited a more maladaptive LV remodeling and a higher incidence of cardiovascular death and heart transplantation.
Highlights
Cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to cardiovascular magnetic resonance (CMR) is conducted in children with LVNC
Study patients We prospectively recruited a cohort of consecutive children with isolated LVNC who were referred to the Fuwai Hospital for CMR between June 2006 and December 2013
The diagnosis of LVNC was made on the basis of previously defined CMR and clinical criteria: [9] (a) appearance of 2 distinct myocardial layers; (b) prominent myocardial trabeculations and deep intertrabecular recesses communicating with the LV cavity; (c) end-diastolic ratio of non-compacted-to-compacted (NC:C) myocardium >2.3:1, and (d) absence of other known co-existing cardiac abnormalities
Summary
Cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to CMR is conducted in children with LVNC. This study was performed to characterize and compare CMR features and clinical outcomes in children with LVNC with and without late gadolinium enhancement (LGE). Left ventricular non-compaction (LVNC) is a genetically and clinically heterogeneous cardiomyopathy characterized by numerous prominent trabeculations, progressive myocardial dysfunction, malignant ventricular arrhythmias, and early mortality [1, 2]. We have assembled a relatively large cohort of children with LVNC, defined by eligibility for CMR, to characterize and compare CMR features and clinical outcomes in children with LVNC with and without LGE
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