Comparison of cardiovascular magnetic resonance characteristics and clinical consequences in children and adolescents with isolated left ventricular non-compaction with and without late gadolinium enhancement.

BACKGROUND: Although 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). METHODS: A 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. RESULTS: The 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). CONCLUSIONS: The 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.

Transcatheter occlusion of azygos/hemiazygos vein in patients with systemic venous collateral development after the bidirectional Glenn procedure.

OBJECTIVES: To elucidate the feasibility and treatment effects of transcatheter occlusion of the azygos/hemiazygos vein in patients with progressive cyanosis after performing the bidirectional Glenn procedure. METHODS: From January 2007 to May 2011, transcatheter closure was performed on 9 patients (7 males and 2 females) aged 5-15 years (median 9 years). RESULTS: A total of 7 azygos veins and 3 hemiazygos veins were occluded successfully. Coils were employed in 4 of the procedures, patent ductus arteriosus (PDA) occluders in 3, atrial septal defect occluders in 2 and a PDA occluder together with coils in 1 procedure. Immediate complete occlusions were achieved in 9 (81.8%) procedures and trivial residual shunt in 2 (18.2%). The femoral artery oxygen saturation (SaO2) rose from 81 [interquartile range (IQR) 75-86%] to 88% (IQR 84-91%). CONCLUSIONS: Patients with superior vena cava azygos vein collateral channels can be treated successfully by transcatheter occlusion with multiple devices. The device for embolization depends on the extent of the severity of the shunt, the size of the lumen of the azygos vein/hemiazygos vein and the candidate location for occlusion.

The relationship between electrocardiographic changes and CMR features in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy.

To investigate the relationship between electrocardiographic (ECG) abnormalities and left ventricular (LV) segmental hypertrophy and myocardial fibrosis assessed by cardiovascular magnetic resonance (CMR) in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy (HCM). 118 asymptomatic or mildly symptomatic patients with HCM were examined with late gadolinium enhancement (LGE) CMR, 12-lead ECG, and echocardiography. The distribution and magnitude of LV segmental hypertrophy and LGE were assessed and analyzed in relation to ECG abnormalities. Abnormal electrocardiograms were found in 113 of 118 (95%) patients. Negative T waves were associated with greater apical septal thickness (P = 0.009) and an increased ratio of LV septum to free wall thickness (P = 0.01). Giant negative T waves (GNT) were found in 19 patients (16%), and were associated with apical HCM (P < 0.001), greater apical thickness (P = 0.004), and increased ratio of LV apical to basal wall thickness (P < 0.001). However, no significant association was demonstrated between GNT and apical LGE (P = 0.71). Abnormal Q waves were associated with greater basal anteroseptal thickness (P = 0.001), maximal basal thickness (P = 0.004), and more segments with extensive LGE (>75% wall thickness involved) (P = 0.001). LV hypertrophy was related to greater LV mass (P = 0.002) and LV end diastolic volume (P = 0.002). In addition, a modest but significant correlation was observed between maximum LV wall thickness and the Romhilt-Estes score (r = 0.41, P < 0.001). GNT were associated with apical HCM and an increased ratio of LV apical to basal wall thickness. Abnormal Q waves were related to basal anteroseptal hypertrophy and segmental extensive LGE.