Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

Abstract

BackgroundLittle is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM).Materials and methodsWe retrospectively evaluated 15 patients (8 ± 6 years, 6 males) with diagnosis of DCM who underwent cardiac magnetic resonance since 2014. All scans were performed with a 1.5 T system (Aera, Siemens). Study protocol included cine steady-state free precession sequences, followed by administration of 0.2 mmol/kg of gadolinium-based contrast agent. Inversion recovery Turbo Flash sequences, in the same position of cine images, were acquired 10–15 min after the injection of contrast agent, in order to assess the presence of LGE. The latter was considered positive with a signal intensity >6 SD from normal myocardial tissue. Indexed end-diastolic volume (EDVi) and end-systolic volume (ESVi), and left ventricle (LV) ejection fraction (EF) were calculated by using dedicated software on off-line workstation. Global longitudinal strain and diastolic function were evaluated by echocardiography. Clinical follow-up, including death, transplant, and listing for heart transplant [major adverse cardiac events (MACE)], were evaluated. Patients were divided into two different subgroups: negative (Group A) and positive (Group B) for presence of LGE. Statistical analysis was performed by using Mann–Whitney U test (p < 0.05 considered as statistically significant).ResultsSeven patients (47%) showed LGE. A global diffuse subendocardial pattern was evident in all patients presenting LGE (7/7, 100%). The following main LV indexes were observed in the two subgroups. Group A: EDVi = 96 ± 33 ml, ESVi = 56 ± 29 ml, LV EF = 45 ± 10%, global longitudinal strain = −16 ± 5%, E/e′ ratio = 10 ± 3, MACE = 1. Group B: EDVi = 130 ± 60 ml, ESVi = 89 ± 43 ml, LV EF = 31 ± 6%, global longitudinal strain = −13 ± 4%, E/e′ ratio = 9 ± 3, MACE = 3. There was no statistically significant difference between the two groups, in terms of EDVi (p: 0.2), ESVi (p: 0.2), and E/e′ ratio (0.9), whereas a significant difference of LV EF, presence of significative mitral regurgitation, and global longitudinal strain were observed (respectively, p: 0.03, p: 0.009, and p: 0.03).ConclusionIn our population of children with DCM, LGE shows a global diffuse subendocardial pattern. Presence of LGE seems to play a role in these patients determining a worst global systolic function.

Mathematical modeling of cardiac function to evaluate clinical cases in adults and children

“…Cardiac output and ejection fraction reduced from healthy values to the values characterized as impaired [72,73]. The left ventricular volume signal over a cardiac cycle increased as a result of dilatation and cause dilation in the left atrial volume, as reported in [74]. Left ventricular end-diastolic and end-systolic diameters and right ventricular diameter were similar to patient-specific values reported in [73] and remained within the reported range in the children for the studied age interval [75].…”

Midwall Fibrosis and Cardiac Mechanics: Rigid Body Rotation Is a Novel Marker of Disease Severity in Pediatric Primary Dilated Cardiomyopathy

“…The findings of our study are comparable with previously published reports on adults with DCM in whom LGE was detected with a frequency of up to 39%, while others described lower presence of LGE ( 2 – 5 ). Data on LGE in children and adolescents with DCM are sparse ( 21 , 22 ). In the study by Latus et al, LGE was present in only 16% of the pediatric DCM cohort despite marked LV dilation and dysfunction.…”

“…Muscogiuri found LGE in 47% of children with DCM. In all patients, LGE occurred in a global diffuse subendocardial pattern compatible with primary endocardial fibroelastosis, and was associated with reduced global LV systolic function ( 22 ). In line with these data, it has been shown that LGE is related to functional impairment ( 23 ), and is a predictor of arrhythmias, cardiovascular hospitalization and death in adult DCM ( 2 , 3 ).…”

SCMR expert consensus statement for cardiovascular magnetic resonance of acquired and non-structural pediatric heart disease

“…CMR studies for pediatric DCM have focused on the early detection of myocardial involvement in children at high risk for developing ventricular dysfunction, such as muscular dystrophy patients [23][24][25] and pediatric cancer survivors exposed to anthracyclines and radiation [26,27]. Myocardial fibrosis demonstrated by LGE was seen in association with ventricular dysfunction in the DCM or muscular dystrophy group [28][29][30], but not in the pediatric cancer survivor group [27,31]. In a study of long-term survivors of childhood leukemia, LGE was associated with LV and right ventricular (RV) diastolic dysfunction [32].…”