Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy

Abstract

Background: Little is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM).

Materials and methods: We 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).

Results: Seven 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).

Conclusion: In 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.

Keywords: cardiac magnetic resonance; dilated cardiomyopathy; late gadolinium enhancement; systolic function; ventricular mechanics.

Figure 1

Seventeen-year-old patient with dilated cardiomyopathy (DCM) (A,B). Cine steady-state free precession (SSFP), in two chambers view, shows dilated left ventricle (LV) (A) in absence of late gadolinium enhancement (LGE) (B). Three years old patient with DCM (C,D). Dilated LV in two chambers view on cine SSFP (C). Diffuse subendocardial LGE is visible in two chambers view [arrows (D)].

Figure 2

One-year-old patient with dilated cardiomyopathy. Cine steady-state free precession on short axis view (A) shows dilated left ventricle with associated diffuse global subendocardial late gadolinium enhancement [arrows (B)].