title: 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).
  
  
  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-WhitneyUtest (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), andE/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, andp: 0.03).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Imaging, Bambino Gesù - Children's Hospital IRCCS, Rome, Italy; Department of Clinical and Molecular Medicine, University of Rome "Sapienza", Rome, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Muscogiuri
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Ciliberti
authors.affiliation: Department of Neurosciences, Imaging and Clinical Sciences, Diagnostic Imaging and Therapy, University "G. D'Annunzio" , Chieti , Italy.
authors.forename: Domenico
authors.initials: D
authors.lastname: Mastrodicasa
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Marcello
authors.initials: M
authors.lastname: Chinali
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Rinelli
authors.affiliation: Department of Imaging, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Teresa Pia
authors.initials: TP
authors.lastname: Santangelo
authors.affiliation: Department of Imaging, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Carmela
authors.initials: C
authors.lastname: Napolitano
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Leonardi
authors.affiliation: Department of Imaging, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.
authors.forename: Aurelio
authors.initials: A
authors.lastname: Secinaro
keywords: cardiac magnetic resonance; dilated cardiomyopathy; late gadolinium enhancement; systolic function; ventricular mechanics