title: [Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. Results of a multicenter study].
abstract: BACKGROUND
  Non-compaction of the ventricular myocardium (NCVM) is a rare congenital heart disease. Heightened awareness has resulted in increased detection of the morphological features of NCVM in routine clinical practice.
  
  
  METHODS
  Multicentre study including paediatric patients affected by NCVM according to the echocardiographic criteria of Chin and Jenni.
  
  
  RESULTS
  A total of 29 patients were included, 15 female and 14 male, the median age at diagnosis was 5 years and 7 months (birth to 17 years). Sixteen patients (55%) presented as an isolated lesion, 8 (27.5%) had a ventricular septal defect, one of them associated with aortic coarctation, 3 (10%) had an inborn error of metabolism, 1 (3.5%) had Juvenile Idiopathic Arthritis and 1 (3.5%) has a syndrome being studied. The location of the trabeculae has been predominantly at the apex, but also affected the left ventricle free wall in 11 patients (40%) and right ventricle in 2 (7%). No complications were present in 12 patients (41%), with cardiac failure in 12 patients (41%), an implantable cardioverter defibrillator was placed for ventricular arrhythmias in 2 patients (7%), stroke, 1 patient (3,5%) and death, 2 patients (7%), both of them less than 6 months of age (P<.05). Median follow up was 12 months (2 months to 8 years). Current treatment includes carvedilol, ACEI's and ASA, and one patient is waiting for a cardiac transplantation.
  
  
  CONCLUSIONS
  Early onset of symptoms is associated with a poor prognosis. Clinical and prognostic heterogeneity is described.
authors: Unidad de Cardiología Pediátrica, Hospital Universitari Vall d'Hebron, Barcelona, España. annasabaterotes@gmail.com
authors: A
authors: A
authors: Sabaté Rotés
authors: V M
authors: VM
authors: Huertas-Quiñones
authors: P
authors: P
authors: Betrián
authors: J
authors: J
authors: Carretero
authors: L
authors: L
authors: Jiménez
authors: J
authors: J
authors: Girona
authors: S
authors: S
authors: Uriel
authors: R
authors: R
authors: Perich
authors: R
authors: R
authors: Collell
authors: F
authors: F
authors: Prada
authors: D C
authors: DC
authors: Albert
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000175:diagnosis*; D008297:Male; D011379:Prognosis