Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. Results of a multicenter study

• Published in: Anales de pediatría (Barcelona, Spain : 2003) Spain : 2003), 2012-12, Vol.77 (6), p.360-365
• Main Authors: Sabaté Rotés, A, Huertas-Quiñones, V M, Betrián, P, Carretero, J, Jiménez, L, Girona, J, Uriel, S, Perich, R, Collell, R, Prada, F, Albert, D C
• Format: Article
• Language: Spanish
• Subjects: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Isolated Noncompaction of the Ventricular Myocardium - diagnosis; Male; Prognosis
• ISSN: 1695-9531
• DOI: 10.1016/j.anpedi.2011.08.005

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. Multicentre study including paediatric patients affected by NCVM according to the echocardiographic criteria of Chin and Jenni. 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