Resumen de Miocardiopatía no compactada: características clínicas, evolutivas y pronósticas en edad pediátrica. Resultados de un estudio multicéntrico

A. Sabaté Rotés, Manuel Huertas, Pilar Betrián, Juan M. Carretero, Lorenzo Jiménez, Josep Girona, Susana Uriel Prat, Rosa M. Perich Duran, Rosa Collell, Fredy Prada, D.C. Albert Brotons

• Introduction: 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.

  Patients and 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 de?brillator 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.