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

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 echoc...

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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
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Language:Spanish
Subjects:
Adolescent
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Isolated Noncompaction of the Ventricular Myocardium - diagnosis
Male
Prognosis
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container_title Anales de pediatría (Barcelona, Spain : 2003)
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creator 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
description 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
doi_str_mv 10.1016/j.anpedi.2011.08.005
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Results of a multicenter study</title><source>ScienceDirect Additional Titles</source><creator>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</creator><creatorcontrib>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</creatorcontrib><description>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&lt;.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. Early onset of symptoms is associated with a poor prognosis. 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subjects Adolescent
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Isolated Noncompaction of the Ventricular Myocardium - diagnosis
Male
Prognosis
title Non-compacted cardiomyopathy: clinical characteristics, evolution and prognostic data in childhood. Results of a multicenter study
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