Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis

Dilated cardiomyopathy is the most common type of heart muscle disease in children with idiopathic etiology in the majority of cases. Idiopathic dilated cardiomyopathy (IDCM) is a severe illness which carries a high mortality rate in the pediatric population. In order to characterize IDCM evolut...

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Main Author: Inas Abdullsattar Saad
Format: Article
Language:English
Published: Taylor & Francis Group 2007-01-01
Series:Libyan Journal of Medicine
Subjects:
Online Access:http://www.ljm.org.ly/articles/AOP/AOP070501/AOP070501.pdf
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author Inas Abdullsattar Saad
author_facet Inas Abdullsattar Saad
author_sort Inas Abdullsattar Saad
collection DOAJ
description Dilated cardiomyopathy is the most common type of heart muscle disease in children with idiopathic etiology in the majority of cases. Idiopathic dilated cardiomyopathy (IDCM) is a severe illness which carries a high mortality rate in the pediatric population. In order to characterize IDCM evolution and identify prognostic predictors in our pediatric cardiology center in the western province of Saudi Arabia, 55 patients with IDCM were evaluated clinically and by echocardiography. They were followed for a minimum of one year and a maximum of four and a half years. Patients less than two years of age represented 69% of the cohort. Gender distribution revealed 65.5% female and 35.5% male. Outcomes were divided into four groups: 25 patients (45.5%) improved (Group I), 17 patients (31%) had a stationary course (Group II), 13 patients (23.6%) deteriorated (Group III), and eventually 11 patients (from Group III) died. Survival rate was 80% with a mean follow-up period of 36.2 ± 22.1 months. The older the age at presentation, the worse the prognosis, with P value= 0.029.In this study, we found a significant correlation of prognosis with end diastolic volume (EDV) (P=0.05) as well as stroke volume (SV) (P=0.04) on presentation. However, fractional shortening of ejection fraction on presentation could not be correlated statistically to the prognosis. Also results suggested that higher z-score of intraventricular septum & Left ventricular posterior wall dimensions in diastole significantly correlated to favorable outcomes and higher z-score of Left ventricular end diastolic dimension (LVEDD) was significantly related to unfavorable outcome. We concluded that further multi-center studies are necessary to verify predictors of outcome in IDCM patients. Identification of markers affecting early myocardial function is essential to achieving improvements in treatments and consequently outcomes in this pediatric population.
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spelling doaj.art-d6a35f6434b24ef787de469a74570b0a2022-12-21T20:11:37ZengTaylor & Francis GroupLibyan Journal of Medicine1819-63572007-01-0123AOP:070501Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of PrognosisInas Abdullsattar SaadDilated cardiomyopathy is the most common type of heart muscle disease in children with idiopathic etiology in the majority of cases. Idiopathic dilated cardiomyopathy (IDCM) is a severe illness which carries a high mortality rate in the pediatric population. In order to characterize IDCM evolution and identify prognostic predictors in our pediatric cardiology center in the western province of Saudi Arabia, 55 patients with IDCM were evaluated clinically and by echocardiography. They were followed for a minimum of one year and a maximum of four and a half years. Patients less than two years of age represented 69% of the cohort. Gender distribution revealed 65.5% female and 35.5% male. Outcomes were divided into four groups: 25 patients (45.5%) improved (Group I), 17 patients (31%) had a stationary course (Group II), 13 patients (23.6%) deteriorated (Group III), and eventually 11 patients (from Group III) died. Survival rate was 80% with a mean follow-up period of 36.2 ± 22.1 months. The older the age at presentation, the worse the prognosis, with P value= 0.029.In this study, we found a significant correlation of prognosis with end diastolic volume (EDV) (P=0.05) as well as stroke volume (SV) (P=0.04) on presentation. However, fractional shortening of ejection fraction on presentation could not be correlated statistically to the prognosis. Also results suggested that higher z-score of intraventricular septum & Left ventricular posterior wall dimensions in diastole significantly correlated to favorable outcomes and higher z-score of Left ventricular end diastolic dimension (LVEDD) was significantly related to unfavorable outcome. We concluded that further multi-center studies are necessary to verify predictors of outcome in IDCM patients. Identification of markers affecting early myocardial function is essential to achieving improvements in treatments and consequently outcomes in this pediatric population.http://www.ljm.org.ly/articles/AOP/AOP070501/AOP070501.pdfdilated cardiomyopathychildrennatural historypredictors of prognosisechocardiography
spellingShingle Inas Abdullsattar Saad
Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
Libyan Journal of Medicine
dilated cardiomyopathy
children
natural history
predictors of prognosis
echocardiography
title Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
title_full Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
title_fullStr Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
title_full_unstemmed Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
title_short Idiopathic Dilated Cardiomyopathy in Children: Natural History and Predictors of Prognosis
title_sort idiopathic dilated cardiomyopathy in children natural history and predictors of prognosis
topic dilated cardiomyopathy
children
natural history
predictors of prognosis
echocardiography
url http://www.ljm.org.ly/articles/AOP/AOP070501/AOP070501.pdf
work_keys_str_mv AT inasabdullsattarsaad idiopathicdilatedcardiomyopathyinchildrennaturalhistoryandpredictorsofprognosis