Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience

Introduction and Aims : Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective wa...

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Main Authors: Gousia Mukhtar, Bijulal Sasidharan, Kavassery Mahadevan Krishnamoorthy, Harikrishnan K N. Kurup, Arun Gopalakrishnan, Deepa Sasikumar, Sankara Sarma, Ajit Kumar Valaparambil, Sivasankaran Sivasubramonian
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-01-01
Series:Annals of Pediatric Cardiology
Subjects:
Online Access:http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=175;epage=181;aulast=Mukhtar
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author Gousia Mukhtar
Bijulal Sasidharan
Kavassery Mahadevan Krishnamoorthy
Harikrishnan K N. Kurup
Arun Gopalakrishnan
Deepa Sasikumar
Sankara Sarma
Ajit Kumar Valaparambil
Sivasankaran Sivasubramonian
author_facet Gousia Mukhtar
Bijulal Sasidharan
Kavassery Mahadevan Krishnamoorthy
Harikrishnan K N. Kurup
Arun Gopalakrishnan
Deepa Sasikumar
Sankara Sarma
Ajit Kumar Valaparambil
Sivasankaran Sivasubramonian
author_sort Gousia Mukhtar
collection DOAJ
description Introduction and Aims : Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results : We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5–9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value– 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion : A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.
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spelling doaj.art-1633cfc246e643d99477b31a78aad3022023-10-26T05:47:19ZengWolters Kluwer Medknow PublicationsAnnals of Pediatric Cardiology0974-20692023-01-0116317518110.4103/apc.apc_149_22Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experienceGousia MukhtarBijulal SasidharanKavassery Mahadevan KrishnamoorthyHarikrishnan K N. KurupArun GopalakrishnanDeepa SasikumarSankara SarmaAjit Kumar ValaparambilSivasankaran SivasubramonianIntroduction and Aims : Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results : We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5–9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value– 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion : A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=175;epage=181;aulast=Mukhtardilated cardiomyopathyindiaoutcomespediatric
spellingShingle Gousia Mukhtar
Bijulal Sasidharan
Kavassery Mahadevan Krishnamoorthy
Harikrishnan K N. Kurup
Arun Gopalakrishnan
Deepa Sasikumar
Sankara Sarma
Ajit Kumar Valaparambil
Sivasankaran Sivasubramonian
Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
Annals of Pediatric Cardiology
dilated cardiomyopathy
india
outcomes
pediatric
title Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
title_full Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
title_fullStr Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
title_full_unstemmed Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
title_short Clinical profile and outcomes of childhood dilated cardiomyopathy – A single-center three-decade experience
title_sort clinical profile and outcomes of childhood dilated cardiomyopathy a single center three decade experience
topic dilated cardiomyopathy
india
outcomes
pediatric
url http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=175;epage=181;aulast=Mukhtar
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