Cardiomyopathies in children: An overview

Tsatsopoulou, Adalena; Protonotarios, Ioannis; Xylouri, Zafeirenia; Papagiannis, Ioannis; Anastasakis, Aris; Germanakis, Ioannis; Πατριανάκος, Αλέξανδρος; Nyktari, Evangelia; Gavras, Christoforos; Papadopoulos, Georgios; Meditskou, Soultana; Lazarou, Emilia; Miliou, Antigoni; Lazaros, George

doi:10.1016/j.hjc.2023.02.007

Cited by 11 publications

(13 citation statements)

References 124 publications

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“…In our study, 15 patients (18.8%) presented systolic dysfunction with EF < 55%. A prior study demonstrated that the survival rate exhibited a significant decline within two years after diagnosis with a proportion of 71.2% patients at <1 year of age [ 5 ]. However, our findings did not show an association between the risk of death and age at diagnosis < 1 year or in other age groups.…”

Section: Discussionmentioning

confidence: 99%

“…Eligible participants amongst patients with CM were limited to those who displayed inherent myocardial abnormalities. The inclusion criteria for DCM were left ventricular dilation (left ventricular end diastolic diameter ≥ 2 SD above normal for body-surface area), simultaneously reduced left ventricular systolic function (left ventricular ejection fraction (LVEF) ≤ 2 SD below normal for age), and an absence of secondary causes of ventricular dilation [ 5 ]. Diagnosis of HCM was based on left ventricular hypertrophy (a wall thickness ≥ 2 SD above the normal population mean for body surface area), excluding defined hemodynamic causes such as congenital heart disease, hypertension, or exposure to drugs known to result in cardiac hypertrophy [ 6 ].…”

Section: Methodsmentioning

confidence: 99%

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The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

Yuan,

Jia,

et al. 2024

Eur J Pediatr

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Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases in children. This study aimed to identify demographic features, clinical presentation and prognosis of children with CM. Clinical characteristics and prognostic factors associated with mortality were evaluated by Cox proportional hazards regression analyses. Genetic testing was also conducted on a portion of patients. Among the 317 patients, 40.1%, 25.2%, 24.6% and 10.1% were diagnosed with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular noncompaction cardiomyopathy (LVNC) and restrictive cardiomyopathy (RCM), respectively. The most common symptom observed was dyspnea (84.2%). Except for HCM, the majority of patients were classified as NYHA/Ross class III or IV. The five-year survival rates were 75.5%, 67.3%, 74.1% and 51.1% in DCM, HCM, LVNC and RCM, respectively. The ten-year survival rates were 60.1%, 56.1%, 57.2% and 41.3% in DCM, HCM, LVNC and RCM, respectively. Survival was inversely related to NYHA/Ross class III or IV in patients with DCM, HCM and RCM. Out of 42 patients, 32 were reported to carry gene mutations.Conclusions: This study demonstrates that CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.Trial registration: MR-50-23-011798. What is Known:• Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases and one of the leading causes of heart failure in children due to the lack of effective treatments.• There remains scarce data on Asian pediatric populations though emerging studies have assessed the clinical characteristics and outcomes of CM. What is New:• A retrospective study was conducted and the follow-up records were established to investigate the clinical characteristics, the profile of gene mutations and prognostic outcomes of children with CM in Western China.• CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

Yuan,

Jia,

et al. 2024

Eur J Pediatr

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“…The inclusion criteria for DCM were left ventricular dilation (left ventricular end diastolic diameter ≥ 2 SD above normal for body-surface area), simultaneously reduced left ventricular systolic function (left ventricular ejection fraction (LVEF) ≤ 2 SD below normal for age), and an absence of secondary causes of ventricular dilation [4]. Diagnosis of HCM was based on left ventricular hypertrophy (a wall thickness ≥ 2 SD above the normal population mean for body surface area), excluding de ned hemodynamic causes such as congenital heart disease, hypertension, or exposure to drugs known to result in cardiac hypertrophy [5].…”

Section: Patients and Data Collectionmentioning

confidence: 99%

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single- center retrospective study

Yuan,

Jia,

et al. 2023

Preprint

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Background: Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases in children. This study aimed to identify demographic features, clinical presentation and prognosis of children with CM. Methods: Clinical characteristics and prognostic factors associated with mortality were evaluated by Cox proportional hazards regression analyses. Genetic testing was also conducted on a portion of patients. Results: Among the 317 patients, 40.1%, 25.2%, 24.6%, and 10.1% were diagnosed with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular noncompaction cardiomyopathy (LVNC) and restrictive cardiomyopathy (RCM), respectively. The most common symptom observed was dyspnea (84.2%). Except for HCM, the majority of patients were classified as NYHA/Ross class III or IV. The five- and ten- year survival rates were 75.5% and 60.1%, 67.3% and 56.1%, 74.1% and 57.2%, and 51.1% and 41.3% in DCM, HCM, LVNC, and RCM, respectively. Survival was inversely related to NYHA/Ross class III or IV in patients with DCM, HCM and RCM. Out of 42 patients, 32 were reported to carry gene mutations. Conclusions: This study demonstrates that CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.

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“…The spectrum of CMPs in children differs from that in adults, being heterogeneous in origin. Although sarcomeric and cytoskeleton mutations can also cause pediatric cardiomyopathies, syndromic, metabolic, or neuromuscular disorders are also relevant [ 2 , 3 ]. The most common morphological phenotypes are dilated (DCM) and hypertrophic (HCM) cardiomyopathy, whereas arrhythmogenic (ACM), restrictive (RCM), and LV non-compaction (LVNC) cardiomyopathy occur less frequently [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of Pediatric Cardiomyopathy in a Mediterranean Population

Bagkaki,

Parthenakis,

Chlouverakis

et al. 2024

Children

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Background. Our knowledge regarding the epidemiology of pediatric cardiomyopathy is based on large national population studies reporting an annual incidence of 1 case per 100,000 children, with a higher incidence observed in infancy and among selected populations. The aim here is to document the epidemiology of pediatric cardiomyopathy in a Mediterranean population. Methods. Children younger than 18 years of age living on the Mediterranean island of Crete, Greece, who have been evaluated since the establishment of tertiary pediatric cardiology services (2002–2022) were included in this retrospective study. Results. A total of 40 children were included, corresponding to an average annual incidence of pediatric cardiomyopathy of 1.59 cases (95% CI: 1.4–2.3) and a prevalence of 26 cases per 100,000 children. In decreasing order of frequency, most cases corresponded to dilated (50%), followed by hypertrophic (42.5%), arrhythmogenic (5%), and restrictive (2.5%) cardiomyopathy. An etiology was identified in 40%, including a genetic diagnosis in 22.5%. Conclusions. The incidence of pediatric cardiomyopathy in the Mediterranean island of Crete is higher compared with that reported previously for other Caucasian populations. Further study is needed to investigate the exact prevalence and specific genetic factors associated with the epidemiology of pediatric cardiomyopathy in Mediterranean populations.

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Cardiomyopathies in children: An overview

Cited by 11 publications

References 124 publications

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single- center retrospective study

Epidemiology of Pediatric Cardiomyopathy in a Mediterranean Population

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