Incidence, Causes, and Outcomes of Dilated Cardiomyopathy in Children

Towbin, Jeffrey A.; Lowe, April M.; Colan, Steven D.; Sleeper, Lynn A.; Orav, E. John; Clunie, Sarah K.; Messere, Jane; Cox, Gerald F.; Lurie, Paul R.; Hsu, Daphne T.; Canter, Charles E.; Wilkinson, James D.; Lipshultz, Steven E.

doi:10.1001/jama.296.15.1867

Cited by 850 publications

(793 citation statements)

References 34 publications

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“…This annual incidence was similar to earlier reports 1, 6, 16. Other studies have shown that the annual incidence of DCM in children up to 18 years of age was 0.57 16 and 0.73 6 per 100 000 person years in North America and Australia, respectively .…”

Section: Discussionsupporting

confidence: 91%

“…The Finnish study reported corresponding rates of 65%, 56% and 51%, respectively 7. In North American and Australian studies, the survival rates were 69% to 72% at one year and 54% to 63% at five years 16, 17. The high mortality observed in our study during the first year after diagnosis was similar to previous reports 7, 16, 17, 18, as so was the gradual decline with time 16, 17.…”

Section: Discussionsupporting

confidence: 89%

“…Other studies have shown that the annual incidence of DCM in children up to 18 years of age was 0.57 16 and 0.73 6 per 100 000 person years in North America and Australia, respectively . A nationwide study in Finland carried out between 1980 and 1991 showed an annual incidence of 0.34 per 100 000 person years for idiopathic DCM 13.…”

Section: Discussionmentioning

confidence: 91%

“…In North American and Australian studies, the survival rates were 69% to 72% at one year and 54% to 63% at five years 16, 17. The high mortality observed in our study during the first year after diagnosis was similar to previous reports 7, 16, 17, 18, as so was the gradual decline with time 16, 17. In the Finnish study 7, heart transplants were virtually unavailable, yet the mortality pattern was similar when compared to transplant‐free survival rates in other studies.…”

Section: Discussionmentioning

confidence: 97%

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The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

et al. 2017

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AimThe aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children.MethodsWe reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015.ResultsIn total, 69 cases (61% males) were identified. The combined incidence of DCM and LVNC was 0.77 (95% CI 0.59‐0.96) per 100 000 person years. Children were divided into six groups, and their outcomes were analysed depending on their aetiology. Idiopathic DCM was reported in 43%, and familial dilated and left ventricular noncompaction aetiology was present in 32%. DCM due to various diseases occurred in 8%. DCM associated with neuromuscular diseases was present in 16%. The overall risk of death or receiving transplants in children with idiopathic and familial DCM was 30% over the study period, and 21% died in the first year after diagnosis.ConclusionThe combined incidence of DCM and LVNC was similar to previous reports. Most children with idiopathic DCM presented during infancy, and mortality was highest during the first year after diagnosis.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 97%

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The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

et al. 2017

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“…In children with heart failure, a familial origin is frequently identified 9, 10. In unselected adult heart failure populations, prevalence of heart failure caused by (monogenic) cardiomyopathies is probably lower compared with that in paediatric populations.…”

Section: Familial (Genetic) Predisposition For Heart Failurementioning

confidence: 99%

Genetic determinants of heart failure: facts and numbers

2018

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The relevance of gene mutations leading to heart diseases and hence heart failure has become evident. The risk for and the course of heart failure depends on genomic variants and mutations underlying the so‐called genetic predisposition. Genetic contribution to heart failure is highly heterogenous and complex. For any patient with a likely inherited heart failure syndrome, genetic counselling is recommended and important. In the last few years, novel sequencing technologies (named next‐generation sequencing – NGS) have dramatically improved the availability of molecular testing, the efficiency of genetic analyses, and moreover reduced the cost for genetic testing. Due to this development, genetic testing has become increasingly accessible and NGS‐based sequencing is now applied in clinical routine diagnostics. One of the most common reasons of heart failure are cardiomyopathies such as the dilated or the hypertrophic cardiomyopathy. Nearly 100 disease‐associated genes have been identified for cardiomyopathies. The knowledge of a pathogenic mutation can be used for genetic counselling, risk and prognosis determination, therapy guidance and hence for a more effective treatment. Besides, family cascade screening for a known familial, pathogenic mutation can lead to an early diagnosis in affected individuals. At that timepoint, a preventative intervention could be used to avoid or delay disease onset or delay disease progression. Understanding the cellular basis of genetic heart failure syndromes in more detail may provide new insights into the molecular biology of physiological and impaired cardiac (cell) function. As our understanding of the molecular and genetic pathophysiology of heart failure will increase, this might help to identify novel therapeutic targets and may lead to the development of new and specific treatment options in patients with heart failure.

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Racial and Ethnic Disparities in All-Cause and Cause-Specific Mortality Among US Youth

Wolf,

Rivara,

Orr

et al. 2024

JAMA

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ImportanceMortality rates in US youth have increased in recent years. An understanding of the role of racial and ethnic disparities in these increases is lacking.ObjectiveTo compare all-cause and cause-specific mortality trends and rates among youth with Hispanic ethnicity and non-Hispanic American Indian or Alaska Native, Asian or Pacific Islander, Black, and White race.Design, Setting, and ParticipantsThis cross-sectional study conducted temporal analysis (1999-2020) and comparison of aggregate mortality rates (2016-2020) for youth aged 1 to 19 years using US Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research database. Data were analyzed from June 30, 2023, to January 17, 2024.Main Outcomes and MeasuresPooled, all-cause, and cause-specific mortality rates per 100 000 youth (hereinafter, per 100 000) for leading underlying causes of death were compared. Injuries were classified by mechanism and intent.ResultsBetween 1999 and 2020, there were 491 680 deaths among US youth, including 8894 (1.8%) American Indian or Alaska Native, 14 507 (3.0%) Asian or Pacific Islander, 110 154 (22.4%) Black, 89 251 (18.2%) Hispanic, and 267 452 (54.4%) White youth. Between 2016 and 2020, pooled all-cause mortality rates were 48.79 per 100 000 (95% CI, 46.58-51.00) in American Indian or Alaska Native youth, 15.25 per 100 000 (95% CI, 14.75-15.76) in Asian or Pacific Islander youth, 42.33 per 100 000 (95% CI, 41.81-42.86) in Black youth, 21.48 per 100 000 (95% CI, 21.19-21.77) in Hispanic youth, and 24.07 per 100 000 (95% CI, 23.86-24.28) in White youth. All-cause mortality ratios compared with White youth were 2.03 (95% CI, 1.93-2.12) among American Indian or Alaska Native youth, 0.63 (95% CI, 0.61-0.66) among Asian or Pacific Islander youth, 1.76 (95% CI, 1.73-1.79) among Black youth, and 0.89 (95% CI, 0.88-0.91) among Hispanic youth. From 2016 to 2020, the homicide rate in Black youth was 12.81 (95% CI, 12.52-13.10) per 100 000, which was 10.20 (95% CI, 9.75-10.66) times that of White youth. The suicide rate for American Indian or Alaska Native youth was 11.37 (95% CI, 10.30-12.43) per 100 000, which was 2.60 (95% CI, 2.35-2.86) times that of White youth. The firearm mortality rate for Black youth was 12.88 (95% CI, 12.59-13.17) per 100 000, which was 4.14 (95% CI, 4.00-4.28) times that of White youth. American Indian or Alaska Native youth had a firearm mortality rate of 6.67 (95% CI, 5.85-7.49) per 100 000, which was 2.14 (95% CI, 1.88- 2.43) times that of White youth. Black youth had an asthma mortality rate of 1.10 (95% CI, 1.01-1.18) per 100 000, which was 7.80 (95% CI, 6.78-8.99) times that of White youth.Conclusions and RelevanceIn this study, racial and ethnic disparities were observed for almost all leading causes of injury and disease that were associated with recent increases in youth mortality rates. Addressing the increasing disparities affecting American Indian or Alaska Native and Black youth will require efforts to prevent homicide and suicide, especially those events involving firearms.

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Incidence, Causes, and Outcomes of Dilated Cardiomyopathy in Children

Abstract: In children, DCM is a diverse disorder with outcomes that depend largely on cause, age, and heart failure status at presentation. Race, sex, and age affect the incidence of disease. Most children do not have a known cause of DCM, which limits the potential for disease-specific therapies.

Cited by 850 publications

References 34 publications

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

Genetic determinants of heart failure: facts and numbers

Racial and Ethnic Disparities in All-Cause and Cause-Specific Mortality Among US Youth

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