The Epidemiology of Childhood Cardiomyopathy in Australia

Nugent, Alan; Daubeney, Piers E.F.; Chondros, Patty; Carlin, John B.; Cheung, Michael; Wilkinson, Lynette C.; Davis, Andrew M.; Kahler, Stephen G.; Chow, C. W.; Wilkinson, James L.; Weintraub, Robert G.

doi:10.1056/nejmoa021737

Cited by 640 publications

(285 citation statements)

References 26 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%

“…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: 92%

“…Both DCM and LVNC were included in the same review, because both conditions are still considered idiopathic 6, 14 in most patients. Some of the records were further reviewed regarding echocardiographic examinations and results.…”

Section: Methodsmentioning

confidence: 99%

“…For example, both the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases and the World Health Organization (WHO) define LVNC as an unclassified cardiomyopathy, whereas the American Heart Association defines LVNC as a primary genetically determined cardiomyopathy, which reflects the embryonic structure of the human heart due to an arrest in the compaction process during the first trimester. Previous studies have reported incidences of DCM between 0.57 and 0.73 per 100 000 person years among children aged between birth and 18 2, 6, 13. The occurrence of DCM and LVNC during childhood has not previously been described in Sweden, and we therefore wanted to assess rates and outcomes for these two uncommon conditions.…”

Section: Introductionmentioning

confidence: 99%

“…Dilated cardiomyopathy (DCM) is characterised by dilatation and impaired contractility of the left or both ventricles, and the usual presentation is heart failure 2, 3. DCM has been associated with significant morbidity and mortality and has been reported to account for more than half of all cardiac transplants in children and adolescents 4, 5, 6, 7, 8. Left ventricular noncompaction cardiomyopathy (LVNC) is still referred to as an unclassified cardiomyopathy that may develop into dilated cardiomyopathy–the LVNC‐dilated phenotype 9, 10, 11, 12.…”

Section: Introductionmentioning

confidence: 99%

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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: Discussionmentioning

confidence: 92%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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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Incidence, Causes, and Outcomes of Dilated Cardiomyopathy in Children

Towbin¹,

Lowe²,

Colan³

et al. 2006

JAMA

850

731

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Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids)

et al. 2019

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IMPORTANCE Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. OBJECTIVE To develop and validate an SCD risk prediction model that provides individualized risk estimates. DESIGN, SETTING, AND PARTICIPANTS A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. EXPOSURES The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. MAIN OUTCOMES AND MEASURES A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). RESULTS Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. CONCLUSIONS AND RELEVANCE This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

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The Epidemiology of Childhood Cardiomyopathy in Australia

Abstract: Lymphocytic myocarditis and left ventricular noncompaction are important causes of childhood cardiomyopathy in Australia. The timing and severity of presentation in children with cardiomyopathy are related to the type of cardiomyopathy, as well as to genetic and ethnic factors.

Cited by 640 publications

References 26 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

Incidence, Causes, and Outcomes of Dilated Cardiomyopathy in Children

Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids)

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