ContextDilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and cause of cardiac transplantation in children. However, the epidemiology and clinical course of DCM in children are not well established.ObjectiveTo provide a detailed description of the incidence, causes, outcomes, and related risk factors for DCM in children.Design and SettingLongitudinal study based on a population-based, prospective cohort of children diagnosed as having DCM since January 1, 1996, at 89 pediatric cardiac centers and a retrospectively collected cohort of patients seen primarily at large tertiary care centers in North America and who had diagnoses between January 1, 1990, and December 31, 1995, and were enrolled through February 2003.ParticipantsA total of 1426 children from the United States and Canada diagnosed as having DCM at younger than 18 years. Primary DCM was determined by strict echocardiographic and/or pathologic criteria. Patients with disease due to endocrine, immunologic, drug toxicity, and other causes were excluded.Main Outcome MeasuresAnnual incidence per 100 000 children; mortality; cardiac transplantation.ResultsThe annual incidence of DCM in children younger than 18 years was 0.57 cases per 100 000 per year overall. The annual incidence was higher in boys than in girls (0.66 vs 0.47 cases per 100 000; P<.001), in blacks than in whites (0.98 vs 0.46 cases per 100 000; P<.001), and in infants (<1 year) than in children (4.40 vs 0.34 cases per 100 000; P<.001). The majority of children (66%) had idiopathic disease. The most common known causes were myocarditis (46%) and neuromuscular disease (26%). The 1- and 5-year rates of death or transplantation were 31% and 46%, respectively. Independent risk factors at DCM diagnosis for subsequent death or transplantation were older age, congestive heart failure, lower left ventricular fractional shortening Z score, and cause of DCM (P<.001 for all).ConclusionsIn 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.

https://jamanetwork.com/journals/jama/articlepdf/203681/JOC60133.pdf

Incidence, causes, and outcomes of dilated cardiomyopathy in children.

http://icnapedia.org/guidelines/open/Chest-2008-Monagle-887S-968S.pdf

Antithrombotic Therapy in Neonates and Children: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition)

Background Population-based data on the incidence of pediatric cardiomyopathy are rare because of the lack of large, prospective studies. Methods Since 1996 the Pediatric Cardiomyopathy Registry sponsored by the National Heart, Lung, and Blood Institute has collected data on all children with newly diagnosed cardiomyopathy in New England and the Central Southwest region (Texas, Oklahoma, and Arkansas) of the United States. We report on all children in these regions who received this diagnosis between 1996 and 1999. Results We identified 467 cases of cardiomyopathy, for an overall annual incidence of 1.13 per 100,000 children (95 percent confidence interval, 1.03 to 1.23). The incidence was significantly higher among infants younger than 1 year old than among children and adolescents who were 1 to 18 years old (8.34 vs. 0.70 per 100,000, P<0.001). The annual incidence of cardiomyopathy was lower among white children (upper-bound estimate, 1.06 cases per 100,000) than among black children (lower-bound estim...

https://www.nejm.org/doi/pdf/10.1056/NEJMoa021715

The incidence of pediatric cardiomyopathy in two regions of the United States.

Aims Left-ventricular non-compaction (LVNC) is characterized by excessive and prominent left-ventricular (LV) trabeculations and may be associated with systolic dysfunction in advanced disease. We sought to determine the proportion of patients fulfilling LVNC criteria in an adult population referred to a heart failure clinic using current diagnostic criteria.

Methods and results One hundred and ninety-nine patients [age 63.5 ± 15.9 years, 124 (62.3%) males] with LV systolic impairment were studied. All underwent clinical examination, electrocardiography, and 2-D echocardiography. The number of patients fulfilling diagnostic criteria for LVNC was retrospectively determined using three published definitions. Results were compared with 60 prospectively evaluated normal controls (age 35.7 ± 13.5 years; 31 males, 30 blacks). Forty-seven patients (23.6%) fulfilled one or more echocardiographic definitions for LVNC. Patients fulfilling LVNC criteria were younger ( P = 0.002), had larger LV end-diastolic dimension ( P < 0.001), and smaller left atrial size ( P = 0.01). LVNC was more common in black individuals (35.5 vs. 16.2%, P = 0.003). Five controls (four blacks) fulfilled one or more LVNC criteria.

Conclusions This study demonstrates an unexpectedly high percentage of patients with heart failure fulfilling current echocardiographic criteria for LVNC. This might be explained by a hitherto underestimated cause of heart failure, but the comparison with controls suggests that current diagnostic criteria are too sensitive, particularly in black individuals.

/pdf/diagnosis-of-left-ventricular-non-compaction-in-patients-4eock21k7x.pdf

Diagnosis of left-ventricular non-compaction in patients with left-ventricular systolic dysfunction: time for a reappraisal of diagnostic criteria?

Antithrombotic therapy in children: the Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy.

Background The incidence and age distribution of primary cardiomyopathy in children are not well defined. We undertook a population-based, retrospective cohort study in Australia to document the epidemiology of childhood cardiomyopathy. Methods We analyzed all cases of primary cardiomyopathy in children who presented between 1987 and 1996 and who were younger than 10 years of age. Children were recruited from multiple sources, and cases of cardiomyopathy were classified according to World Health Organization guidelines. Results Over the 10-year period, 314 new cases of primary cardiomyopathy were identified, for an annual incidence of 1.24 per 100,000 children younger than 10 years of age (95 percent confidence interval, 1.11 to 1.38). Dilated cardiomyopathy made up 58.6 percent of cases, hypertrophic cardiomyopathy 25.5 percent, restrictive cardiomyopathy 2.5 percent, and left ventricular noncompaction 9.2 percent of cases. The incidence of all types of cardiomyopathy except restrictive declined rapidly ...

https://www.nejm.org/doi/pdf/10.1056/NEJMoa021737

The epidemiology of childhood cardiomyopathy in Australia.

Background

Low and moderate dose anthracycline therapy used in current treatment protocols, may be associated with subclinical abnormalities of cardiac function and late presentation with congestive heart failure. Exercise echocardiography is a useful and non-invasive means of uncovering latent cardiac dysfunction in other settings. The purpose of this study is to determine whether exercise echocardiography has the potential to detect subclinical abnormalities of cardiac function in children treated with anthracyclines.



Procedure

One hundred ten children previously treated with anthracycline, in remission and off treatment for >12 months were assessed, together with 31 control subjects. Each subject had a resting ECG and echocardiogram performed, and following exercise on a treadmill according to the Bruce protocol, a repeat echocardiogram was performed.



Results

Cumulative anthracycline dose was the only patient variable related to any of the outcome measures. Resting fractional shortening was normal in the majority of treated patients but was inversely related to cumulative anthracycline dose (rate of decline 1.2%/100 mg/m2). Following peak exercise, the inverse relationship between fractional shortening and anthracycline dose was more pronounced (rate 2.7%/100 mg/m2).



Conclusions

Higher anthracycline doses are associated with a greater difference in peak fractional shortening between treated subjects and controls. Exercise echocardiography is a simple, relatively inexpensive tool that may enhance the detection of latent cardiac dysfunction after anthracycline administration during childhood. © 2004 Wiley-Liss, inc.

Lynette C. Wilkinson

Papers

The epidemiology of childhood cardiomyopathy in Australia.

Exercise echocardiography reflects cumulative anthracycline exposure during childhood.