The Incidence of Pediatric Cardiomyopathy in Two Regions of the United States

Lipshultz, Steven E.; Sleeper, Lynn A.; Towbin, Jeffrey A.; Lowe, April M.; Orav, E. John; Cox, Gerald F.; Lurie, Paul R.; McCoy, Kristina; McDonald, Melissa A.; Messere, Jane; Colan, Steven D.

doi:10.1056/nejmoa021715

Cited by 745 publications

(396 citation statements)

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“…1,12 In brief, patients Ͻ18 years of age newly diagnosed with cardiomyopathy are eligible for inclusion in the registry. Diagnosis of HCM is based on the finding of regional or global left ventricular hypertrophy based on strict criteria (wall thickness Ͼ2 SD above the normal population mean for body surface area 1 ) in the absence of a defined hemodynamic cause such as hypertension, congenital heart disease, or exposure to drugs known to cause cardiac hypertrophy.…”

Section: Methodsmentioning

confidence: 99%

“…12 The overall annual incidence of pure HCM (nϭ246) in the prospective regional cohort was 4.7 (95% CI, 4.1 to 5.3) per 1 million children (Table 1). There was a higher incidence in the New England than in the central Southwest region (Pϭ0.009), in boys than in girls (PϽ0.001), and in children diagnosed at Ͻ1 year of age than in older children (PϽ0.001).…”

Section: Incidencementioning

confidence: 99%

“…15 Incidence estimates are based on the prospective cohort from the 2 geographic regions for 1996 through 2000 using methods described elsewhere. 12 The distributions of categorical variables by groups were compared using the Fisher exact test unless otherwise noted. Two-and 3-group comparisons for normal continuous variables were conducted using Student t test and ANOVA, respectively, whereas skewed data were compared using the Wilcoxon rank sum and Kruskal-Wallis tests.…”

Section: Colan Et Al Hypertrophic Cardiomyopathy In Childrenstatisticmentioning

confidence: 99%

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Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

et al. 2007

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Background-Current information on the epidemiology and outcomes of hypertrophic cardiomyopathy (HCM) in children is limited by disease diversity and small case series. Methods and Results-The Pediatric Cardiomyopathy Registry has collected prospective and retrospective data on children diagnosed with HCM since 1990. We identified the various causes of HCM in childhood and determined the relationship between outcomes, cause, and age at presentation. Of 855 patients Ͻ18 years of age with HCM, 8.7% (nϭ74) had inborn errors of metabolism, 9.0% (nϭ77) had malformation syndromes, 7.5% (nϭ64) had neuromuscular disorders, and 74.2% (nϭ634) had idiopathic HCM. Children with HCM associated with inborn errors of metabolism and malformation syndromes have significantly worse survival than the other 2 groups. Patients with idiopathic HCM diagnosed before 1 year of age (nϭ227) had worse survival from the time of diagnosis than those diagnosed after 1 year of age (nϭ407). Patients with idiopathic HCM who survived to at least 1 year of age, however, had an annual mortality rate of 1% that was similar regardless of whether they were diagnosed before or after 1 year of age. Conclusions-In children, HCM is a diverse disorder with outcomes that depend largely on cause and age. Patients presenting before 1 year of age have the broadest spectrum of causes and the poorest outcome. In those children with idiopathic HCM who survive beyond age 1, however, survival is independent of age at diagnosis, with an annual mortality rate (1%) that is much lower than previously reported in children and is not different from has been found in population-based studies in adults.

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Section: Methodsmentioning

confidence: 99%

Section: Incidencementioning

confidence: 99%

Section: Colan Et Al Hypertrophic Cardiomyopathy In Childrenstatisticmentioning

confidence: 99%

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Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

et al. 2007

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“…The PCMR data, gathered from 18 and 20 pediatric centers in New England and Central Southwest U.S. respectively, reported an annual incidence of pediatric cardiomyopathies of 1.13 cases per 100,000 children [1]. The incidence was significantly higher in infants < 1 year estimated at 8.34 incidence in 100,000, in boys (1.32) compared to girls (0.92), and in blacks (1.47) compared to whites (1.06), which were determined using DCM and HCM functional phenotypes [21,22]. However, the incidence might have been underestimated because the definition of pediatric cardiomyopathies excluded children who suffered from sudden cardiac death, findings from pathologists and medical examiners in the original protocol, and children and adolescent asymptomatic of left ventricular dysfunction who did not seek medical attention [22].…”

Section: Based On Pediatric Cardiomyopathy Registrymentioning

confidence: 99%

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

Albakri¹

2018

Pediatr Dimensions

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Cardiomyopathies define a heterogeneous group of heart muscle disorders. They are an important cause of heart failure and a leading indication for heart transplantation in pediatric patients. Research on cardiomyopathy has however focused on the adult populations with fewer dedicated clinical trials investigating pediatric cardiomyopathy. Diagnosis and clinical management of pediatric cardiomyopathy are also challenging because of the lack of specific methods for diagnosis and treatment. Assessment of ventricular structure using absolute values in adults are not useful in children because of their constantly changing body surface area. Therapies developed for adults applied to pediatric patients also have had varied clinical outcomes. This paper therefore reviews existing research evidence on pediatric cardiomyopathy, with the objective of broadening its clinical understanding and management.

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“…In addition to this fact are cardiomyopathies, which are diseases that affect the heart muscle. In Pediatrics, they have an incidence of 1.13 cases per 100,000 and a prevalence of 2.6 cases per 100,000 children younger than 18 years old 11 . Dilated cardiomyopathies of idiopathic origin (DCM) is the most common cause and the main reason for heart transplantation in both adults and children 12,13 .…”

Section: Introductionmentioning

confidence: 99%

Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades

Becker

Besa

Riveros

et al. 2017

Rev. chil. pediatr.

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Introduction: Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. Objectives: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Material and Methods: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Results: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33,3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. Conclusions: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed urgently and 34.5% died on the waiting list.

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The Incidence of Pediatric Cardiomyopathy in Two Regions of the United States

Abstract: The estimated incidence of pediatric cardiomyopathy in two large regions of the United States is 1.13 cases per 100,000 children. Most cases are identified at an early age, and the incidence varies according to sex, region, and racial or ethnic origin.

Cited by 745 publications

References 31 publications

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

Resultados de un programa nacional de trasplante cardiaco pediátrico: fortalezas y debilidades

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