Characteristics and Outcomes of Heart Failure–Related Intensive Care Unit Admissions in Children With Cardiomyopathy

Shamszad, Pirouz; Hall, Matthew W.; Rossano, Joseph W.; Denfield, Susan W.; Knudson, Jarrod D.; Penny, Daniel J.; Towbin, Jeffrey A.; Cabrera, Antonio G.

doi:10.1016/j.cardfail.2013.08.006

Cited by 44 publications

(35 citation statements)

References 32 publications

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“…96 Many children require hospitalization at the time of diagnosis because of advanced heart failure. 97, 98 In a large population-based study of admissions for new-onset heart failure in children with cardiomyopathy, 54% received intravenous inotropic support, 41% were placed on mechanical ventilation, 13% were treated with extracorporeal membrane oxygenation, and 11% underwent urgent transplantation. 98 Compared to adults, children with cardiomyopathy hospitalized with heart failure had greater morbidity and mortality and utilized advanced heart failure therapies more frequently.…”

Section: Dilated Cardiomyopathymentioning

confidence: 99%

Pediatric Cardiomyopathies

Lee

Hsu

Kantor

et al. 2017

Circulation Research

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224

218

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Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1–1.5 per 100,000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent. Pediatric cardiomyopathies often occur in the absence of co-morbidities such as atherosclerosis, hypertension, renal dysfunction, and diabetes; as a result, they offer insights into the primary pathogenesis of myocardial dysfunction. Large international registries have characterized the epidemiology, etiology, and outcomes of pediatric cardiomyopathies. Although adult and pediatric cardiomyopathies have similar morphologic and clinical manifestations, their outcomes differ significantly. Within two years of presentation, normalization of function occurs in 20% of children with dilated cardiomyopathy, and 40% die or undergo transplantation. Infants with hypertrophic cardiomyopathy have a two-year mortality of 30%, whereas death is rare in older children. Sudden death is rare. Molecular evidence indicates that gene expression differs between adult and pediatric cardiomyopathies, suggesting treatment response may differ as well. Clinical trials to support evidence-based treatments and the development of disease-specific therapies for pediatric cardiomyopathies are in their infancy. This compendium summarizes current knowledge of the genetic and molecular origins, clinical course, and outcomes of the most common phenotypic presentations of pediatric cardiomyopathies, and highlights key areas where additional research is required.

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Section: Dilated Cardiomyopathymentioning

confidence: 99%

Pediatric Cardiomyopathies

Lee

Hsu

Kantor

et al. 2017

Circulation Research

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224

218

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“…Cardiomyopathies are major causes of morbidity and mortality and over the past twenty-five years, limited improvements in outcome have been reported [1, 2]. However, improvement in the understanding of the major forms of cardiomyopathy has occurred over that time, in large part due to advances in imaging, genetics and genomics [3, 4].…”

Section: Introductionmentioning

confidence: 99%

Inherited Cardiomyopathies

Towbin

2014

Circ J

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156

148

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Cardiomyopathies, diseases of the heart muscle, are major causes of morbidity and mortality. A significant percentage of patients with cardiomyopathies have genetic-based, inheritable disease and, over the past two decades the genetic causes of these disorders have been increasingly discovered. The genes causing these disorders when they are mutated appear to encode proteins that frame a “final common pathway” for that specific disorder but the specifics of the phenotype, including age of onset, severity, and outcome is variable for reasons not yet understood. The “final common pathways” for the classified forms of cardiomyopathy include the sarcomere in the primarily diastolic dysfunction disorders hypertrophic cardiomyopathy (HCM) and restrictive cardiomyopathy (RCM), the linkage of the sarcomere and sarcolemma in the systolic dysfunction disorder dilated cardiomyopathy (DCM), and the desmosome in arrhythmogenic cardiomyopathy (AVC). Left ventricular noncompaction cardiomyopathy (LVNC) is an overlap disorder and appears that any of these “final common pathways” can be involved depending on the specific form of LVNC. The genetics and mechanisms responsible for these clinical phenotypes will be described.

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“…According to several recent investigations, DCM is still one of the major causes of morbidity and mortality, especially in youngsters and those in their productive years of life. 13,14) Thus, a better understanding of the pathogenesis of DCM is essential to further improve its survival. Human TIM gene family, which contains TIM1, TIM3 and TIM4, is located on chromosome 5q23-35, a region that has been repeatedly linked with immune dysfunction, which may subsequently lead to autoimmune diseases, allergic diseases, chronic viral infections, immune rejections, and tumors.…”

Section: Discussionmentioning

confidence: 99%

Associations Between <i>TIM1</i> Polymorphisms and Dilated Cardiomyopathy in a Han Chinese Population

Xie

Zhou

et al. 2016

Int. Heart J.

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SummaryImmune dysfunction is implicated in dilated cardiomyopathy (DCM). Previous studies found that TIM1 polymorphisms were associated with immune dysfunction. However, the associations between TIM1 polymorphisms and DCM have not been investigated. Therefore, we conducted the present study to evaluate whether TIM1 polymorphisms were associated with DCM in the Han Chinese population. A total of 396 DCM patients and 403 healthy controls were enrolled in this case-control study. Two promoter region single nucleotide polymorphisms (SNPs) of TIM1 gene, -416G>C and -1454G>A, were genotyped by PCR-RFLP. The associations between two SNPs genotyped and the overall survival (OS) of DCM patients were evaluated with Kaplan-Meier analysis and Cox regression analysis. Plasma TIM-1 levels were further measured by ELISA. We found that the C allelic frequency of -416G>C and A allelic frequency of -1454G>A were higher in DCM patients than that in controls (P < 0.001). The genotypic frequencies of both SNPs were associated with DCM susceptibility in the codominant, dominant, and overdominant models (P < 0.01). They were also associated with the OS of DCM patients in the dominant, recessive, and overdominant models (P < 0.001). The CC genotype of -416G>C and AA genotype of -1454G>A were associated with the worst prognosis (P < 0.001). In addition, the plasma TIM-1 levels in DCM patients were higher than that in controls (259.0 pg/mL versus 149.8 pg/mL, P = 0.035). The CC genotype of 416G>C and AA genotype of -1454G>A were associated with the highest TIM-1 production (P < 0.01). Overall, our findings suggest that TIM1 polymorphisms are associated with DCM susceptibility and prognosis in this Han Chinese population. (Int Heart J 2016; 57: 742-746) Key words: Single nucleotide polymorphisms, Survival analysis, Enzyme-linked immunosorbent assay D ilated cardiomyopathy (DCM), characterized by progressive ventricular chamber enlargement and contractional dysfunction in the absence of associated conditions such as hypertension, ischemic heart disease, valvular heart disease, congenital heart disease, and pulmonary heart disease, is the most common type of cardiomyopathy, accounting for approximately 55-60% of cardiomyopathies with an estimated prevalence of 1 in 2,500 individuals.1) It is the leading non-ischemic cause of congestive heart failure and sudden cardiac death, and is the most frequent indication for cardiac transplantation in adults and children. 2)In the last two decades, extensive studies have been conducted to investigate the etiology of DCM. However, the exact cause of the disease is still largely unknown. Recently, immune dysfunction was demonstrated to be involved in the pathogenesis of DCM. Firstly, it was found that T suppressor (Ts) cells are decreased while T helper (Th) cells are increased in DCM patients.3) Secondly, several cardiac-specific antibodies were detected in serum of DCM patients.4-7) And thirdly, immunosuppressive therapy was shown to be associated with symptomatic and functional improvements of DCM...

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Characteristics and Outcomes of Heart Failure–Related Intensive Care Unit Admissions in Children With Cardiomyopathy

Cited by 44 publications

References 32 publications

Pediatric Cardiomyopathies

Pediatric Cardiomyopathies

Inherited Cardiomyopathies

Associations Between <i>TIM1</i> Polymorphisms and Dilated Cardiomyopathy in a Han Chinese Population

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