Circulating microRNA as a biomarker for recovery in pediatric dilated cardiomyopathy

Miyamoto, Shelley D.; Karimpour-Fard, Anis; Peterson, Valencia; Auerbach, Scott R.; Stenmark, Kurt R.; Stauffer, Brian L.; Sucharov, Carmen C.

doi:10.1016/j.healun.2015.01.979

Cited by 69 publications

(49 citation statements)

References 41 publications

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“…Only 5% of the pediatric patients enrolled in the American Pediatric Cardiomyopathy Registry received beta blockers in the 1990s compared with 18% after 2000 . An increasing burden of studies about the pathophysiological differences of pediatric and adult HF mechanisms characterizes the current literature . This contributes to our understanding of the different age‐related responses to therapy.…”

Section: Discussionmentioning

confidence: 99%

Natural History of Dilated Cardiomyopathy in Children

Puggia

Merlo

Barbati

et al. 2016

JAHA

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BackgroundThe long‐term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long‐term progression of pediatric and adult DCM populations.Methods and ResultsBetween 1988 and 2014, 927 DCM patients were consecutively enrolled. The pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). At presentation, the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (P=0.03), shorter duration of heart failure (P=0.04), lower systolic blood pressure (P=0.01), decreased presence of left bundle‐branch block (P=0.001), and increased left ventricular ejection fraction (P=0.03). Despite these baseline differences, long‐term longitudinal trends of New York Heart Association class III to IV, left ventricular dimensions, left ventricular ejection fraction, and restrictive filling pattern were similar between the 2 populations. Regarding survival analysis, because of the size difference between the 2 populations, we compared the pediatric population with a sample of adult patients randomly matched using the above‐mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). During a median follow‐up of 110 months, survival free from heart transplantation was significantly lower among pediatric patients compared with adults (P<0.001). Furthermore, pediatric age (ie, <18 years) was found to be associated with an increasing risk of both death from pump failure and life‐threatening arrhythmias.ConclusionsDespite the pediatric DCM population having higher baseline left ventricular ejection fraction and similar long‐term echocardiographic progression compared with the adult DCM population, the pediatric DCM patients had worse cardiovascular prognosis.

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Section: Discussionmentioning

confidence: 99%

Natural History of Dilated Cardiomyopathy in Children

Puggia

Merlo

Barbati

et al. 2016

JAHA

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“…Potential targets for investigation include genetic variants, circulating or imaging markers of inflammation or stress, neurohormonal abnormalities, microRNA, viral genome on endomyocardial biopsy, and markers of extracardiac impairment. 51, 73, 74, 94, 101, 102, 108, 109 Identifying biomarkers that are important for pediatric patients is the focus of a large ongoing study by the PCMR and is an area of research need.…”

Section: Dilated Cardiomyopathymentioning

confidence: 99%

Pediatric Cardiomyopathies

Lee

Hsu

Kantor

et al. 2017

Circulation Research

246

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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“…Gap junctions are at the center of these events and allow for proper electrical coupling (8,9,13,14). Gap junctions are cell passages that help exchange micro-molecular metabolites to coordinate electrical activity (10,13). They rely on connexin isoforms in order to carry out cardiac conduction (6–8,14,15).…”

Section: Gap Junctions Are Essential For Cell Communication and Cx43 mentioning

confidence: 99%

“…Gap junction integrity is important to maintain normal heart function, and changes in its levels can lead to pathologic cardiac remodeling (6,8–10,13). Pathologic remodeling may result in a decrease in Cx43 expression, which can interrupt fundamental cell-to-cell communication processes (5,6,13).…”

Section: Future Research Directions For Cardiovascular Diseasementioning

confidence: 99%