Pediatric and adult dilated cardiomyopathy represent distinct pathological entities

Patel, Meghna; Mohan, J.; Schneider, C.; Bajpai, Geetika; Purevjav, Enkhsaikhan; Canter, Charles E.; Towbin, Jeffrey A.; Bredemeyer, Andrea L.; Lavine, Kory J.

doi:10.1172/jci.insight.94382

Cited by 53 publications

(50 citation statements)

References 74 publications

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“…The inability of pediatric CM patients to upregulate N2BA expression might be a reflection of their limited capability to adapt to altered cardiac demands. This is in line with Patel et al who recently published limited adverse remodeling in pediatric CM patients compared to adult onset DCM (Patel et al, 2017 ). They showed that hypertrophy and perivascular and interstitial fibrosis was increased in adult DCM but not in pediatric CM patients compared age-matched controls.…”

Section: Discussionsupporting

confidence: 89%

“…They showed that hypertrophy and perivascular and interstitial fibrosis was increased in adult DCM but not in pediatric CM patients compared age-matched controls. In addition, they showed sarcomere thickness was increased in adult DCM, but not in pediatric CM (Patel et al, 2017 ). Limited cardiac remodeling in pediatric CM patients might hamper the hearts to cope with altered cardiac demands.…”

Section: Discussionmentioning

confidence: 99%

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Cardiomyocyte Hypocontractility and Reduced Myofibril Density in End-Stage Pediatric Cardiomyopathy

Bollen¹,

Meulen

Goede³

et al. 2017

Front. Physiol.

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Dilated cardiomyopathy amongst children (pediatric cardiomyopathy, pediatric CM) is associated with a high morbidity and mortality. Because little is known about the pathophysiology of pediatric CM, treatment is largely based on adult heart failure therapy. The reason for high morbidity and mortality is largely unknown as well as data on cellular pathomechanisms is limited. Here, we assessed cardiomyocyte contractility and protein expression to define cellular pathomechanisms in pediatric CM. Explanted heart tissue of 11 pediatric CM patients and 18 controls was studied. Contractility was measured in single membrane-permeabilized cardiomyocytes and protein expression was assessed with gel electrophoresis and western blot analysis. We observed increased Ca2+-sensitivity of myofilaments which was due to hypophosphorylation of cardiac troponin I, a feature commonly observed in adult DCM. We also found a significantly reduced maximal force generating capacity of pediatric CM cardiomyocytes, as well as a reduced passive force development over a range of sarcomere lengths. Myofibril density was reduced in pediatric CM compared to controls. Correction of maximal force and passive force for myofibril density normalized forces in pediatric CM cardiomyocytes to control values. This implies that the hypocontractility was caused by the reduction in myofibril density. Unlike in adult DCM we did not find an increase in compliant titin isoform expression in end-stage pediatric CM. The limited ability of pediatric CM patients to maintain myofibril density might have contributed to their early disease onset and severity.

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Cardiomyocyte Hypocontractility and Reduced Myofibril Density in End-Stage Pediatric Cardiomyopathy

Bollen¹,

Meulen

Goede³

et al. 2017

Front. Physiol.

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“…32 Fibrosis appears to play a less prominent role in the progression of heart failure in children with DCM compared with their adult counterparts. 33,34 Circulating factors that may influence disease progression are also likely to be unique to the pediatric age group. 35,36 Furthermore, pediatric patients with heart failure secondary to SV CHD demonstrate unique b-adrenergic adaptation, 37 phosphodiesterase expression 32 and activity, 38 and fibrotic gene expression profiles.…”

Section: Challenges In the Diagnosis Of Heart Failurementioning

confidence: 99%

Pediatric Heart Failure: An Evolving Public Health Concern

Miyamoto

Price

Rossano

et al. 2020

The Journal of Pediatrics

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“…2 To date, dilated cardiomyopathy is the most common indication for heart transplantation in the paediatric population. 20 Diagnosis is usually made by echocardiography, electrocardiography, cardiac MRI, or cardiac catheterisation. Screening studies in children who have a familial history of dilated cardiomyopathy, an inborn error of metabolism, a neuromuscular disorder, or a sentinel phenotypic trait are especially relevant.…”

Section: Dilated Cardiomyopathymentioning

confidence: 99%

Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology

et al. 2020

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Background:Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies.Methods:An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines.Results:From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4–9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases; hypertrophic cardiomyopathy, 12.3% (n = 11); restrictive cardiomyopathy, 8.9% (n = 8); non-compacted cardiomyopathy, 7.8% (n = 7); arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6); and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.

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Pediatric and adult dilated cardiomyopathy represent distinct pathological entities

Cited by 53 publications

References 74 publications

Cardiomyocyte Hypocontractility and Reduced Myofibril Density in End-Stage Pediatric Cardiomyopathy

Cardiomyocyte Hypocontractility and Reduced Myofibril Density in End-Stage Pediatric Cardiomyopathy

Pediatric Heart Failure: An Evolving Public Health Concern

Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology

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