Fibroblast-Cardiomyocyte Interaction in Pediatric Restrictive Cardiomyopathy

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Conduction abnormalities in pediatric patients with restrictive cardiomyopathy.

BACKGROUND Pediatric restrictive cardiomyopathy carries a poor prognosis secondary to a high risk of sudden death previously attributed to ventricular tachyarrhythmias. The extent of conduction abnormalities in this population and their relationship to life-threatening events has not been previously reported. METHODS AND RESULTS A retrospective study of pediatric patients with restrictive car...

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Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a myocardial disorder that usually results from increased myocardial stiffness that leads to impaired ventricular filling. Biventricular chamber size and systolic function are usually normal or nearnormal until later stages of the disease. Affecting either or both ventricles, RCM may cause signs or symptoms of left or right heart failure. Arrhythmias and cond...

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Molecular Characterization of Pediatric Restrictive Cardiomyopathy from Integrative Genomics

Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated...

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Pediatric Cardiology Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype A Report From the Pediatric Cardiomyopathy Registry

Steven A. Webber, MBChB; Steven E. Lipshultz, MD; Lynn A. Sleeper, ScD; Minmin Lu, MS; James D. Wilkinson, MD; Linda J. Addonizio, MD; Charles E. Canter, MD; Steven D. Colan, MD; Melanie D. Everitt, MD; John Lynn Jefferies, MD; Paul F. Kantor, MD; Jacqueline M. Lamour, MD; Renee Margossian, MD; Elfriede Pahl, MD; Paolo G. Rusconi, MD; Jeffrey A. Towbin, MD; on behalf of the Pediatric Cardiomyop...

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Desmin-related restrictive cardiomyopathy.

Restrictive cardiomyopathies may have different etiologies, among which we can point out storage diseases by accumulation of different materials such as desmin. Desminopathies are uncommon diseases that progress with conduction abnormalities, peripheral myopathies, and ventricular dysfunction. The present report describes a patient with complete atrioventricular block as the initial event; he l...

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ژورنال

عنوان ژورنال: Circulation Journal

سال: 2021

ISSN: 1346-9843,1347-4820

DOI: 10.1253/circj.cj-21-0100