By Corinna Brunckhorst, Firat Duru, Ardan M. Saguner
Edited and written via the world over famous specialists, present options in Arrhythmogenic correct Ventricular Cardiomyopathy/Dysplasia (ARVC/D) offers very important insights to all elements of this certain disorder and may function a important advisor to aid readers give you the absolute best take care of their sufferers. dialogue of ARVC/D through the specialists includes:
- Basic and scientific science.
- Pathophysiology, molecular mechanisms, and genetic background.
- The mechanisms of ailment development resulting in a variety of ailment phenotypes.
- Challenges within the medical environment with admire to prognosis, hazard stratification, and therapy.
"The editors...have enlisted as authors those that first well-known and named the affliction, and such a lot of these liable for the hot advances during this attention-grabbing sector. the result's a great, complete yet very readable textual content facing this more and more vital spectrum of ailments. it's a precise booklet that are meant to be came upon at the cabinets of each person who seeks to regulate sufferers with cardiac arrhythmia simply because among those that consult there are guaranteed to be sufferers with this demanding disease."
- From the foreword through A. John Camm, MD
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Extra resources for Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia
Generally, RYR2 mutations lead to catecholaminergic polymorphic VT without structural abnormalities. 36 Transforming growth factors β (TGF-βs) regulate the production of extracellular matrix components and modulates expression of genes encoding desmosomal proteins. The gene TGFβ3 has been mapped to chromosome 14. A mutation in the 5' UTR promoter region of TGFβ3, with a predicted inhibitory effect, was found in clinically affected members of one large ARVC/D family. 37 These observations implicated that regulatory mutations resulting in overexpression and enhanced activity of TGFβ3 may lead to fibrosis and thereby contribute to the development of ARVC/D.
Basic Res Cardiol. 2013;108:348. 45. Rhett JM, Jourdan J, Gourdie RG. Connexin 43 connexon to gap junction transition is regulated by zonula occludens-1. Mol Biol Cell. 2011;22:1516-1528. 46. Rhett JM, Ongstad EL, Jourdan J, Gourdie RG. 5 in the cardiomyocyte perinexus. J Membr Biol. 2012;245:411-422. 47. Agullo-Pascual E, Reid DA, Keegan S, et al. Super-resolution fluorescence microscopy of the cardiac connexome reveals plakophilin-2 inside the connexin43 plaque. Cardiovasc Res. 2013;100:231-240.
Studying arrhythmogenic right ventricular dysplasia with patient-specific iPSCs. Nature. 2013;494: 105-110. Bonny A, Lellouche N, Ditah I, et al. C-reactive protein in arrhythmogenic right ventricular dysplasia/cardiomyopathy and relationship with ventricular tachycardia. Cardiol Res Pract. 2010;2010:919783. Fressart V, Duthoit G, Donal E, et al. Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice. Europace.
Current Concepts in Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia by Corinna Brunckhorst, Firat Duru, Ardan M. Saguner