Download e-book for kindle: Arrhythmogenic right ventricular cardiomyopathy dysplasia: by Frank I. Marcus, Andrea Nava, Gaetano Thiene

By Frank I. Marcus, Andrea Nava, Gaetano Thiene

ISBN-10: 8847004896

ISBN-13: 9788847004894

ISBN-10: 884700490X

ISBN-13: 9788847004900

This e-book covers all elements (biological, pathological, genetics, medical and therapeutical) of arrhythmogenic correct ventricular cardiomyopathy/dysplasia, a contemporary cardiomyopathy which represents a really excessive threat of surprising loss of life within the younger and in athletes. The monograph provides the result of 5-year study software on ARVC/D supported by means of provides of either the eu fee and the NIH, which enabled the invention of seven disease-causing genes, hence establishing new avenues for the early identity of affected sufferers and for surprising demise prevention. A panel of best scientists, either eu and americans, contributed to this quantity, in order to be a necessary reference for pros and citizens in cardiology, radiology, human genetics, and activity drugs.

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Extra info for Arrhythmogenic right ventricular cardiomyopathy dysplasia: Recent Advances

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Syrris P, Ward D, Asimaki A et al (2006) Clinical expression of plakophilin-2 mutations in familial arrhythmogenic right ventricular cardiomyopathy. Circulation 113:356-364 23. Antoniades L, Tsatsopoulou A, Anastasakis A et al (2006) Arrhythmogenic right ventricular cardiomyopathy caused by deletions in plakophilin-2 and plakoglobin (Naxos disease) in families from Greece and Cyprus: Genotype-phenotype relations, diagnostic features and prognosis. Eur Heart J 27:22082216 24. Sporn A, Roberts AB (1992) Transforming growth factor-beta: Recent progress and new challenges.

In nine subjects arrhythmic symptoms CHAPTER 3 • Genotype-Phenotype Correlations were not present at the first visit and appeared during follow-up. The twelve-lead ECG showed normal sinus rhythm, normal atrioventricular conduction, and normal QT interval in all mutation carriers. The signal-averaged ECG showed late potentials in five subjects (12%), whereas the 24-h ambulatory ECG revealed sporadic monomorphic ventricular complexes in four subjects (9%). In 17 (39%) gene mutation carriers, the 2D echocardiogram showed kinetic alterations of the RV, whereas mild structural abnormalities as trabecular thickening and/or highly reflective moderator band were found in 26 affected subjects (60%).

It is not clear whether the presence of viruses represents a cause and effect relationship or rather that the diseased myocardium is more susceptible to viral infection. Nevertheless, the inflammatory phenomenon may trigger abrupt electrical instability and cause arrhythmic death as well as lead to progression to heart failure and clinically mimic dilated cardiomyopathy [4, 7, 8, 11]. Myocardial apoptosis is another potential mechanism of myocardial cell death and atrophy and subsequent replacement by fatty or fibro-fatty tissue in ARVC/D.

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Arrhythmogenic right ventricular cardiomyopathy dysplasia: Recent Advances by Frank I. Marcus, Andrea Nava, Gaetano Thiene


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