Sodium Channelopathy Underlying Familial Sick Sinus Syndrome With Early Onset and Predominantly Male Characteristics

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Sodium channelopathy underlying familial sick sinus syndrome with early onset and predominantly male characteristics.

BACKGROUND Sick sinus syndrome (SSS) is a common arrhythmia often associated with aging or organic heart diseases but may also occur in a familial form with a variable mode of inheritance. Despite the identification of causative genes, including cardiac Na channel (SCN5A), the pathogenesis and molecular epidemiology of familial SSS remain undetermined primarily because of its rarity. METHODS ...

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A family with sick sinus syndrome is presented: a mother and her 2 daughters affected with sinus node dysfunction. Electrophysiological studies revealed abnormal atrioventricular conduction in the 2 daughters. All 3 individuals were implanted with a permanent pacemaker to relieve their symptoms. Endomyocardial biopsy from the right ventricle of one daughter showed disarrangement, degeneration, ...

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The sick sinus syndrome.

1. a. SSS b. SA block c. SA arrest Sick sinus syndrome is a disorder characterized by a dysfunctional sinus node. It is often idiopathic and a result of degenerative fibrosis of nodal tissue. Certain disorders such as amyloidosis, connective tissue disease, Chagas disease, and hemochromatosis are examples of intrinsic causes. Hypertensive heart disease and cardiomyopathies account for a smaller...

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ژورنال

عنوان ژورنال: Circulation: Arrhythmia and Electrophysiology

سال: 2014

ISSN: 1941-3149,1941-3084

DOI: 10.1161/circep.113.001340