Inherited bundle-branch block may not manifest in early childhood and can present asymptomatically, whereas familial AV block can lead to severe symptoms and premature death.
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May inform family counseling on variable onset in inherited conduction disease; leaves open screening timing and risk stratification.
Four cases of complete right bundle-branch block and one case of atrioventricular heart block, all occurring within three generations of the same family, are described. The 4 patients with bundle-branch block had no symptoms, whereas the patient with atrioventricular heart block suffered Adams-Stokes attacks from the age of 13 and died at 47 years of age. A 33-year-old man with bundle-branch block had a normal electrocardiogram at the age of 7, suggesting that inherited bundle-branch block does not necessarily manifest itself during the first years of life.
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Simonsen et al. (1970) studied this question.
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