Key result
Familial idiopathic dilatation of the right atrium associated with complete atrio-ventricular block was identified in two siblings, representing a potentially new syndrome.
This report describes a potentially new syndrome characterized by familial idiopathic dilatation of the right atrium and complete atrio-ventricular block.
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May prompt familial screening in right atrial dilatation with AV block; leaves open confirmation as a distinct genetic syndrome.
Familial occurrence of idiopathic dilatation of the right atrium is extremely rare. This is the first description of a family in which 2 siblings had a syndrome of idiopathic dilatation of the right atrium associated with complete atrio-ventricular block. The family workup did not show other family members to be affected, and the question we raise is whether or not this might be a new syndrome.
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Blondheim et al. (2000) studied this question. Familial idiopathic dilatation of the right atrium associated with complete atrio-ventricular block was identified in two siblings, representing a potentially new syndrome.
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