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Benign congenital polycystic tumors of the atrioventricular (A-V) node are an unusual but not very rare cause of heart block. Two such cases are presented and discussed in conjunction with the reported experience of other. The tumor is always within and only very near the A-V node, seldom involves more than the proximal end of the His bundle, and has not been reported to occur in the sinus node. Although sudden death has been reported in conjunction with these tumors, a surprising number of other patients have lived to old age and died of causes unrelated to the A-V nodal tumor. As a corollary it is important to consider a diagnosis of A-V nodal tumor in any patient of any age who presents with otherwise unexplained heart block or syncope. From accumulated experience it appears that such patients do not tolerate electronic pacing safely and some possible explanations for this are discussed. Escape rhythm in all reported cases has been characterized by QRS complexes which are narrow and a ventricular rate which is from half to two-thirds of the sinus rate. Reasons why an A-V junctional rhythm which is 66% of sinus rate may be especially stable are discussed.

circulation

De subitaneis mortibus. XXVI. Fatal electrical instabiltiy of the heart associated with benign congenital polycystic tumor of the atrioventricular node.