Familial sudden unexpected death may be associated with minor anatomical defects of the conduction system that predispose to fatal arrhythmias.
May suggest heritable conduction anomalies in familial sudden death; hypothesis-generating and should not alter practice without confirmatory studies.
Ten instances of sudden unexpected deaths were identified in three generations. Average age at death was 21 years, and in one generation 30% died abruptly at an average age of 13. Syncopal episodes heralded death in 80%, but no other signs or symptoms of cardiovascular disease existed. No abnormalities were found in 22 members of recent generations. Serial study of the conduction systems of two patients revealed minor variances from normal. Perhaps a nonsex-linked gene has produced minor anatomical defects of the conduction system which could predispose to the development of fatal arrhythmias by a variety of environmental factors. The Lev technique for bundle studies of the conduction system should be used in similar instances of unexplained death with grossly normal hearts.
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J. Russell Green (1969) studied this question.
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