In 1901 Morquio described a family with 8 siblings, 5 of whom experienced syncope and early death due to bradycaria. Since this first description of familial arrhythemias, Osler in 1903 described a family in which most of the members had a slow pulse rate and Adams-Stokes syncopes. Aylward in 1928 reported the two cases of complete A-V block in two sisters, and these cases were first cases of congenital A-V block fulfilled the criteria of Yater. Yater, in 1929, collected 40 acceptable cases and laid down the criteria necessary for the diagnosis of congenital complete heart block. These criteria are: 1. The heart block shall be established by means of graphic records in relatively young individuals, 2. A slow pulse rate must have been ascertained at early age, 3. There must be no history of any infection which may be thought to have caused the heart block after birth(Diphtheria or Rheumatism).
We had the opportunity of examining a family spanning three generations, in which at least three members had A-V heart block. Complete A-V blocks were found in 3 of 10 family members, and fulfilled the criteria of Yater. Mother of the propositus, who died of sudden cardiac arrest was suspected to have had an A-V block. So we report this family to have familial cardiac conduction defect with the confidence.
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