The first two decades of life were ignored, because the registration of juvenile mortality in the 19th century may have been incomplete. Ancestors, who died at young age, had a decreased change to pass their mutation on to present generations. Therefore, primary analyses were on all persons with 50% probability of being affected. The overall SMR was 1.32 (n=250). Analysis restricted to definitely affected persons yielded (n=118) an SMR of 1.59. Apparently, early death by FH did not occur or did not affect the identification of cases.