Linkage Study of Long QT Syndrome In An Amish Kindred
To screen by electrocardiography the entire population of 1,400 individuals in seven Amish Mennonite communities in order to perform genetic linkage studies of long QT syndrome (LQTS).
Long QT Syndrome
|Study Design:||Observational Model: Natural History|
|Study Start Date:||January 1990|
|Estimated Study Completion Date:||December 1992|
LQTS is a severe heart disorder leading to sudden death due to ventricular tachycardia or ventricular fibrillation. The syndrome segregated as a Mendelian recessive in the highly inbred population under study and resulted in the sudden death of several members of the population.
The entire population was screened to identify cases of LQTS. In addition, 29 obligate heterozygotes were studied by 24-hour Holter monitoring to determine whether these gene carriers had any subtle phenotypes. The data were subjected to complex segregation and linkage analysis to establish the mode of inheritance and penetrance of LQTS.