Presentation and outcome of water-related events in children with long QT syndrome

摘要

Long QT syndrome (LQTS) occurs in about 1 in 2500 of the general population.1 Of the 12 genotypes, LQTS type 1 is the most common type and is classically associated with exercise-related events, especially swimming,2 and the QT interval prolongs with Epinephrine infusion3 and after exercise.4 Mutations are in KCNQ1 and the cardiac potassium channel I_Ks is defective, such that the action potential is prolonged. Of the first 388 index cases with LQTS at the Mayo clinic, 28 (11%) had a history of swimming triggered events; 85% of these were due to LQTS type 1, 6% to . LQTS type 2 and 3% were genotype negative.