Congenital long QT syndrome (LQTS) causes life-threatening cardiac arrhythmias and is the leading cause of sudden cardiac death in young people. Measurements of QT prolongation during exercise or postural change have been recommended to assist in the diagnosis of LQTS, particularly in those with hidden phenotypes. However, most evidence has come from single-center studies without external validation in an independent cohort. Inter-study heterogeneity leads to significant difficulties in interpreting and applying consistent diagnostic criteria for LOTS. A comprehensive systematic review is critically needed to summarize the evidence and validate the diagnostic performance of QT intervals during exercise or postural change across a variety of studies. In this study, we review cross-sectional and cohort studies evaluating the efficacy and feasibility of exercise tests or postural changes in diagnosing LQTS, and propose possible problems resulting from exercise tests.
