In a founder population of long QT syndrome type 1 (LQT1), which avoids the confounding factors due to different mutations and segregates the malignant KCNQ1-A341V mutation (Brink et al., 2005; Crotti et al., 2007; Brink and Schwartz, 2009), the characterization of cardiac autonomic control and baroreflex function was found to be useful to improve the risk stratification of arrhythmic events (Schwartz et al., 2008b; Crotti et al., 2012; Bari et al., 2014a,b, 2015; Porta et al., 2015). Here, KCNQ1 is linked to long QT syndrome 1.