• long QT syndrome;
  • dynamic QT–RR coupling;
  • Holter recording;
  • QT adaptation;
  • QT parameters

Background: The patients with the long QT syndrome type-1 (LQT-1) have an impaired adaptation of the QT interval to heart rate changes. Yet, the description of the dynamic QT–RR coupling in genotyped LQT-1 has never been thoroughly investigated.

Method: We propose a method to model the dynamic QT–RR coupling by defining a transfer function characterizing the relationship between a QT interval and its previous RR intervals measured from ambulatory Holter recordings. Three parameters are used to characterize the QT–RR coupling: a fast gain (GainF), a slow gain (GainL), and a time constant (τ). We investigated the values of these parameters across genders, and in genotyped LQT-1 patients with normal QTc interval duration (QTc  <  470 ms).

Results: The QT–RR dynamic profiles are significantly different between LQT-1 patients (97) and controls (154): LQT-1 have longer QTc interval (453  ± 35 vs. 384  ± 26 ms, P  <  0.0001), and an increased dependency of the QT interval to previous RR changes revealed by a larger GainL (0.22  ± 0.06 vs. 0.18  ± 0.07, P  <  0.0001) and GainF (0.05  ± 0.02 vs. 0.03  ± 0.01, P  <  0.0001). Importantly, LQT-1 patients have a faster QT dynamic response to previous RR changes described by τ: 122  ± 44 vs. 172  ± 92 beats (P  <  0.0001). This faster QT dynamic response of the QT–RR dynamic coupling remained in LQT-1 patients with QTc in a normal range (<430 ms).

Conclusions: The measurement of QT–RR dynamic coupling could be used in patients suspected to carry a concealed form of the LQT-1 syndrome, or to provide insights into the types of arrhythmogenic triggers a patient may be prone to.