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.