Objectives
To assess whether heart rate (HR) reduction following an exercise stress test (ExStrT), an easily quantifiable marker of vagal reflexes, might identify high and low risk long QT syndrome (LQTS) type 1 (LQT1) patients.
Background
Identification of LQTS patients more likely to be symptomatic remains elusive. We have previously shown that depressed baroreflex sensitivity (BRS), an established marker of reduced vagal reflexes, predicts low probability of symptoms among LQT1.
Methods
We studied 169 LQTS genotype-positive patients below age 50 who performed an ExStrT with the same protocol, on and off β-blockers including 47 South African LQT1 patients all harboring the KCNQ1-A341V mutation and 122 Italian LQTS patients with impaired (IKs−, LQT1, n=66) or normal (IKs+, 50 LQT2 and 6 LQT3) IKs current.
Results
Despite similar maximal HR and workload, by the first minute after cessation of exercise the symptomatic patients in both IKs− groups had a greater HR reduction compared to the asymptomatic (19±7 vs 13±5 and 27±10 vs 20±8 bpm, both p=0.009). By contrast, there was no difference between the IKs+ symptomatic and asymptomatic patients (23±9 vs 26±9 bpm, p=0.47). LQT1 patients in the upper tertile for HR reduction had a higher risk of being symptomatic (OR 3.28, 95% CI 1.3–8.3, p=0.012).
Conclusions
HR reduction following exercise identifies LQT1 patients at high or low arrhythmic risk, independently of β-blocker therapy, and contributes to risk stratification. Exercise training, which potentiates vagal reflexes, should be avoided by LQT1 patients.
Objectives
We aimed to evaluate the relationship between mitral annular disjunction (MAD) severity and myocardial interstitial fibrosis at the left ventricular (LV) base in patients with mitral valve prolapse (MVP), and to assess the association between severity of interstitial fibrosis and the occurrence of ventricular arrhythmic events.
Background
In MVP, MAD has been associated with myocardial replacement fibrosis and arrhythmia, but the importance of interstitial fibrosis remains unknown.
Methods
In this retrospective study, 30 patients with MVP and MAD (MVP–MAD) underwent cardiovascular magnetic resonance (CMR) with assessment of MAD length, late gadolinium enhancement (LGE), and basal segments myocardial extracellular volume (ECVsyn). The control group included 14 patients with mitral regurgitation (MR) but no MAD (MR-NoMAD) and 10 patients with normal CMR (NoMR-NoMAD). Fifteen MVP–MAD patients underwent 24 h-Holter monitoring.
Results
LGE was observed in 47% of MVP–MAD patients and was absent in all controls. ECVsyn was higher in MVP–MAD (30 ± 3% vs 24 ± 3% MR-NoMAD, p < 0.001 and vs 24 ± 2% NoMR-NoMAD, p < 0.001), even in MVP–MAD patients without LGE (29 ± 3% vs 24 ± 3%, p < 0.001 and vs 24 ± 2%, p < 0.001, respectively). MAD length correlated with ECVsyn (rho = 0.61, p < 0.001), but not with LGE extent. Four patients had history of out-of-hospital cardiac arrest; LGE and ECVsyn were equally performant to identify those high-risk patients, area under the receiver operating characteristic (ROC) curve 0.81 vs 0.83, p = 0.84). Among patients with Holter, 87% had complex ventricular arrhythmia. ECVsyn was above the cut-off value in all while only 53% had LGE.
Conclusion
Increase in ECVsyn, a marker of interstitial fibrosis, occurs in MVP–MAD even in the absence of LGE, and was correlated with MAD length and increased risk of out-of-hospital cardiac arrest. ECV should be includedin the CMR examination of MVP patients in an effort to better assess fibrous remodelling as it may provide additional value beyond the assessment of LGE in the arrhythmic risk stratification.
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