Suppression of implantable cardioverter defibrillator therapy during magnetic resonance imaging.
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Aged, 80 and over↗
Biomedical subjects
Jan Nemec
Publications and source records attributed to Jan Nemec.
Heart rate dependence of the QT interval duration: differences among congenital long QT syndrome subtypes.
INTRODUCTION: The heart rate dependence of QT interval duration is abnormal in patients with congenital long QT syndrome. Patients with LQT1 have a defective I(Ks) current, a major determinant of QT response to heart rate. METHODS AND RESULTS: We studied the heart rate dependence of QT interval duration in different long QT syndrome genotypes and control subjects using computerized QT measurements obtained from Holter recordings. The dependence of QT duration on heart rate is steeper in long QT syndrome than in control subjects (0.347 +/- 0.263 vs 0.162 +/- 0.083 at heart rate 100 beats/min; P < 0.05). In addition, QT interval is significantly longer in LQT2 and LQT3 than in LQT1 patients at slow (533 +/- 23 ms vs 468 +/- 30 ms at heart rate 60 beats/min; P < 0.0001) but not at rapid heart rate. The heart rate dependence of QT interval is steeper in LQT2 and LQT3 than in LQT1 (0.623 +/- 0.245 vs 0.19 +/- 0.079 at heart rate 100 beats/min; P < 0.05). For a given heart rate, the QT intervals vary more in LQT2 and LQT3 than in LQT1 patients (25.98 +/- 11.18 ms vs 14.39 +/- 1.55 ms; P < 0.01). CONCLUSION: Individual long QT syndrome genotypes differ with respect to QT interval dependence on heart rate. These differences may relate to the propensity of LQT2 and LQT3 patients to develop arrhythmias during bradycardia.
Adult↗
Catecholamine-provoked microvoltage T wave alternans in genotyped long QT syndrome.
Macrovoltage T wave alternans (TWA) has been described in congenital long QT syndrome (LQTS). Microvoltage T wave alternans (microV-TWA) at low heart rate (HR) is a marker of arrhythmogenic risk in many conditions, but its significance in LQTS has not been established. Twenty-three genotypically heterogeneous patients with LQTS and 16 control subjects were studied at rest and during phenylephrine and dobutamine provocation. Genotyping was established by PCR amplification and DNA sequencing of the three most common LQTS genes; KCNQ1/KVLQT1 (LQT1), KCNH2/HERG (LQT2), and SCN5A (LQT3). microV-TWA was determined using Fast Fourier transform. Precluded by ectopy, microV-TWA could not be assessed in 8 of 23 patients with LQTS. In the remaining 15 patients with LQTS, microV-TWA occurred at lower HR in LQTS than in controls (117 +/- 49 vs 153 +/- 37 beats/min; P < 0.05). Patients with LQTS developed microV-TWA at HR < 150 beats/min more often than controls (10/15 vs 2/16; P = 0.003). However, microV-TWA was not detected in the 3 individuals with a history of out-of-hospital cardiac arrest including a 14-year-old male with an F339del-KVLQT1 mutation (LQT1) who had dobutamine-provoked polymorphic ventricular tachycardia requiring external defibrillation. Catecholamine-provoked microV-TWA occurs at lower HR in patients with LQTS than in healthy people but does not identify high risk subjects.
Adolescent↗
Effect of phenylephrine provocation on dispersion of repolarization in congenital long QT syndrome.
INTRODUCTION: Syncope and sudden death are associated with sympathetic stimulation in LQT1 while LQT2 patients are more susceptible to arrhythmias during nonexertional states. Abnormal spatial (QTd)- and transmural (TDR)-dispersion of repolarization may indicate increased arrhythmogenicity. This study compares the effect of phenylephrine on QTd and TDR in genotyped LQTS to control (C). METHODS AND RESULTS: Seventeen LQT1, 12 LQT2, and 18 age- and sex-matched normal controls received 2 mcg/kg of phenylephrine intravenously. At baseline and peak phenylephrine effect, BP, QT, RR, Bazett's QTc, precordial QTd (QTmax-QTmin), and T-peak to T-end (Tp-e) intervals were determined blinded to the patient's clinical and genotype status. Baseline QT intervals and QTc were significantly longer in LQT1 and LQT2 compared to C. Baseline QTd and Tp-e were greater in LQT2 than either LQT1 or C: QTd=79+/-29 ms (LQT2), 53+/-26 (LQT1), and 45+/-15 (C) and Tp-e=120+/-30 ms (LQT2), 99+/-20 (LQT1), and 90+/-11 (C). Overall, phenylephrine exerted no significant effect on either QTd or Tp-e except with subgroup analysis of symptomatic LQTS where LQT1 and LQT2 patients had a divergent response with TDR. CONCLUSIONS: Phenylephrine-induced bradycardia decreased TDR in symptomatic LQT1 but increased TDR in symptomatic LQT2. The observed effects of phenylephrine are consistent with the protective effect of beta-blocker in LQT1 and the increased arrhythmogenicity noted during nonexertional states in LQT2.
Adolescent↗
Antiarrhythmic drugs: new agents and evolving concepts.
Properties of several new antiarrhythmic drugs are summarised in this review article. Recent concepts concerning their safety and efficacy of antiarrhythmics are discussed. A brief perspective on possible future strategies for pharmacotherapy of arrhythmias is provided.
Angiotensin Receptor Antagonists↗
Catecholamine-induced T-wave lability in congenital long QT syndrome: a novel phenomenon associated with syncope and cardiac arrest.
OBJECTIVE: To determine the effects of phenylephrine and dobutamine on repolarization lability in patients with genotyped long QT syndrome (LQTS). PATIENTS AND METHODS: Between December 1998 and August 2000, 23 patients with genotyped LQTS (13 LQT1, 7 LQT2, and 3 LQT3) and 16 controls underwent electrocardiographic stress testing at the Mayo Clinic in Rochester, Minn. Aperiodic repolarization lability was quantified from digitized electrocardiograms recorded during catecholamine stress testing with phenylephrine and dobutamine. T-wave lability was quantified as a root-mean-square of the differences between corresponding signal values of subsequent beats. The magnitude of aperiodic T-wave lability was quantified by using a newly derived T-wave lability index (TWLI). RESULTS: The TWLI was significantly greater in patients with LQTS than in controls (0.0945 +/- 0.0517 vs 0.0445 +/- 0.0123; P < .003). Marked T-wave lability (TWLI > or = 0.095) was detected in all 3 LQTS genotypes (10/23) but in no controls (P < .003). There was no correlation between the TWLI and the baseline corrected QT interval. All high-risk patients having either a history of out-of-hospital cardiac arrest or syncope had a TWLI of 0.095 or greater. CONCLUSIONS: Beat-to-beat nonalternating T-wave lability occurs in LQT1, LQT2, and LQT3 patients during catecholamine provocation and is associated with a history of prior cardiac events. The quantification of this novel phenomenon may assist in identifying LQTS patients with increased risk of sudden cardiac death.
Adolescent↗