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Takashi Noda

Publications and source records attributed to Takashi Noda.

At least 19 recordsLinked to original sources
PubMed2006

Genotype-specific onset of arrhythmias in congenital long-QT syndrome: possible therapy implications.

BACKGROUND: The identification of the molecular-genetic substrate underlying the various forms of the congenital long-QT syndrome (LQTS) has sparked studies into possible genotype-phenotype correlations with the aim of developing genotype-tailored therapy. The onset of torsade de pointes (TdP) may differ among LQTS patients, being pause dependent in some but not all. This disparity may point to different arrhythmia mechanisms and may affect therapy strategies. We studied whether the proportion of pause-dependent TdP onset varies among LQTS genotypes. METHODS AND RESULTS: We studied all LQT1 (n=10), LQT2 (n=34), and LQT3 (n=6) patients from 4 centers for whom ECGs of TdP onset were available and analyzed whether pauses preceded TdP onset (first available ECG per patient). Pauses preceded TdP significantly more often in LQT2 (68%) than in LQT1 (0%), and the interval immediately before TdP (pause interval) was significantly longer in LQT2 than in LQT1. The proportion of pause dependence in LQT3 (33%) appeared intermediate, but this group was too small for statistical analysis. CONCLUSIONS: Pause dependence of TdP onset is predominant in LQT2 but absent or rare in LQT1. It is suggested that disparities in pause dependence of TdP onset may reflect different arrhythmia mechanisms.

Adrenergic beta-Antagonists↗
PubMed2006

Diagnostic and prognostic value of a type 1 Brugada electrocardiogram at higher (third or second) V1 to V2 recording in men with Brugada syndrome.

To evaluate the diagnostic and prognostic value of an electrocardiogram (ECG) recorded at a higher (third or second) intercostal space, 98 men (17 to 76 years of age, mean +/- SD 47 +/- 13; with documented ventricular fibrillation [VF] in 22 and syncope in 32) were categorized into 3 groups; 68 men had a spontaneous type 1 ECG in standard leads V(1) and V(2) (S group), 19 had a spontaneous type 1 ECG only in the higher V(1) and V(2) leads (H group), and 11 had a type 1 ECG only after receiving class Ic sodium channel blockers (Ic group). There were no significant differences in baseline clinical characteristics, including VF episodes, syncope, atrial fibrillation, family history, late potentials, and inducibility of VF during electrophysiologic study across the 3 groups. During prospective follow-up periods (779 +/- 525, 442 +/- 282, and 573 +/- 382 days, respectively), subsequent cardiac events occurred in 11 men (16%) within the S group, in 2 men (11%) in the H group, and in 0 men (0%) in the Ic group (p = NS, S vs H group). In men with previous episodes of VF, subsequent cardiac events occurred in 7 (44%) within the S group and in 2 (50%) in the H group (p = NS). In conclusion, men with a spontaneous type 1 Brugada ECG recorded only at higher leads V(1) and V(2) showed a prognosis similar to that of men with a type 1 ECG in using standard leads V(1) and V(2).

Adolescent↗
PubMed2006

"Left-variant" atypical atrioventricular nodal reentrant tachycardia: electrophysiological characteristics and effect of slow pathway ablation within coronary sinus.

INTRODUCTION: Recent anatomical and electrophysiological studies have demonstrated the presence of leftward posterior nodal extension (LPNE); however, its role in the genesis of atrioventricular nodal reentrant tachycardia (AVNRT) is poorly understood. This study was performed to characterize successful slow pathway (SP) ablation site and to elucidate the role of LPNE in genesis of atypical AVNRT with eccentric activation patterns within the coronary sinus (CS). METHODS AND RESULTS: Among 45 patients with atypical AVNRT (slow-slow/fast-slow/both = 20/22/3 patients) with concentric (n = 37, 82%) or eccentric CS activation (n = 8, 18%), successful ablation site was evaluated. Among 35/37 patients (95%) with concentric CS activation, ablation at the conventional SP region outside CS eliminated both retrograde SP conduction and AVNRT inducibility. Among eight patients with eccentric CS activation, the earliest retrograde atrial activation was found at proximal CS 16 +/- 4 mm distal to the ostium during AVNRT. The earliest retrograde activation site was located at inferior to inferoseptal mitral annulus, consistent with the presumed location of LPNE. Ablation at the conventional SP region with electroanatomical approach only rendered AVNRT nonsustained without elimination of retrograde SP conduction in seven of eight patients (88%). Ablation targeted to the earliest retrograde atrial activation site within proximal CS (15 +/- 4 mm distal to the ostium); however, eliminated retrograde SP conduction and rendered AVNRT noninducible in six of eight patients (75%). CONCLUSION: In 75% of "left-variant" atypical AVNRT, ablation within proximal CS was required to eliminate eccentric retrograde SP conduction and render AVNRT noninducible, suggesting LPNE formed retrograde limb of reentrant circuit.

Adult↗
PubMed2006

Stable top-up operation at SPring-8.

Top-up operation allows SPring-8 to provide highly stable X-ray beams with arbitrary filling patterns. The implementation of top-up operation is described, with a focus on the simultaneous achievement of stability of stored current, beam orbit, purity of an isolated single bunch, and beam injection efficiency. Stored-current fluctuations have been routinely reduced to a level of 10(-3). Stored-beam oscillation on frequent beam injection, which was originally regarded as the most serious problem, has been successfully suppressed to a sufficiently low level that it never perturbs imaging experiments. Current impurities in nominally empty buckets have been reduced to a level of 10(-9) over more than one week of operation, making possible the measurement of time-resolved spectra using high-current bunches. Finally, excellent injection efficiency, higher than 80%, is routinely obtained, even for small undulator gaps, which is critical for preventing radiation damage to insertion-device magnets and to reduce leakage radiation. The process of achieving highly stabilized top-up operation at SPring-8 and its utility for user experiments are described.

Electrons↗
PubMed2006

Cellular basis for trigger and maintenance of ventricular fibrillation in the Brugada syndrome model: high-resolution optical mapping study.

OBJECTIVES: We examined how repolarization and depolarization abnormalities contribute to the development of extrasystoles and subsequent ventricular fibrillation (VF) in a model of the Brugada syndrome. BACKGROUND: Repolarization and depolarization abnormalities have been considered to be mechanisms of the coved-type ST-segment elevation (Brugada-electrocardiogram [ECG]) and development of VF in the Brugada syndrome. METHODS: We used high-resolution (256 x 256) optical mapping techniques to study arterially perfused canine right ventricular wedges (n = 20) in baseline and in the Brugada-ECG produced by administration of terfenadine (5 micromol/l), pinacidil (2 micromol/l), and pilsicainide (5 micromol/l). We recorded spontaneous episodes of phase 2 re-entrant (P2R)-extrasystoles and subsequent self-terminating polymorphic ventricular tachycardia (PVT) or VF under the Brugada-ECG condition and analyzed the epicardial conduction velocity and action potential duration (APD) restitutions in each condition. RESULTS: Forty-one episodes of spontaneous P2R-extrasystoles in the Brugada-ECG were successfully mapped in 9 of 10 preparations, and 33 of them were originated from the maximum gradient of repolarization (GR(max): 176 +/- 54 ms/mm) area in the epicardium, leading to PVT (n = 12) or VF (n = 5). The epicardial GR(max) was not different between PVT and VF. Wave-break during the first P2R-extrasystole produced multiple wavelets in all VF cases, whereas no wave-break or wave-break followed by wave collision and termination occurred in PVT cases. Moreover, conduction velocity restitution was shifted lower and APD restitution was more variable in VF cases than in PVT cases. CONCLUSIONS: Steep repolarization gradient in the epicardium but not endocardium develops P2R-extrasystoles in the Brugada-ECG condition, which might degenerate into VF by further depolarization and repolarization abnormalities.

Action Potentials↗
PubMed2006

Unique electrophysiologic characteristics of atrioventricular nodal reentrant tachycardia with different ventriculoatrial block patterns: effects of slow pathway ablation and insights into the location of the reentrant circuit.

BACKGROUND: The electrophysiologic mechanisms of different ventriculoatrial (VA) block patterns during atrioventricular nodal reentrant tachycardia (AVNRT) are poorly understood. OBJECTIVES: The purpose of this study was to characterize AVNRTs with different VA block patterns and to assess the effects of slow pathway ablation. METHODS: Electrophysiologic data from six AVNRT patients with different VA block patterns were reviewed. RESULTS: All AVNRTs were induced after a sudden AH "jump-up" with the earliest retrograde atrial activation at the right superoparaseptum. Different VA block patterns comprised Wenckebach His-atrial (HA) block (n = 4), 2:1 HA block (n = 1), and variable HA conduction times during fixed AVNRT cycle length (CL) (n = 1). Wenckebach HA block during AVNRT was preceded by gradual HA interval prolongation with fixed His-His (HH) interval and unchanged atrial activation sequence. AVNRT with 2:1 HA block was induced after slow pathway ablation for slow-slow AVNRT with 1:1 HA conduction, and earliest atrial activation shifted from right inferoparaseptum to superoparaseptum without change in AVNRT CL. The presence of a lower common pathway was suggested by a longer HA interval during ventricular pacing at AVNRT CL than during AVNRT (n = 5) or Wenckebach HA block during ventricular pacing at AVNRT CL (n = 1). In four patients, HA interval during ventricular pacing at AVNRT CL was unusually long (188 +/- 30 ms). Ablations at the right inferoparaseptum rendered AVNRT noninducible in 5 (83%) of 6 patients. CONCLUSION: Most AVNRTs with different VA block patterns were amenable to classic slow pathway ablation. The reentrant circuit could be contained within a functionally protected region around the AV node and posterior nodal extensions, and different VA block patterns resulted from variable conduction at tissues extrinsic to the reentrant circuit.

Adolescent↗
PubMed2006

Assessment of ability of activation mapping by duodecapolar catheter to diagnose complete isthmus block utilizing electroanatomical mapping system.

UNLABELLED: Duodecapolar catheters (DPCs) have been widely used to diagnose isthmus block after ablation in patients with atrial flutters. The purpose of this study was to assess the ability of DPC to diagnose isthmus block utilizing electroanatomical mapping system (CARTO). METHODS: Sixty-two patients with common atrial flutter underwent isthmus ablation during CS pacing while DPC was positioned at lateral wall of RA along tricuspid annulus (TA). When activation sequence of DPC recording changed exclusively counter-clockwise after ablation, or did not even after ablations targeting single potentials on ablation line (Ab-L), only lateral side of Ab-L was remapped using CARTO to assess whether complete block (CB) was established. RESULTS: After ablation, DPC recording suggested CB and incomplete block (ICB) in 53 (85%) and 9 patients (15%), respectively. In 51/53 patients (96%) with CB suggested by DPC recordings, CARTO remap also demonstrated CB, however, in the remaining two patients (4%), demonstrated ICB with residual isthmus conduction that was slow enough to allow wavefront conducting around TA to arrive at distal dipole of DPC earlier, mimicking CB. In 4/9 patients (44%) with ICB suggested by DPC recordings, CARTO remap also demonstrated ICB, however, in the remaining five patients (56%), demonstrated CB with earlier arrival of wavefront traversing posterior wall at just lateral to Ab-L than that conducting around TA, mimicking ICB. Sensitivity, specificity, positive, and negative predictive values of DPC to diagnose CB were 91, 67, 96, and 44%, respectively. CONCLUSIONS: Mapping using DPC would not be sufficient for diagnosis of CB and ICB.

Aged↗
PubMed2006

Site-specific influence of transversal conduction across crista terminalis on recognition of isthmus block.

BACKGROUND: Transversal conduction across crista terminalis (CT) is commonly observed during low-rate coronary sinus (CS) pacing after isthmus ablation and sometimes mimics incomplete clockwise isthmus block (IB). Site-specific influence of trans-cristal conduction gap on recognition of clockwise IB has been poorly understood. METHODS: Forty-five patients with common-type atrial flutter underwent mapping of CT and free wall lateral to CT during CS pacing of 100 ppm using CARTO after verification of IB, while duodecapolar catheter was positioned along tricuspid annulus to map periannular activation. RESULTS: A total of 43 gaps were demonstrated at upper (n = 15, 35%), middle (n = 17, 40%), and lower one-thirds of CT (n = 11, 25%) in 36 of 45 patients (80%). Gaps were single in 31 (69%) and multiple in 5 patients (11%). Activation patterns of free wall lateral to CT in CARTO maps were descending pattern without gaps (n = 9, 20%), collision pattern with a single gap (n = 31, 69%), and simultaneous pattern with multiple gaps (n = 5, 11%). Activation sequence of duodecapolar catheter was complete block pattern in 41 (91%) and incomplete block pattern in 4 patients (9%), masquerading as persistent clockwise isthmus conduction. The incomplete block pattern in duodecapolar catheter was exclusively associated with a gap at the lower CT (0/15, 0/17, and 4/11 gaps at upper, middle, and lower CT, respectively; P < 0.01) and was attributable to faster conduction across CT gaps than in complete block pattern. CONCLUSIONS: Trans-cristal conduction was commonly observed during low-rate CS pacing. Rapid transversal conduction exclusively across lower CT masqueraded as incomplete clockwise IB.

Atrial Flutter↗
PubMed2006

Pilsicainide-induced verapamil sensitive idiopathic left ventricular tachycardia.

A 20-year-old man was admitted to our hospital for treatment of verapamil sensitive idiopathic left ventricular tachycardia (ILVT). During the electrophysiologic study (EPS), no sustained ventricular tachycardia (VT) could be induced both at baseline and after infusion of isoproterenol. However, sustained clinical VT could be easily induced with single ventricular extrastimulation following intravenous administration of pilsicainide, a class Ic sodium channel blocker. The arrhythmia was ablated with radiofrequency catheter ablation.

Adult↗
PubMed2006

Spatial distribution of repolarization and depolarization abnormalities evaluated by body surface potential mapping in patients with Brugada syndrome.

BACKGROUND: Mutations in sodium channel gene, SCN5A, have been identified in Brugada syndrome, but it is still unclear as to how sodium channel dysfunction relates to arrhythmogenesis. We examined spatial distribution of both repolarization and depolarization abnormalities in patients with Brugada syndrome by using 87-leads body surface potential mapping (BSPM). METHODS: BSPM was recorded under baseline condition and after pharmacological interventions in 28 patients with Brugada syndrome (27 males, 49 +/- 14 years). The ST-segment amplitude 20 ms after the end of QRS (ST20), QRS duration, and corrected recovery time (RTc) were measured in all 87-leads, and averaged among 6-leads (D-F, 5-6) reflecting right ventricular outflow tract (RVOT) potentials and the other 81-leads. RESULTS: The ST20 was elevated at baseline, normalized by isoproterenol, and augmented by pilsicainide in only the RVOT. The RTc was longer at baseline and increased by pilsicainide in only the RVOT. On the other hand, the QRS duration was slightly widened at baseline, further increased by pilsicainide, but not changed by isoproterenol in both leads. CONCLUSIONS: The ST-segment elevation and the RTc prolongation were localized and modulated by agents only in the RVOT region, while the slight QRS widening at baseline and further increase by pilsicainide were observed homogeneously. Our data suggest that depolarization abnormalities are distributed homogeneously, whereas repolarization abnormalities are localized in the RVOT.

Adolescent↗
PubMed2006

Catheter ablation of stable and unstable ventricular tachycardias in patients with arrhythmogenic right ventricular dysplasia.

INTRODUCTION: A reentrant circuit within an area of abnormal myocardium is suspected as the origin of ventricular tachycardia (VT) in patients with arrhythmogenic right ventricular dysplasia (ARVD). OBJECTIVES: To examine the relationship between the reentrant circuits of VT and the abnormal electrograms in ARVD, and to assess the feasibility of a block line formation in the reentrant circuit isthmus utilizing electroanatomical mapping system (CARTO) guidance. METHODS AND RESULTS: An electrophysiological study and catheter ablation (CA) were performed in 17 ARVD patients (13 men, 47 +/- 17 year) using CARTO. Endocardial mapping during sinus rhythm demonstrated electrogram abnormalities extended from the tricuspid annulus (TA) or the right ventricular outflow tract in 16 of 17 patients. In 13 hemodynamically stable VTs, the reentrant circuits and critical slow conduction sites for the CA were investigated during VTs. The entire macro-reentrant pathway was identified in 6/13 stable VTs (figure-of-8 in 4, single loop in 2). In the remaining seven VTs, a focal activation pattern was found in four and an unidentifiable pattern in three. CA successfully abolished all the macro-reentrant and focal tachycardias, however, not effective in three unidentifiable VTs. In the 13 cases with unstable VT, the linear conduction block zone was produced between the sites with abnormal electrograms and the TA. Ultimately, 23/26 VTs (88%) became noninducible after the CA. During follow-up (26 +/- 15 months), 13/17 patients remained free from any VT episodes. CONCLUSIONS: CARTO is useful for characterizing the anatomical and electrophysiological substrates, and for identifying the optimal ablation sites for VT associated with ARVD.

Adult↗
PubMed2005

Malignant entity of idiopathic ventricular fibrillation and polymorphic ventricular tachycardia initiated by premature extrasystoles originating from the right ventricular outflow tract.

OBJECTIVES: The aim of this study was to assess the clinical characteristics and the efficacy of radiofrequency catheter ablation (RFCA) for idiopathic ventricular fibrillation (VF) and/or polymorphic ventricular tachycardia initiated by ventricular extrasystoles originating from the right ventricular outflow tract (RVOT). BACKGROUND: Ventricular fibrillation and/or polymorphic ventricular tachycardia are occasionally initiated by ventricular extrasystoles originating from the RVOT in patients without structural heart disease. METHODS: Among 101 patients without structural heart disease in whom RFCA was conducted for idiopathic ventricular tachyarrhythmias arising from the RVOT, we examined the clinical characteristics and the efficacy of RFCA in 16 patients with spontaneous VF and/or polymorphic ventricular tachycardia initiated by the ventricular extrasystoles originating from the RVOT. RESULTS: Among 16 patients, spontaneous episodes of VF were documented in 5 patients, and 11 patients had prior episodes of syncope. Holter recordings showed frequent isolated ventricular extrasystoles with the same morphology as that of initiating ventricular extrasystoles, and non-sustained polymorphic ventricular tachycardia with short cycle length (mean of 245 +/- 28 ms) in all 16 patients. Radiofrequency catheter ablation by targeting the initiating ventricular extrasystoles eliminated episodes of syncope, VF, and cardiac arrest in all patients during follow-up periods of 54 +/- 39 months. CONCLUSIONS: Our data suggest that the malignant entity of idiopathic VF and/or polymorphic ventricular tachycardia was occasionally present in patients with idiopathic ventricular arrhythmias arising from the RVOT. Radiofrequency catheter ablation was effective as a treatment option for this entity.

Adult↗
PubMed2005

Cellular and ionic mechanism for drug-induced long QT syndrome and effectiveness of verapamil.

OBJECTIVES: We examined the cellular and ionic mechanism for QT prolongation and subsequent Torsade de Pointes (TdP) and the effect of verapamil under conditions mimicking KCNQ1 (I(Ks) gene) defect linked to acquired long QT syndrome (LQTS). BACKGROUND: Agents with an I(Kr)-blocking effect often induce marked QT prolongation in patients with acquired LQTS. Previous reports demonstrated a relationship between subclinical mutations in cardiac K+ channel genes and a risk of drug-induced TdP. METHODS: Transmembrane action potentials from epicardial (EPI), midmyocardial (M), and endocardial (ENDO) cells were simultaneously recorded, together with a transmural electrocardiogram, at a basic cycle length of 2,000 ms in arterially perfused feline left ventricular preparations. RESULTS: The I(Kr) block (E-4031: 1 micromol/l) under control conditions (n = 5) prolonged the QT interval but neither increased transmural dispersion of repolarization (TDR) nor induced arrhythmias. However, the I(Kr) blocker under conditions with I(Ks) suppression by chromanol 293B 10 micromol/l mimicking the KCNQ1 defect (n = 10) preferentially prolonged action potential duration (APD) in EPI rather than M or ENDO, thereby dramatically increasing the QT interval and TDR. Spontaneous or epinephrine-induced early afterdepolarizations (EADs) were observed in EPI, and subsequent TdP occurred only under both I(Ks) and I(Kr) suppression. Verapamil (0.1 to 5.0 micromol/l) dose-dependently abbreviated APD in EPI more than in M and ENDO, thereby significantly decreasing the QT interval, TDR, and suppressing EADs and TdP. CONCLUSIONS: Subclinical I(Ks) dysfunction could be a risk of drug-induced TdP. Verapamil is effective in decreasing the QT interval and TDR and in suppressing EADs, thus preventing TdP in the model of acquired LQTS.

Action Potentials↗
PubMed2004

Classification and mechanism of Torsade de Pointes initiation in patients with congenital long QT syndrome.

AIMS: To examine the initiating mode of Torsade de Pointes (TdP) in patients with congenital long QT syndrome (LQTS). METHODS AND RESULTS: We evaluated 111 episodes of TdP recorded on the electrocardiograms of 24 patients with congenital LQTS, and clarified the initiating mode, the three consecutive preceding RR intervals defined as C(2), C(1), and C(0), the timing of initiating premature ventricular contraction (PVC) and the cycle length (CL) of TdP. Three different initiating patterns were observed: (1) a "short-long-short" sequence (SLS) pattern (23 patients, 72 TdP, 65%) defined as one or more short-long cardiac cycles followed by an initiating short-coupled PVC (C(1)>C(2) and C(0)), (2) an "increased sinus rate" (ISR) pattern (8 patients, 28 TdP, 25%) defined as a gradual increase in sinus rate with or without T-wave alternans (C(2)>/=C(1)>/=C(0)), and (3) a "changed depolarization" (CD) pattern (5 patients, 11 TdP, 10%) defined as a sudden long-coupled PVC or fusion beat followed by short-coupled PVC. The C(0) was shorter in ISR than SLS and CD (mean C(0): 488 vs. 587 and 603 ms, respectively; P<0.05). Therefore, the initiating PVC appeared near the T-wave peak of the last beat before onset in ISR, while it occurred after the T-wave peak in SLS and CD. The CL of TdP was shorter in ISR than in SLS (256 vs. 295 ms, P<0.05). CONCLUSIONS: Our data show the existence of three predominant initiating modes of TdP in patients with congenital LQTS and suggests a differential mechanism of initiation of TdP for each mode.

Adolescent↗
PubMed2004

Diagnostic value of epinephrine test for genotyping LQT1, LQT2, and LQT3 forms of congenital long QT syndrome.

OBJECTIVES: The aim of this study was to test the hypothesis that epinephrine test may have diagnostic value for genotyping LQT1, LQT2, and LQT3 forms of congenital long QT syndrome (LQTS). BACKGROUND: A differential response of dynamic QT interval to epinephrine infusion between LQT1, LQT2, and LQT3 syndromes has been reported, indicating the potential diagnostic value of the epinephrine test for genotyping the three forms. METHODS: The responses of 12-lead ECG parameters to epinephrine were retrospectively examined in 15 LQT1, 10 LQT2, 8 LQT3, and 10 healthy volunteers to select the best ECG criteria for separating the four groups. The epinephrine test then was prospectively conducted in 42 probands clinically affected with LQTS, their 67 family members, and 10 new volunteers. The best criteria were applied in a blinded fashion to prospectively separate a different group of 31 LQT1, 23 LQT2, 6 LQT3, and 30 Control patients (10 genotype-negative LQT1, 10 genotype-negative LQT2 family members, and 10 volunteers). RESULTS: The sensitivity (penetrance) by ECG diagnostic criteria was lower in LQT1 (68%) than in LQT2 (83%) or LQT3 (83%) before epinephrine and was improved with steady-state epinephrine in LQT1 (87%) and LQT2 (91%) but not in LQT3 (83%), without the expense of specificity (100%). The sensitivity and specificity to differentiate LQT1 from LQT2 were 97% and 96%, those from LQT3 were 97% and 100%, and those from Control were 97% and 100%, respectively, when Delta mean corrected Q-Tend >/=35 ms at steady state was used. The sensitivity and specificity to differentiate LQT2 from LQT3 or Control were 100% and 100%, respectively, when Delta mean corrected Q-Tend >/=80 ms at peak was used. CONCLUSIONS: Epinephrine infusion is a powerful test to predict the genotype of LQT1, LQT2, and LQT3 syndromes as well as to improve the clinical diagnosis of genotype-positive patients, especially those with LQT1 syndrome.

Adolescent↗
PubMed2003

Epinephrine unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome.

OBJECTIVES: This study was designed to test the hypothesis that epinephrine infusion may be a provocative test able to unmask nonpenetrant KCNQ1 mutation carriers. BACKGROUND: The LQT1 form of congenital long QT syndrome is associated with high vulnerability to sympathetic stimulation and appears with incomplete penetrance. METHODS: The 12-lead electrocardiographic parameters before and after epinephrine infusion were compared among 19 mutation carriers with a baseline corrected QT interval (QTc) of > or =460 ms (Group I), 15 mutation carriers with a QTc of <460 ms (Group II), 12 nonmutation carriers (Group III), and 15 controls (Group IV). RESULTS: The mean corrected Q-Tend (QTce), Q-Tpeak (QTcp), and Tpeak-end (Tcp-e) intervals among 12-leads before epinephrine were significantly larger in Group I than in the other three groups. Epinephrine (0.1 microg/kg/min) increased significantly the mean QTce, QTcp, Tcp-e, and the dispersion of QTcp in Groups I and II, but not in Groups III and IV. The sensitivity and specificity of QTce measurements to identify mutation carriers were 59% (20/34) and 100% (27/27), respectively, before epinephrine, and the sensitivity was substantially improved to 91% (31/34) without the expense of specificity (100%, 27/27) after epinephrine. The mean QTce, QTcp, and Tcp-e before and after epinephrine were significantly larger in 15 symptomatic than in 19 asymptomatic mutation carriers in Groups I and II, and the prolongation of the mean QTce with epinephrine was significantly larger in symptomatic patients. CONCLUSIONS: Epinephrine challenge is a powerful test to establish electrocardiographic diagnosis in silent LQT1 mutation carriers, thus allowing implementation of prophylactic measures aimed at reducing sudden cardiac death.

Adolescent↗