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Introduction: Most patients with Brugada syndrome (BrS) are first diagnosed in their 40s, with sudden cardiac death (SCD) often occurring in their 50s. Ventricular fibrillation (VF) may occur in some patients with BrS despite having been asymptomatic for a long period. This study aimed to assess the incidence and risk factors for late life-threatening arrhythmias in patients with BrS., Methods: Patients with BrS (n = 523; mean age, 51 ± 13 years; male, n = 497) were enrolled. The risk of late life-threatening arrhythmia was investigated in 225 patients who had experienced no cardiac events (CEs: SCD or ventricular tachyarrhythmia) for at least 10 years after study enrollment. The incidence of CEs during the follow-up period was examined., Results: During the follow-up of the 523 patients, 59 (11%) experienced CEs. The annual incidences of CEs were 2.87%, 0.77%, and 0.09% from study enrollment to 3, 3-10, and after 10 years, respectively. Among 225 patients who had experienced no CEs for at least 10 years after enrollment, four patients (1.8%) subsequently experienced CEs. Kaplan-Meier analysis revealed significant differences in the incidence of late CEs between patients with and without a history of symptoms (p = .032). The positive and negative predictive values of late CEs for the programmed electrical stimulation (PES) test were 2.9% and 100%, respectively., Conclusion: Our results suggest that patients with BrS who are asymptomatic and have no ventricular tachycardia/VF inducibility by PES are at extremely low risk of experiencing late life-threatening arrhythmias., (© 2024 Wiley Periodicals LLC.)

Background: The prognostic value of programmed electrical stimulation (PES) in Brugada syndrome (BrS) remains controversial. Asymptomatic BrS patients generally have a better prognosis than those with symptoms. The purpose of this study was to evaluate the value of nonaggressive PES with up to two extra stimuli and predict clinical factors for risk stratification in asymptomatic BrS patients., Methods: The study enrolled 193 consecutive asymptomatic BrS patients with type 1 ECG (mean age: 50 ± 13 years, 180 males) who underwent PES using a nonaggressive uniform protocol. Cardiac events (CEs: sudden cardiac death or ventricular tachyarrhythmia) during the follow-up period were examined., Results: During a mean follow-up of 101 ± 48 months, seven asymptomatic patients (3.6%) had a CE. The incidence of CEs was not different between patients with and without inducible ventricular tachyarrhythmia by PES (p = .51). The clinical significance of risk factor combinations, including spontaneous type 1 ECG, family history of sudden cardiac death, QRS duration in lead V 2 , and presence of J wave, was evaluated. Using the Kaplan-Meier method according to the number of risk factors, the prevalence of CE in patients with three or four risk factors was determined to be significantly higher than in those with one risk factor (p = .02 and p = .004, respectively)., Conclusions: The present study suggests that inducibility of ventricular tachyarrhythmia does not predict future CEs in asymptomatic BrS patients. Combination analysis of the other four clinical risk parameters may be effective for risk assessment., (© 2020 Wiley Periodicals LLC.)

Background: The aim is to evaluate the efficacy of additional radiofrequency ablation (RFCA) for spontaneous dissociated pulmonary vein activity (DPV-spike) after PV isolation (PVI) in patients with paroxysmal atrial fibrillation (AF)., Methods: One hundred fifty-two consecutive patients with paroxysmal AF referred for RFCA were enrolled. When DPV-spike was documented after PVI, we randomly assigned these patients to receive additional RFCA for DPV-spike or only PVI. We divided them into 4 groups: 87 patients without DPV-spike after PVI (No-spike group), 31 without DPV-spike after additional RFCA (Successful group), 8 with remaining DPV-spike after additional RFCA (Unsuccessful group), and 26 with DPV-spike after only PVI (Spike group). AF recurrence was evaluated among the 4 groups., Results: After PVI, DPV-spike was documented in 87 PVs (14%) from 65 patients. During 16 ± 9 months of follow-up, the incidence of the freedom from AF was significantly higher in the No-spike group than that in the Spike group and Unsuccessful group (P < 0.05), and tended to be higher in the Successful group than that in the Spike group and Unsuccessful group (P = 0.08 and 0.11, respectively). In a multivariate analysis, the remaining PV-spike after ablation was an independent predictor of AF recurrence (HR 2.44; CI 1.10-5.43, P < 0.05). No major complications including PV stenosis were observed during the follow-up., Conclusions: DPV-spike after PVI may be associated with higher electrical activity within the PVs and may be one of the risk factors for AF recurrence. Additional RFCA for DPV-spike was effective to reduce the AF recurrence after PVI., (© 2013 Wiley Periodicals, Inc.)

Introduction: The presence of early repolarization (ER) recently has been considered as a prognostic marker for sudden cardiac death in patients with idiopathic ventricular fibrillation (IVF), but there are certain numbers of IVF patients lacking ER. We aimed to clarify the clinical and electrocardiographic characteristics of the patients with IVF in the presence and absence of ER., Methods and Results: We studied 64 consecutive IVF patients from the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS) registry, which subjected with at least one episode of documented VF in the absence of structural heart diseases and excluding Brugada syndrome. We assessed clinical and electrophysiological characteristics in the IVF patients with and without ER. ER was defined as J-point elevation of >0.1 mV in either inferior or lateral leads. Twelve-lead electrocardiogram (ECG) demonstrated 24 (38%) of 64 patients with ER (ER[+] group) and the remaining 40 (62%) patients without ER (ER[-] group ). ER[+] group had a male predominance (92% for males) and ER[-] group revealed nearly equal distribution in both sexes. While no patients in ER[+] group showed intraventricular conduction disturbance (CD) with abnormal axis deviation and/or bundle branch block in ECG, 9 in ER[-] group had signs of CD (ER[-]/CD[+] subgroup). ER[-]/CD[+] subgroup had prolonged P-R interval and QRS duration compared to other patient groups., Conclusion: We found 3 distinct ECG patterns in IVF patients. In addition to the presence and absence of ER, there is a subgroup without ER demonstrating intraventricular CD, which represents a distinct clinical entity of IVF., (© 2013 Wiley Periodicals, Inc.)

Introduction: Patients with type 1 Brugada electrocardiogram (ECG) and an episode of syncope are diagnosed as symptomatic Brugada syndrome; however, all episodes of syncope may not be due to ventricular tachyarrhythmia., Methods and Results: Forty-six patients with type 1 Brugada ECG (all males, 51 +/- 13 years, 29 spontaneous, 17 Ic-drug induced), 20 healthy control subjects (all males, 35 +/- 11 years), and 15 patients with suspected neurally mediated syncope (NMS; 9 males, 54 +/- 22 years) underwent the head-up tilt (HUT) test. During the HUT test, 12-lead ECGs were recorded in all patients, and the heart rate variability was investigated in some patients. Sixteen (35%) of 46 patients with Brugada ECG, 2 (10%) of 20 control subjects, and 10 (67%) of 15 patients with suspected NMS showed positive responses to the HUT test. Although no significant differences were observed in HUT-positive rate among Brugada patients with documented VT (7/14; 50%), syncope (5/19; 26%) and asymptomatic patients (4/13; 31%), the HUT-positive rate was significantly higher in patients with documented VT (50%) and those with VT or no symptoms (11/27, 41%) compared to that in control subjects (10%) (P < 0.05). Augmentation of ST-segment amplitude (> or =0.05 mV) in leads V1-V3 was observed in 11 (69%) of 16 HUT-positive patients with Brugada ECG during vasovagal responses, and was associated with augmentation of parasympathetic tone following sympathetic withdrawal., Conclusion: Thirty-five percent of patients with Brugada ECG showed vasovagal responses during the HUT test, suggesting that some Brugada patients have impaired balance of autonomic nervous system, which may relate to their syncopal episodes.

Background: Neither the clinical characteristics nor risk stratification in Brugada syndrome have been clearly determined. We compared the clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for high-risk patients., Methods: A total of 188 consecutive individuals with Brugada syndrome (mean age 53 +/- 14 years, 178 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were evaluated in three groups of patients: Ventricular fibrillation (VF) group: patients with documented VF (N = 33); Syncope (Sy) group: patients with syncope without documented VF (N = 57); and asymptomatic (As) group: subjects without symptoms (N = 98). Their prognostic parameters were evaluated over a 3-year follow-up period., Results: (1) Clinical characteristics: incidence of past history of atrial fibrillation (AF) was significantly higher in the VF and Sy groups than in the AS group (P = 0.04). (2) On 12-lead ECG, r-J interval in lead V2 and QRS duration in lead V6 were longest in the VF group (P = 0.001, 0.002, respectively). (3) Clinical follow-up: during a mean follow-up period of 37 +/- 16 months, incidences of cardiac events (sudden death and/or VF) were higher in the symptomatic (VF/Sy) groups than in the As group (P < 0.0001). The r-J interval in lead V2 >/= 90 ms and QRS duration in lead V6 >/= 90 ms were found to be possible predictors of recurrence of cardiac events in symptomatic patients., Conclusions: Prolonged QRS duration in precordial leads was prominent in symptomatic patients. This ECG marker may be useful for distinguishing high- from low-risk patients with Brugada syndrome.

Introduction: The clinical phenotype is 8 to 10 times more prevalent in males than in females in patients with Brugada syndrome. Brugada syndrome has been reported to be thinner than asymptomatic normal controls. We tested the hypothesis that higher testosterone level associated with lower visceral fat may relate to Brugada phenotype and male predominance., Methods and Results: We measured body-mass index (BMI), body fat percentage (BF%), and several hormonal levels, including testosterone, in 48 Brugada males and compared with those in 96 age-matched control males. Brugada males had significantly higher testosterone (631 +/- 176 vs 537 +/- 158 ng/dL; P = 0.002), serum sodium, potassium, and chloride levels than those in control males by univariate analysis, and even after adjusting for age, exercise, stress, smoking, and medication of hypertension, diabetes, and hyperlipidemia, whereas there were no significant differences in other sex and thyroid hormonal levels. Brugada males had significantly lower BMI (22.1 +/- 2.9 vs 24.6 +/- 2.6 kg/m(2); P < 0.001) and BF% (19.6 +/- 4.9 vs 23.1 +/- 4.7%; P < 0.001) than control males. Testosterone level was inversely correlated with BMI and BF% in both groups, even after adjusting for the confounding variables. Conditional logistic regression models analysis showed significant positive and inverse association between Brugada syndrome and hypertestosteronemia (OR:3.11, 95% CI:1.22-7.93, P = 0.017) and BMI (OR:0.72, 95% CI:0.61-0.85, P < 0.001), respectively., Conclusions: Higher testosterone level associated with lower visceral fat may have a significant role in the Brugada phenotype and male predominance in Brugada syndrome.

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.

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.

Introduction: It often is difficult to determine the optimal ablation site for idiopathic ventricular tachycardia (VT) originating from the right ventricular outflow tract (RVOT) when the VT or premature ventricular complex (PVC) does not occur frequently. The aim of our study was to evaluate the usefulness of a multielectrode basket catheter for ablation of idiopathic VT originating from the RVOT., Methods and Results: Radiofrequency (RF) catheter ablation was performed using a 4-mm tip, quadripolar catheter in 50 consecutive patients with 81 VTs originating from the RVOT with (basket group = 25 patients with 45 VTs) or without (control group = 25 patients with 36 VTs) predeployment of a multielectrode basket catheter composed of 64 electrodes. Deployment of the multielectrode basket catheter was possible and safe in all 25 patients in the basket group. Ablation was successful in 25 (100%) of 25 patients in the basket group and in 22 (88%) of 25 patients in the control group. The total number of RF applications and the number of RF applications per PVC morphology did not differ between the two groups. However, both the fluoroscopic and ablation procedure times per PVC morphology were shorter in the basket group than in the control group (36.8+/-14.1 min vs 52.0+/-32.5 min, P = 0.04; 60.0+/-14.6 vs 81.5+/-51.2 min, P = 0.05). This difference was more pronounced in the 29 patients in whom VT or PVC was not frequently observed., Conclusion: The multielectrode basket catheter is safe and useful for determining the optimal ablation site in patients with idiopathic VT originating from the RVOT, especially in those without frequent VT or PVC.

Introduction: Brugada syndrome is characterized by an ST segment elevation in leads V1-V3 and a high incidence of ventricular fibrillation (VF). A mutation in a cardiac Na+ channel gene, SCN5A, has been linked to Brugada syndrome, and sodium channel blockers have been shown to be effective in unmasking the syndrome when concealed. The aim of this study was to examine the effects of Na+ channel blockers on ST segment elevation, QRS, corrected QT (QTc) interval, and ventricular arrhythmias in patients with Brugada syndrome., Methods and Results: We examined the effects of three different Na+ channel blockers (flecainide, disopyramide, and mexiletine) on the amplitude of the ST segment 20 msec after the end of QRS (ST20), QRS duration, QTc interval measured from 12-lead ECG, and ventricular arrhythmias in 12 Brugada and 10 control patients. Maximum ST20 observed in the V2 or V3 leads under baseline conditions was greater in the Brugada patients than in control patients, whereas QRS duration and maximum QTc interval were no different between the two groups. Flecainide and disopyramide, but not mexiletine, significantly increased maximum ST20 and QRS duration in both groups, although these effects were much more pronounced in the Brugada patients. The increases in ST20 and QRS duration with flecainide were significantly larger than those with disopyramide. An increase of 0.15 mV in ST20 with flecainide separated the two groups without overlap. Ventricular premature complexes developed only with flecainide in Brugada patients (3/12) displaying a marked ST elevation but not widening of QRS., Conclusion: Our findings suggest that Na+ channel blockers amplify existing I(Na) and possibly other ion channel defects, with a potency inversely proportional to the rate of dissociation of the drug from the Na+ channel, thus causing a prominent elevation of the ST segment and, in some cases, prolongation of QRS duration in patients with Brugada syndrome.

Introduction: The clinical usefulness of QT dispersion in 12-lead ECG has been controversial in identifying subjects at risk for sustained ventricular tachycardia (VT) in patients with idiopathic dilated cardiomyopathy (DCM). We hypothesized that increasing the spatial resolution of the ECG improves the accuracy of risk stratification. The purpose of this study was to test the ability of recovery time dispersion measured from 87-lead body surface potential mapping (BSPM) to identify patients at risk for sustained VT in idiopathic DCM., Methods and Results: We obtained 87-lead BSPM and 12-lead ECG in 33 patients with idiopathic DCM (15 patients with a history of sustained VT [VT(+) group] and 18 patients without a history of sustained VT [VT(-) group]) and in 20 normal control subjects. We measured the corrected QT dispersion and corrected recovery time dispersion from 12-lead ECG (QTc-12 dispersion and RTc-12 dispersion, respectively) and 87-lead BSPM (QTc-87 dispersion and RTc-87 dispersion, respectively). Signal-averaged ECG also was recorded in 25 patients. Neither the QTc-12 nor QTc-87 dispersion discriminated between the VT(+) and VT(-) groups patients. The VT(+) group patients had a larger but insignificant RTc-12 dispersion than the VT(-) group patients. In contrast, the RTc-87 dispersion was significantly larger in the VT(+) group patients than in the VT(-) group patients (236 +/- 39 msec vs 184 +/- 28 msec, P < 0.001). Receiver operating curve analysis indicated that the RTc-87 dispersion was as good as late potentials in predicting susceptibility to sustained VT; its sensitivity, specificity, and negative predictive value were 73%, 76%, and 76%, respectively (cutoff value 200 msec). RTc-87 dispersion >200 msec combined with positive late potentials provide high sensitivity (92%) and high negative predictive value (88%) for sustained VT., Conclusion: The RTc-87 dispersion is a useful tool to identify subjects at risk for sustained VT in patients with idiopathic DCM.

Introduction: The clinical characteristics of three patients with spontaneous or inducible ventricular fibrillation (VF) without apparent heart disease, who presented with J wave and ST segment elevation in inferior leads, are described., Methods and Results: All patients were male and experienced syncope. Their symptoms occurred at night or early in the morning. Holter ECG revealed infrequent premature ventricular complexes. Injection with disopyramide 2 mg/kg augmented ST segment elevation., Conclusion: These characteristics were very similar to those of patients with Brugada syndrome. These three patients with these specific features might have a variant of Brugada syndrome.

Introduction: Body surface distribution and magnitude of ST segment elevation and their reflection in 12-lead ECGs have not been clarified in Brugada syndrome., Methods and Results: Eighty-seven-lead body surface potential mapping and 12-lead ECGs were recorded simultaneously in 25 patients with Brugada syndrome and 40 control patients. The amplitude of the ST segment 20 msec after the end of QRS (ST20) was measured from all 87 leads, and an ST isopotential map was constructed. The maximum ST elevation (maxST20) was distributed in an area of the right ventricular outflow tract in all Brugada patients, and it was larger than that in control patients (0.37 +/- 0.13 vs 0.12 +/- 0.04 mV; P < 0.0005). The maximum was observed on the level of the parasternal fourth intercostal space, on which the V1 and V2 leads of the standard 12-lead ECG were located, in 18 of the 25 Brugada patients in whom typical coved- or saddleback-type ST elevation was seen in leads V1 and V2. The maximum was located on the second intercostal space in the remaining seven Brugada patients in whom only a mild saddleback-type ST elevation was seen in leads V1 and V2 of the 12-lead ECG. Typical ST segment elevation was recognized in leads V1 and V2, which were recorded on the second or third intercostal space. ST elevation in Brugada patients was dramatically normalized by isoproterenol, a beta-adrenergic agonist (maxST20 = 0.17 +/- 0.08 mV; P < 0.0005 vs control conditions), and accentuated by disopyramide, an Na+ channel blocker (maxST20 = 0.50 +/- 0.15 mV; P < 0.0005 vs control conditions), without any change in the location of the maxST20., Conclusion: Our data indicate that recordings of leads V1-V3 of the 12-lead ECG on the parasternal second or third intercostal space would be helpful in diagnosing suspected patients with Brugada syndrome. The data suggest that Na+ channel blockers are capable of accentuating ST elevation in leads V1-V3.

We report a case of verapamil-sensitive idiopathic ventricular tachycardia in which a mid-diastolic potential (MDP) 45 msec preceding the Purkinje potential (P potential) was recorded. Pacing during the tachycardia caused concealed entrainment, and the stimulus-QRS interval was equal to the P potential-QRS interval. The interval between the last pacing stimulus and the next P potential (postpacing interval) was longer than the ventricular tachycardia cycle length, but the MDP was orthodromically activated. These findings suggest that the MDP was on the reentrant circuit and the P potential was not on the reentrant circuit, but a bystander.

We present a patient with Brugada syndrome in whom 12-lead ECGs were recorded just before and after an episode of ventricular fibrillation (VF). A progressive elevation of both the RS-T segment and J waves just preceding and following the VF, and a close relationship between the amplitude of the RS-T segment and the preceding R-R intervals during atrial fibrillation, were documented. These findings support the hypothesis that RS-T elevation and a subsequent VF are related to a transient outward current-mediated spike-and-dome morphology of the epicardial action potential.

Introduction: The role of increased dispersion of repolarization in the genesis of torsades de pointes in patients with long QT syndrome has been clarified, but its role in the genesis of idiopathic ventricular fibrillation (VF) is not yet known. To investigate the pathogenesis of VF, we recorded monophasic action potentials (MAPs) from two siblings (48- and 36-year-old males) with familial idiopathic VF., Methods and Results: The elder brother (patient 1) showed a late r' wave in lead V1 and ST segment elevation in leads V1 through V3. The younger brother (patient 2) had late r' waves and ST segment elevation in leads II, III, and aVF, and the configurations were very similar to those of patient 1. MAPs were recorded from several sites in the right ventricular (RV) and left ventricular (LV) endocardium during constant right atrial pacing. The repolarization time (RT) was defined as the sum of the activation time (AT) and action potential duration (APD) at 90% repolarization. In patient 1, marked prolongation of the AT (140 msec) and the RT (380 msec) was recorded in the RV septum of the outflow tract, and the RT dispersion was markedly increased (125 msec). In contrast, patient 2 showed prolongation of the AT (80 msec) and RT (310 msec), and fractionated electrograms in the RV floor of the inflow tract. The RT dispersion was also increased (80 msec). VF and nonsustained polymorphic ventricular tachycardia were induced by double premature stimulation in patients 1 and 2, respectively. Chronic amiodarone therapy decreased the RT dispersion and suppressed the induction of ventricular tachyarrhythmias in patient 2, although late r' waves and slight ST segment elevation were unmasked in leads V1 and V2., Conclusion: Our data suggest that the increased dispersion of the RT, which was due mainly to a localized conduction delay in the RV, created an arrhythmogenic substrate in the two patients with familial idiopathic VF.

Introduction: Sympathetic stimulation plays an important role in the genesis of QT(U) prolongation and ventricular arrhythmias in congenital long QT syndrome (LQTS). Permanent pacemaker as well as beta blockers are reported to be effective in the management of this syndrome. The purpose of this study was to examine influence of epinephrine (alpha- and beta-adrenergic stimulation), propranolol (beta blocker), and atrial pacing on the spatial distribution of repolarization using body surface recovery time (RT) in congenital LQTS., Methods and Results: Body surface mapping was recorded in 16 patients with congenital LQTS and 20 control patients before and after epinephrine infusion (0.1 microg/kg per min), oral propranolol (1 to 2 mg/kg per day), addition of epinephrine during oral propranolol, atrial pacing at a cycle length of 600 or 750 msec, and addition of epinephrine during atrial pacing. The RT, that is, the interval between the QRS onset and the maximum dV/dt point in the ST-T segment, was measured automatically by a computer from each of the 87 mapping leads, and the corrected RT (RTc) was calculated using Bazett's method. In patients with congenital LQTS, epinephrine markedly changed the T(U) wave morphology and spatial distribution of RT, especially the distribution of maximum RT of the left anterior chest and back. Epinephrine prolonged the maximum RTc and the minimum RTc in 87 leads and increased the RTc dispersion (difference between maximum and minimum RTc in each patient). Neither propranolol nor atrial pacing changed the T(U) wave morphology, spatial distribution of RT, or any RTc parameters at rest. Propranolol prevented the influences of epinephrine on the T(U) wave morphology, spatial distribution of RT, and RTc parameters, whereas atrial pacing did not. In control patients, marked changes of the T(U) wave morphology and RTc parameters were not recognized during the entire protocol., Conclusions: Our results indicate that epinephrine markedly changes the spatial distribution of repolarization and increases the dispersion of repolarization, which probably are linked to arrhythmogenesis in congenital LQTS. The data suggest that propranolol but not atrial pacing are effective to suppress repolarization abnormalities during sympathetic stimulation.