Long-term prognosis in patients with non-type 1 Brugada ECG: results from a large Japanese cohort of Brugada syndrome

Abstract Background Most recent consensus conference report recommends Brugada syndrome (BrS) is diagnosed in patients with ST segment elevation with spontaneous, drug-induced or fever-induced type 1 morphology. Prognosis in patients with type 2 or 3 ECG without drug-induced or fever-induced type 1 ECG is still unknown. Purpose To evaluate a long-term prognosis in patients with non-type 1 Brugada ECG in a large Japanese cohort of BrS (The Japan Idiopathic Ventricular Fibrillation Study [J-IVFS]). Methods From 528 patients in J-IVFS, a total of 28 consecutive non-type 1 patients (54±14 years, all male, previous sustained ventricular tachyarrhythmias (VTs) 1, syncope 11, asymptomatic 16) were enrolled. Cardiac events (CI: sudden cardiac death or VTs) during the follow-up period were evaluated, and risk factors for the cardiac events were assessed. Results During a mean follow-up period of 111±91 months (median 134 months), 4 patients experienced cardiac events (1.5%/yr), who all had received implantable cardioverter defibrillator implantation. There was no statistically significant clinical risk factor for cardiac events. However, the incidences of cardiac events tended to be higher in symptomatic patients (CI: 25.0, non-CI: 6.3%, p=0.17), those with wide QRS duration >90 msec in lead V2 (CI: 30.0, non-CI: 6.3%, p=0.11), and those with inducible VTs (CI: 21.1, non-CI: 0%, p=0.20), as determined by the Kaplan-Meier method. The annual incidences of cardiac events in patients with symptom, wide QRS duration >90msec in lead V2, or inducible VTs were 2.8, 3.5, and 2.0%/yr, respectively. The incidences of cardiac events were significantly higher in patients with all these 3 factors (9.9%/yr) than those without (p=0.01). Conclusions Our large-scaled multicentre study revealed long-term prognosis in patients with non-type 1 Brugada ECG. The combination of symptom, wide QRS duration in lead V2, and inducible VTs may be useful to evaluate risk for cardiac events. The patients with all these parameters showed high risk for cardiac events and need to be carefully followed. Funding Acknowledgement Type of funding source: None

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Long-term prognosis of asymptomatic individuals with spontaneous or drug-induced type 1 electrocardiographic phenotype of Brugada syndrome

Journal of Electrocardiology ◽

10.1016/j.jelectrocard.2010.12.007 ◽

2011 ◽

Vol 44 (3) ◽

pp. 346-349 ◽

Cited By ~ 12

Author(s):

Konstantinos P. Letsas ◽

Reinhold Weber ◽

Michael Efremidis ◽

Panagiotis Korantzopoulos ◽

Klaus Astheimer ◽

...

Keyword(s):

Brugada Syndrome ◽

Drug Induced ◽

Long Term Prognosis ◽

Asymptomatic Individuals

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Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1

Journal of the American Heart Association ◽

10.1161/jaha.119.015709 ◽

2020 ◽

Vol 9 (17) ◽

Cited By ~ 1

Author(s):

Hideki Itoh ◽

Takashi Hisamatsu ◽

Takuhisa Tamura ◽

Kazuhiko Segawa ◽

Toshiaki Takahashi ◽

...

Keyword(s):

Sudden Death ◽

Myotonic Dystrophy ◽

Cardiac Conduction ◽

Myotonic Dystrophy Type 1 ◽

Myotonic Dystrophy Type ◽

Cardiac Events ◽

Conduction Disorders ◽

Qrs Duration

Background Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. Methods and Results This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine‐thymine‐guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow‐up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow‐up period of 87 months (Q1–Q3, 37–138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22–15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9–7.19, P <0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62–33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Conclusions Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.

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Abstract 2640: Clinical Characteristics and Risk Stratification in Symptomatic and Asymptomatic Patients with Brugada Syndrome - Multi-center Study in Japan-

Circulation ◽

10.1161/circ.116.suppl_16.ii_585-a ◽

2007 ◽

Vol 116 (suppl_16) ◽

Author(s):

Masahiko Takagi ◽

Yasuhiro Yokoyama ◽

Kazutaka Aonuma ◽

Naohiko Aihara ◽

Masayasu Hiraoka

Keyword(s):

Sudden Death ◽

Risk Stratification ◽

Brugada Syndrome ◽

Clinical Characteristics ◽

Low Risk ◽

Cardiac Events ◽

Asymptomatic Patients ◽

Risk Patients ◽

Qrs Duration

Background Neither the clinical characteristics nor risk stratification of symptomatic and asymptomatic patients with Brugada syndrome have been clearly determined. We compared clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for distinguishing high- from low-risk patients. Methods A total of 216 consecutive individuals with Brugada syndrome (mean age 52±14 years, 197 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were compared among 3 groups of patients: VF group; patients with aborted sudden death and documented VF (N=34), Syncope (Sy) group; patients with syncope without documented VF (N=70), and Asymptomatic (As) group; subjects without symptoms (N=112). Comparisons were made among the 3 groups as well as between the symptomatic (VF/Sy) and asymptomatic (As) groups. Short-term prognosis was also compared among the 3 groups, and between the VF/Sy and As groups. Results 1) Clinical characteristics: incidence of past history of AF was significantly higher in the VF and Sy groups than in the AS group (26, 26, and 12 %, respectively; [p=0.04]), though no other clinical parameters differed among the groups. 2) On resting 12-lead ECG, r-J interval (interval from QRS onset to J point) in lead V2 and QRS duration in lead V6 were highest in the VF group (104, 98, and 92 msec in V2 [p<0.001]; 106, 103, and 94 msec in V6 [p<0.0001], respectively, VF vs. Sy vs. As). 3) Positive late potential and inducibility of VF by EPS did not differ in incidence among the 3 groups. 4) Clinical follow-up: during a mean follow-up of 36±16 months, incidence of cardiac events (sudden death and/or VF) was higher in the VF/Sy groups than in the As group (29, 8, and 0 %, respectively [p<0.001]). Multivariate analysis showed that the frequencies of r-J interval ≥ 90 msec in lead V2 and QRS duration ≥ 90 msec in lead V6 were significantly higher in patients with cardiac events (p=0.02, 0.02, respectively). Conclusions In symptomatic patients, prolonged ventricular depolarization in precordial leads of the ECG was prominent in the VF group, and this sign can be used to distinguish high- from low-risk patients with Brugada syndrome.

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