Clinical and molecular differences between Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy

2021 Fiscal Year Final Research Report

• Project/Area Number: 19K08572
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 53020:Cardiology-related
• Research Institution: National Cardiovascular Center Research Institute
• Principal Investigator: NAGASE SATOSHI 国立研究開発法人国立循環器病研究センター, 病院, 部長 (50397907)
• Co-Investigator(Kenkyū-buntansha): 片岡 直也 富山大学, 学術研究部医学系, 助教 (10791634)
• Project Period (FY): 2019-04-01 – 2022-03-31
• Keywords: 不整脈源性右室心筋症 / ブルガダ症候群 / 心室細動 / 心不全 / 心室頻拍 / 突然死 / 埋込型除細動器

Outline of Final Research Achievements

This research found that the prevalence of the Brugada ECG pattern was quite low despite a long-term follow-up period and repeated ECG with upper intercostal recordings in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). The appearance of the Brugada ECG pattern was transient. The Brugada ECG pattern in ARVC patients was associated with a longer PQ interval, longer QRS duration, and cardiac events, including cardiac death and HF hospitalization. Unfortunately, genetic analysis was not performed in all ARVC patients with Brugada ECG pattern. Accordingly, the relationship between genetic mutation and Brugada type ECG in ARVC patients could not be evaluated. Further prospective, multicenter studies, which include a larger number of ARVC patients, are warranted to confirm these findings. However, at this point, the hypothesis that Brugada syndrome is an early stage of ARVC seems negative.

Free Research Field

循環器内科、不整脈

Academic Significance and Societal Importance of the Research Achievements

ブルガダ症候群患者は不整脈源性右室心筋症（ARVC）患者の初期段階であるという仮説があるが、本研究結果からはそれは否定的で、むしろかなり進行したARVC患者において一過性にブルガダ型心電図が出現する傾向を認めた。またその心電図波形も一般的な症候性ブルガダ症候群と比べて非典型的であった。ブルガダ症候群とARVCは類似した所見も認めるが基本的に異なる疾患であることが示唆された。