The development of a novel histopathological predictive method of sudden cardiac death in patients with tetralogy of Fallot

2021 Fiscal Year Final Research Report

• Project/Area Number: 20K17085
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 53020:Cardiology-related
• Research Institution: Kyushu University
• Principal Investigator: Ishikita Ayako 九州大学, 大学病院, 学術研究員 (60829232)
• Project Period (FY): 2020-04-01 – 2022-03-31
• Keywords: 成人先天性心疾患 / ファロー四徴症 / 右室心筋傷害 / 突然死

Outline of Final Research Achievements

Right ventricular myocardium specimens by a transcatheter biopsy from adult patients with repaired tetralogy of Fallot were assessed pathologically. As a result, the right ventricular myocardium was moderately hypertrophied, and the right ventricle was moderate to highly fibrotic. In addition, fibrosis in the right ventricle correlated with an index called extracellular volume fraction measured by CT or MRI. Therefore, it was suggested that non-invasive image evaluation could provide the same information as invasive right ventricular myocardium evaluation.
Furthermore, this right ventricular myocardial injury was also confirmed in asymptomatic patients. Thus, preventing sudden death requires regular right ventricular myocardial damage assessment, including non-invasive imaging, and is considered an intervention indicator.

Free Research Field

成人先天性心疾患

Academic Significance and Societal Importance of the Research Achievements

医学の進歩により以前は救命できなかったファロー四徴症患者の多くが若年成人まで生存できるようになった。しかし、無症状で社会的に自立している若年成人患者にも、突然死の危険性があり、解決が急務である。本研究により、無症候のうちから右室心筋傷害が進行していること、および非侵襲検査でも右室心筋傷害を評価できることが示された。外来での、定期的な右室心筋傷害評価の意義が明確となり、より適切なタイミングでの治療介入が予後改善に寄与できると期待される。