Kasiakogias Alexandros, Kaskoutis Christos, Antoniou Christos-Konstantinos, Georgopoulos Stavros, Tsiachris Dimitrios, Arsenos Petros, Kouroutzoglou Alexandrina, Klettas Dimitrios, Vlachopoulos Charalambos, Tsioufis Konstantinos, Gatzoulis Konstantinos
First Department of Cardiology, School of Medicine, National and Kapodistrian University of Athens, Hippokration General Hospital, 11527 Athens, Greece.
J Cardiovasc Dev Dis. 2025 Mar 14;12(3):101. doi: 10.3390/jcdd12030101.
Improving clinical prediction of sudden cardiac death is a crucial step in the management of patients with hypertrophic cardiomyopathy. However, finding the optimal method for risk evaluation has been challenging, given the complexity and the wide variation in clinical phenotypes. This is particularly important, as these patients are often of younger age and defibrillator implantation is associated with a low but tangible long-term risk of adverse events. A number of risk factors, including degree of hypertrophy, presence of syncope and family history of sudden cardiac death, have typically been considered to indicate a higher risk. The European risk score for prediction of sudden cardiac death is widely used; however, it may not apply well in patients with specific forms of the condition, such as those with extreme hypertrophy. Increasing evidence suggests that the presence and extent of myocardial fibrosis assessed with cardiac magnetic resonance imaging should be considered in clinical decision-making. Some research suggests that integrating electrophysiological studies into traditional risk assessment models may further optimize risk prediction and significantly improve accuracy in detecting high risk patients. Novel cardiac imaging techniques, better understanding of the genetic substrate and artificial intelligence-based algorithms may prove promising for risk refinement. The present review article provides an updated and in-depth viewpoint.
改善心脏性猝死的临床预测是肥厚型心肌病患者管理中的关键一步。然而,鉴于临床表型的复杂性和广泛变异性,找到最佳的风险评估方法一直具有挑战性。这一点尤为重要,因为这些患者通常较为年轻,而植入除颤器会伴随着虽低但切实存在的长期不良事件风险。一些风险因素,包括肥厚程度、晕厥的存在以及心脏性猝死家族史,通常被认为表明风险较高。欧洲心脏性猝死预测风险评分被广泛使用;然而,它可能不适用于特定形式疾病的患者,比如那些极度肥厚的患者。越来越多的证据表明,在临床决策中应考虑通过心脏磁共振成像评估的心肌纤维化的存在和程度。一些研究表明,将电生理研究纳入传统风险评估模型可能会进一步优化风险预测,并显著提高检测高危患者的准确性。新型心脏成像技术、对基因底物的更好理解以及基于人工智能的算法可能在风险细化方面很有前景。本综述文章提供了一个更新且深入的观点。