Khayyat Ibrahim M, Ordonez Maria Victoria, Marelli Ariane, Therrien Judith
Adult Cardiology, McGill University Health Center, Montreal, Quebec, Canada.
Heart Failure and Heart Transplant Department, McGill University Health Center, Montreal, Quebec, Canada.
JACC Adv. 2025 Jun;4(6 Pt 1):101758. doi: 10.1016/j.jacadv.2025.101758. Epub 2025 May 14.
Since the initial Fontan procedure introduced in 1968 for tricuspid atresia, significant advancements have expanded its application to various congenital cardiac anomalies where a biventricular circulation is unattainable. Despite improved survival rates, Fontan circulation tends to fail over time leading to late morbidity and mortality. Diastolic dysfunction is increasingly recognized as a significant contributor to circulatory insufficiency and failure in Fontan patients. This review aims to assess the current evidence for diagnosing diastolic dysfunction in adults with failing Fontan circulation, including biomarkers, echocardiography, cardiac magnetic resonance imaging, and catheterization. While advancements have been made in understanding diastolic dysfunction in single ventricles, challenges remain due to the unique anatomy and physiology of Fontan patients. Future research should focus on refining diagnostic parameters and exploring potential therapies tailored to the distinct needs of this population.
自1968年首次引入用于治疗三尖瓣闭锁的Fontan手术以来,重大进展已将其应用扩展到各种无法实现双心室循环的先天性心脏异常。尽管生存率有所提高,但Fontan循环往往会随着时间的推移而失败,导致晚期发病和死亡。舒张功能障碍越来越被认为是Fontan患者循环功能不全和衰竭的重要原因。本综述旨在评估目前关于诊断Fontan循环衰竭的成人舒张功能障碍的证据,包括生物标志物、超声心动图、心脏磁共振成像和心导管检查。虽然在理解单心室舒张功能障碍方面取得了进展,但由于Fontan患者独特的解剖结构和生理功能,挑战仍然存在。未来的研究应专注于完善诊断参数,并探索针对该人群独特需求的潜在治疗方法。