Ericsson Sabina, Paakkanen Riitta, Taipale Marko, Helle Emmi, Peltonen Juha, Kormi Alma, Vepsäläinen Teemu, Mattila Ilkka, Pätilä Tommi, Martelius Laura, Ojala Tiina
New Children's Hospital Pediatric Research Center, Helsinki University Hospital, Helsinki, Finland
Stem Cells and Metabolism Research Program, Faculty of Medicine, University of Helsinki, Helsinki, Finland.
Open Heart. 2025 May 21;12(1):e003306. doi: 10.1136/openhrt-2025-003306.
Cardiac magnetic resonance (CMR) imaging provides critical insight into the prognosis of Fontan patients, enhancing our understanding of their long-term outcomes. This study aimed to investigate the prognostic role of CMR in a carefully selected cohort of Fontan patients with the highest initial likelihood of survival.
This retrospective nationwide cohort study included 148 Fontan patients who underwent post-Fontan CMR imaging in Finland between 2017 and 2023. The primary endpoint was death or listing for heart transplant. The secondary endpoint was myocardial fibrosis determined by native T1 mapping measured by CMR.
The median time from the Fontan procedure to CMR examination was 10.8 years, with a median post-CMR follow-up of 2.55 years. Six patients (4.1%) reached the primary endpoint. Significant haemodynamic risk factors for the primary endpoint included worse global longitudinal strain (p=0.03), worse global circumferential strain (p<0.001) and reduced ejection fraction (p=0.04). Notably, patients with decreased myocardial function showed higher native T1-mapping values. Additional clinical risk factors that were associated with the primary endpoint included arrhythmias (p=0.01), protein-losing enteropathy (p=0.01), New York Heart Association functional class ≥2 (p<0.001) and liver cirrhosis (p=0.01).
CMR provides critical insights into long-term outcomes in Fontan patients. In our prioritised cohort, characterised by an initially high likelihood of survival, the observed risks of adverse outcomes corroborate findings from higher mortality cohorts. This underscores the importance of myocardial function and native myocardial T1 mapping in risk assessment, reaffirming CMR's role in effective risk stratification for this population.
心脏磁共振成像（CMR）为了解Fontan手术患者的预后提供了关键信息，增进了我们对其长期结局的认识。本研究旨在调查CMR在一组精心挑选的、初始生存可能性最高的Fontan手术患者中的预后作用。
这项全国性回顾性队列研究纳入了2017年至2023年期间在芬兰接受Fontan术后CMR成像的148例Fontan手术患者。主要终点是死亡或列入心脏移植名单。次要终点是通过CMR测量的心肌T1 mapping确定的心肌纤维化。
从Fontan手术到CMR检查的中位时间为10.8年，CMR检查后的中位随访时间为2.55年。6例患者（4.1%）达到主要终点。主要终点的显著血流动力学危险因素包括较差的整体纵向应变（p=0.03）、较差的整体周向应变（p<0.001）和射血分数降低（p=0.04）。值得注意的是，心肌功能下降的患者显示出较高的心肌T1 mapping值。与主要终点相关的其他临床危险因素包括心律失常（p=0.01）、蛋白丢失性肠病（p=0.01）、纽约心脏协会功能分级≥2级（p<0.001）和肝硬化（p=0.01）。
CMR为Fontan手术患者的长期结局提供了关键信息。在我们这个初始生存可能性高的优先队列中，观察到的不良结局风险证实了高死亡率队列的研究结果。这强调了心肌功能和心肌T1 mapping在风险评估中的重要性，再次肯定了CMR在该人群有效风险分层中的作用。
