Innocenzi Adriana, Rangel Isabela, Póvoa-Corrêa Mariana, Parente Daniella Braz, Perez Renata, Rodrigues Rosana Souza, Fukuyama Lúcia Tomoko, Barroso Julia Machado, Oliveira Neto Jaime Araújo, Silvestre de Sousa Andréa, Luiz Ronir Raggio, Barbosa Rosa Célia Pimentel, Camargo Gabriel Cordeiro, Moll-Bernardes Renata
D'Or Institute for Research and Education (IDOR), Diniz Cordeiro, 30, Botafogo, Rio de Janeiro, RJ, 22281-100, Brazil.
Instituto Nacional de Cardiologia, Rio de Janeiro, RJ, Brazil.
Pediatr Cardiol. 2025 Apr;46(4):966-975. doi: 10.1007/s00246-024-03522-9. Epub 2024 May 21.
The abnormal hemodynamics in Fontan circulation due to persistently increased systemic venous pressure results in hepatic venous congestion and Fontan-associated liver disease. Combined assessment of cardiac and liver fibrosis and cardiac remodeling using multiparametric MRI in this context have not been fully explored. To evaluate cardiac and liver fibrosis and cardiac remodeling using multiparametric MRI in patients who have undergone Fontan procedures. Thirty-eight patients and 23 controls underwent cardiac and liver MRI examinations in a 3.0-T scanner. Mann-Whitney, Fisher exact test, and Spearman's correlation were applied to evaluate myocardial volumes, function, native cardiac and liver T1 mapping, ECVs and liver stiffness. The mean native cardiac T1 value (p = 0.018), cardiac ECV (p < 0.001), liver native T1 (p < 0.001), liver ECV (p < 0.001), and liver stiffness (p < 0.001) were higher in patients than controls. The indexed end-diastolic volume (EDVi) correlated with the myocardial ECV (r = 0.356; p = 0.033), native liver T1 (r = 0.571; p < 0.001), and with liver stiffness (r = 0.391; p = 0.015). In addition, liver stiffness correlated with liver ECV (r = 0.361; p = 0.031) and native liver T1 (r = 0.458; p = 0.004). An association between cardiac remodeling and cardiac and liver fibrosis were found in this population. The usefulness of MRI to follow cardiac and liver involvement in these patients is critical to improve treatment strategies and to prevent the need for combined liver and heart transplantation.
由于体循环静脉压力持续升高，Fontan循环中异常的血流动力学导致肝静脉淤血和Fontan相关肝病。在此背景下，使用多参数MRI对心脏和肝脏纤维化以及心脏重塑进行联合评估尚未得到充分探索。本研究旨在使用多参数MRI评估接受Fontan手术患者的心脏和肝脏纤维化以及心脏重塑情况。38例患者和23例对照在3.0-T扫描仪上接受了心脏和肝脏MRI检查。采用Mann-Whitney检验、Fisher精确检验和Spearman相关性分析来评估心肌容积、功能、心脏和肝脏的固有T1映射、细胞外容积分数（ECV）以及肝脏硬度。患者的平均心脏固有T1值（p = 0.018）、心脏ECV（p < 0.001）、肝脏固有T1（p < 0.001）、肝脏ECV（p < 0.001）和肝脏硬度（p < 0.001）均高于对照组。指数化舒张末期容积（EDVi）与心肌ECV（r = 0.356；p = 0.033）、肝脏固有T1（r = 0.571；p < 0.001）以及肝脏硬度（r = 0.391；p = 0.015）相关。此外，肝脏硬度与肝脏ECV（r = 0.361；p = 0.031）和肝脏固有T1（r = 0.458；p = 0.004）相关。在这一人群中发现了心脏重塑与心脏和肝脏纤维化之间的关联。MRI对于追踪这些患者的心脏和肝脏受累情况很有用，这对于改进治疗策略以及避免肝心联合移植的需求至关重要。
