Jacquemyn Xander, Rao Sruti, Doshi Ashish N, Danford David A, Kutty Shelby
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, MD, USA; Department of Cardiovascular Sciences, KU Leuven & Congenital and Structural Cardiology, UZ Leuven, Leuven, Belgium.
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
Heart Lung Circ. 2025 Jul 22. doi: 10.1016/j.hlc.2025.03.014.
We aimed to identify a progressive phenotype in repaired tetralogy of Fallot (rTOF), characterised by accelerated changes in right ventricular (RV) parameters on cardiac magnetic resonance (CMR) and assess its association with an accelerated course to pulmonary valve replacement (PVR).
This was a retrospective study of patients with rTOF who had two or more CMRs (2005-2022). Clinical variables and CMR data were collected, and annualised changes in CMR data were calculated. "Rapid progressors" were classified as those with annual RV ejection fraction decreases>3.5% or RV end-diastolic volume index (RVEDVi) increases>3.5 mL/m.
A total of 112 patients with rTOF (median age 14.7 years, 63 males) were included, with 60 identified as rapid progressors (12 based on RV ejection fraction, 48 based on the RVEDVi criteria). Baseline RV volumes and function were similar between groups, although rapid progressors had a higher pulmonary regurgitant fraction (PRF) (38.2±16.3 vs 31.4±15.4%; p=0.033). Pulmonary regurgitant fraction (PRF) (odds ratio 1.03; 95% confidence interval [CI] 1.00-1.05; p=0.037) and RV end-diastolic volume/left ventricular end-diastolic volume ratio (odds ratio 2.75; 95% CI 1.13-7.35; p=0.032) were significant predictors of the rapid progressive phenotype. During follow-up (median 9.3 years, interquartile range 6.8-13.8), 48 patients underwent PVR. Rapid progressors had a five-fold increased risk of PVR (hazard ratio 5.44; 95% CI 2.75-10.8; p<0.001). Adjusting for age, sex, transannular patch use, baseline RVEDVi, and PRF, the risk of PVR remained over five-fold higher in rapid progressors (hazard ratio 5.18; 95% CI 4.39-5.97; p<0.001).
This study identifies imaging biomarkers in the rTOF subpopulation that were more likely to have an early PVR.
我们旨在确定法洛四联症修复术后（rTOF）的一种进展性表型，其特征为心脏磁共振成像（CMR）显示右心室（RV）参数加速变化，并评估其与肺动脉瓣置换术（PVR）加速进程的相关性。
这是一项对有两次或更多次CMR检查（2005 - 2022年）的rTOF患者的回顾性研究。收集临床变量和CMR数据，并计算CMR数据的年化变化。“快速进展者”被定义为每年右心室射血分数降低>3.5%或右心室舒张末期容积指数（RVEDVi）增加>3.5 mL/m²的患者。
共纳入112例rTOF患者（中位年龄14.7岁，男性63例），其中60例被确定为快速进展者（12例基于右心室射血分数，48例基于RVEDVi标准）。两组间基线右心室容积和功能相似，尽管快速进展者的肺动脉反流分数（PRF）更高（38.2±16.3%对31.4±15.4%；p = 0.033）。肺动脉反流分数（PRF）（比值比1.03；95%置信区间[CI] 1.00 - 1.05；p = 0.037）和右心室舒张末期容积/左心室舒张末期容积比值（比值比2.75；95% CI 1.13 - 7.35；p = 0.032）是快速进展性表型的显著预测因素。在随访期间（中位9.3年，四分位间距6.8 - 13.8年），48例患者接受了PVR。快速进展者接受PVR的风险增加了五倍（风险比5.44；95% CI 2.75 - 10.8；p < 0.001）。在调整年龄、性别、跨环补片使用、基线RVEDVi和PRF后，快速进展者接受PVR的风险仍高出五倍多（风险比5.18；95% CI 4.39 - 5.97；p < 0.00 !）。
本研究确定了rTOF亚组中更可能早期接受PVR的影像学生物标志物。
