Fogel Mark A, Faerber Jennifer A, Mahmood Abdullah, Reddy Keerthi, Zhang Xuemei, Goldmuntz Elizabeth, Harris Matthew A, Biko David, Partington Sara, Paridon Stephen, Mcbride Michael, Ferrari Victor, Whitehead Kevin K, Mercer-Rosa Laura
Division of Cardiology, Department of Pediatrics The Children's Hospital of Philadelphia/The Perelman School of Medicine at The University of Pennsylvania School of Medicine 3401 Civic Center Blvd Philadelphia 19104 PA USA.
Department of Radiology The Children's Hospital of Philadelphia/The Perelman School of Medicine at The University of Pennsylvania School of Medicine Philadelphia PA USA.
J Am Heart Assoc. 2025 May 20;14(10):e037983. doi: 10.1161/JAHA.124.037983. Epub 2025 May 13.
Few large scale prediction models of clinical outcomes in repaired tetralogy of Fallot (rTOF) exist. Further, contraction fraction, a novel parameter indexing stroke volume by mass reflecting myocardial efficiency, has not been studied. The goals of this study were to develop and validate an rTOF prediction model of clinical outcome from a single center, the SCOUT-TOF (Single Center Outcomes Using Cardiac Magnetic Resonance in Tetralogy of Fallot) registry, using readily available cardiac magnetic resonance parameters and explore novel metrics.
We retrospectively reviewed cardiac magnetic resonance parameters of patients with rTOF undergoing cardiac magnetic resonance from 2005 to 2021. Composite outcome 1 (CO1) included death, transplantation, ventricular tachycardia, and pacemaker placement, and composite outcome 2 (CO2) added cardiovascular hospitalizations. An elastic net was used to identify significant variables to enter a best subsets logistic regression. A group of 761 patients with rTOF were studied with a median follow-up of 4.15 years; 31 and 44 CO1 and CO2 events occurred respectively. Right ventricular (RV) contraction fraction was the most significant predictor for CO1 (area under the curve, 0.72; odds ratio [OR], 0.54; =0.01) and CO2 (area under the curve, 0.69; OR, 0.60; =0.01). RV contraction fraction was lower for those met that CO1 and CO2 end points (median 1.84 [1.48-2.39] versus 2.34 [1.72-3.02] and 1.88 [1.51-2.53] versus 2.34 [1.72-3.02] cc×cm/g×m, <0.01 respectively). Additional significant predictors for CO1 were indexed RV mass/volume and left ventricular ejection fraction whereas for CO2, left ventricular global function index and left ventricular mass were additional predictors.
In rTOF, RV contraction fraction, a novel biomarker of ventricular efficiency, may be used to possibly improve risk stratification. In addition, not only RV but left ventricular measures of remodeling should be considered in the follow-up of these patients.
目前存在的法洛四联症修复术后（rTOF）临床结局的大规模预测模型较少。此外，收缩分数作为一种通过质量反映心肌效率来索引每搏输出量的新参数，尚未得到研究。本研究的目的是利用单中心的法洛四联症心脏磁共振单中心结局（SCOUT-TOF）注册研究中的现成心脏磁共振参数，开发并验证一个rTOF临床结局预测模型，并探索新的指标。
我们回顾性分析了2005年至2021年接受心脏磁共振检查的rTOF患者的心脏磁共振参数。复合结局1（CO1）包括死亡、移植、室性心动过速和起搏器植入，复合结局2（CO2）增加了心血管住院。采用弹性网法识别进入最佳子集逻辑回归的显著变量。对761例rTOF患者进行了研究，中位随访时间为4.15年；分别发生了31例和44例CO1和CO2事件。右心室（RV）收缩分数是CO1（曲线下面积，0.72；比值比[OR]，0.54；P=0.01）和CO2（曲线下面积，0.69；OR，0.60；P=0.01）的最显著预测因子。达到CO1和CO2终点的患者的RV收缩分数较低（中位数分别为1.84[1.48-2.39]对2.34[1.72-3.02]以及1.88[1.51-2.53]对2.34[1.72-3.02]cc×cm/g×m，P均<0.01）。CO1的其他显著预测因子是索引化的RV质量/体积和左心室射血分数，而对于CO2，左心室整体功能指数和左心室质量是额外的预测因子。
在rTOF中，RV收缩分数作为一种新的心室效率生物标志物，可能用于改善风险分层。此外，在这些患者的随访中，不仅应考虑RV，还应考虑左心室重构指标。
