O'Meagher Shamus, Seneviratne Martin, Skilton Michael R, Munoz Phillip A, Robinson Peter J, Malitz Nathan, Tanous David J, Celermajer David S, Puranik Rajesh
Faculty of Medicine, The University of Sydney, Sydney, Australia,
Pediatr Cardiol. 2015 Aug;36(6):1225-31. doi: 10.1007/s00246-015-1150-4. Epub 2015 Mar 21.
The relationship between exercise capacity and right ventricular (RV) structure and function in adult repaired tetralogy of Fallot (TOF) is poorly understood. We therefore aimed to examine the relationships between cardiac MRI and cardiopulmonary exercise test variables in adult repaired TOF patients. In particular, we sought to determine the role of RV mass in determining exercise capacity. Eighty-two adult repaired TOF patients (age at evaluation 26 ± 10 years; mean age at repair 2.5 ± 2.8 years; 23.3 ± 7.9 years since repair; 53 males) (including nine patients with tetralogy-type pulmonary atresia with ventricular septal defect) were prospectively recruited to undergo cardiac MRI and cardiopulmonary exercise testing. As expected, these repaired TOF patients had RV dilatation (indexed RV end-diastolic volume: 153 ± 43.9 mL/m(2)), moderate-severe pulmonary regurgitation (pulmonary regurgitant fraction: 33 ± 14 %) and preserved left (LV ejection fraction: 59 ± 8 %) and RV systolic function (RV ejection fraction: 51 ± 7 %). Exercise capacity was near-normal (peak work: 88 ± 17 % predicted; peak oxygen consumption: 84 ± 17 % predicted). Peak work exhibited a significant positive correlation with RV mass in univariate analysis (r = 0.45, p < 0.001) and (independent of other cardiac MRI variables) in multivariate analyses. For each 10 g higher RV mass, peak work was 8 W higher. Peak work exhibits a significant positive correlation with RV mass, independent of other cardiac MRI variables. RV mass measured on cardiac MRI may provide a novel marker of clinical progress in adult patients with repaired TOF.
成人法洛四联症(TOF)修复术后运动能力与右心室(RV)结构和功能之间的关系尚不清楚。因此,我们旨在研究成人TOF修复术后患者心脏磁共振成像(MRI)与心肺运动试验变量之间的关系。特别是,我们试图确定右心室质量在决定运动能力方面的作用。前瞻性招募了82例成人TOF修复术后患者(评估时年龄26±10岁;平均修复年龄2.5±2.8岁;修复后23.3±7.9年;53例男性)(包括9例室间隔缺损合并四联症型肺动脉闭锁患者),进行心脏MRI和心肺运动测试。正如预期的那样,这些TOF修复术后患者存在右心室扩张(右心室舒张末期容积指数:153±43.9 mL/m²)、中重度肺动脉反流(肺动脉反流分数:33±14%),左心室(左心室射血分数:59±8%)和右心室收缩功能(右心室射血分数:51±7%)保留。运动能力接近正常(峰值功:预测值的88±17%;峰值耗氧量:预测值的84±17%)。在单变量分析中,峰值功与右心室质量呈显著正相关(r = 0.45,p < 0.001),在多变量分析中(独立于其他心脏MRI变量)也是如此。右心室质量每增加10 g,峰值功增加8 W。峰值功与右心室质量呈显著正相关,独立于其他心脏MRI变量。心脏MRI测量的右心室质量可能为成人TOF修复术后患者的临床进展提供一个新的标志物。