Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Division of Pediatrics, P. Universidad Catolica de Chile, Santiago, Chile.
Division of Cardiology, Department of Pediatrics, The Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Division of Pediatric Cardiology, Perth Children´s Hospital, Perth, Australia.
Am Heart J. 2019 Jul;213:8-17. doi: 10.1016/j.ahj.2019.03.012. Epub 2019 Apr 8.
Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes.
Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups.
The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ± 6.4% vs. 48.5 ± 7.2%, P = .81) and exercise capacity (% predicted peak VO:82.5 ± 17.7% vs. 75.6 ± 20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ± 3.9 vs.-25.7 ± 4.4, P < .001).
Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.
在肺动脉瓣反流（PR）导致的右心室（RV）扩张后，常见于肺动脉瓣狭窄（PS）或闭锁合并完整室间隔（PA/IVS）的介入治疗后。目前尚不清楚与法洛四联症（rToF）修复后的患者相比，PR 和 RV 扩张对这些患者的 RV 功能和运动能力是否有类似的影响。本研究的目的是比较非 ToF 与 rToF 患儿中，肺动脉瓣反流导致 RV 显著扩张且 RV 容积相当患者的运动耐量、RV 功能和心肌力学。
回顾性选取 30 例因肺动脉瓣反流导致 RV 显著扩张（非 ToF 组）且 RV 舒张末期容积指数（RVEDVi）和年龄与 30 例 rToF 患者相匹配的 PS 或 PA/IVS 患儿。比较两组患者的临床特征、超声心动图和 CMR 评估的 RV 功能、心电图和运动能力。
两组 RVEDVi 和年龄相匹配。两组的 RV 整体功能（RVEF：48.7±6.4% vs. 48.5±7.2%，P=0.81）和运动能力（%预测峰值 VO：82.5±17.7% vs. 75.6±20.4%，P=0.27）均显著降低。RVEDVi 与两组的 RVEF 均呈负相关（非 ToF：r=-0.39，P=0.04；rToF：r=-0.40，P=0.03）。rToF 患者的 QRS 间期较宽，且与 RVEF 呈负相关（非 ToF：r=-0.77，P<0.001；rToF：r=-0.69，P<0.001）。与整体功能相反，rToF 患者的 RV 纵向应变较非 ToF 患者低（-20.1±3.9 vs.-25.7±4.4，P<0.001）。
在通过 RVEDVi 匹配后，非 ToF 和 rToF 患者 RV 严重扩张时，RV 整体功能和运动能力均显著降低，这表明 RV 扩张对 RV 整体功能的影响相当。rToF 患者 RV 纵向功能降低和同步性更差的意义需要进一步探讨。
