Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA.
J Am Soc Echocardiogr. 2013 Jul;26(7):746-55. doi: 10.1016/j.echo.2013.03.019. Epub 2013 Apr 25.
Long-term outcome in repaired tetralogy of Fallot (TOF) is related to chronic pulmonary insufficiency (PI), right ventricular (RV) dilation, and deterioration of RV function. The aim of this study was to characterize clinical differences between restrictive and nonrestrictive RV physiology in young patients with repaired TOF.
Patients were prospectively enrolled from February 2008 to August 2009. Each had a clinic visit, brain natriuretic peptide assessment, exercise test, cardiac magnetic resonance study, and echocardiographic examination with assessment of regional myocardial mechanics. Consistent antegrade diastolic pulmonary arterial flow with atrial contraction identified restrictive RV physiology.
Twenty-nine patients (median age, 12 years; range, 8-33 years; nine male patients) were studied. Twelve had restrictive RV physiology. The median time since initial TOF repair was 12 years (range, 5-27 years). Restrictive physiology appeared more prevalent after transannular patch repair and was not influenced by other demographic features. The restrictive group had more PI (46% vs 28%, P = .002), larger RV end-diastolic volumes (128 vs 98 mL/m(2), P = .046), but similar ejection fractions, brain natriuretic peptide levels, New York Heart Association classes, and exercise capacity. RV basal and mid free wall peak diastolic strain rate differed between groups, negatively correlating with exercise time and positively correlating with PI in patients with restrictive physiology.
Restrictive RV physiology correlates with a larger right ventricle and increased PI after TOF repair but does not negatively affect other markers of myocardial health. Diastolic regional RV myocardial mechanics, particularly diastolic velocity and peak diastolic strain rate, differ for postoperative TOF patients with restrictive and nonrestrictive RV physiology; longitudinal study is necessary to understand the relationship of regional myocardial mechanics and patients' clinical status.
法洛四联症（TOF）修复术后的长期预后与慢性肺功能不全（PI）、右心室（RV）扩张和 RV 功能恶化有关。本研究旨在描述修复后 TOF 年轻患者中 RV 限制型和非限制型生理学之间的临床差异。
2008 年 2 月至 2009 年 8 月，前瞻性招募患者。每位患者均接受了门诊检查、脑利钠肽评估、运动试验、心脏磁共振检查和超声心动图检查，并评估了局部心肌力学。心房收缩时出现一致的顺行舒张肺动脉血流可确定 RV 限制型生理学。
29 例患者（中位数年龄为 12 岁；范围为 8-33 岁；9 例为男性）被纳入研究。12 例患者存在 RV 限制型生理学。TOF 初次修复后中位时间为 12 年（范围为 5-27 年）。跨瓣环补片修复后，RV 限制型生理学更为常见，且不受其他人口统计学特征的影响。限制型组的 PI 更多（46% vs. 28%，P =.002），RV 舒张末期容积更大（128 比 98 ml/m²，P =.046），但射血分数、脑利钠肽水平、纽约心脏协会心功能分级和运动能力相似。RV 基底段和中段游离壁舒张末期峰值应变率在两组间存在差异，与运动时间呈负相关，与限制型组的 PI 呈正相关。
TOF 修复术后 RV 限制型生理学与更大的右心室和增加的 PI 相关，但不会对其他心肌健康标志物产生负面影响。RV 限制型和非限制型术后 TOF 患者的 RV 局部心肌舒张力学不同，特别是舒张速度和舒张末期峰值应变率；需要进行纵向研究以了解局部心肌力学与患者临床状况之间的关系。
