Heng Ee Ling, Gatzoulis Michael A, Uebing Anselm, Sethia Babulal, Uemura Hideki, Smith Gillian C, Diller Gerhard-Paul, McCarthy Karen P, Ho Siew Yen, Li Wei, Wright Piers, Spadotto Veronica, Kilner Philip J, Oldershaw Paul, Pennell Dudley J, Shore Darryl F, Babu-Narayan Sonya V
From Adult Congenital Heart Disease Centre, (E.L.H., M.A.G., A.U.., B.S., H.U., W.L., V.S., P.O., D.F.S., S.V.B.-N.), Cardiac Morphology Unit (K.P.M., S.Y.H.), and Non-Invasive Cardiology Department (P.W.), Royal Brompton Hospital, London, United Kingdom; National Institute for Health Research Cardiovascular Biomedical Research Unit, Royal Brompton & Harefield NHS Foundation Trust and Imperial College London, United Kingdom (E.L.H., M.A.G., G.C.S., P.J.K., D.J.P., D.F.S., S.V.B.-N.); Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital of Münster, Germany (G.-P.D.); and Department of Thoracic and Cardiovascular Sciences, University of Padua, Italy (V.S.).
Circulation. 2017 Oct 31;136(18):1703-1713. doi: 10.1161/CIRCULATIONAHA.117.027402.
Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling.
Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y).
There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m; <0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m and RVESVi ≤82 mL/m. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; <0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm/m; <0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (=0.03) over a median of 9.5 years of follow-up.
Significant right heart structural reverse remodeling takes place immediately after PVR, followed by a continuing process of further biological remodeling manifested by further reduction in RVESVi. PVR before RVESVi reaches 82 mL/m confers optimal chances of normalization of RV function.
法洛四联症修复术后患者进行肺动脉瓣置换术（PVR）可改善症状并减少右心室（RV）容量。然而，关于心室结构和功能适应性变化率的数据却很少。我们旨在评估PVR术后即刻和中期的变化以及逆向重构的预测因素。
前瞻性纳入57例接受PVR的法洛四联症修复术后患者（年龄≥16岁；平均年龄35.8±10.1岁；男性38例），在PVR术前（pPVR）、术后即刻（中位数为6天）和术后中期（mPVR；中位数为3年）进行心血管磁共振检查。
校正后的RV舒张末期容积和RV收缩末期容积（RVESVi）在术后即刻和中期均降低（校正后的RV舒张末期容积：pPVR与术后即刻与mPVR相比，分别为156.1±41.9、104.9±28.4、104.2±34.4 mL/m²；RVESVi：pPVR与术后即刻与mPVR相比，分别为74.9±26.2、57.4±22.7、50.5±2l.7 mL/m²；P<0.01）。70%的患者术后舒张期和收缩期RV容积达到正常（主要终点），术前校正后的RV舒张末期容积≤158 mL/m²和RVESVi≤82 mL/m²可预测这一情况。RVESVi从基线到术后即刻再到中期随访呈逐渐下降趋势，表明PVR术后RV固有功能持续改善。左心室射血分数提高（pPVR与mPVR相比，分别为59.4±7.6%与61.9±6.8%；P<0.01），右心房发生逆向重构（pPVR与mPVR相比，分别为15.2±3.4与13.8±3.6 cm/m²；P<0.01）。术前RV流出道瘢痕较大与PVR术后RV/左心室射血分数改善较小相关。RV射血分数和峰值摄氧量可预测中位随访9.5年期间的死亡率（P=0.03）。
PVR术后即刻发生显著的右心结构逆向重构，随后是一个持续的进一步生物学重构过程，表现为RVESVi进一步降低。在RVESVi达到82 mL/m²之前进行PVR可使RV功能正常化的机会最佳。
