Great Ormond Street Hospital for Children, NHS Trust, London, UK (A.F., M.H., A.T., J.M., A.G., T.-Y.H., K.B.); University College of London, Institute of Cardiovascular Sciences, London, UK (A.F., A.T.); and Adult Congenital Heart Disease Research Network, UK (M.T.).
Circulation. 2013 Oct 22;128(17):1861-8. doi: 10.1161/CIRCULATIONAHA.113.001600. Epub 2013 Sep 24.
Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR.
Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8-5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4-57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m(2)) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak o2=-0.91±1.3; z e/ co2=0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mm Hg), pulmonary annulus diameters <0.5z, and unobstructed branch pulmonary arteries.
An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z.
法洛四联症修复术后通常需要进行肺动脉瓣置换（PVR），这是一项繁琐的手术。然而，在关注 PVR 适应证的文献中，对于无晚期 PVR 且运动能力良好的患者的详细解剖和生理特征，描述得并不充分。
在 1964 年至 2009 年期间，在一家机构接受标准法洛四联症修复术的 1085 例连续患者中，追踪了生存情况和无 PVR 生存情况。152 例总死亡中，100 例发生在术后 1 年内。10 至 50 岁的存活患者的年死亡风险是同龄正常人群的 4 倍（置信区间，2.8-5.4）。迄今为止，189 例患者在平均年龄 20±13 岁时（40 岁时存活的患者中有 36%）接受了二次 PVR。对 50 例无 PVR 的随机幸存者（年龄 4-57 岁）进行了心血管磁共振、超声心动图和运动试验。这些患者的右心室轻度扩张（右心室舒张末期容积=101±26 mL/m2），收缩功能良好（右心室射血分数=59±7%）。大多数患者的运动能力在正常范围内（z 峰值 o2=-0.91±1.3；z e/ co2=0.20±1.5）。在年龄>35 岁且运动能力正常的患者中，存在轻度残余的右心室流出道梗阻（平均梯度，24±13mmHg）、肺动脉瓣环直径<0.5z 和无阻塞的肺动脉分支。
在法洛四联症修复术后，相当一部分患者需要晚期进行 PVR。无 PVR 且运动能力正常的存活至 35 岁的患者可能已经接受了确定性的初次修复术；他们的右心室流出道表现为轻度残余梗阻和肺动脉瓣环直径<0.5z。
