Kim Yuli Y, Ruckdeschel Emily
Division of Cardiology, The Hospital of the University of Pennsylvania, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA Division of Cardiology, The Children' Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
Heart. 2016 Oct 1;102(19):1520-6. doi: 10.1136/heartjnl-2015-309067. Epub 2016 Jun 21.
Residual right ventricular outflow tract and pulmonary valve disease is common in adults with repaired tetralogy of Fallot. Chronic severe pulmonary regurgitation as a result of surgical repair can lead to myriad complications including right ventricular dysfunction, decreased exercise tolerance, right heart failure and symptomatic arrhythmias. The aim of restoring pulmonary valve integrity is to preserve right ventricular size and function with the intent of mitigating the development of symptoms and poor long-term outcomes. Right ventricular size thresholds by cardiac MRI have emerged beyond which reverse right ventricular remodelling after pulmonary valve replacement is less likely. Though pulmonary valve replacement has been shown to improve right ventricular dimensions and symptoms, no consistent improvement in right ventricular ejection fraction or objective measures of exercise capacity have been demonstrated. Furthermore, there are no long-term studies showing that normalisation of right ventricular size results in improved clinical outcomes. New transcatheter techniques of percutaneous pulmonary valve replacement have emerged with good short-term and mid-term outcomes, further adding to the complexity in determining 'when' and 'how' right ventricular outflow tract and pulmonary valve intervention should occur. With improved survival of these patients, the trend towards earlier pulmonary valve replacement at smaller right ventricular size and rapidly evolving transcatheter pulmonary valve techniques, the clinician must balance the goals of preserving right ventricular size and function in an attempt to prevent untoward outcomes with risks of multiple interventions in a patient's lifetime.
法洛四联症修复术后的成人中，残留的右心室流出道及肺动脉瓣疾病很常见。手术修复导致的慢性重度肺动脉反流可引发多种并发症，包括右心室功能障碍、运动耐量下降、右心衰竭及有症状的心律失常。恢复肺动脉瓣完整性的目的是维持右心室大小和功能，以减轻症状发展及不良长期预后。心脏磁共振成像得出的右心室大小阈值已出现，超过此阈值，肺动脉瓣置换术后右心室逆向重塑的可能性较小。尽管肺动脉瓣置换已显示可改善右心室大小及症状，但尚未证实右心室射血分数或运动能力客观指标有持续改善。此外，尚无长期研究表明右心室大小正常化会带来更好的临床结局。经皮肺动脉瓣置换的新型经导管技术已出现，短期和中期效果良好，这进一步增加了确定右心室流出道及肺动脉瓣干预“何时”及“如何”进行的复杂性。随着这些患者生存率的提高、在较小右心室大小时更早进行肺动脉瓣置换的趋势以及经导管肺动脉瓣技术的快速发展，临床医生必须在维持右心室大小和功能以预防不良后果的目标与患者一生中多次干预风险之间取得平衡。
