Khairy Paul, Landzberg Michael J, Gatzoulis Michael A, Lucron Hugues, Lambert Jean, Marçon François, Alexander Mark E, Walsh Edward P
Boston Adult Congenital Heart and Electrophysiology Services, Department of Cardiology, Children's Hospital Boston, 300 Longwood Ave, Boston, Mass 02115, USA
Circulation. 2004 Apr 27;109(16):1994-2000. doi: 10.1161/01.CIR.0000126495.11040.BD. Epub 2004 Mar 29.
Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain.
A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5+/-9.6 and 6.5+/-4.5 years after corrective surgery and electrophysiological testing, respectively. Clinical VT and/or SCD occurred in 24.6%. Sustained monomorphic VT and polymorphic VT were induced in 30.2% and 4.4%. Including polymorphic VT in the definition of inducibility improved sensitivity (66.1+/-6.0% versus 77.4+/-5.3%, P=0.0082) with a marginal reduction in specificity (81.6+/-2.8% versus 79.5+/-2.9%, P=0.0455). Positive and negative predictive values were 55.2+/-5.3% and 91.5+/-2.2%. Independent risk factors for inducibility were age at study > or =18 years (OR, 3.3), palpitations (OR, 2.8), prior palliative surgery (OR, 3.1), modified Lown criteria > or =2 (OR, 5.6), and cardiothoracic ratio > or =0.6 (OR, 3.3). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively (P<0.0001). Both inducible monomorphic VT [relative risk (RR), 5.0; P=0.0002] and polymorphic VT (RR, 12.9; P<0.0001) predicted future clinical VT and SCD. In a multivariate analysis, inducible sustained VT was an independent risk factor for subsequent events (RR, 4.7; 95% CI, 1.2 to 18.5; P=0.0268).
Programmed ventricular stimulation is of diagnostic and prognostic value in risk stratifying patients with repaired tetralogy of Fallot. In this patient population, inducible sustained polymorphic VT should not be disregarded as nonspecific.
研究一直表明，室性心动过速（VT）和心源性猝死（SCD）会使法洛四联症修复术后的长期预后复杂化，然而电生理检查在该人群中的诊断和预测价值尚不确定。
对252例接受法洛四联症修复术并进行程控心室刺激的多中心队列患者分别在矫正手术和电生理检查后随访了18.5±9.6年和6.5±4.5年。临床VT和/或SCD发生率为24.6%。持续性单形性VT和多形性VT的诱发率分别为30.2%和4.4%。将多形性VT纳入诱发率的定义可提高敏感性（66.1±6.0%对77.4±5.3%，P=0.0082），特异性略有降低（81.6±2.8%对79.5±2.9%，P=0.0455）。阳性和阴性预测值分别为55.2±5.3%和91.5±2.2%。诱发率的独立危险因素为研究时年龄≥18岁（OR，3.3）、心悸（OR，2.8）、既往姑息手术（OR，3.1）、改良洛恩标准≥2（OR，5.6）和心胸比率≥0.6（OR，3.3）。非诱发组和诱发组患者1年、5年、10年和15年的无事件生存率分别为97.9%、92.8%、89.3%和89.3%，而79.4%、62.6%、58.7%和50.3%（P<0.0001）。诱发的单形性VT[相对危险度（RR），5.0；P=0.0002]和多形性VT（RR，12.9；P<0.0001）均预测未来临床VT和SCD。在多因素分析中，诱发的持续性VT是后续事件的独立危险因素（RR，4.7；95%CI，1.2至18.5；P=0.0268）。
程控心室刺激在法洛四联症修复术后患者的危险分层中具有诊断和预后价值。在该患者群体中，诱发的持续性多形性VT不应被视为非特异性而被忽视。
