From the Division of Pediatric Cardiology (T.E.D., D.J.G., C.R., J.R., M.J.G., J.J.R., A.C.G.), Department of Surgery (S.F., T.L.S., J.W.G.), and Department of Anesthesiology and Critical Care (L.M.M., J.M.S., S.C.N.), The Perelman School of Medicine at the University of Pennsylvania, and Cardiac Center at The Children's Hospital of Philadelphia; Division of Cardiology, University of Texas, Houston (L.S.R.); and Division of Critical Care, Lurie Children's Hospital, Chicago, IL (K.Y.A.).
Circ Cardiovasc Interv. 2017 Sep;10(9). doi: 10.1161/CIRCINTERVENTIONS.116.004924.
There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.
All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; =0.006) and lateral tunnel Fontan (HR, 4.3; =0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; =0.007), Norwood operation (HR, 2.3; =0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; =0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; <0.001) and hypoplastic left heart syndrome (HR, 2.0; =0.01).
In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.
对 Fontan 手术后长期生存者的手术和基于导管的再干预进行随访研究的资料有限。
对 1992 年至 2009 年期间在我院行 Fontan 手术的 773 例患者进行了回顾性分析。获得了 70%的 Fontan 后干预的现有信息。Fontan 后 20 年,65%的患者经历了手术或经导管干预。首次再干预的中位时间为 9.8 年。15 年时无再手术生存率为 69%,20 年时为 63%。最常见的手术为安置起搏器和 Fontan 修正术。安置起搏器的危险因素包括系统性左心室(风险比[HR],2.2;=0.006)和侧隧道 Fontan(HR,4.3;=0.001)。15 年时无介入导管化生存率为 53%,20 年时为 50%。最常见的操作是开窗关闭和肺动脉干预。解剖学适应证的导管介入与 Fontan 后 2002 年(HR,2.1;=0.007)、Norwood 手术(HR,2.3;=0.001)和更长的心肺转流时间(HR,每 10 分钟增加 1.1;=0.001)相关。生理学适应证的导管介入与 Fontan 后胸腔引流延长(HR,4.0;<0.001)和左心发育不良综合征(HR,2.0;=0.01)相关。
在这项 Fontan 生存者的研究中,三分之二的患者在 20 年内需要手术或基于导管的再干预。应告知患者家属,Fontan 通常不是单心室姑息治疗的最后阶段。