Department of Cardiac Surgery, Royal Children's Hospital, the University of Melbourne and the Murdoch Children's Research Institute, Melbourne, Australia.
Eur J Cardiothorac Surg. 2010 Jul;38(1):59-65. doi: 10.1016/j.ejcts.2010.01.031. Epub 2010 Mar 4.
To determine outcomes of patients undergoing a bidirectional cavo-pulmonary shunt (BCPS) in the Fontan era.
From 1990 to 2000, 212 patients underwent a BCPS in a single institution at a mean age of 2.6+/-5 years.
Hospital mortality was 6% (13 patients). Five patients were lost to follow-up (3%). After a mean of 9+/-4 years, 15 patients had not yet been referred for Fontan surgery and their status was deemed satisfactory (11 1(1/2) ventricle repair, three BCPS and one patient converted to a bi-ventricular repair). Out of the remaining 179 patients who could potentially have reached a Fontan status, 141 had undergone the procedure and seven were awaiting Fontan completion. The expected completion rate of Fontan surgery after BCPS was 83% (148/179). Thirteen patients died and heart transplantation was necessitated in nine patients. Nine were deemed unsuitable for Fontan surgery for cardiac (seven), respiratory (one) or neurologic (one) reasons. After BCPS, the only predictors of late failure (death, heart transplantation or NYHA class III-IV) were not reaching Fontan status and the presence of bilateral superior vena cava (SVC). Ten-year survival was 99% (95% confidence interval (CI): 95-99%) for Fontan patients and 67% (95%CI: 50-80%) for those left with a BCPS.
In the present era, staging with BCPS represents a selection process in the Fontan pathway. Almost a fifth of the patients will not reach Fontan status. Outstanding results after Fontan surgery may hide a high attrition rate between BCPS and Fontan.
确定在 Fontan 时代行双向腔静脉肺动脉吻合术（BCPS）患者的结局。
1990 年至 2000 年，在一家机构中，212 例患者在平均年龄 2.6±5 岁时行 BCPS。
院内死亡率为 6%（13 例）。5 例患者失访（3%）。平均 9±4 年后，15 例患者尚未转诊行 Fontan 手术，其状况被认为是满意的（11 1（1/2）心室修复、3 例 BCPS 和 1 例患者转为双心室修复）。在其余 179 例可能达到 Fontan 状态的患者中，141 例已行该手术，7 例正在等待 Fontan 完成。BCPS 后行 Fontan 手术的预期完成率为 83%（148/179）。13 例患者死亡，9 例需要心脏移植。9 例因心脏（7 例）、呼吸（1 例）或神经（1 例）原因不适合行 Fontan 手术。BCPS 后，晚期失败（死亡、心脏移植或 NYHA 心功能分级 III-IV）的唯一预测因素是未达到 Fontan 状态和双侧上腔静脉（SVC）的存在。Fontan 患者的 10 年生存率为 99%（95%置信区间（CI）：95-99%），而遗留 BCPS 的患者为 67%（95%CI：50-80%）。
在当前时代，BCPS 分期代表了 Fontan 途径中的一个选择过程。近五分之一的患者将无法达到 Fontan 状态。Fontan 手术后的出色结果可能掩盖了 BCPS 与 Fontan 之间较高的淘汰率。
