Department of Cardiovascular Surgery, Kyoto University, Graduate School of Medicine, Kyoto, Japan.
Eur J Cardiothorac Surg. 2010 Jun;37(6):1264-70. doi: 10.1016/j.ejcts.2009.12.026. Epub 2010 Feb 6.
Fontan completion in patients with atrial isomerism, in which the inferior vena cava (IVC) and the hepatic vein (HV) drain separately, is technically challenging. Herein, we review our surgical approach to these patients.
The medical records of 50 consecutive patients with atrial isomerism who underwent Fontan completion between 1998 and 2008 were reviewed retrospectively.
Separate HV drainage was present in 17 patients. Patients with interrupted IVC were excluded. Patient characteristics were as follows: median age, 26 months (range 15-149); median weight, 9.6 kg (range 8.1-47.2); right atrial isomerism, 16 patients; and left atrial isomerism, one. The IVC and the separate HV at the level of diaphragm were contralateral in 16 patients, and ipsilateral in one. The surgical procedures for directing blood flow from the IVC and the separate HV to the pulmonary arteries were as follows: en bloc resection of the IVC and the HV and anastomosing these veins to an extracardiac conduit in 10 patients; connecting the IVC to the HV in a side-to-side fashion before anastomosing them to an extracardiac conduit in one; and lateral tunnel in another. When the IVC and the HV were widely separated by the vertebrae, we chose an intra-extracardiac conduit (intra-atrial septation) in four patients and an extracardiac conduit for the IVC and the right HV and lateral tunnel for the separate left HV in one. There was no mortality. Five re-operations were performed (pacemaker in two patients; one each of fenestration, release of outflow obstruction and ligation of collateral arteries). Sixteen patients underwent follow-up catheterisation, which revealed central venous pressure of 12.0 + or - 2.0 mmHg and arterial oxygen saturation of 92% + or - 6%.
The mid-term results of the Fontan completion in patients with atrial isomerism and separate HV drainage were excellent. The distance between the IVC and the separate HV and the position of the vertebrae should be considered when choosing a surgical technique.
在腔静脉（IVC）和肝静脉（HV）分别引流的房间隔异构患者中，Fontan 完成术具有一定的技术挑战性。在此，我们回顾了我们对这些患者的手术方法。
回顾性分析了 1998 年至 2008 年间接受 Fontan 完成术的 50 例连续房间隔异构患者的病历资料。
17 例患者存在 HV 单独引流。排除了 IVC 中断的患者。患者特征如下：中位年龄 26 个月（范围 15-149）；中位体重 9.6kg（范围 8.1-47.2）；右房异构 16 例；左房异构 1 例。IVC 和膈肌水平的单独 HV 在 16 例患者中为对侧，1 例为同侧。将血液从 IVC 和单独 HV 引导至肺动脉的手术方法如下：10 例患者整块切除 IVC 和 HV，并将这些静脉吻合至心外管道；1 例患者先将 IVC 与 HV 连接，然后再将其吻合至心外管道；1 例患者采用侧隧道。当 IVC 和 HV 被脊柱广泛隔开时，我们在 4 例患者中选择了心内-心外管道（房间隔切开），在 1 例患者中选择了 IVC 和右 HV 的心外管道以及单独左 HV 的侧隧道。无死亡病例。5 例患者行再手术（2 例患者行起搏器植入术；1 例患者行开窗术，1 例患者行流出道梗阻松解术，1 例患者行侧支动脉结扎术）。16 例患者接受了随访心导管检查，显示中心静脉压为 12.0+/-2.0mmHg，动脉血氧饱和度为 92+/-6%。
房间隔异构和单独 HV 引流患者的 Fontan 完成术中期结果良好。在选择手术技术时，应考虑 IVC 和单独 HV 之间的距离和脊柱的位置。
