Hopkins R A, Armstrong B E, Serwer G A, Peterson R J, Oldham H N
J Thorac Cardiovasc Surg. 1985 Sep;90(3):391-8.
The original Fontan procedure included a classic superior vena cava-to-right pulmonary artery (Glenn) shunt. Subsequent experience demonstrated that this anastomosis was not essential and was an unnecessary commitment of the larger right pulmonary circulation to the smaller blood volume of the superior vena caval return. With application of the Fontan principle to more complex cardiac malformations, there has been a reconsideration of possible benefits of a cavopulmonary shunt in selected patients. A modified shunt from the divided end of the superior vena cava to the side of the undivided right pulmonary artery utilized in 21 patients is described. This shunt is designed to allow bidirectional pulmonary arterial distribution of both superior vena caval inflow and right atrial outflow after completion of the Fontan procedure. Twelve patients had the bidirectional shunt performed prior to a Fontan operation; five of these had a subsequent atriopulmonary connection and seven await operation. Eight patients had construction of this shunt at the time of their Fontan procedure. One patient had a bidirectional shunt constructed following atriopulmonary anastomosis to help relieve right atrial outflow obstruction. Two patients with univentricular heart undergoing simultaneous Fontan procedure and a bidirectional shunt died while in the hospital. The remaining 19 patients have been followed up for 2 months to 9 years with one late sudden death at 9 years. There have been no bidirectional cavopulmonary shunt failures, stenoses, kinks, or recognized pulmonary arteriovenous malformations. Postoperatively, eight patients had assessment of pulmonary distribution of shunt blood flow by angiography. Seven of these patients were also evaluated by radionuclide angiography. Superior vena caval blood flow via the bidirectional cavopulmonary shunt tended to be greater to the right lung, but bilateral pulmonary flow was documented in all but one patient. After Fontan operation, six of seven patients tested also demonstrated bilateral distribution of atriopulmonary flow. We concluded from our experience that this modified shunt provides excellent relief of cyanosis, allows bidirectional pulmonary distribution of both superior vena caval return and also the right atrial blood flow after atriopulmonary connection, and may be done before, with, or after a Fontan procedure and is compatible with all currently recommended modifications. Perioperative hemodynamic adjustments to the Fontan procedure may be improved by reducing atrial volume, and this may also be of potential benefit in the long-term adaptation to Fontan physiology by minimizing atrial distention.
最初的Fontan手术包括经典的上腔静脉至右肺动脉（Glenn）分流术。随后的经验表明，这种吻合并非必要，而且将较大的右肺循环用于较小容量的上腔静脉回流是不必要的。随着Fontan原则应用于更复杂的心脏畸形，对于特定患者行腔肺分流术可能带来的益处有了重新审视。本文描述了一种改良分流术，该分流术将上腔静脉的断端与未分隔的右肺动脉侧壁相连，应用于21例患者。这种分流术旨在使Fontan手术完成后，上腔静脉流入血和右心房流出血能在肺动脉内双向分布。12例患者在Fontan手术前行双向分流术；其中5例随后进行了心房-肺动脉连接，7例等待手术。8例患者在Fontan手术时构建了这种分流术。1例患者在心房-肺动脉吻合术后构建双向分流术以缓解右心房流出道梗阻。2例单心室心脏患者在同时进行Fontan手术和双向分流术时死于医院。其余19例患者随访2个月至9年，1例在9岁时发生晚期猝死。未出现双向腔肺分流失败、狭窄、扭结或公认的肺动静脉畸形。术后，8例患者通过血管造影评估了分流血流的肺部分布。其中7例患者还接受了放射性核素血管造影评估。通过双向腔肺分流的上腔静脉血流往往更多地流向右肺，但除1例患者外，所有患者均记录到双侧肺血流。在Fontan手术后，7例接受检测的患者中有6例也显示心房-肺动脉血流呈双侧分布。我们从经验中得出结论，这种改良分流术能显著缓解紫绀，使上腔静脉回流和心房-肺动脉连接后的右心房血流在肺内双向分布，可在Fontan手术前、术中或术后进行，且与目前所有推荐的改良方法兼容。通过减少心房容量可改善Fontan手术的围手术期血流动力学调整，这在长期适应Fontan生理状态方面也可能具有潜在益处，可将心房扩张降至最低。
