Department of Cardiac Surgery, Royal Children's Hospital, Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Eur J Cardiothorac Surg. 2011 Oct;40(4):1011-5. doi: 10.1016/j.ejcts.2011.01.069. Epub 2011 Mar 6.
To determine the late incidence of pulmonary arteriovenous malformations after bidirectional Glenn in patients with azygos continuation of the inferior vena cava (Kawashima operation).
From 1990 to 2006, 21 patients underwent a Kawashima procedure at a median age of 2.3 years (0.5-8 years). Underlying anatomy included atrioventricular septal defect [14], double-outlet right ventricle [13], pulmonary atresia [3], pulmonary stenosis [11], total anomalous pulmonary venous drainage [4] and bilateral superior vena cavae [14].
There was one hospital death after Kawashima due to low output syndrome. Follow-up was complete in all but one patient. Two patients died 23 days and 4 years after Kawashima following cardiac reinterventions (one atrioventricular valve replacement and one Fontan completion). Thirteen patients developed pulmonary arteriovenous malformations after a median of 4 years (2-9 years) after Kawashima. Freedom from development of arteriovenous malformations was 47% at 5 years (95% CI: 23-69%). A total of 16 patients underwent Fontan completion, 12 for cyanosis related to pulmonary arteriovenous malformations and four for decreased exercise capacity. Only three patients were left without Fontan completion at 4, 9 and 13 years after Kawashima. The two patients who had more than 9 years of follow-up after Kawashima had antegrade flow preserved between the ventricle and the pulmonary arteries.
Unless some hepatic blood flow is directed to both lungs, most, if not all patients with a Kawashima procedure will ultimately develop pulmonary arteriovenous malformations. Elective non-fenestrated Fontan completion in the years following Kawashima procedure should be recommended.
确定下腔静脉奇静脉续连（川岛法）双向 Glenn 术后肺动脉静脉畸形的迟发发生率。
1990 年至 2006 年，21 例患者在中位年龄 2.3 岁（0.5-8 岁）时接受川岛法手术。基础解剖结构包括房室间隔缺损[14]、双出口右心室[13]、肺动脉闭锁[3]、肺动脉瓣狭窄[11]、完全性肺静脉异位引流[4]和双侧上腔静脉[14]。
川岛法术后 1 例因低心输出量综合征死亡。除 1 例外，所有患者均完成随访。2 例患者在川岛术后 23 天和 4 年后因心脏再次介入（1 例房室瓣置换和 1 例 Fontan 完成术）死亡。川岛术后中位数 4 年（2-9 年）后，13 例患者出现肺动脉静脉畸形。5 年无畸形发生率为 47%（95%CI：23-69%）。16 例患者接受 Fontan 完成术，12 例因肺动脉静脉畸形相关紫绀，4 例因运动能力下降。川岛术后 4、9 和 13 年后，仍有 3 例患者未完成 Fontan 完成术。川岛术后随访时间超过 9 年的 2 例患者，心室与肺动脉之间仍存在前向血流。
除非部分肝血流同时流向两肺，否则大多数（如果不是全部）接受川岛法手术的患者最终将出现肺动脉静脉畸形。建议在川岛法手术后的几年内选择非开窗 Fontan 完成术。
