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目的 回顾总结改良外管道Fontan术治疗无脾或多脾综合征伴发的复杂型先天性心脏病(先心病)经验。方法 青紫型复杂先心病病儿11例,无脾9例(右房异构,单心室合并共同房室瓣6例,房室连接不一致的右室双出口2例,纠正性大动脉错位1例) ;多脾2例(左房异构,右室双出口合并共同房室瓣)。平均年龄(6 3±3 7)岁,平均体重(2 1 0±5 5 )kg。直接行改良外管道Fontan术3例,双向Glenn术后行二期外管道Fontan术8例。结果 术后早期发生低心排出量综合征2例,肾衰1例,室上性心动过速1例,胸腔引流(12 0ml d) >10d 2例。超声提示上腔吻合口通畅(流速0 6～0 8m s) ,下腔血流速度0 3～0 5m s ;动脉血氧饱和度9例在0 92～0 95 ,2例为0 86 ;活动能力明显改善,无早期死亡。随访中超声显示上腔静脉血流速度0 8m s,下腔静脉血流速度0 4～0 7m s ,随呼吸而变化。外管道通畅,无血栓形成,管道窗口直径0 34cm ,血流右向左分流。肺静脉回流无梗阻,心室射血分数>0 6 0。心电图未见明显心律失常。生长发育正常,活动自如,经皮氧饱和度>0 90 ,无慢性渗出、蛋白丢失肠病等并发症,无中期死亡。结论 改良外管道Fontan术适用于治疗无脾或多脾综合征型复杂先心病,术后心律失常发生率低。
OBJECTIVE: To review the experience with modified Fontan’s technique in the treatment of complex congenital heart disease (CHD) associated with splenomegaly or splenomegaly. Methods 11 cases of cyanotic complex congenital heart disease children, 9 cases without spleen (right atrial heterogeneous, single ventricular combined common atrioventricular valve in 6 cases, atrioventricular anomalies in two cases of right ventricular outlet in two cases, the correction of aortic dislocation in 1 case) ; Splenomegaly 2 cases (left atrial isomerism, right ventricular double outlet with common atrioventricular valve). The average age was (63 ± 37) years old, mean body weight (210 ± 5 5) kg. Three patients underwent Fontan procedure, and two patients underwent Glenn operation underwent Fontan procedure. Results In the early postoperative period, low cardiac output syndrome occurred in 2 cases, renal failure in 1 case, supraventricular tachycardia in 1 case, chest drainage (120ml)> 10d in 2 cases. Ultrasound showed that the anastomosis of the superior vena cava was unobstructed (flow rate of 0 6 ~ 0 8m s), and the velocity of flow of the inferior vena cava was 0 3 ~ 0 5m s. The arterial oxygen saturation was 0 92 ~ 0 95 in 9 cases and 0 86 in 2 cases Significantly improved abilities without early death. Follow-up ultrasound showed that the superior vena cava flow velocity 0 8m s, inferior vena cava flow velocity 0 4 ~ 0 7m s, with the change of breathing. External patency, no thrombosis, the diameter of the pipe window 0 34cm, right to left shunt of blood flow. Pulmonary venous return without obstruction, ventricular ejection fraction> 0 6 0. No significant arrhythmia ECG. Growth and development of normal, freely, percutaneous oxygen saturation> 0 90, no chronic exudate, protein and other complications of intestinal diseases, no medium-term death. Conclusion Improved Fontan operation is suitable for the treatment of complicated congenital heart disease without spleen or splenomegaly and the incidence of postoperative arrhythmia is low.