Kawai T, Wada Y, Enmoto T, Nakajima S, Nishiyama K, Kitaura K, Sato S, Oka T
Second Division of Surgery, Children's Research Hospital, Kyoto, Japan.
Surg Today. 1995;25(6):525-31. doi: 10.1007/BF00311309.
Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.
1985年至1993年间，对13例患有与右位异构相关的复杂心血管畸形的儿科患者进行了姑息性手术。患者包括2名新生儿、10名婴儿和1名儿童，根据是否存在完全性肺静脉异位连接（TAPVC）分为两组。第1组由6例患有TAPVC的患者组成，第2组由7例没有TAPVC的患者组成。在第1组中，手术操作包括2例单独进行TAPVC修复，2例将TAPVC修复与改良的Blalock-Taussig分流术联合进行，1例将TAPVC修复与肺动脉环扎术联合进行，1例单独进行改良的Blalock-Taussig分流术。该组有5例住院死亡和1例晚期死亡：2例患者发生肺静脉梗阻，2例新生儿围手术期心肌衰竭，2例患者因肺血流量增加导致充血性心力衰竭。在第2组中，所有患者均接受了体肺分流术，有1例住院死亡和3例晚期死亡，其中2例患者的死因不明，1例患者分别死于分流失败和肺炎。这些结果表明，对于患有梗阻性TAPVC的幼儿，右位异构的手术姑息治疗效果不佳。因此，我们得出结论，如果临床诊断为肺静脉梗阻，应立即进行TAPVC修复。考虑到婴儿管理困难，对于婴儿来说，在无体外循环（CPB）的情况下解决肺静脉梗阻可能更可取。体肺分流术应采用低流量型，特别是如果存在共同房室瓣反流。如果婴儿在手术后存活，由于存在猝死或感染风险，必须对其进行长期仔细随访。
