Hraska V, Sagat M, Sojak V, Kostolny M, Nosal M, Kantorova A, Nagi A, Krajci M, Siman J, Kunovsky P
Department of Pediatric Cardiosurgery, Pediatric Cardiocenter, Pediatric University Hospital, Bratislava, Slovakia.
Bratisl Lek Listy. 1999 Dec;100(12):657-61.
Total abnormal pulmonary venous return (TAPVR), mainly the obstructive type represents the most riskful critical congenital heart defect requiring urgent surgery immediately after birth.
Analysis of surgical correction of TAPVR results performed from December 1992 to December 1998.
Twenty-seven patients underwent surgery for TAPVR. 13 of them (48%) presented with hemodynamically severe obstruction. Mean age in the group with obstruction was 3.6 +/- 3.2 days with mean weight of 3282 +/- 537 grams.
From the 27 studied patients 5 (18.5%) died. Mean duration of the study in the whole group is 1.91 +/- 2.01 years. Actuarial survival in the first month is 85%, in the second month 81% and remains identical in the 1., 2., 3., 4., 5., 6. year of the study. Univariate analysis identified operation before the year 1996 (p = 0.0056) as a risk factor of immediate mortality. Introduction of ultrafiltration significantly eliminated mortality (p = 0.0101). Remaining variables (age, weight, sex, obstructive TAPVR, TAPVR, extracorporeal circulation duration, pulmonary hypertension) did not significantly influence the survival (p more than 0.05). Multivariate analysis defined operation before the year 1996 as the sole risk factor of mortality (p = 0.0033). In patients operated on in the year 1996 (n = 15) was the survival in the studied period 100%.
Since the year 1996 the results of surgical treatment of TAPVR significantly improved. The key role in the improvement have better urgent diagnostic and surgery, improvement of surgical technique and myocardial protection, introduction of modified ultrafiltration and the quality of postoperative care. Psychomotor development of children after correction is comparable with healthy population, all patients are in NYHA I class. (Tab. 2, Fig. 4, Ref. 9.)
完全性肺静脉异位引流（TAPVR），主要是梗阻型，是最危险的严重先天性心脏缺陷，出生后需立即进行紧急手术。
分析1992年12月至1998年12月期间TAPVR手术矫正的结果。
27例患者接受了TAPVR手术。其中13例（48%）存在血流动力学严重梗阻。梗阻组的平均年龄为3.6±3.2天，平均体重为3282±537克。
在27例研究患者中，5例（18.5%）死亡。全组的平均研究时长为1.91±2.01年。第一个月的精算生存率为85%，第二个月为81%，在研究的第1、2、3、4、5、6年保持不变。单因素分析确定1996年前手术（p = 0.0056）是即刻死亡的危险因素。引入超滤显著降低了死亡率（p = 0.0101）。其余变量（年龄、体重、性别、梗阻性TAPVR、TAPVR、体外循环时长、肺动脉高压）对生存率无显著影响（p＞0.05）。多因素分析确定1996年前手术是唯一的死亡危险因素（p = 0.0033）。1996年接受手术的患者（n = 15）在研究期间的生存率为100%。
自1996年以来，TAPVR的外科治疗结果显著改善。改善的关键作用在于更好的紧急诊断和手术、手术技术和心肌保护的改进、改良超滤的引入以及术后护理质量。矫正术后儿童的精神运动发育与健康人群相当，所有患者均处于纽约心脏协会I级。（表2，图4，参考文献9）
