[Surgical treatment of total anomalous pulmonary venous return]

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.)