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Background  The small size of the pulmonary veins in infants increases the risk of pulmonary vein obstruction (PVO) after surgical repair of type IV total anomalous pulmonary venous connection (TAPVC). Here, we described the outcomes of our strategy, which delayed total correction after initial partial correction.    Methods  We reviewed the data of patients who underwent total correction for type IV TAPVC. In total, 11 out of 103 patients with TAPVC had type IV TAPVC with biventricular physiology. Of these 11 patients, we retrospectively reviewed the data of 7 patients who underwent two-stage correction. Major pulmonary venous confluent chambers, with the exception of the left superior pulmonary vein (LSPV), were initially anastomosed to the left atrium (LA), followed by anastomosis between the LSPV and the LA auricle.    Results  The median weight, age, and LSPV size were 4.3 kg (range, 3.5–5.4 kg), 40 days (range, 20–103 days), and 4.5 mm (range, 3.0–5.4 mm), respectively, during the first operation and 12.2 kg (range, 8.5–31.5 kg), 1,165 days (range, 280–3,250 days), and 9.8 mm (range, 8.0–12.3 mm), respectively, during the second operation. The median Qp/Qs was 1.61 (range, 1.22–1.65) and the median cardiothoracic ratio was 0.52 (range, 0.49–0.57) at second operation. The median interval between the operations was 1,094 days (range, 196–3,226 days). The median follow-up period was 22 month (range, 7–59 month). No mortality or major morbidities occurred after either operation. The median V max  at the LSPV anastomosis site was 1.0 m/s (range, 0.8–1.3 m/s) on predischarge echocardiography. This patency was maintained at the last follow-up, showing an identical median V max  of 1.0 m/s (range, 0.8–1.3 m/s). All 7 patients who underwent two-stage correction were in good condition, without any clinical symptoms of PVO.    Conclusions  Our results suggest that leaving the isolated LSPV uncorrected during infancy and performing a second operation when the LSPV has grown adequately is a viable treatment option for patients with type IV TAPVC.

Two-stage correction of type IV total anomalous pulmonary venous connection