The natural and surgically modified history of anomalous pulmonary veins from the left lung

Background Reconstruction of anomalous left pulmonary veins (ALPV) requires an anastomosis at a nonanatomic position, posing the potential risk of pulmonary vein obstruction (PVO). Methods The 514 patients who were diagnosed with a pulmonary vein abnormality from 1990 to 2010 were reviewed. Thirty-eight patients (7.4%; median age, 1.4 years; interquartile range, 0.1 to 5.7 years) were identified. ALPV was diagnosed as an isolated anomaly in 23 (61%) or as part of mixed total anomalous pulmonary venous drainage in 15 (39%). Patients were divided into 3 groups (group 1: partial ALPV, treated; group 2: total ALPV, treated; or group 3: partial ALPV, untreated). Freedom from poor clinical (death/reoperation) and functional (any PVO, mean pressure gradient > 3 mm Hg) outcomes were analyzed. Results Repair in 30 ALPV patients (79%) was performed with direct anastomosis to the left atrium (n = 26 [68%]) or by sutureless repair (n = 4 [11%]). Two deaths occurred in group 2 (heart failure and PVO). The 4 reoperations in group 2 were prompted by PVO and occurred within 6 months of the initial repair. There was a nonsignificant trend of lower freedom from poor outcomes in group 2 (74.6%) vs group 1 (100%) at 10 years (p = 0.105). There was no difference in the incidence of any left PVO among the groups (p = 0.381). Severe left PVO did not develop in group 3 (n = 8 [21%]). Conclusions Total ALPV carries a high risk of early PVO. Thus, the optimal surgical approach remains elusive. Untreated partial ALPV remained unobstructed during midterm follow-up. Therefore, surgical treatment may not be necessary in patients with partial ALPV.