Surgical Repair of total anomalous pulmonary venous connection is the optimal treatment option. We studied the incidence of Preoperative and postoperative pulmonary venous obstruction in patients eighteen years and younger in a single institute. Ninety-one patient's medical records from January 2004 to December 2014 were retrospectively reviewed. The average age 3 months and weight 5 kg at presentation. The majority of patients presented with supracardiac type (53%). Six patients had reoperation show younger age (p = 0.013) and weight (p= 0.035). Four out of thirty-five readmissions were readmitted because of pulmonary venous obstruction. The cohort mortality rate was 16%. The average age for mortality was 11 months and average weight was 5 kg. Fifty percent of that mortality had supracardiac type. For those who died, 64 % had preoperative pulmonary venous obstruction. Preoperative Pulmonary venous obstruction is common in patients with total anomalous pulmonary venous connection. Surgical repair carries a good prognosis. Need for reoperation is not common but carries a high mortality.

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