Left-Sided Partial Anomalous Pulmonary Venous Connections

Background: Left-sided partial anomalous pulmonary venous connection (PAPVC) is a congenital defect where pulmonary veins from the left lung drain into the right atrium. If left untreated, PAPVC may result in severe right ventricular failure and pulmonary vascular disease. We sought to determine the effectiveness and long-term outcome after surgical correction of this anomaly. Methods: From 1954 to 2006, 376 patients were treated at our institution for PAPVC. Of these, 27 (7.1%) patients underwent surgical repair for left-sided PAPVC. Thirteen patients were treated with a minimally invasive approach and 14 were treated with median sternotomy and cardiopulmonary bypass (CPB). Results: Mean age at time of operation was 33 ± 18 years. Eleven patients had complete left-sided PAPVCs; patients with one anomalous vein had a significantly higher right ventricular systolic pressure (RVSP) than those with two (31 vs 42 mm Hg, p = 0.003). There was a trend toward early surgical treatment in patients with two anomalous veins (27.5 vs 34.6 years, p = 0.14). Patients who underwent off-pump correction were younger (p = 0.04), presented later in our surgical experience (p = 0.004), and had more tricuspid regurgitation (TR) (p = 0.04). Repair of left-sided PAPVC was performed by left thoracotomy without CPB in 13 patients. There were no early or late deaths. Three patients were rehospitalized for cardiovascular reasons during the follow-up period. All patients had right-sided chamber reduction at follow-up, decreased RVSP (p = 0.02), and decreased TR (p = 0.04). Patients who presented with higher grades of TR showed evidence of pulmonary vascular disease at follow-up (p = 0.017). Conclusions: Repair of PAPVC can be completed successfully with low morbidity. Patients with left-sided PAPVC, right-sided chamber enlargement, evidence of TR, or clinical symptoms should undergo surgical repair.