Introduction: A association between partial anomalous pulmonary venous connection (PAPVC) and systemic venous anomalies has been described in the literature, but the true incidence is yet to be elucidated. At our institution, we sought to find the incidence of a persistent left superior vena cava (PLSVC) in patients undergoing an operation for PAPVC. Methods: A retrospective review of all pediatric and adult patients with PAPVC who underwent surgical repair from February 2006 to February 2016. All clinical, radiographic, and operative data were reviewed. Results: Eighty-five patients underwent surgical repair. A PLSVC was identified in 15 (17.6%) patients. Every PLSVC drained/connected to the coronary sinus. A bridging vein was present in only 26.7% (4 of 15). Intraoperative management of the PLSVC consisted of direct cannulation in nine (60%) patients, temporary occlusion in one (6.7%) patient, and ligation in one (6.7%) patient. In the 15 PLSVC patients, 6 (40%) had a secundum atrial septal defect (ASD), 2 (13.3%) had a patent foramen ovale (PFO), 10 (66.6%) had a sinus venosus type defect, and 3 (20%) had both an ASD and sinus venosus–type defect. Of the 15 patients with PAPVC and PLSVC, 14 (93%) had anomalous drainage of pulmonary vein(s) on the right side, whereas 1 (7%) had veins on both sides with anomalous drainage. Conclusion: Persistent left superior vena cava is present in 17.6% of patients undergoing an operation for PAPVC. Awareness of this association as well as the intraoperative management of PLSVC is advised for those that perform operations for PAPVC. As improper myocardial protection and cardiopulmonary bypass strategies in the presence of a PLSVC can result in deleterious outcomes.
Use of multi-modality imaging in a patient with a persistent left superior vena cava, partial anomalous pulmonary venous connection, and sinus venosus-type atrial septal defect
