Partial Anomalous Pulmonary Venous Connection Repair: Customized Approach and Outcomes

Alternative options for the correction of partial anomalous pulmonary venous connection (PAPVC) have been proposed. Each can be associated with variable risk for dysrhythmias, caval or pulmonary venous (PV) obstruction. A selective customized strategy to address PAPVC taking into account atrial shunt (AS) and growth potential was pursued. Between September 2014 and August 2018 21 PAPVC patients were identified. Two levels of reference determined the chosen repair strategy; azygous vein (AzV) and cavoatrial junction (CAJ). Six (Group-A) with PAPVC entering SVC cephalad to AV underwent a combined in-situ cavoatrial autologous reconstruction with atrial appendage advancement flap (CARAF). PAPVC entering caudally to AzV (Group-B) underwent alternative repair (caval division/Warden-type or intraatrial rerouting) (n = 15). Age was 8.3 (IQR:4.2-18.5) years for Group-A (vs 11.9; IQR:8.8-34.7 in Group-B) (p = 0.07). In Group-A 5(83%) had AS (vs 12[80%] Group-B; p = 0.9). None had left SVC in Group-A (vs 1 in Group-B; p = 0.9). Preoperative advanced imaging and echocardiographic hemodynamic evaluation was undertaken. Follow-up was complete (median 2.9; IQR:1.2-3.7 years). Freedom from atrial dysrhythmias, caval or PV obstruction was assessed. There were no early or late deaths. ICU and hospital length of stay were 1.8 ± 1.1 and 3.2 ± 0.5 days, respectively. No atrial dysrhythmias occurred postoperatively in Group-A (vs 1 in Group-B; p = 0.9). No permanent pacemaker was implanted. All patients remained in normal sinus rhythm. There were no early or late caval/PV obstruction. A customized approach reserves the advantages of each technique tailored to patient's needs. Expanding surgical capacity with favorable outlook for all PAPVC variations, irrespective of association with AS, can maximize efficiency and reproducibility paired with the lowest morbidity.