Early Outcomes for In Situ Pericardial Roll Repair for Distant Anomalous Pulmonary Venous Return

Repair of anomalous pulmonary venous return (APVR) when veins are remote from the left atrium (LA) is challenging and may eventuate in a higher prevalence of pulmonary vein stenosis, superior vena cava stenosis, or intracardiac baffle obstruction. We describe our experience in 6 patients with a technique, using both anterior and posterior in situ pericardial roll repairs, which reduces these complications.

Six patients underwent in situ pericardial roll repair of APVR at Cleveland Clinic between 2018 and 2019. Median age was 40 years (range, 0.25-65 years). Three patients had partial APVR of right upper and middle veins into superior vena cava high above the right pulmonary artery without atrial septal defect; 2 had scimitar syndrome, and the infant had heterotaxy with unbalanced atrioventricular canal and mixed obstructed total APVR. The anomalous pulmonary vein drained into the respective cava far from the LA, which was not ideal for traditional repair techniques. In situ pericardial roll directed anomalous pulmonary vein to the LA. Most patients had concomitant complex cardiac procedures.

There was no mortality. Median hospital stay was 23 days (range, 4-60 days) and median follow-up was 20 months (range, 1-36 months). The infant required percutaneous dilatation and stenting of LA anastomosis but since underwent ventricular switch. At last follow-up, pulmonary veins were unobstructed and adult patients were asymptomatic with excellent functional status.

In situ autologous pericardial roll is a useful technique that abrogates the need for mobilization of distant anomalous pulmonary vein with direct anastomosis or complex intracardiac baffles. It is suitable for multiple anatomic configurations and can be used in infants and adults.