Repair of sinus venosus defects with partial anomalous pulmonary venous connection in adult: Four surgical procedures for a large armamentarium

Background Surgical repair of sinus venosus defects with partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) has been associated with venous return obstructions, and sinus node dysfunction. Purpose We aimed to evaluate the safety of four different surgical procedures, for repair of sinus venosus defects with PAPVC, in 23 adult patients, adapted to each patient\u0027s specific anatomy assessed by 3D computed tomography. Methods Between March 2010 and May 2019, we performed anatomic repair of sinus venosus defects with a single patch (n = 6; 26.1%), 2-patch (n = 9; 39.1%), Warden (n = 3; 13%), or modified Warden procedure with reconstruction of the divided SVC using a PTFE conduit (n = 5; 21.7%) ( Fig. 1 ). Median age was 40 years [range, 15 to 73 years]. Main associated procedures were: creation of atrial septal defect (n = 1), tricuspid valvuloplasty (n = 2), tricuspid valve replacement (n = 1), pulmonary valve replacement (n = 1), monoblock “cut and sew” of pulmonary veins and superior vena cava (n = 1). Results Follow-up was 3.26 years [range, 2 months to 10 years]. There were no mortality and no obstruction of SVC and pulmonary veins at the time of discharge and during follow-up. Aortic clamping was shorter (60 ± 39 min) with the single patch technique and longer (112 ± 31 min) with the modified Warden procedure. Nineteen patients (82.6%) had regained sinus rhythm at discharge; a patient with paroxysmal atrial fibrillation had regained a sinus rhythm after the “cut and sew” isolation technique of the superior vena cava and pulmonary veins. All patients were NYHA 1. Conclusions PAPVC can safely be performed with an appropriate technique, and low morbidity. The single patch technique, when anatomically possible, offers good results but now competes with transcatheter correction. When PAPVC is far from the SVC-right atrium junction, the modified Warden procedure offers an effective and obligatory surgical repair, without any increase in complications.