Is Restrictive Atrial Septal Defect a Risk in Partial Anomalous Pulmonary Venous Drainage Repair

Background The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. Methods 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild = 3 to 5 mm Hg; severe = 6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). Results There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p  = 0.002), and no differences were found in freedom from severe PVS ( p  = 0.103), any SVCS ( p  = 0.419), or severe SVCS ( p  = 0.373). Group A patients had more PVS-related reoperations ( p  = 0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS ( p  = 0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD ( p  = 0.018). Conclusions Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVS-related reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.