Partial Pulmonary Venous Return Repair with and Without Atrial Septal Defects: A Multicenter Analysis

Background: There is scant data, which evaluates the perioperative differences in those undergoing surgical repair of partial anomalous pulmonary venous return (PAPVR) along with ASD closure versus isolated PAPVR repair. The objective of this study is to identify independent factors that differentiate patients undergoing PAPVR repair with ASD and isolated PAPVR repair.

Methods: The Pediatric Health Information System data set was utilized in this retrospective, multicenter, cohort analysis of surgically repaired PAPVR with and without ASD in children from 2006-2020. Continuous variables were summarized with medians and interquartile ranges and categorical variables were summarized with counts and percentages. Differences between the PAPVR only and PAPVR + ASD group were assessed using the Kruskal-Wallis test (continuous variables), the χ2 / Fisher exact test for categorical variables. Multivariable logistic regression was used to investigate independent factors that differentiate patients with only PAPVR versus PAPVR + ASD repair. Multiple models were utilized to confirm the robustness of the identified variables.

Results: A total of 1291 patients underwent surgical repair of PAPVR. Of these 1108 (85.8%) patients underwent additional ASD repair. Median age for the PAPVR + ASD group was five years compared to nine years for PAPVR only group (P < 0.001). Of the patients with PAPVR + ASD group that underwent surgical repair, 53.9% were prior to age five, compared to 30.6% with isolated PAPVR (P < 0.001). Overall, post-operative complications included pleural effusion (9.3%), heart block (1.2%), arrhythmia (14.3%), and pneumothorax (7.2%). On univariate analysis, the PAPVR + ASD group were noted to have less genetic abnormalities (3.2% vs. 15.8%; P < 0.001), congenital lung abnormalities (1.8% vs. 12%; P < 0.001), asthma (7.2% vs. 14.8%; P < 0.001) and post-operative advanced heart block (0.7% vs. 3.8%; P = 0.002). Multivariable analysis confirmed that patients with PAPVR + ASD repair have less genetic abnormalities (OR- 0.24; 95% CI- 0.13, 0.43; P < 0.001), asthma (OR- 0.57; 95% CI- 0.34, 0.97; P = 0.033), and advanced heart block (OR- 0.16; 95% CI- 0.05, 0.55; P = 0.003). Differences in outcomes between the two groups is summarized in Table 2.

Conclusion: Overall, the most common post-operative complications were arrhythmia, pleural effusion, and pneumothorax. The two investigated groups differed significantly for specific pre- and post-operative factors. Among pre-operative factors, genetic abnormalities, congenital lung abnormalities, and asthma were less likely among those undergoing PAPVR + ASD repair compared to those undergoing isolated PAPVR repair. Interestingly, patients with PAVPR repair alone were more likely to have post-operative complication of advanced heart block compared to patients with PAPVR + ASD repair.