Results
Population study and early post-operative outcome.
Between October 1990 and December 2019, 115 patients (male: 63, 54.8%), underwent surgical repair of PAPVC associated with sinus venosus ASD. The median age at the time of surgery was 8.8 years (IQ 4.3-23.6) (Table 1). In 80 cases (70.4%) the repair was performed in pediatric age (<16 years old), while the remaining 35 (29.6%) patients were operated in adulthood (Figure 1). Surgical technique entailed single-patch repair in 49 patients (42.6%), double-patch repair in 65 patients (56.5%) and one Warden procedure (0.9 %) (Table 1). No perioperative mortality was observed.
For 84 patients (57 in the pediatric group and 28 in the adult group), complete data regarding their postoperative period were available. Overall, the mean post-operative length of stay was 8.5±2.8 days.
Hemodynamically significant early-onset arrhythmias were reported in 6 patients, namely one patient suffered from supraventricular tachycardia, 1 had brady-tachy syndrome requiring implantation of a pacemaker and 4 had AF. In addition, 15 patients presented early-onset EAR without hemodynamic consequences, and this was by far the commonest postoperative arrhythmia.
Early post-operative course according to age.
Post-operative complications were not different between the pediatric and the adult groups patient (Table 1). Nonetheless, in the adult group the rate of early-onset arrhythmias with hemodynamic compromise was significantly higher compared to the pediatric group, 17.8% vs 1.7%, respectively (p=0.006). Four adult patients indeed experienced AF and another one had SND which required permanent pacemaker. The percentage of EAR was certainly increased among pediatric patients compared to the adults (21.4% vs 10.7%), albeit without reaching statistical significance (p=0.2) (Table 1).
Early postoperative course according to surgical repair.
When comparing patients who underwent single-patch repair to patients who underwent double-patch repair, we did not find any statistically significant difference in terms of age at surgical repair (Table 2). Early post-operative results were also similar in these 2 groups. In particular, the early postoperative incidence of EAR was not different between the single patch group and the double patch. Even when taking into account only the pediatric population, this difference was still not significant.
Follow-up outcome (> 30 days).
Forty-five patients were lost at follow-up, thus 70 patients (23 adults and 47 pediatrics) were enrolled in the subsequent statistical analysis. There was no statistical difference between patients lost at follow-up and the remaining population in terms of gender, age and type of surgical repair (supplementary table 1).
Median follow-up time was 52 months (15.1-113) and median age at follow-up was 16 years (10.5- 35.7). SVC obstruction was detected at echocardiogram and confirmed with cardiac magnetic resonance imaging in 2 pediatric patients both operated with the double-patch technique, although additional surgical or interventional procedure was required in these cases. We didn’t observe any mortality in our follow-up group.
At their last follow-up 58 patients (82.9%) were in sinus rhythm, 41 in the pediatric group and 17 in the adult group (p=0.1). The other 12 patients (17.1%) instead had developed late-onset rhythm disturbances. Five children had non-hemodynamically significant EAR. Among the adults, 2 had SND with subsequent pacemaker implantation, 2 had frequent premature ventricular contractions requiring antiarrhythmic drugs, another 2 had AF and finally 1 adult patient had second degree AV block. Significant statistical difference (p= 0.04) was therefore observed in terms of types of late-onset rhythm disturbance between the pediatric patients and the adult patients (Table 3). Nonetheless, in regard to the type of surgical repair, no statistical difference (p=0.2) in occurrence of late-onset arrhythmias was highlighted when comparing the single-patch (24.1%) and the double-patch group (12.5%).
Cumulative rhythm anomalies analysis.
We finally evaluated the overall occurrence of any rhythm disturbance among operated patients, considering both early-onset arrhythmias and late-onset arrhythmias (Figure 2). A total of 29 (33,3%) patients had experienced at least one arrhythmia, either in the early postoperative period or at follow-up (Table 4).
Surgical technique alone did not seem to affect the overall occurrence of all rhythm disturbances (41% in single patch group and 35% in double patch groups, p=0.5) and of EAR alone (29% in single patch group and 24.4 % in double patch group, p=0.4). On the other hand, cumulative onset of EAR was significantly more frequent (p=0.02) among the pediatric patients compared to the adults, 32% vs 8%, respectively. Finally, as possibly expected from the separate early-onset and late-onset analysis, we observed that overall most of the arrhythmias with hemodynamic compromise occurred in the adult population, and this result was statistically significant (p= 0.006) (Table 1).