Materials and methods
This was a single-centre, retrospective study approved by the
Institutional Review Board of our hospital. All patients who underwent
surgical repair of PAPVC associated with sinus venosus ASD between 1990
and 2019 were identified. Patients presenting with PAPVC and no sinus
venosus ASD or with other associated significant congenital heart
anomalies were excluded from the study, whereas patients with associated
minor abnormalities such as persistent left SVC or bicuspid aortic valve
were included. Surgical procedures and all reported postoperative
complications were reviewed, while follow-up data were obtained from
hospital records. The reports of the last echocardiogram performed were
evaluated to identify any residual shunt or SVC obstruction.
Electrocardiograms (ECG) including Holter ECG were reviewed to detect
rhythm disturbances, which were classified as follows: sinus nodal
dysfunction (SND), defined as “persistent sinus bradycardia
inappropriate to hemodynamic requests”, frequently in the form of
brady-tachy syndrome (16); ectopic atrial rhythm (EAR) likely caused by
iatrogenic SA node injury; atrio-ventricular (AV) block, ventricular
arrhythmias and atrial fibrillation (AF). According to the timing of
their occurrence, rhythm disturbances were divided in early-onset and
late-onset, as to whether they developed within 30 days after surgery or
over 30 days post-surgery, respectively.
Surgical repair
All procedures were carried out through a median sternotomy, under
moderate hypothermic cardiopulmonary bypass (CPB). Cold blood antegrade
cardioplegia was used in all cases. Glutaraldehyde-treated autologous
pericardial patches were employed to perform ASD closure, PVs rerouting
and cavo-atrial junction enlargement, when required. In a single case of
Warden procedure, a direct SVC-right atrial appendage anastomosis was
performed to correct the upper venous drainage. The surgical technique
for the PAPVC repair was chosen according to the site of abnormal
drainage of the PVs. When the connection was at the level of the
superior cavo-atrial junction close to the ASD, a single-patch repair
was performed. Conversely, when the anomalous PVs drained into the SVC,
well above the cavo-atrial junction, a double-patch repair was preferred
in nearly all cases, except from a single Warden procedure which was
performed in view of surgeon’s personal preference. In all cases,
surgical access to the right atrium was obtained through a longitudinal
atriotomy parallel to the atrioventricular groove, or through an
L-shaped atriotomy. In patients where an extensive superior cavo-atrial
enlargement was required (double-patch group), the incision was carried
out as close as possible to the site of PVs connection to the SVC, in
order to limit iatrogenic damage to the SA node. When single-patch
technique was adopted instead, a more limited superior cavo-atrial
incision was performed.
Data analysis
Continuous variables are expressed as mean ± standard deviations or as
median and interquartile ranges, while categorical variables are
expressed as absolute numbers and percentages. Analysis of continuous
variables was performed by analysis of variance (ANOVA) test or Mann
Whitney test, as appropriate. The Pearson’s chi-square test and the
t-test were used to analyze categorical variables.
A p value < 0.05 was considered statistically significant.