Results
No early or late deaths occurred in this series; no patients required reoperation, and none had residual ASDs. Of the 9 patients with PAPVC involving the RA-SVC junction or RA, none exhibited late stenosis of either the SVC or pulmonary veins on follow-up echocardiography, and none required the Warden procedure. Two patients, P4 and P7, exhibited junctional rhythm on early ECG (Figure 23) and underwent two-patch repair. This junctional rhythm reverted to normal sinus rhythm on late ECG in the hospital. The remaining 8 patients exhibited normal sinus rhythm on early ECG. The association between technique (single- versus two-patch) and rhythm was not significant (p > 0.999; Figure 24). The gender distribution of patients and type of superior vena cava are illustrated in Figures 25 and 26.)

Discussion

The repair of SVASD with PAPVC involving the SVC has been a surgical challenge since the earliest reports of this anomaly [5,6]. Numerous surgical modifications have been made to repair the defect and redirect pulmonary venous return. Although the early problems of persistent PAPVC and residual ASD have largely been eliminated, problems with SVC stenosis, pulmonary vein stenosis, and sinus node dysfunction remain, as reported in many surgical series. In our study, PAPVC involving the SVC at a low level, the RA, or both was treated by septal transposition [7] or by redirection of the anomalous pulmonary venous flow to the left atrium through the ASD through the use of a patch applied from within the RA [8]. We did not encounter cases in which PAPVC involved the SVC at a high level (1 cm or more above the cavoatrial junction) with or without an associated ASD.
In a literature review, we found that use of the two-patch technique was associated with a very low incidence of stenosis of the SVC or pulmonary veins. According to the literature, the incidence of sinus node dysfunction after two-patch repair is significant; this technique entails an incision that crosses the SVC-RA junction, on the anterior surface or laterally, which puts the sinus node at risk for damage. DeLeon and associates [8], in a review of 40 patients who underwent repair between 1979 and 1991, found that repair with an RA appendage flap that included a continuous incision through the anterior RA-SVC junction was associated with a higher incidence of sinus node dysfunction than was repair involving either no incision of the SVC or an end-to-side appendage to an SVC anastomosis without any incision near the SVC-RA junction.
Stewart and coworkers [7] studied 15 patients in whom SVASD and PAPVC involving the SVC were repaired with a single-patch technique, which included an incision from the atrial appendage superiorly along the SVC to the level of the PAPVC, thus directly across the sinus node. They noted that 6 (40%) of these patients had early sinus node dysfunction but, of interest, all these postoperative rhythm problems resolved before hospital discharge. In one of our patients (P9), early sinus node dysfunction returned to normal sinus rhythm.
All 7 patients who underwent two-patch repair continue to be monitored at our cardiac center. Two patients exhibited junctional rhythm on ECG early in the postoperative period, but this later reverted to normal sinus rhythm. The remaining 5 patients exhibited normal sinus rhythm on early ECG.
Because patients who underwent two-patch repair continued to exhibit normal sinus rhythm on late ECG with no turbulence in the SVC-RA junction pathway and with no arrhythmias, we advocate two-patch repair over single-patch repair.
According to our review of the literature, the Warden procedure involves no incisions near the sinus node or the sinus nodal artery, and no sinus node dysfunction has occurred after this procedure. Gustafson et al. and Warden et al. have reported on their growing series of patients; the initial patient underwent surgery in 1967 [9,10]. As of the most recent report, they had performed the Warden procedure in 40 patients, of whom only 1 (2.5%) exhibited continuing sinus node dysfunction [10]. Two other patients exhibited early sinus node dysfunction, but normal sinus rhythm was achieved by 5 days and 6 months after surgery.
Shahriari and colleagues [11] reported the results of SVASD repair in 54 patients, of whom 27 underwent single-patch repair, 12 underwent two-patch repair, and 13 underwent the Warden procedure. The rate of arrhythmias in the entire series was very low; 100% of the patients who underwent the Warden procedure remained in normal sinus rhythm. Similarly, Gaynor and colleagues [12] reported on a series of 11 patients who underwent the Warden procedure between 1987 and 1995, none of whom exhibited sinus node dysfunction. DiBardino and associates [13] reported a single case of transient sinus bradycardia among 16 patients who underwent the Warden procedure between 1995 and 2004 [14]. In summary, the literature indicates that the Warden procedure essentially eliminates the possibility of sinus node injury and thus sinus node dysfunction.
In the 2 patients who underwent single-patch repair, turbulence was noted in the SVC-RA junction pathway: Patient P1 exhibited a peak pressure of 4 mm Hg (mean, 2 mm Hg), and P2 exhibited a peak pressure of 18 mm Hg (mean, 18 mm Hg). No rhythm abnormalities occurred after single-patch repair [15,16].
In our series, patient P10 had left-sided isolated PAPVC with ASD and pulmonary stenosis. In this patient, we rerouted the PAPVC by creating an anastomosis between the left atrial appendage and the vertical vein with flush ligation of the vertical vein and the innominate vein, leaving the hemiazygos vein in the systemic pathway. We used a glutaraldehyde-treated pericardial patch to close the ASD and performed pulmonary valvotomy.
Patient P4 underwent preoperative evaluation with dynamic cardiac CT because intracranial arteriovenous malformations were suspected of causing dilatation of the SVC.
In the combined series reported by Shariari et al., Gaynor et al., and DiBardino et al., no patients had SVC stenosis, and only 2 had pulmonary vein stenosis. Of interest is that in both patients, who were treated at different hospitals, pulmonary vein stenosis appeared to result from untreated shrinkage of an autologous pericardial patch. The authors recommended using a polytetrafluoroethylene intra-atrial baffle. In our series, we used pericardium patches without any evidence of pulmonary vein stenosis caused by patch shrinkage.
Limitations
A limitation of this study was its retrospective design. Because the natural history of SVASD is not assessable, and because no historical control group is available for comparison, the natural history of unoperated SVASD is unknown; however, it is probably similar to that of large ASDs.
The presence of atrial arrhythmias decreases the ability to detect sinus node dysfunction; thus the frequency of sinus node dysfunction detection may have been underestimated.

Conclusions

The decision to surgically repair SVASD should be individualized. However, such intervention is rarely necessary or advisable in an infant younger than 1 year or in very elderly patients. Our findings confirm that the two-patch technique remains the procedure of choice for patients with a SVASD and PAPVC involving the RA or the SVC-RA junction. In cases of bilateral SVC without innominate vein connection, single-patch repair can be attempted without causing much turbulence in the SVC-RA junction, in comparison with cases of single SVC.
The Warden procedure should be an option in pediatric cases of SVASD with PAPVC involving high levels of the SVC because it provides unobstructed drainage of the SVC and pulmonary veins and virtually eliminates the problem of sinus node dysfunction that is common with the two-patch repair.
Despite the complexity of the lesion, repair of SVASD with associated APVC is associated with low rates of morbidity and mortality, even in patients older than 40 years. Survival rates are similar to those of a matched population, and although repair is suggested as early as possible, it should be considered whenever repair may affect survival or symptoms .
ACKNOWLEDGEMENTS
We are thankful to –
  1. Mrs. Mamta Roshan Patel, Bio- Statistician, Central Research services, Bhaikaka University, Karamsad.Email : mamtarp@charutarhealth.org
  2. Mr. Naresh Jayantibhai Fumakiya, Echo-Cardiographer, Bhanubhai and Madhuben Patel Cardiac Centre, Shree Krishna Hospital, Karamsad.
  3. Mr. Khushal N. Vankar, Office Assistant, Department of Diagnostic Services, Shree Krishna Hospital, Bhaikaka University, Karamsad.Email : khushalnv@charutarhealth.org

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