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 –
- Mrs. Mamta Roshan Patel, Bio- Statistician, Central Research services,
Bhaikaka University, Karamsad.Email : mamtarp@charutarhealth.org
- Mr. Naresh Jayantibhai Fumakiya, Echo-Cardiographer, Bhanubhai and
Madhuben Patel Cardiac Centre, Shree Krishna Hospital, Karamsad.
- Mr. Khushal N. Vankar, Office Assistant, Department of Diagnostic
Services, Shree Krishna Hospital, Bhaikaka University, Karamsad.Email : khushalnv@charutarhealth.org
References
- Peacock TB:
Malformations of
the heart. In: Peacock TB, ed. On Malformations, &c., of the Human
Heart: With Original Cases. London, UK: John Churchill. 1858:11-102.
10.1017/S1047951100010349
- Oliver JM, Gallego P, Gonzalez A, Dominguez FJ, Aroca A, Mesa JM:
Sinus venosus
syndrome: atrial septal defect or anomalous venous conection? A
multiplane transoesophageal approach. Heart. 2002, 88:634-638.
10.1136/heart.88.6.634
- Van Praagh S, Carrera ME, Sanders SP, Mayer JE, Van Praagh R:
Sinus venosus
defects: unroofing of the right pulmonary veins: anatomic and
echocardiographic findings and surgical treatment. Am Heart J. 1994,
128:365-379.
10.1016/0002-8703(94)90491-X
- Kirklin JW, Barratt-Boyes BG, eds:
Cardiac Surgery:
Morphology, Diagnostic Criteria, Natural History, Techniques, Results,
and IndicationsNew York. NY: Churchill Livingstone; 1993. 1:609-644.
- Schuster SR, Gross RE, Colodny AH:
Surgical
management of anomalous right pulmonary venous drainage to the
superior vena cava, associated with superior marginal defect of the
atrial septum. Surgery. 1962, 51:805-8.
10.5555/uri:pii:0039606062903252
- Kyger ER III, Frazier H, Cooley DA, et al.:
Sinus venosus
atrial septal defect: early and late results following closure in 109
patients. Ann Thorac Surg. 1978, 25:44-50.
10.1016/S0003-4975(10)63485-6
- Stewart S, Alexson C, Manning J:
Early and late
results of repair of partial anomalous pulmonary venous connection to
the superior vena cava with a pericardial baffle. Ann Thorac Surg.
1986, 41:498-501.
10.1016/S0003-4975(10)63026-3
- DeLeon SY, Freeman JE, Ilbawi MN, et al.:
Surgical
techniques in partial anomalous pulmonary veins to the superior vena
cava. Ann Thorac Surg. 1993, 55:1222-6.
10.1016/0003-4975(93)90038-J
- Gustafson RA, Warden HE, Murray GF, Hill RC, Rozar GE:
Partial
anomalous pulmonary venous connection to the right side of the heart.
J Thorac Cardiovasc Surg. 1989, 98:861-8.
10.1016/S0022-5223(19)34264-3
- Gustafson RA, Warden HE, Murray GF:
Partial
anomalous pulmonary venous connection to the superior vena cava. Ann
Thorac Surg. 1995, 60:5614-7.
10.1016/j.ejcts.2011.05.043
- Shahriari A, Rodefeld MD, Turrentine MW, Brown JW:
Caval
division technique for sinus venosus atrial septal defect with partial
anomalous pulmonary venous connection. Ann Thorac Surg. 2006,
81:224-30.
10.1016/j.athoracsur.2005.07.015
- Gaynor JW, Burch M, Dollery C, Sullivan ID, Deanfield JE, Elliott MJ:
Repair of
anomalous pulmonary venous connection to the superior vena cava. Ann
Thorac Surg. 1995, 59:1471-5.
10.1016/0003-4975(95)00150-J
- DiBardino DJ, McKenzie ED, Heinle JS, Su JT, Fraser CD Jr:
The Warden
procedure for partially anomalous pulmonary venous connection to the
superior caval vein. Cardiol Young. 2004, 14:64-7.
10.1017/S1047951104001118
- Warden HE, Gustafson RA, Tarnay TJ, Neal WA:
An alternative
method for repair of partial anomalous pulmonary venous connection to
the superior vena cava. Ann Thorac Surg. 1984, 38:601-5.
10.1016/S0003-4975(10)62317-X
- Jost CH, Connolly HM, Danielson GK, et al.:
Sinus
venosus atrial septal defect. long-term postoperative outcome for 115
patients. Circulation. 2005, 112:1953-1958.
10.1161/CIRCULATIONAHA.104.493775
- Stewart RD, Bailliard F, Kelle AM, Backer CL, Young L, Mavroudis C:
Evolving
Surgical Strategy for Sinus Venosus Atrial Septal Defect: Effect on
Sinus Node Function and Late Venous Obstruction. Ann Thorac Surg.
2007, 84:1651-5.
10.1016/j.athoracsur.2007.04.130