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Ann Thorac Surg 1996;62:1188-1190
© 1996 The Society of Thoracic Surgeons

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Case Report

Operative Technique for Persistent Left Superior Vena Cava Draining Into the Left Atrium

Department of Thoracic and Cardiovascular Surgery, Wakayama Medical College, Wakayama, Japan

Accepted for publication April 14, 1996.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
We applied a surgical method with an inverted flap of the left atrial appendage to construct an internal conduit in a 4-year-old girl with a persistent left superior vena cava draining into the left atrium associated with an atrial septal defect. The remaining defect of the septum was closed with autologous pericardium. The short-term result is satisfactory, although the long-term result remains to be ascertained.

	Introduction

Top Footnotes Abstract Introduction Comment References

 
Persistent left superior vena cava (LSVC) is a common anomaly often found associated with other congenital heart diseases [1]. It usually connects to the coronary sinus, but in rare instances it drains directly into the left atrium (LA). Although many surgical methods have been applied in efforts to correct this rare anomaly, no established method has been reported so far. We applied a surgical method for correction of this anomaly associated with atrial septal defect and absent coronary sinus using an inverted left atrial appendage flap.

A 4-year-old girl with the diagnosis of atrial septal defect and persistent LSVC was referred to our surgical team for the corrective operation. At birth she had been a premature baby (28 weeks of gestation; birth weight, 802 g). She was noted to have a heart murmur soon after birth and was diagnosed with cardiac anomalies and atresia ani. At 2 years of age she underwent a corrective operation for the atresia ani. Although the growth rate of her body was below normal and she was prone to catch cold, her mental development and physical condition were fairly good. A systolic murmur and the fixed splitting of the second heart sound were heard at the left sternal border.

At the age of 4 years she underwent cardiac catheter study. Her heart showed situs solitus and had a normal atrioventricular and ventriculoarterial relationship. The catheter proceeded easily from the right into the left atrium. The innominate vein was not found. Left atriography revealed the LSVC connecting to the LA at the upper and left quadrant. The pulmonary-to-systemic arterial flow ratio was 3.0, and left-to-right and right-to-left shunt were 77.4% and 32.0%, respectively.

Echocardiography revealed left-to-right shunt flow at the atrial level. Contrast medium administered from the left arm vein first reached the LA, showing the LSVC connecting to the LA. Magnetic resonance imaging also demonstrated the LSVC draining directly into the LA at the roof.

Operation was performed through a median sternotomy. The LSVC was found to return directly to the LA at the portion just cephalad to the LA appendage. When the LSVC was temporarily occluded, the left jugular venous pressure significantly increased to more than 30 mm Hg. Thus, we decided to reconstruct the drainage root of the LSVC. After cardiopulmonary bypass was established and cardioplegic arrest was obtained, the right atriotomy was placed. A large atrial septal defect without lower margin and a small foramen ovale were found with the remnant tissue of the atrial septum between them. The third venous cannula was inserted into the LSVC from the inside of the atrium. We inverted the left atrial appendage and sewed the edge of that appendage flap to the posterior wall of the left atrium around the third cannula to construct the conduit between the left caval orifice and the right atrium. Then the remnant of the atrial septum was swung to the inside to roof the conduit. When the LSVC was rerouted to the right atrium, we closed the remaining defect of the septum with autologous pericardium (Fig 1). After cardiopulmonary bypass was disconnected the pressure of the left jugular vein was as low as that of the right. The postoperative clinical course was uneventful. Echocardiography 1 month after the operation showed smooth flow from the LSVC into the right atrium through the interatrial tunnel (Fig 2).

View larger version (19K): [in this window] [in a new window]   Fig 1. . Diagram of the operation. We used the reversed flap of left atrial appendage and the remnant of atrial septum to construct an interatrial tunnel. (ASD = atrial septal defect; IVC = inferior vena cava; LA = left atrium; LSVC = left superior vena cava; RA = right atrium; RSVC = right superior vena cava.)

View larger version (77K): [in this window] [in a new window]   Fig 2. . Postoperative contrast echocardiogram. The contrast medium administered from the left arm vein reached the left atrium (LA), and then through the intraatrial tunnel it reached the right atrium (RA). (LV = left ventricle.)

	Comment

Top Footnotes Abstract Introduction Comment References

Simple ligation is the procedure of choice to remove the abnormal shunt, but when temporary occlusion of the LSVC leads to elevation of the left jugular venous pressure to more than 30 mm Hg, this procedure might cause an intracranial complication postoperatively [4]. Several other procedures have been reported. They include intraatrial roofing [5], intraatrial baffle rerouting [4], reimplantation into the right atrium or pulmonary artery [6], and graft interposition to the right atrium [1, 4]. The use of a graft is not applicable for young children because of their growth. Baffle rerouting seems beneficial, but the long-term patency is questionable because most of the tunnel wall consists of artificial or other nongrowing materials. One can expect prolonged patency with intraatrial roofing using the patient's own LA wall, but the essential pathology of this lesion is the developmental "defect" of the tissue known as the left atriovenous fold, which normally forms the interior wall of the coronary sinus. Thus, it would be better to augment the tissue with some additional material.

By use of our method employing the inverted LA appendage flap one can avoid all of these disadvantages. Because the wall of the tunnel consists entirely of the patient's own viable pedicled tissue, it has superior properties in terms of anticoagulability and growth, and with it favorable short- and long-term patency can be expected. The LA appendage is used as part of a conduit for the repair of truncus arteriosus [7] and tetralogy of Fallott with pulmonary atresia [8]. We also experienced a neonatal case of truncus arteriosus repaired using the LA appendage. Two years later the conduit was shown to have sufficient growth in follow-up cardiac catheterization.

In conclusion, we obtained satisfactory short-term results using the inverted left atrial appendage to construct the intraatrial drainage route of persistent LSVC connecting to the LA. Although the long-term results remain to be appraised, we think that this technique is suitable for young children with persistent LSVC to the LA.

	Footnotes

Top Footnotes Abstract Introduction Comment References

 
Address reprint requests to Dr Komai, Department of Thoracic and Cardiovascular Surgery, Wakayama Medical College, 27, 7-bancho, Wakayama, 640 Japan.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Shumacker HB Jr, King H, Waldhausen JA. The persistent left superior vena cava: surgical implications, with special reference to caval drainage into the left atrium. Ann Surg 1967;165:797–805.[Medline]
2. Lucas RV Jr, Krabill KA. Anomalous venous connections, pulmonary and systemic. In: Adams FH, Emmanouilides GC, eds. Heart disease in infants, children and adolescents. Baltimore: Williams & Wilkins, 1983:580–617.
3. Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P Jr, Edwards JE. Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus; a developmental complex. Circulation 1965;31:906–18.[Abstract/Free Full Text]
4. De Leval MR, Ritter DG, McGoon DC, Danielson GK. Anomalous systemic venous connection: surgical considerations. Mayo Clin Proc 1975;50:599–610.[Medline]
5. Rastelli GC, Ongley PA, Kirklin JW. Surgical correction of common atrium with anomalously connected persistent left superior vena cava: report of a case. Mayo Clin Proc 1965;40:528–32.[Medline]
6. Foster ED, Baesa OR, Farina MF, Shaher RM. Atrial septal defect associated with drainage of left superior vena cava to the left atrium and absence of the coronary sinus. J Thorac Cardiovasc Surg 1978;76:718–20.[Abstract]
7. Barbero-Marcial M, Riso A, Atik E, Jatene A. A technique for correction of truncus arteriosus types I and II without extracardial conduits. J Thorac Cardiovasc Surg 1990;99:364–9.[Abstract]
8. Kitagawa T, Katoh I, Chikugo F, et al. Technique for constructing the pulmonary trunk for tetralogy of Fallot with pulmonary atresia. Ann Thorac Surg 1995;59:1245–8.[Abstract/Free Full Text]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Keiichi Fujiwara Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Komai, H. Articles by Fujiwara, K. Search for Related Content PubMed PubMed Citation Articles by Komai, H. Articles by Fujiwara, K.