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Ann Thorac Surg 1995;59:1471-1475
© 1995 The Society of Thoracic Surgeons

Repair of Anomalous Pulmonary Venous Connection to the Superior Vena Cava

J. William Gaynor, MD, Michael Burch, MRCP, Claire Dollery, MD, Ian D. Sullivan, MD, John E. Deanfield, FRCP, Martin J. Elliott, FRCS

Cardiothoracic Unit, The Hospital for Sick Children, London, England

Accepted for publication February 17, 1995.


   Abstract
Top
 Footnotes
 Abstract
Introduction
 Material and Methods
 Results
 Comment
 References
 
Complex forms of anomalous pulmonary venous connection to the superior vena cava (SVC) can be difficult to correct surgically. Since 1987, 11 patients have undergone repair of anomalous pulmonary venous connection to the SVC by diversion of the pulmonary venous drainage to the left atrium using a baffle with division of the SVC and reimplantation on the right atrial appendage to restore normal systemic venous drainage. Total anomalous pulmonary venous connection was present in 3 patients and partial anomalous pulmonary venous connection, in 8. All patients are alive and asymptomatic at a mean follow-up of 2.3 ± 1.4 years. Postoperative echocardiograms (8 patients) revealed pulmonary venous obstruction requiring reoperation in 1 patient. No patient has clinical evidence of SVC obstruction, and all are in sinus rhythm. This is a safe and effective technique for repair of complex forms of anomalous pulmonary venous connection to the SVC, and the incidence of postoperative venous obstruction and rhythm disturbances is low.


   Introduction
Top
 Footnotes
 Abstract
 Introduction
Material and Methods
 Results
 Comment
 References
 
Complex variants of anomalous pulmonary venous connection (APVC) to the superior vena cava (SVC) can be difficult to correct. Total anomalous pulmonary venous connection (TAPVC) to the SVC occurs when all of the pulmonary veins drain to the SVC without a connection to the left atrium. Partial anomalous pulmonary venous connection (PAPVC) is present when some of the pulmonary veins drain anomalously to the SVC, but at least one pulmonary vein drains to the left atrium. Supracardiac TAPVC with a posterior confluence of pulmonary veins draining to the SVC through a vertical vein can be repaired by direct anastomosis of the confluence to the left atrium with ligation of the vertical vein. However, if all of the pulmonary veins drain directly to the SVC, surgical correction may not be straightforward.

Anomalous pulmonary venous connection to the SVC is often associated with either a sinus venosus or secundum atrial septal defect (ASD), although the atrial septum may be intact. A variety of techniques have been suggested for correction of APVC to the SVC; however, postoperative complications including arrhythmias and obstruction of the SVC or pulmonary veins still occur [1–6]. Patch enlargement of the SVC with an incision in the region of the sinus node or SVC-right atrial junction is frequently required. The sinus node artery may encircle the SVC, and any incision in this region, even a posterolateral incision, may damage the sinus node artery, thus leading to sinus node dysfunction. Variants of APVC to the SVC that are difficult to repair include drainage of all of the pulmonary veins directly to the SVC without a pulmonary venous confluence, drainage of one or more pulmonary veins to the high SVC near the innominate vein, and drainage of one or more pulmonary veins to a small right SVC (usually in association with a persistent left SVC) without a bridging vein [4].

Creation of an atrial wall baffle to redirect pulmonary venous and SVC drainage across an ASD to the left atrium with division of the SVC above the pulmonary veins and reimplantation on the right atrium to maintain normal systemic venous drainage has been proposed for difficult cases of APVC to the SVC [7–11]. Although this technique has not been widely used, previous reports of similar techniques have documented a low operative mortality as well as a low incidence of postoperative SVC and pulmonary venous obstruction in these patients with complex anatomy [7–11]. No incision is necessary in the region of the sinoatrial node or SVC-right atrial junction, and the reported incidence of postoperative arrhythmias is low.


   Material and Methods
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
Results
 Comment
 References
 
Since 1987, 11 patients (6 male and 5 female) have undergone correction of APVC to the SVC at our institution using the method just described at a mean age of 11.2 ± 12.3 years (range, 0.1 to 41.2 years) (Table 1Go). Four patients were 2 years of age or younger, and 3 were less than 6 months of age. Total anomalous pulmonary venous connection to the SVC without a posterior pulmonary venous confluence was present in 3 patients and PAPVC to the SVC, in the remainder. A sinus venosus ASD was present in 4 patients, a secundum ASD in 3 patients, and a patent foramen ovale in 3 patients. The atrial septum was intact in 1 patient.


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  		Table 1. . Summary of Patient Data
 
The operation is performed through a median sternotomy. The thymus is resected and dissection of the SVC carried to the innominate vein. The SVC and anomalous pulmonary veins must be fully mobilized and the azygos vein divided to provide maximum mobility of the SVC. The SVC cannula is placed at the junction of the innominate vein and SVC, and the inferior vena cava cannula is placed at the right atrial-inferior vena cava junction, below the pericardial reflection.

After institution of total cardiopulmonary bypass and cardioplegic arrest, a J-shaped right atriotomy is performed (Fig 1Go). When an ASD is present, it can be enlarged if necessary to ensure unobstructed drainage of the pulmonary veins to the left atrium (Fig 2Go). If the atrial septum is intact, an ASD must be created. The posterior margin of the atriotomy incision is sutured to the anterior edge of the ASD and around the orifice of the SVC, thus baffling the SVC and pulmonary venous drainage to the left atrium (see Fig 2Go). The atriotomy is closed by approximating its anterior margin to the posterior portion of the atrial wall that serves as the baffle (Fig 3Go). This step moves the right atrial appendage toward the SVC, thereby allowing the subsequent anastomosis to be performed without tension.



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  		Fig 1. . Dissection of superior vena cava (SVC) is carried to innominate vein. The SVC and anomalous pulmonary veins are mobilized, and the azygos vein is divided. Direct bicaval cannulation is performed with SVC pursestring placed at junction of SVC and innominate vein. The inferior vena cava (IVC) cannula is placed at right atrial-IVC junction, below the pericardial reflection. After institution of total cardiopulmonary bypass and cardioplegic arrest, a J-shaped right atriotomy is performed.

 


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  		Fig 2. . The atrial septum is inspected and the pulmonary venous connection, confirmed. If necessary, the atrial septal defect (ASD) is enlarged to ensure unobstructed drainage of pulmonary veins. The posterior margin of atriotomy is sutured around the orifice of the superior vena cava and to the anterior edge of the ASD, thus baffling the pulmonary venous drainage to the left atrium.

 


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  		Fig 3. . The atriotomy is closed by suturing its anterior margin to portion of atrial wall serving as the baffle, thus directing inferior vena cava flow to the tricuspid valve and bringing the right atrial (RA) appendage toward the superior vena cava (SVC). The SVC is divided above the pulmonary veins. The tip of the RA appendage is excised. It is very important to divide all trabeculations inside the appendage, as this will increase the length of appendage and may reduce risk of SVC obstruction.

 
The SVC is divided above the anomalous pulmonary veins and oversewn proximally. The tip of the right atrial appendage is excised. It is very important to divide all muscular trabeculations inside the appendage to increase the length of the appendage and reduce the risk of SVC obstruction (see Fig 3Go). The repair is completed by anastomosing the distal end of the SVC to the open right atrial appendage (Fig 4Go). The patient is rewarmed and separated from cardiopulmonary bypass in the usual fashion.



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  		Fig 4. . The repair is completed by oversewing proximal end of superior vena cava and anastomosing distal end to open right atrial appendage.

 

   Results
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
Comment
 References
 
There were no operative deaths, and there have been no late deaths at a mean follow-up of 2.3 ± 1.4 years (range, 0.1 to 5.4 years) (Table 2Go). Nine patients underwent repair using an atrial wall baffle, and in 2 patients a pericardial baffle was used (see Table 1Go). Pulmonary venous obstruction developed in 1 patient operated on early in the series in whom a pericardial baffle was used. In that patient, fatigue, decreased appetite, and weight loss developed 3 months postoperatively. A pulmonary angiogram was normal; however, transesophageal echocardiography revealed pulmonary venous obstruction. Revision of the baffle was undertaken using a Gore-Tex patch (W. L. Gore & Assoc, Flagstaff, AZ). A Gore-Tex patch was used to minimize the need of mobilization of the right atrium and SVC at the second operation. The patient is now asymptomatic and in sinus rhythm.


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  		Table 2. . Summary of Latest Follow-up Data
 
At last follow-up, all patients were asymptomatic and in sinus rhythm on resting electrocardiogram. No patient has had a documented arrhythmia or symptoms suggestive of arrhythmia (see Table 2Go). Eight patients have undergone postoperative echocardiography; 6 had normal flow in the SVC with unobstructed drainage of the pulmonary veins to the left atrium. In 2 patients, the SVC flow velocity was slightly increased (1.2 and 1.4 m/s), but there was no clinical evidence of SVC obstruction. Postoperative echocardiograms are not available for 3 patients.


   Comment
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
References
 
Substantial anatomic variability exists among patients with APVC to the SVC. Surgical repair of APVC to the SVC can be complicated by obstruction of the SVC, obstruction of the pulmonary veins, and postoperative arrhythmias [1–6]. A variety of techniques have been suggested for correction of APVC to the SVC including use of a baffle to redirect the pulmonary venous drainage from the SVC across an ASD, partitioning of the SVC, and division of the anomalous pulmonary veins with reanastomosis to the left atrium [2, 5, 12–14]. However, application of these techniques to complex variants of APVC to the SVC can result in an increased incidence of postoperative complications, and some forms are difficult to correct using standard methods [9–11, 15].

Repair of even simple forms of APVC to the SVC can be complicated by obstruction of the SVC or pulmonary veins and postoperative rhythm disturbances [1, 4–6, 12]. Use of a single patch to baffle the pulmonary venous drainage from the SVC to the right atrium can be associated with narrowing of the SVC and subsequent SVC obstruction [12]. Schuster and colleagues [12] used a second patch to enlarge the junction between the SVC and the right atrium to decrease the incidence of postoperative SVC obstruction. Subsequently, Friedli and associates [4] reported SVC obstruction after repair of PAPVC despite enlargement of the SVC-right atrial junction. The risk of SVC obstruction was increased when a left SVC was present, and the right SVC was smaller than usual [4].

DeLeon and co-workers [3] recently reported follow-up of 40 patients after repair of PAPVC to the SVC at a mean age of 6 ± 2 years. In 18 patients (group I), an atriotomy was performed through the right atrial appendage and extended through the sinus node to the SVC. A baffle was used to redirect the pulmonary veins, and the SVC was enlarged using an atriocavoplasty. In 17 patients (group II), redirection of the pulmonary veins was accomplished through a right atriotomy without SVC enlargement, and in 5 patients (group III), the right atrial appendage was anastomosed end-to-side to the SVC after rerouting of the pulmonary veins with a patch. Two patients in group I had development of sinus bradycardia. Superior vena cava obstruction and pulmonary venous obstruction developed in 1 patient in group II, and heart block was seen in 1 patient in group III.

Experimental anastomosis of the SVC to the right atrium was first reported by Gerbode and colleagues [16] in 1949. Correction of APVC to the SVC using an atrial wall baffle with reimplantation of the SVC on the right atrial appendage was proposed by Lewis [7] in 1958, and in the same year, Ehrenhaft and associates [17] reported the repair of APVC to the SVC using a similar technique. Groves [8] used a posterior atrial wall baffle to redirect pulmonary venous drainage with division of the SVC and reimplantation on the right atrium in 3 children with APVC to the SVC (2 with PAPVC and 1 with TAPVC). Clinical evidence of SVC obstruction developed in 1 patient.

Warden and associates [10] repaired PAPVC to the SVC in 15 patients by coaptation of the anterior border of the ASD to the intracardiac orifice of the SVC, thus diverting the SVC and pulmonary venous drainage to the left atrium. The SVC was divided above the anomalous veins and anastomosed to the right atrium. One death occurred in a patient with pulmonary hypertension, and SVC obstruction developed in 1 patient.

Williams and colleagues [9] used a pericardial patch to baffle the SVC and pulmonary veins to the left atrium, with division of the SVC above the pulmonary veins and reanastomosis to a conduit created from the right atrial appendage in 6 children. All of the children survived in sinus rhythm without SVC or pulmonary venous obstruction. Vargas and Kreutzer [11] corrected TAPVC to the SVC in 3 children using a J-shaped right atriotomy to create a posterior flap of the right atrial wall that was sutured to the anterior border of the ASD and around the orifice of the SVC to divert the pulmonary venous drainage to the left atrium. The SVC was divided above the pulmonary veins, and SVC-right atrial continuity was reestablished by direct anastomoses. All 3 children survived without evidence of pulmonary venous or SVC obstruction at follow-up.

Complex forms of APVC to the SVC, particularly those when all of the pulmonary veins drain directly to the SVC or when pulmonary veins drain to a high SVC near the innominate vein or when the right SVC is small, present a difficult surgical challenge [9–11, 16]. The use of an atrial wall baffle to redirect the pulmonary venous flow to the left atrium with division and reimplantation of the SVC on the right atrial appendage is an attractive option for these difficult patients [8, 11]. Advantages include creation of wide, unobstructed pathways for both SVC and pulmonary venous drainage and avoidance of any incision in the region of the sinus node and its artery. The technique is applicable to both TAPVC and PAPVC, even in neonates. Reimplantation of the SVC on the right atrium is required as well as creation of two atrial suture lines. However, follow-up in this series and reported series reveals a low incidence of pulmonary venous and SVC obstruction and a low incidence of postoperative rhythm disturbances. Primary anastomosis of the SVC to the right atrial appendage was accomplished in every patient without the use of atrial appendage pedicled conduits or prosthetic grafts. Although this repair is not necessary for all patients with APVC to the SVC, it should be considered for patients with complex anatomy.


   Footnotes
Top
 Footnotes
Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 
Address reprint requests to Dr Gaynor, Pediatric Cardiothoracic Surgery, The Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104-4399.


   References
Top
 Footnotes
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 

  1. Clark EB, Roland JA, Varghese PJ, Neill CA, Haller JA. Should the sinus venosus type ASD be closed? A review of the atrial conduction defects and surgical results in twenty-eight children [Abstract]. Am J Cardiol 1975;35:127.
  2. Chartrand C, Payot M, Davignon A, Guerin R, Stanley P. A new surgical approach for correction of partial anomalous pulmonary venous drainage into the superior vena cava. J Thorac Cardiovasc Surg 1976;71:29–34.[Abstract]
  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.[Abstract]
  4. Friedli B, Guerin R, Davignon A, Fouron JC, Stanley P. Surgical treatment of partial anomalous pulmonary venous drainage: a long-term follow-up study. Circulation 1972;45:159–70.[Abstract/Free Full Text]
  5. Kyger ER III, Frazier OH, 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.[Abstract]
  6. Trusler GA, Kazenelson G, Freedom RM, Williams WG, Rowe RD. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 1980;79:776–81.[Medline]
  7. Lewis FJ. High defects of the atrial septum. J Thorac Surg 1958;36:1–11.
  8. Groves LK. Correction of anomalous pulmonary venous drainage into the superior vena cava. Ann Thorac Surg 1967;4:301–17.[Medline]
  9. Williams WH, Zorn-Chelton S, Raviele AA, et al. Extracardiac atrial pedicle conduit repair of partial anomalous pulmonary venous connection to the superior vena cava in children. Ann Thorac Surg 1984;38:345–55.[Abstract]
  10. 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;38:601–15.[Abstract]
  11. Vargas FJ, Kreutzer GO. A surgical technique for correction of total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1985;90:410–3.[Abstract]
  12. 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.[Medline]
  13. Robicsek F, Daugherty HK, Cook JW, Selle JG. Sinus venosus type of atrial septal defect with partial anomalous pulmonary venous return. J Thorac Cardiovasc Surg 1979;78:559–62.[Abstract]
  14. Long DM, Rios MV, Elias DO, Meier MA, DuBrow IW. Parietal and septal atrioplasty for total correction of anomalous pulmonary venous connection with superior vena cava. Ann Thorac Surg 1974;18:466–71.[Medline]
  15. Zoltie N, Walker DR. Total anomalous pulmonary venous drainage into the right superior vena cava. J Cardiovasc Surg (Torino) 1983;24:537–9.[Medline]
  16. Gerbode F, Yee J, Rundle FF. Experimental anastomoses of vessels to the heart: possible application to superior vena caval obstruction. Surgery 1949;25:556–65.[Medline]
  17. Ehrenhaft JL, Theilen EO, Lawrence MS. The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 1958;148:249–58.[Medline]



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