- Email: [email protected]
Outcome of Caval Division Techniques for Partial Anomalous Pulmonary Venous Connections to the Superior Vena Cava
PEDIATRIC CARDIAC
Outcome of Caval Division Techniques for Partial Anomalous Pulmonary Venous Connections to the Superior Vena Cava Sameh M. Said, MD, Harold M. Burkhart, MD, Joseph A. Dearani, MD, Ben Eidem, MD, Paul Stensrud, MD, Sabrina D. Phillips, MD, and Hartzell V. Schaff, MD Divisions of Cardiovascular Surgery, Cardiovascular Diseases and Cardiac Anesthesia, Mayo Clinic, Rochester, Minnesota
Background. Correction of a high partial anomalous pulmonary venous (PV) connection to the superior vena cava (SVC) may be complicated by sinus node dysfunction, or SVC or PV obstruction. We have used the caval division technique when the anomalous PV insert high above the cavoatrial junction and report our long-term outcome. Methods. Between 1990 and 2009, 178 consecutive patients underwent operations for a partial anomalous PV connection. Caval division was used in 41 patients (23%). Median age was 34 years (range, 1.3 to 76 years). Thirty patients (73%) had an atrial septal defect. Eighteen patients (44%) underwent Warden procedure in which the SVC was divided, the upper end was connected to the right atrial appendage, and the remaining SVC served as a conduit for PV drainage to the left atrium through the atrial septal defect. A modified Warden was performed in 17 patients (41%) using a short ringed interposition graft between the SVC and the right atrial appendage. Six patients (15%) with persistent left SVC underwent right SVC division without reimplantation.
Results. No early deaths occurred. One Warden group patient (5%) required early reoperation for SVC obstruction. One patient (2%) with atrial fibrillation underwent a concomitant Maze procedure and permanent pacemaker implantation for tachybrady syndrome. All other patients remained in sinus rhythm at follow-up. Follow-up was complete in all patients (mean, 2 ⴞ 2.6 years; maximum, 14 years). One noncardiac-related death occurred at 14 years. SVC obstruction developed in 3 Warden group patients (17%) and was managed with angioplasty and stenting. No patient had pulmonary vein stenosis on follow-up. Conclusions. Caval division techniques appear to be effective, with low morbidity and mortality. The modified Warden procedure may decrease the risk of SVC obstruction. Late arrhythmias or PV obstruction are uncommon.
P
structed way. Many techniques have been described [3–9], the most common being the single-patch technique, in which the APVs are baffled by one atrial patch into the left atrium through a native or surgically created ASD. The concern with any of these surgical techniques is the development of SVC or pulmonary venous obstruction, as well as sinus node dysfunction. This is particularly important if the APVs are committed to the SVC. In 1984, Warden and colleagues [10] described the caval division technique, in which the SVC is divided above the entrance of the APV, the upper end is connected to the right atrial appendage (RAA), and the lower end carrying the anomalous vein is baffled through an internal patch to the left atrium through the ASD. This Warden procedure was designed to decrease the incidence of SVC, or pulmonary venous obstruction as well as sinus node dysfunction. We reviewed our experience with the caval division technique for PAPVC.
artial anomalous pulmonary venous connection (PAPVC) is characterized by a failure of 1 to 3 of the pulmonary veins to connect with the left atrium during fetal development. Most commonly, the APV is connected to the superior vena cava (SVC) near the cavoatrial junction or to the right atrium [1]. Other congenital heart defects, the most common of which is an atrial septal defect (ASD), is present in more than 80% of the patients. An intact atrial septum is present in 18% [2]. The most common anomalous veins combinations are the right upper and middle lobe PVs. Isolated anomalous right upper lobe venous drainage is less common. The ASD associated with PAPVC is not amenable to percutaneous device closure due to its complexity, and surgical intervention remains the primary method for repair of these anomalies. The goal of the operation is to divert the APV drainage to the left atrium in an unobAccepted for publication April 1, 2011. Presented at the Forty-seventh Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 31–Feb 2, 2011. Address correspondence to Dr Burkhart, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: [email protected].
© 2011 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2011;92:980 –5) © 2011 by The Society of Thoracic Surgeons
Patients and Methods This retrospective review was approved by the Mayo Clinic and Foundation Institutional Review Board. 0003-4975/$36.00 doi:10.1016/j.athoracsur.2011.04.110
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Patient Population
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Between February 1990 and August 2009, 178 patients underwent surgical repair of PAPVC, and we identified 41 (23%) with high insertion of the APVs into the SVC above the cavoatrial junction. The mean age was 35 ⫾ 24 years (range, 1.3 to 76 years; median age, 34 years), and 11 patients (27%) were children. There were 22 males and 19 females. Eighteen patients (44%) underwent the traditional Warden procedure (group A), 17 (41%) underwent a modified version of Warden (group B), and the remaining 6 (15%) underwent caval division without reimplantation (group C; Fig 1).
Preoperative Data The main indications of operation were the presence of symptoms or evidence of right ventricular volume overload on preoperative echocardiography. Seventeen patients (41%) were asymptomatic. Preoperative echocardiographic data were obtained in all patients, and cardiac catheterization was performed in 11 (27%). A native ASD was present in 30 patients (73%), including sinus venosus type in 22 (73%), ostium secundum type in 8 (27%), and patent foramen ovale in 7 (23%). The most common APV drainage combination was the right upper and middle lobe veins in 26 patients (43%). The mean number of the APVs was 2.2 (range, 1 to 4). Persistent left SVC was present in 9 patients (22%), with 6 of these having well-developed bilateral SVC with a well-developed communicating vein (group C). Three patients (7%) had an associated left PAPVC.
Fig 2. Intraoperative photograph shows partial anomalous pulmonary veins from the right upper and middle lobes (white arrows) draining into the superior vena cava above the cavoatrial junction.
Surgical Technique A standard median sternotomy was preformed. The entrance level of the APVs into the SVC was adequately visualized after opening the right pleura (Fig 2). The pericardium was opened and the heart was exposed. The SVC was dissected out to the innominate vein, and the azygous and the APVs were adequately exposed. Cardiopulmonary bypass was established using ascending aortic and bicaval cannulation. The SVC cannula
18 16 14 12 10 8 6 4 2 0 Warden
Modified Warden
Caval Division only
Fig 1. Graph shows number of patients who underwent each procedure: the traditional Warden procedure was preformed in 18 patients, the modified Warden in 17, and caval division only in 6.
was placed as high as possible in the SVC or in the innominate vein. A persistent left SVC was managed by an additional suction line inserted into the coronary sinus through the right atrium. The mean cardiopulmonary bypass time was 89 ⫾ 35 minutes, with a mean aortic cross-clamp time of 40 ⫾ 18 minutes. Normothermia was used in 30 patients (73%). The azygous vein was divided, and the right SVC was cross-clamped and divided above the insertion of the highest APV with oversewing of its caudal end. A surgical ASD was created in those patients who had an intact atrial septum (Fig 3) by excising the fossa ovalis and extending the incision upwards with removal of a portion of the septum secundum, as needed, to ensure an adequately sized ASD with an unobstructed pathway for the APVs. In cases of an associated secundum-type ASD, we typically used a patch or primarily closed the inferior half of the ASD. We then extended the ASD superiorly as described above. The APVs were baffled with an internal patch through the ASD into the left atrium. The most commonly used patch materials were untreated autologous pericardium in 25 patients (61%) and bovine pericardium in 14 patients (34%). Gore-Tex (W.L. Gore and Assoc, Flagstaff, AZ) and glutaraldehyde-treated autologous pericardium were used in 1 patient each (2%). To avoid isolating the sinus node from the rest of the right atrial wall during suturing of
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Fig 3. Intraoperative photograph shows (A) native sinus venosus atrial septal defect and (B) a surgically created atrial septal defect in the presence of an intact atrial septum. PEDIATRIC CARDIAC
the patch and to minimize the risk of sinus node dysfunction, we believe that the depth of the suture bites should be partial thickness. These basic surgical principles did not differ among the three groups (Fig 4A and B). In group A, the proximal portion of the SVC was anastomosed directly to the RAA after cutting all of the trabeculations (Fig 4C). We have used polypropylene as our suture material of choice, and to avoid the pursestring effect, we use a continuous technique for the Fig 4. (A) The surgically created atrial septal defect is shown through the right atriotomy. (B) Bovine pericardial patch is used as an intraatrial baffle to redirect the pulmonary venous drainage through the atrial septal defect into the left atrium. (C) The traditional Warden procedure creates a direct anastomosis between the proximal superior vena cava and the right atrial appendage. (D) The modified Warden procedure is shown using a short ringed Gore-Tex graft interposition.
posterior wall and interrupt the anterior wall of the SVC-RAA anastomosis. In group B, a short piece of a ringed Gore-Tex graft was used as an interposition graft between the proximal SVC and the RAA (Fig 4D). The graft sizes were between 14 and 20 mm. These patients received anticoagulation with Coumadin (Bristol-Myers Squibb, Princeton, NJ) for 3 months postoperatively. In group C, with bilateral well-developed SVC and a communicating vein, the right SVC was divided and did not
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Fig 5. Posterior view of the heart is shown in the 6 patients who had bilateral superior vena cavae with a well-developed communicating vein. These patients underwent right superior vena caval division without reimplantation. The systemic venous drainage is directed through the communicating vein into the left superior vena cava and then through the coronary sinus into the right atrium. (A) Preoperatively. (B) Postoperatively. (ASD ⫽ atrial septal defect; L ⫽ left; LA ⫽ left atrium; R ⫽ right; RA ⫽ right atrium; SVC ⫽ superior vena cava; v ⫽ vein.)
require reimplantation into the RAA. The venous drainage from the head and neck was through the left SVC into the right atrium through the coronary sinus (Fig 5). The most common associated procedures were a modified Cox-Maze procedure using cryoablation in 8 patients (20%), tricuspid valve annuloplasty in 6 (15%), ligation of the left atrial appendage in 4 (10%), and pulmonary valve replacement in 2 (5%).
Follow-Up The mean follow-up time from the operation to the last assessment was 2 ⫾ 2.6 years, maximum of 14 years. This was shorter in the modified Warden group. This difference is only because we began applying this technique at our institution in 2006. Follow-up echocardiographic data and electrocardiograms (ECG) were available in all patients. ECG was considered early if it is obtained before hospital discharge. Late ECG was obtained after discharge. The ECG was interpreted as normal sinus rhythm, junctional rhythm, and supraventricular arrhythmias, which included atrial flutter, atrial fibrillation, and multifocal atrial tachycardias.
Results There were no early deaths. Three patients (7%) required early reoperation: One patient underwent reoperation for postoperative bleeding, and another underwent sternal debridement and rewiring due to sternal dehiscence. The third patient had early postoperative SVC obstruction despite adequate SVC and RAA mobilization and tension-free anastomosis and required reoperation on the same day, with conversion to a modified Warden procedure with insertion of a 16-mm ringed Gore-Tex interposition graft. Normal sinus rhythm in 8 patients (20%) changed to junctional rhythm. This recovered at time of discharge, with all but 1 patient remaining in sinus
rhythm at late follow-up. One patient required permanent pacemaker placement for tachybrady syndrome. This patient underwent a concomitant modified CoxMaze procedure for preoperative atrial fibrillation at the time of PAPVC repair. Two patients (5%) had postpericardiotomy syndrome that was managed by antiinflammatory medications. No patient had pulmonary venous stenosis among the three groups. One late death occurred: an 82 year-old woman died of a noncardiac-related cause 14 years after the operation. No patient required late reoperation. Late SVC obstruction occurred in 3 patients (17%) from the traditional Warden group. The first 2 patients were 2-year-old girls who were diagnosed with SVC obstruction at 10 and 8 months postoperatively, and the third patient was a 47-year-old woman who was diagnosed 20 months postoperatively. All SVC obstructions were managed with percutaneous balloon angioplasty and stent placement, with no residual obstruction.
Comment Several surgical techniques have been described for repair of PAPVC. These include the 1-patch or 2-patch techniques and various other modifications. De Leon and colleagues [11] described a modification using the RAA as a flap across the cavoatrial junction. Because it was close to the sinus node, this was again modified into 2 separate incisions. The concern with any of these techniques is the risk of sinus node dysfunction, SVC, or pulmonary venous stenosis [12–14]. We have used the Warden procedure for high insertion of the APVs into the SVC. Higher insertion of the APV into the SVC with a resulting shorter SVC after division adds to the complexity of the repair. Gustafson and colleagues [15] reported only 1 patient with SVC obstruction and sick sinus syndrome postoperatively among a series of 40 patients who underwent
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the Warden procedure. In the 5 patients who underwent Warden procedure as reported by Stewart and colleagues [16], SVC obstruction developed in 1 patient 2 years after a Warden procedure, but they attributed that to a technical problem caused by failure of excision of all the RAA trabeculations, as Warden and colleagues [10] reported in their original article. In our series, one early (5%) and three late cases (17%) of SVC obstructions occurred in the traditional Warden group. The median age of our patients was 34 years, which seems to be older than the age in other reports. This may be due to referral patterns, but we did not notice an effect for age on the late development of SVC stenoses. In fact, 2 of the 3 patients with late SVC stenoses were children. Late SVC stenoses occurred despite adequate mobilization of the proximal portion of the SVC and the RAA to create a tension-free anastomosis as well as excision of all RAA trabeculations. These patients will require regular follow-up for early detection of SVC obstruction. No SVC obstructions occurred in our patients in the modified Warden group, suggesting this may represent a good alternative in adults with high PAPVC. No sinus node dysfunction was detected in any of the 11 patients in a series undergoing the Warden procedure reported by Gaynor and colleagues [17]. Stewart and colleagues [16] did not report sinus node dysfunction in any of the 5 patients in their series who underwent the Warden procedure. Caval division techniques are associated with a low incidence of sinus node dysfunction, and all the patients in our series but 1 remained in normal sinus rhythm at time of discharge and at late follow-up. This is consistent with other authors’ experiences with the 1-patch or 2-patch technique where the incisions are placed close to the sinus node or its arterial supply [16, 18]. The primary advantage of this procedure is that a right atrial incision extending across the cavoatrial junction or near the sinus node is not necessary. In the Gaynor and colleagues [17] series, only 1 patient had pulmonary venous obstruction. Shahriari and colleagues [19] reported 13 patients who underwent the Warden repair, with only 1 early pulmonary venous obstruction. In this series the authors initially used an untreated autologous pericardial patch and then switched to a Gore-Tex patch for diversion of the pulmonary venous return due to fear of patch contraction and PV obstruction. In our series, 41 patients underwent a caval division procedure, and none of them had any early or late pulmonary venous obstruction. We have not encountered any pulmonary venous obstruction due to patch contraction among any of our patients, regardless of the patch material that was used. This same conclusion regarding untreated autologous pericardium was reported by Stewart and colleagues [16]. In conclusion, caval division techniques for treatment of high PAPVC are effective and associated with low mortality and morbidity. The Warden procedure— or its modification— creates an unobstructed passage for the pulmonary veins, and the chance of sinus node dysfunction is very low. The modified Warden procedure may decrease the risk of SVC obstruction. Caval division
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without reimplantation of the right SVC appears to be a reasonable choice in patients with a well-developed bilateral SVC and a communicating vein.
References 1. Hijii T, Fukushige J, Hara T. Diagnosis and management of partial anomalous pulmonary venous connection. A review of 28 pediatric cases. Cardiology 1998;89:148 –51. 2. Gustafson RA, Warden HE, Murray GF, et al. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg 1989;98:861– 8. 3. Lewis FJ, Taufic M, Varco RL, et al. The surgical anatomy of atrial septal defects: experience with repair under direct vision. Ann Surg 1955;142:401–15. 4. Kirklin JW, Ellis FH Jr., Wood EH. Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 1956;39:389 –98. 5. Bahnson HT, Spencer FC, Neill CA. Surgical treatment of thirty-five cases of drainage of pulmonary veins to the right side of the heart. J Thorac Surg 1958;36:777–99. 6. Ehrenhaft JL, Theilen EO, Lawrence MS. The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 1958;148:249 –58. 7. 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. 8. Puig-Massana M, Murtra M, Revuelta JM. A new technique in the correction of partial anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1972;64:108 –13. 9. Lewin AN, Zavanella C, Subramanian S. Sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage: surgical repair. Ann Thorac Surg 1978;26:185– 8. 10. Warden HE, Gustafson RA, Tarnay TJ, et al. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601–5. 11. 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. 12. Stewart S, Alexon 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. 13. Freiedli B, Guerin R, Davignon A, et al. Surgical treatment of partial anomalous pulmonary venous drainage: a long-term follow-up study. Circulation 1972;45:159 –70. 14. Trusler GA, Kazenelson G, Freedom RM, et al. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 1980;79:776 – 81. 15. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60(suppl):614 –7. 16. Stewart RD, Bailliard F, Kelle AM, et al. 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. 17. Gaynor JW, Burch M, Dollery C, et al. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;59:1471–5. 18. Nicholson IA, Chard RB, Nunn GR, et al. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg 2000;119:741– 4. 19. Shahriari A, Rodefeld MD, Turrentine MW, et al. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg 2006;81:224 –30.
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DR DUKE CAMERON (Baltimore, MD): How often do you use this technique? How many of your partial vein cases require this as opposed to just a simple patch from the anomalous vein orifice to the sinus venosus atrial septal defect (ASD)? DR SAID: We have adopted this technique for all adult patients with high insertion of the anomalous veins at least 1 cm above the cavoatrial junction. We started using this modification in 2006. In our partial veins series we use this technique about 20% of the time. DR ROBERT JAQUISS (Durham, NC): Do you use any sort of anticoagulation when you put the piece of Gore-Tex in with the modified procedure? And have you gone to using the modified procedure for all of your Wardens, since you had a fairly significant incidence of superior vena cava (SVC) stenosis with an unmodified Warden? DR SAID: We use anticoagulation for 3 months with Coumadin (Bristol-Myers Squibb, Princeton, NJ) for all adults with an interposition graft. We have a low threshold to use this procedure in adult patients with high insertion of the anomalous pulmonary vein. We have noticed that even with extensive mobilization of the proximal SVC and the right atrial append-
age, there are still some cases with anastomotic tension that could predispose them to SVC obstruction. DR SHIVAPRAKASH SESHADRI (Detroit, MI): I work with Dr Walters from Children’s Hospital of Michigan in Detroit. We are in the process of writing a paper on this. We have 13 cases. Dr Walters modifies this technique in a different way. He performs the posterior hemicircumference of the cavoatrial anastomosis with running polydioxanone suture and augments the anterior aspect of the anastomosis with a diamond-shaped patch of fresh autologous pericardium, using a vertical counter-incision in the anterior aspect of the SVC and the right atrial appendage. We have done 13 patients, 6 of whom had bilateral SVC with an associated small right SVC. We have experienced no caval obstruction in any of the patients in our series. DR SAID: We are aware of your technique and thank you for sharing your experience. How long was your follow-up? Did you change any of your anastomotic technique? What type of sutures did you use? DR SESHADRI: No. We used in situ pericardium. Once we do posterior, we put a kind of diamond-shaped pericardial patch.
PEDIATRIC CARDIAC
DISCUSSION
Outcome of Caval Division Techniques for Partial Anomalous Pulmonary Venous Connections to the Superior Vena Cava Sameh M. Said, MD, Harold M. Burkhart, MD, Joseph A. Dearani, MD, Ben Eidem, MD, Paul Stensrud, MD, Sabrina D. Phillips, MD, and Hartzell V. Schaff, MD Divisions of Cardiovascular Surgery, Cardiovascular Diseases and Cardiac Anesthesia, Mayo Clinic, Rochester, Minnesota
Background. Correction of a high partial anomalous pulmonary venous (PV) connection to the superior vena cava (SVC) may be complicated by sinus node dysfunction, or SVC or PV obstruction. We have used the caval division technique when the anomalous PV insert high above the cavoatrial junction and report our long-term outcome. Methods. Between 1990 and 2009, 178 consecutive patients underwent operations for a partial anomalous PV connection. Caval division was used in 41 patients (23%). Median age was 34 years (range, 1.3 to 76 years). Thirty patients (73%) had an atrial septal defect. Eighteen patients (44%) underwent Warden procedure in which the SVC was divided, the upper end was connected to the right atrial appendage, and the remaining SVC served as a conduit for PV drainage to the left atrium through the atrial septal defect. A modified Warden was performed in 17 patients (41%) using a short ringed interposition graft between the SVC and the right atrial appendage. Six patients (15%) with persistent left SVC underwent right SVC division without reimplantation.
Results. No early deaths occurred. One Warden group patient (5%) required early reoperation for SVC obstruction. One patient (2%) with atrial fibrillation underwent a concomitant Maze procedure and permanent pacemaker implantation for tachybrady syndrome. All other patients remained in sinus rhythm at follow-up. Follow-up was complete in all patients (mean, 2 ⴞ 2.6 years; maximum, 14 years). One noncardiac-related death occurred at 14 years. SVC obstruction developed in 3 Warden group patients (17%) and was managed with angioplasty and stenting. No patient had pulmonary vein stenosis on follow-up. Conclusions. Caval division techniques appear to be effective, with low morbidity and mortality. The modified Warden procedure may decrease the risk of SVC obstruction. Late arrhythmias or PV obstruction are uncommon.
P
structed way. Many techniques have been described [3–9], the most common being the single-patch technique, in which the APVs are baffled by one atrial patch into the left atrium through a native or surgically created ASD. The concern with any of these surgical techniques is the development of SVC or pulmonary venous obstruction, as well as sinus node dysfunction. This is particularly important if the APVs are committed to the SVC. In 1984, Warden and colleagues [10] described the caval division technique, in which the SVC is divided above the entrance of the APV, the upper end is connected to the right atrial appendage (RAA), and the lower end carrying the anomalous vein is baffled through an internal patch to the left atrium through the ASD. This Warden procedure was designed to decrease the incidence of SVC, or pulmonary venous obstruction as well as sinus node dysfunction. We reviewed our experience with the caval division technique for PAPVC.
artial anomalous pulmonary venous connection (PAPVC) is characterized by a failure of 1 to 3 of the pulmonary veins to connect with the left atrium during fetal development. Most commonly, the APV is connected to the superior vena cava (SVC) near the cavoatrial junction or to the right atrium [1]. Other congenital heart defects, the most common of which is an atrial septal defect (ASD), is present in more than 80% of the patients. An intact atrial septum is present in 18% [2]. The most common anomalous veins combinations are the right upper and middle lobe PVs. Isolated anomalous right upper lobe venous drainage is less common. The ASD associated with PAPVC is not amenable to percutaneous device closure due to its complexity, and surgical intervention remains the primary method for repair of these anomalies. The goal of the operation is to divert the APV drainage to the left atrium in an unobAccepted for publication April 1, 2011. Presented at the Forty-seventh Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 31–Feb 2, 2011. Address correspondence to Dr Burkhart, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: [email protected].
© 2011 by The Society of Thoracic Surgeons Published by Elsevier Inc
(Ann Thorac Surg 2011;92:980 –5) © 2011 by The Society of Thoracic Surgeons
Patients and Methods This retrospective review was approved by the Mayo Clinic and Foundation Institutional Review Board. 0003-4975/$36.00 doi:10.1016/j.athoracsur.2011.04.110
Ann Thorac Surg 2011;92:980 –5
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Patient Population
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Between February 1990 and August 2009, 178 patients underwent surgical repair of PAPVC, and we identified 41 (23%) with high insertion of the APVs into the SVC above the cavoatrial junction. The mean age was 35 ⫾ 24 years (range, 1.3 to 76 years; median age, 34 years), and 11 patients (27%) were children. There were 22 males and 19 females. Eighteen patients (44%) underwent the traditional Warden procedure (group A), 17 (41%) underwent a modified version of Warden (group B), and the remaining 6 (15%) underwent caval division without reimplantation (group C; Fig 1).
Preoperative Data The main indications of operation were the presence of symptoms or evidence of right ventricular volume overload on preoperative echocardiography. Seventeen patients (41%) were asymptomatic. Preoperative echocardiographic data were obtained in all patients, and cardiac catheterization was performed in 11 (27%). A native ASD was present in 30 patients (73%), including sinus venosus type in 22 (73%), ostium secundum type in 8 (27%), and patent foramen ovale in 7 (23%). The most common APV drainage combination was the right upper and middle lobe veins in 26 patients (43%). The mean number of the APVs was 2.2 (range, 1 to 4). Persistent left SVC was present in 9 patients (22%), with 6 of these having well-developed bilateral SVC with a well-developed communicating vein (group C). Three patients (7%) had an associated left PAPVC.
Fig 2. Intraoperative photograph shows partial anomalous pulmonary veins from the right upper and middle lobes (white arrows) draining into the superior vena cava above the cavoatrial junction.
Surgical Technique A standard median sternotomy was preformed. The entrance level of the APVs into the SVC was adequately visualized after opening the right pleura (Fig 2). The pericardium was opened and the heart was exposed. The SVC was dissected out to the innominate vein, and the azygous and the APVs were adequately exposed. Cardiopulmonary bypass was established using ascending aortic and bicaval cannulation. The SVC cannula
18 16 14 12 10 8 6 4 2 0 Warden
Modified Warden
Caval Division only
Fig 1. Graph shows number of patients who underwent each procedure: the traditional Warden procedure was preformed in 18 patients, the modified Warden in 17, and caval division only in 6.
was placed as high as possible in the SVC or in the innominate vein. A persistent left SVC was managed by an additional suction line inserted into the coronary sinus through the right atrium. The mean cardiopulmonary bypass time was 89 ⫾ 35 minutes, with a mean aortic cross-clamp time of 40 ⫾ 18 minutes. Normothermia was used in 30 patients (73%). The azygous vein was divided, and the right SVC was cross-clamped and divided above the insertion of the highest APV with oversewing of its caudal end. A surgical ASD was created in those patients who had an intact atrial septum (Fig 3) by excising the fossa ovalis and extending the incision upwards with removal of a portion of the septum secundum, as needed, to ensure an adequately sized ASD with an unobstructed pathway for the APVs. In cases of an associated secundum-type ASD, we typically used a patch or primarily closed the inferior half of the ASD. We then extended the ASD superiorly as described above. The APVs were baffled with an internal patch through the ASD into the left atrium. The most commonly used patch materials were untreated autologous pericardium in 25 patients (61%) and bovine pericardium in 14 patients (34%). Gore-Tex (W.L. Gore and Assoc, Flagstaff, AZ) and glutaraldehyde-treated autologous pericardium were used in 1 patient each (2%). To avoid isolating the sinus node from the rest of the right atrial wall during suturing of
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Fig 3. Intraoperative photograph shows (A) native sinus venosus atrial septal defect and (B) a surgically created atrial septal defect in the presence of an intact atrial septum. PEDIATRIC CARDIAC
the patch and to minimize the risk of sinus node dysfunction, we believe that the depth of the suture bites should be partial thickness. These basic surgical principles did not differ among the three groups (Fig 4A and B). In group A, the proximal portion of the SVC was anastomosed directly to the RAA after cutting all of the trabeculations (Fig 4C). We have used polypropylene as our suture material of choice, and to avoid the pursestring effect, we use a continuous technique for the Fig 4. (A) The surgically created atrial septal defect is shown through the right atriotomy. (B) Bovine pericardial patch is used as an intraatrial baffle to redirect the pulmonary venous drainage through the atrial septal defect into the left atrium. (C) The traditional Warden procedure creates a direct anastomosis between the proximal superior vena cava and the right atrial appendage. (D) The modified Warden procedure is shown using a short ringed Gore-Tex graft interposition.
posterior wall and interrupt the anterior wall of the SVC-RAA anastomosis. In group B, a short piece of a ringed Gore-Tex graft was used as an interposition graft between the proximal SVC and the RAA (Fig 4D). The graft sizes were between 14 and 20 mm. These patients received anticoagulation with Coumadin (Bristol-Myers Squibb, Princeton, NJ) for 3 months postoperatively. In group C, with bilateral well-developed SVC and a communicating vein, the right SVC was divided and did not
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Fig 5. Posterior view of the heart is shown in the 6 patients who had bilateral superior vena cavae with a well-developed communicating vein. These patients underwent right superior vena caval division without reimplantation. The systemic venous drainage is directed through the communicating vein into the left superior vena cava and then through the coronary sinus into the right atrium. (A) Preoperatively. (B) Postoperatively. (ASD ⫽ atrial septal defect; L ⫽ left; LA ⫽ left atrium; R ⫽ right; RA ⫽ right atrium; SVC ⫽ superior vena cava; v ⫽ vein.)
require reimplantation into the RAA. The venous drainage from the head and neck was through the left SVC into the right atrium through the coronary sinus (Fig 5). The most common associated procedures were a modified Cox-Maze procedure using cryoablation in 8 patients (20%), tricuspid valve annuloplasty in 6 (15%), ligation of the left atrial appendage in 4 (10%), and pulmonary valve replacement in 2 (5%).
Follow-Up The mean follow-up time from the operation to the last assessment was 2 ⫾ 2.6 years, maximum of 14 years. This was shorter in the modified Warden group. This difference is only because we began applying this technique at our institution in 2006. Follow-up echocardiographic data and electrocardiograms (ECG) were available in all patients. ECG was considered early if it is obtained before hospital discharge. Late ECG was obtained after discharge. The ECG was interpreted as normal sinus rhythm, junctional rhythm, and supraventricular arrhythmias, which included atrial flutter, atrial fibrillation, and multifocal atrial tachycardias.
Results There were no early deaths. Three patients (7%) required early reoperation: One patient underwent reoperation for postoperative bleeding, and another underwent sternal debridement and rewiring due to sternal dehiscence. The third patient had early postoperative SVC obstruction despite adequate SVC and RAA mobilization and tension-free anastomosis and required reoperation on the same day, with conversion to a modified Warden procedure with insertion of a 16-mm ringed Gore-Tex interposition graft. Normal sinus rhythm in 8 patients (20%) changed to junctional rhythm. This recovered at time of discharge, with all but 1 patient remaining in sinus
rhythm at late follow-up. One patient required permanent pacemaker placement for tachybrady syndrome. This patient underwent a concomitant modified CoxMaze procedure for preoperative atrial fibrillation at the time of PAPVC repair. Two patients (5%) had postpericardiotomy syndrome that was managed by antiinflammatory medications. No patient had pulmonary venous stenosis among the three groups. One late death occurred: an 82 year-old woman died of a noncardiac-related cause 14 years after the operation. No patient required late reoperation. Late SVC obstruction occurred in 3 patients (17%) from the traditional Warden group. The first 2 patients were 2-year-old girls who were diagnosed with SVC obstruction at 10 and 8 months postoperatively, and the third patient was a 47-year-old woman who was diagnosed 20 months postoperatively. All SVC obstructions were managed with percutaneous balloon angioplasty and stent placement, with no residual obstruction.
Comment Several surgical techniques have been described for repair of PAPVC. These include the 1-patch or 2-patch techniques and various other modifications. De Leon and colleagues [11] described a modification using the RAA as a flap across the cavoatrial junction. Because it was close to the sinus node, this was again modified into 2 separate incisions. The concern with any of these techniques is the risk of sinus node dysfunction, SVC, or pulmonary venous stenosis [12–14]. We have used the Warden procedure for high insertion of the APVs into the SVC. Higher insertion of the APV into the SVC with a resulting shorter SVC after division adds to the complexity of the repair. Gustafson and colleagues [15] reported only 1 patient with SVC obstruction and sick sinus syndrome postoperatively among a series of 40 patients who underwent
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the Warden procedure. In the 5 patients who underwent Warden procedure as reported by Stewart and colleagues [16], SVC obstruction developed in 1 patient 2 years after a Warden procedure, but they attributed that to a technical problem caused by failure of excision of all the RAA trabeculations, as Warden and colleagues [10] reported in their original article. In our series, one early (5%) and three late cases (17%) of SVC obstructions occurred in the traditional Warden group. The median age of our patients was 34 years, which seems to be older than the age in other reports. This may be due to referral patterns, but we did not notice an effect for age on the late development of SVC stenoses. In fact, 2 of the 3 patients with late SVC stenoses were children. Late SVC stenoses occurred despite adequate mobilization of the proximal portion of the SVC and the RAA to create a tension-free anastomosis as well as excision of all RAA trabeculations. These patients will require regular follow-up for early detection of SVC obstruction. No SVC obstructions occurred in our patients in the modified Warden group, suggesting this may represent a good alternative in adults with high PAPVC. No sinus node dysfunction was detected in any of the 11 patients in a series undergoing the Warden procedure reported by Gaynor and colleagues [17]. Stewart and colleagues [16] did not report sinus node dysfunction in any of the 5 patients in their series who underwent the Warden procedure. Caval division techniques are associated with a low incidence of sinus node dysfunction, and all the patients in our series but 1 remained in normal sinus rhythm at time of discharge and at late follow-up. This is consistent with other authors’ experiences with the 1-patch or 2-patch technique where the incisions are placed close to the sinus node or its arterial supply [16, 18]. The primary advantage of this procedure is that a right atrial incision extending across the cavoatrial junction or near the sinus node is not necessary. In the Gaynor and colleagues [17] series, only 1 patient had pulmonary venous obstruction. Shahriari and colleagues [19] reported 13 patients who underwent the Warden repair, with only 1 early pulmonary venous obstruction. In this series the authors initially used an untreated autologous pericardial patch and then switched to a Gore-Tex patch for diversion of the pulmonary venous return due to fear of patch contraction and PV obstruction. In our series, 41 patients underwent a caval division procedure, and none of them had any early or late pulmonary venous obstruction. We have not encountered any pulmonary venous obstruction due to patch contraction among any of our patients, regardless of the patch material that was used. This same conclusion regarding untreated autologous pericardium was reported by Stewart and colleagues [16]. In conclusion, caval division techniques for treatment of high PAPVC are effective and associated with low mortality and morbidity. The Warden procedure— or its modification— creates an unobstructed passage for the pulmonary veins, and the chance of sinus node dysfunction is very low. The modified Warden procedure may decrease the risk of SVC obstruction. Caval division
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without reimplantation of the right SVC appears to be a reasonable choice in patients with a well-developed bilateral SVC and a communicating vein.
References 1. Hijii T, Fukushige J, Hara T. Diagnosis and management of partial anomalous pulmonary venous connection. A review of 28 pediatric cases. Cardiology 1998;89:148 –51. 2. Gustafson RA, Warden HE, Murray GF, et al. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg 1989;98:861– 8. 3. Lewis FJ, Taufic M, Varco RL, et al. The surgical anatomy of atrial septal defects: experience with repair under direct vision. Ann Surg 1955;142:401–15. 4. Kirklin JW, Ellis FH Jr., Wood EH. Treatment of anomalous pulmonary venous connections in association with interatrial communications. Surgery 1956;39:389 –98. 5. Bahnson HT, Spencer FC, Neill CA. Surgical treatment of thirty-five cases of drainage of pulmonary veins to the right side of the heart. J Thorac Surg 1958;36:777–99. 6. Ehrenhaft JL, Theilen EO, Lawrence MS. The surgical treatment of partial and total anomalous pulmonary venous connections. Ann Surg 1958;148:249 –58. 7. 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. 8. Puig-Massana M, Murtra M, Revuelta JM. A new technique in the correction of partial anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1972;64:108 –13. 9. Lewin AN, Zavanella C, Subramanian S. Sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage: surgical repair. Ann Thorac Surg 1978;26:185– 8. 10. Warden HE, Gustafson RA, Tarnay TJ, et al. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1984;38:601–5. 11. 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. 12. Stewart S, Alexon 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. 13. Freiedli B, Guerin R, Davignon A, et al. Surgical treatment of partial anomalous pulmonary venous drainage: a long-term follow-up study. Circulation 1972;45:159 –70. 14. Trusler GA, Kazenelson G, Freedom RM, et al. Late results following repair of partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. J Thorac Cardiovasc Surg 1980;79:776 – 81. 15. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60(suppl):614 –7. 16. Stewart RD, Bailliard F, Kelle AM, et al. 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. 17. Gaynor JW, Burch M, Dollery C, et al. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;59:1471–5. 18. Nicholson IA, Chard RB, Nunn GR, et al. Transcaval repair of the sinus venosus syndrome. J Thorac Cardiovasc Surg 2000;119:741– 4. 19. Shahriari A, Rodefeld MD, Turrentine MW, et al. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg 2006;81:224 –30.
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DR DUKE CAMERON (Baltimore, MD): How often do you use this technique? How many of your partial vein cases require this as opposed to just a simple patch from the anomalous vein orifice to the sinus venosus atrial septal defect (ASD)? DR SAID: We have adopted this technique for all adult patients with high insertion of the anomalous veins at least 1 cm above the cavoatrial junction. We started using this modification in 2006. In our partial veins series we use this technique about 20% of the time. DR ROBERT JAQUISS (Durham, NC): Do you use any sort of anticoagulation when you put the piece of Gore-Tex in with the modified procedure? And have you gone to using the modified procedure for all of your Wardens, since you had a fairly significant incidence of superior vena cava (SVC) stenosis with an unmodified Warden? DR SAID: We use anticoagulation for 3 months with Coumadin (Bristol-Myers Squibb, Princeton, NJ) for all adults with an interposition graft. We have a low threshold to use this procedure in adult patients with high insertion of the anomalous pulmonary vein. We have noticed that even with extensive mobilization of the proximal SVC and the right atrial append-
age, there are still some cases with anastomotic tension that could predispose them to SVC obstruction. DR SHIVAPRAKASH SESHADRI (Detroit, MI): I work with Dr Walters from Children’s Hospital of Michigan in Detroit. We are in the process of writing a paper on this. We have 13 cases. Dr Walters modifies this technique in a different way. He performs the posterior hemicircumference of the cavoatrial anastomosis with running polydioxanone suture and augments the anterior aspect of the anastomosis with a diamond-shaped patch of fresh autologous pericardium, using a vertical counter-incision in the anterior aspect of the SVC and the right atrial appendage. We have done 13 patients, 6 of whom had bilateral SVC with an associated small right SVC. We have experienced no caval obstruction in any of the patients in our series. DR SAID: We are aware of your technique and thank you for sharing your experience. How long was your follow-up? Did you change any of your anastomotic technique? What type of sutures did you use? DR SESHADRI: No. We used in situ pericardium. Once we do posterior, we put a kind of diamond-shaped pericardial patch.
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DISCUSSION