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Atrial Switch Operation in the Current Era: Modifications and Pitfalls Sachin Talwar, Vinitha Viswambharan Nair, Shiv Kumar Choudhary and Balram Airan World Journal for Pediatric and Congenital Heart Surgery 2012 3: 96 DOI: 10.1177/2150135111422239 The online version of this article can be found at: http://pch.sagepub.com/content/3/1/96
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Review Article
Atrial Switch Operation in the Current Era: Modifications and Pitfalls
World Journal for Pediatric and Congenital Heart Surgery 3(1) 96-103 ª The Author(s) 2012 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135111422239 http://pch.sagepub.com
Sachin Talwar, MCh1, Vinitha Viswambharan Nair, MS1, Shiv Kumar Choudhary, MCh1, and Balram Airan, MCh1
Abstract Although rarely performed today, atrial switch operations continue to have an important role in the management of some forms of congenital heart disease. In developing countries, delayed diagnosis and presentation of patients with transposition of the great arteries is not uncommon. For some patients who are referred for surgery beyond the newborn period, the atrial switch operations are still considered to be the best option. Also, as part of surgical repair of congenitally corrected transposition, an atrial switch operation is combined with arterial switch or the Rastelli procedure as an alternative to physiologic repair. In isolated ventricular inversion (atrioventricular discordance with ventriculoarterial concordance), the atrial switch operation alone leads to complete anatomical correction. Finally, management of late complications of atrial switch operations requires a thorough understanding of the procedures. Keywords congenital heart disease (CHD), congenital heart surgery, adult congenital heart disease, arterial switch operation Submitted May 22, 2011; Accepted August 3, 2011.
Introduction Although rarely performed today, atrial switch operation is not merely of historical interest as there remains a few important indications of this operation: (a) In developing countries, delayed diagnosis and presentation of transposition of great arteries (TGAs) is not uncommon and when patients are seen late in infancy and often in adulthood, the atrial switch operation is still considered a safer and economically viable operation. (b) As part of anatomic correction for congenitally corrected TGAs (ccTGAs), atrial switch operation is combined with arterial switch operation (ASO) or the Rastelli procedure as an alternative to the physiologic repair. (c) In an isolated ventricular inversion where the anomaly involves atrioventricular discordance with ventriculoarterial concordance, the atrial switch operation alone leads to complete anatomical correction. This forms an important indication of atrial switch operation even now.1,2 Therefore, a thorough understanding of the atrial switch procedure is still mandatory for cardiac surgeons dealing with complex congenital cardiac defects. It is also important to be familiar with the technical modifications that may be required in uncommon anatomical situations.
Historical Aspects The first baffle operation for TGAs was described by Lillehei and Varco3 in 1953. It was a partial physiological correction consisting of an anastomosis between the right pulmonary veins to the right atrium and inferior vena cava (IVC) to the left atrium.3 This was modified using an allograft to connect IVC to the left atrium
by Baffles4 in 1956. Thereafter, Ake Senning successfully performed the complete physiological repair or the atrial switch operation5,6 in 1959. He utilized autologous tissue for all the steps of reconstruction. Modifications were suggested by many including Schumacker7 in 1961 (using in situ pericardial technique), Bernard8 in 1962, and Mustard in 1964 (with free pericardial patch augmentation of venous baffles).9 Despite all modifications, a high incidence of baffle obstruction and arrhythmia following the initial atrial switch operation led to the development of ASO which was first performed successfully by Jatene10,11 in 1975 and later modified by Lecompte.12
Technical Considerations In the classic atrial switch operation, Senning utilized autologous atrial tissue for all the steps of venous reconstruction. Atrial septal flap and atrial wall were used to redirect the pulmonary venous return to the tricuspid valve and systemic venous drainage to the mitral valve .This rerouting is done in three layers. As the first step, pulmonary venous drainage is isolated from the mitral valve by suturing atrial septal flap to the left atrial wall anterior to the pulmonary veins and posterior to the mitral 1
Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi, India Corresponding Author: Sachin Talwar, Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi 110029, India Email:
[email protected]
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Acronyms and Abbreviations ASD ASO ccTGAs CPB IVC PTFE SA SVC
atrial septal defect arterial switch operation congenitally corrected transposition of great arteries cardiopulmonary bypass inferior vena cava polytetrafluoroethylene sinoatrial node superior vena cava
valve. This forms the posterior layer of the caval pathway to the mitral valve. The second layer which forms the roof of the systemic venous pathway is created by suturing right edge of right atriotomy incision to the left margin of the atrial septal defect (ASD). The third layer is fashioned in such a manner that the pulmonary venous return exits out of the heart from a generous left atriotomy and is directed toward the tricuspid valve by suturing the left edge of the right atriotomy incision around the left atriotomy incision. In situations where the autologous tissue is not sufficient to create unobstructed venous baffles, Mustard proposed his modification with the use of autologous pericardium. Autologous pericardium was harvested as free patches which were used to create baffles. Although this modification proved to be a good alternative to the classic Senning procedure in the short term, pericardium was seen to shrink during follow-up, and hence this could not be the answer to the problem of long-term baffle obstruction. This led to the revival of the Senning operation.13
Challenges Modern day cardiac surgeons will face many practical problems in performing the atrial switch operation in unfamiliar circumstances. The challenges usually faced are the following: a.
In patients who present beyond infancy and childhood due to unrestricted mixing of oxygenated and deoxygenated blood through a large ASD, it is often difficult to fashion flaps from the small residual interatrial septum to perform a classic Senning procedure. b. In patients with anomalies of situs such as situs inversus and in dextrocardia/mesocardia, the unusual placement of atria and pulmonary veins as well as anomalous position of systemic veins pose difficulties in exposure as well as creation of various baffles. c. Atrial isomerism has a high chance of being associated with other anomalies such as total anomalous pulmonary venous drainage which requires special modification in the procedure for rerouting of pulmonary venous drainage. d. In patients with juxtaposed atrial appendages where both the atrial appendages lie on the same side, deficiency of adequate right atrial volume on one side provides a challenge to the performance of the classic Senning procedure.
e. In cases with bilateral superior vena cava (SVC), atrial switch operation can become more complicated depending on whether the coronary sinus into which the left SVC often drains is unroofed or not. If the coronary sinus is unroofed, coronary sinus flap is usually not available for the venous reconstruction in right atrium, thereby necessitating a larger prosthetic patch, thus leading to redundancy and increased chance of obstruction of the systemic venous baffle.
Technical Modifications in Special Circumstances Atrial Switch Operation in Late Presenters In adult patients with TGAs who survive due to an unobstructed ASD, the interatrial septum may be practically absent and this precludes a classic Senning operation.14 Therefore, modifications are made in fashioning the first and third layers (Figure 1). It is advantageous to cannulate both the vena cavae directly with angled venous cannulae in their extrapericardial course so as to leave the pericardial reflection around the cavae intact. This greatly facilitates the use of in situ pericardial technique for reconstruction of the pulmonary venous baffle. After the right atrium is opened, a Dacron/polytetrafluoroethylene (PTFE)/pericardial patch is sutured to the left atrial wall anterior to the pulmonary veins and posterior to the opening of the left atrial appendage, below and behind the mitral valve with a continuous polypropylene suture. This patch forms the posterior wall of systemic venous pathway and serves to separate the pulmonary venous return from the mitral valve. The second layer is performed as usual by suturing the right edge of right atriotomy incision to the left margin of ASD. The third layer that directs the pulmonary venous return to the tricuspid valve is created using the in situ pedicled pericardial patch technique originally described by Schumacker.15 In this technique, the pericardium in the right edge is sutured to the left edge of right atriotomy incision. Thus, the pulmonary venous blood that exits from the left atrium finds its way to the tricuspid valve through the pericardial well. In essence, the atrial switch operation in an older patient is a combination of the Senning and Mustard operation with the addition of Schumaker modification. The use of in situ pedicled pericardium is probably associated with a reduced incidence of the pulmonary venous baffle obstruction in the long term.
Atrial Switch Operation in Dextrocardia In patients with dextrocardia and situs inversus where the anatomy is mirror image of the usual levocardia situation, the atrial switch operation is accomplished easily by the surgeon from the left side of the operating table, although imagination is required in dealing with such an unusual situation.16 In situations where there is dextrocardia with apicocaval juxtaposition which places both the atria much posteriorly, the right atrium can often be seen only after rotating the heart to the left. The superior and inferior pulmonary veins course posterior to
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Figure 1. (a) Setup for the conduct of the atrial switch. The extrapericardial portions of the superior vena cava (SVC) and the inferior vena cava (IVC) have been cannulated using angled venous cannulae. A to B denote the proposed incision for right atriotomy. P to P’ denote the pericardial reflection on the right side. LA indicates the site of the proposed left atriotomy from where the pulmonary venous return exits into the pericardial well. (b) View of the intracardiac anatomy after right atriotomy. The four pulmonary veins (PV) and the mitral valve (*) are seen through a large atrial septal defect. R and L indicate the right and left edge, respectively, of the atrial septal defect A to B and A’ to B’ indicate the left and right lips, respectively, of right atriotomy. (c) The Dacron patch (shaded and labeled as D) has been sutured in front of the pulmonary veins and behind the mitral valve to form the posterior wall of the systemic venous baffle. The CS has been cut back and directed toward the mitral valve. (d) The anterior wall of the systemic venous baffle is constructed by suturing A’ to B’ to the left edge of the atrial septum (L). The suture line is seen. This directs the SVC and the IVC return (arrows) toward the mitral valve. The opening in the LA has been enlarged. (e) Construction of the pulmonary venous pathway using the in situ pericardial technique. A running suture is used to approximate P to A and P’ to B. Ao indicates aorta; RV, right ventricle; CS, coronary sinus; **, tricuspid valve. Reproduced with permission from Talwar et al.14
the right atrium to drain to the left atrium and can only be visualized after performing this maneuver. After establishing cardiopulmonary bypass (CPB) as usual, a pledgeted 5/0 polypropylene suture is placed at the apex of heart and snared. The left pleural cavity is widely opened and with the snare, the heart is displaced totally into the left pleural cavity. This exposes the posteriorly placed right atrium and the right-sided pulmonary veins. A plane of dissection is developed between the right atrium and pulmonary veins into the interatrial groove by electrocautery dissection so that left atrium is exposed. After cross-clamping the aorta and delivering cardioplegia, the right atrium is opened and the anatomy assessed. Further performance of atrial switch
procedure involves three steps. The first layer can be created with the atrial septal flap or with the use of a patch as described above. Second layer requires augmentation with pericardial/prosthetic patch due to insufficient right atrial volume. The third layer is performed as in Schumaker modification with in situ pedicled pericardium.
Atrial Switch Operation in Bilateral SVC and Unroofed Coronary Sinus A left-sided SVC usually drains to the right atrium through the coronary sinus.16 But when the coronary sinus is unroofed, SVC return reaches the left atrium and causes mixing of
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Figure 2. (a) External cardiac anatomy shows dextrocardia with left isomerism and juxtaposed atrial appendages (arrows). The atria are not seen on the right side as they are covered completely by the ventricular mass. A stay suture (S) has been placed at the cardiac apex to facilitate displacement of the heart to the left. (b) Cannulation and landmarks. A to A’ indicates the site of proposed right atriotomy. LA indicates the site from where blood will exit out of the pulmonary veins. Heart has been dislocated into left pleural cavity using the stay suture (not shown). Note the extrapericardial cannulation of the cavae. The pericardial reflection, P and P’, over the SVC and IVC is left intact. (c) The right atrium has been opened and the large atrial septal defect can be seen along with the openings of the four pulmonary veins and a very small opening of the coronary sinus. X to X’ indicates the left lip of the right atriotomy and Y to Y’ indicates its right lip. (d) Construction of the posterior wall of the systemic venous baffle. A Dacron patch (D) is sutured in front of pulmonary veins and behind the mitral valve. On the right, the patch is sutured to R. The coronary sinus opening (arrow) has been cut back to open into the left atrium. The thatched area between the Dacron patch and the coronary sinus opening indicates the opened coronary sinus which now drains toward the mitral valve. (e) Completion of anterior wall of systemic venous pathway. A patch of bovine pericardium (B) is used to approximate X to Y and X’ to Y’ to complete systemic venous pathway. The coronary sinus (arrow) has been fully opened and directs LSVC return toward the mitral valve. (f) Construction of the pulmonary venous pathway using the in situ pericardial technique. P is approximated to X and P’ to X’. The suture line extends from the pericardium to the atriotomy edges along the SVC and IVC and bites in the cavae must be superficial to avoid narrowing. Also when suturing the pericardium near the SVC, the area around the SA node should be avoided. RSVC indicates right superior vena cava; LSVC, left superior vena cava; IVC, inferior vena cava; Ao, aorta; SA, sinoatrial; S, left edge of atrial septum; R, right edge of septum. Reproduced with permission from Talwar et al.16
systemic and pulmonary blood—this makes rerouting of systemic and pulmonary venous drainage separately difficult while performing an atrial switch procedure (Figure 2). In this situation, the left-sided SVC needs to be cannulated separately in the extrapericardial course. The coronary sinus may be dilated and its opening in the right atrium is either absent or stenosed. Right atriotomy is performed. The coronary
sinus is completely laid open toward the roof of left atrium and the left SVC can be seen draining to it. Then the left atrium is opened and the edges are completely excised up to the opening of the right-sided pulmonary veins so that there is an unobstructed drainage of pulmonary veins to the pericardial cavity. Venous redirection is done as follows: through the right atrium, a Dacron/PTFE/pericardial patch is sutured to the left atrial
100 wall anterior to the pulmonary veins to form the posterior layer of systemic venous pathway. The initial suture line is continued in an anticlockwise direction up to the right edge of the interatrial septum. With the other arm of the suture, the patch is sutured to the inferior edge of the coronary sinus and downward to meet the right edge of the interatrial septum. Thus, the pulmonary veins are isolated from the mitral valve and are beneath the patch, leaving the right SVC, IVC, and the coronary sinus to drain over the patch. Second layer can be constructed either in the usual manner or using a patch, if the right atrial wall is too small to provide an unobstructed venous pathway. The third layer is usually completed with an in situ pedicled pericardial patch technique described earlier. The technical difficulty in this context is that since the coronary sinus opening to right atrium is not available for reconstruction, an accurate cutback and appropriately sized patch is required for reconstruction which will increase the complexity of the baffle created.
Atrial Switch Operation in Bilateral SVC and Coronary Sinus to the Right Atrium In this subset of patients, the coronary sinus opening is larger and a simple cutback of the coronary sinus toward the left atrium creates a wide flap which can be used to fashion the posterior layer of the systemic venous baffle. If a patch is required, the size of this patch can often be limited (and actually should be small) as this reduces the probability of systemic venous obstruction.
Atrial Switch Operation in Juxtaposition of Atrial Appendages In these patients, both the atrial appendages are on the same side (left side) with the right appendage being larger than the left. The challenges faced here are the insufficient right atrial volume, relatively posterior position of the right atrium, difficulty in exposing the left atrium, and frequent association with dextrocardia/mesocardia. All these compromise the exposure and limit the amount of atrial tissue available to create the systemic and pulmonary venous baffles, thereby risking later obstruction. In contrast to the classic Senning procedure, autologous pericardium or a prosthetic patch is used to augment the right atrial volume and fashion the anterior layer of the systemic venous baffle. A Dacron/ PTFE/pericardial patch can serve to form the first layer. Due to very limited volume of the right atrium on the right side, pericardial (autologous or bovine) patch is sutured around the caval orifices and left edge of the interatrial septum to form the second layer. The third layer is formed using the in situ pericardium.
Atrial Switch Operation in Atrial Isomerism All heterotaxy syndromes are commonly associated with anomalies of systemic and pulmonary venous drainage. Left
World Journal for Pediatric and Congenital Heart Surgery 3(1) atrial isomerism is associated with anomalous systemic venous drainage particularly azygos extension of inferior vena cava with or without bilateral SVC. The hepatic veins may directly drain to the right atrium. Here particular attention should be paid that the cannulation should be direct caval and extrapericardial. A larger than usual cannula may be required for SVC if azygos extension of IVC is present. Special care should be taken to place the angled canula in SVC so as not to obstruct the opening of azygos/hemiazygos vein which bears the IVC return. Right atrial isomerism is known for its associated total anomalous pulmonary venous drainage. This should be born in mind while creating the intracardiac baffles.
Atrial Switch Operation in Total Anomalous Pulmonary Venous Drainage to Coronary Sinus or Right Atrium Here the surgical approach is technically easier since the coronary sinus17 and the tricuspid valve are at the same anatomic level. After opening the right atrium,18 interatrial septum is excised in full and an elliptical patch of pericardium is sutured to the coronary sinus wall and all around the tricuspid valve. This will in effect direct the whole of the pulmonary venous return to the tricuspid valve. The right atrium is usually enlarged with another pericardial patch so that the systemic venous return will find its way to the mitral valve through the ASD. In case all pulmonary veins are directly entering the right atrium, a modified Mustard procedure is performed by making a tunnel around the pulmonary venous orifices with a patch, thus isolating the caval orifices and the mitral valve. The right atrium is enlarged with a patch (pericardial/prosthetic; Figure 3).
Atrial Switch Operation in Supracardiac Total Anomalous Pulmonary Venous Drainage In this entity, a standard anastomosis between the pulmonary venous confluence and the left atrial appendage is fashioned if adequate tissue is present (Figure 4). If the latter is not the case and the pulmonary venous confluence does not appear large enough to carry the pulmonary venous return from both the lungs, pericardial enlargement can be used.19 The ASD must be large enough to allow unimpeded flow into the anatomic left atrium.
Atrial Switch Operation in ccTGAs Due to the frequent association of ccTGA with apicocaval juxtaposition, the atrial volume and the atrial tissue available are insufficient for performing a classic Senning procedure.20,21 The heart is displaced into the left pleural cavity for adequate atrial exposure and venous cannulation should be direct and extrapericardial. Pericardial/prosthetic patch is used to augment the first and second layers. The final layer is performed as in Schumaker in situ pericardial technique.
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Figure 3. (a) Intra-atrial anatomy, with all four pulmonary veins (pv) draining into the right atrium. (b) Tunnel rerouting of the caval blood to the mitral valve (modified Mustard operation). asd indicates atrial septal defect. Reproduced with permission from Amodeo et al.18
Atrial Switch Operation in ccTGAs With Pulmonary Stenosis or Atresia The main challenge here is the position of the ventricular mass being anterior to the atria, which will cause tension on the Senning reconstruction.22 Also due to the unfavorable ventricular position, extensive baffle supplementation of the pulmonary venous return is also avoided due to the higher possibility of kinking or folding of baffles. Hence classic Senning method is adopted for creating the roof and floor of systemic venous pathway. Final layer is constructed per Schumacker modification (in situ pericardial technique for routing of pulmonary venous return to tricuspid valve).
Practical Problems of Performing an Atrial Switch Operation In an atrial switch operation, the systemic venous baffle is wedged between the two portions of the pulmonary venous return which exits from the left atrium and is redirected toward the tricuspid valve. In essence, the pulmonary venous baffle encircles the systemic venous baffle in a C-shaped manner. Hence, the systemic venous baffle obstruction is likely to develop if the patch which is used to separate the pulmonary veins from the mitral valve (first layer) is redundant. As the left atrial pressure is higher than the right atrial pressure, the patch, if redundant, will bulge into the systemic venous baffle producing systemic venous obstruction. So the patch used should be always accurately sized and oversizing should be avoided. The sutures for the reconstruction of the third layer are often taken in the vicinity of the IVC and SVC. Care must be taken to take superficial bites in these areas as larger bites
can lead to partial obstruction of the SVC or IVC return. Hence, the needle bites should be placed only very superficially in this region. Arrhythmias—early and late—can result from sinus node dysfunction which may occur because the suture line to create the pulmonary venous baffle often runs in close proximity to the sinoatrial (SA) node. This can be avoided by ensuring superficial bites in this area and a suture line that is well away from the sinus node. This holds true only if Schumaker modification is used. The use of patches to augment the right atrial volume for construction of the second layer is controversial as there are concerns of patch shrinkage (particularly with the pericardial patch), calcification, and growth potential. However, the first two concerns are weighed against the risk of having immediate baffle obstruction due to reduced atrial volume. The growth potential may not be of particular concern because at least part of the circumference of the systemic venous baffle comprises autologous tissue which should preserve the growth potential. Pulmonary venous baffle obstruction can be due to any of the following reasons. a. Restriction at the site of exit of the pulmonary venous return from the left atrium. To prevent this, a generous left atriotomy is made and its edges are widely excised to include a portion of the right-sided pulmonary veins which also should be slit, filleted, and laid open. b. Shrinkage of pericardium when used as a free patch. This is largely avoided by the in situ pedicled pericardial patch technique using Schumaker modification. c. Insufficient height of the pericardial patch used to create the pulmonary venous baffle. To minimize this problem,
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Figure 4. (a) Initial incisions in right atrium and pulmonary venous confluence. The right atrial incision is modeled after that used in Senning procedure. The venous confluence was enlarged as well because of concerns about potential narrowing near the vertical vein. (b) Pulmonary and systemic venous return. The pulmonary venous confluence has been augmented with a patch of pericardium. The atrial septal defect has been enlarged to allow for unobstructed flow from the SVC and IVC. The arrows depict blood flow traversing to enter the mitral valve (MV). The suture line for this is illustrated by the dashed line. (c) Completed repair. Pulmonary venous blood has been diverted to flow into the right ventricle. At the completion of the repair, the VV was ligated. The arrows depict pulmonary blood flow being redirected over the previously constructed baffle and into the tricuspid valve (TV). LUPV indicates left upper pulmonary vein; LLPV, left lower pulmonary vein; VV, vertical vein; RA, right atrium; SVC, superior vena cava; IVC, inferior vena cava. Reproduced with permission from Raff et al.19
the height at which the suturing of the lip of the right atrium to the pericardium is performed should be sufficiently high and preferably nearer to the cut edge of the pericardium on the right side. The obstruction of SVC and IVC is also an important concern following the atrial switch operation. Obstruction of the SVC is more common than IVC obstruction and occurs more frequently following the Mustard operation. In the Mustard operation, creation of a virtual common atrium is performed and it
is important to resect the atrial septum completely along with the ridge of tissue between the SVC entrance and the superior aspect of the atrial septum to prevent caval obstruction.23,24 The bidirectional cavopulmonary anastomosis has been used late in the follow-up to manage SVC obstruction following the atrial switch operation.25 Whether this should be performed prophylactically by routing the IVC to the mitral valve along with the bidirectional cavopulmonary anastomosis and pulmonary venous rerouting toward the tricuspid valve as an initial palliation is a matter of speculation.
Talwar et al Through this review, our aim is to focus on the technical modifications required when an unfavorable anatomy is encountered during atrial switch procedure. If these simple steps and precautions are followed, the early and mid-term outcomes of the atrial switch operation can be expected to be favorable with minimum complications. An obvious limitation of this review is the lack of discussion of results of these techniques. However, it must be realized that there are anecdotal case reports of surgery in such situations and no large series are available to date. As more such patients are operated and more follow-up is obtained, we can look forward to detailed results. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
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