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Eur J Cardiothorac Surg 1998;13:117-123
© 1998 Elsevier Science NL
a Service of Cardiothoracic Surgery, La Timone Childrens Hospital, Boulevard Jean Moulin, 13385 Marseilles Cedex 05, France
b Pediatric Cardiology Unit, Marseilles, France
c Pediatric Cardiology Unit, Montpellier, France
Received 30 September 1997; received in revised form 3 December 1997; accepted 9 December 1997.
Corresponding author. Tel.: +33 491386676; fax: +33 491478170.
| Abstract |
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Key Words: Anatomic correction Atrio-ventricular discordance Senning procedure
| Introduction |
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The purpose of this paper is to report our experience in 8 cases operated upon with this approach, including 3 patients under 6 months, and to discuss results and indications.
| Patients and methods |
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All the others patients had the various classic types of AVD with VA discordance (VAD), 2 patients having the situs inversus (I,D,D) type of segmental approach [9], the others the classic (S,L,L) type [9] of corrected transposition.
A total of 5 patients had undergone one or more previous palliative or curative procedure (patient 2 underwent VSD closure, banding release and tricuspid plasty at age 1 year, 2 years before double switch).
All patients underwent the anatomic correction between the ages of 3 months and 10 years (mean 40 months) under cardiopulmonary by-pass (CPB) with deep hypothermia and occasional periods of circulatory arrest (Table 3Table 4).
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The atrial switch was done by a Senning procedure in 7 cases. It was done under a period of hypothermic circulatory arrest in 4 patients and under hypothermic CPB in 3 patients. The hypothermic circulatory arrest was chosen in cases of low weight and expected difficulties of canulation of both cavae. In 2 of those cases, with situs inversus and levocardia (patients 1 and 6) we have carried out a left-sided Senning, the surgeon standing on the left of the patient. It was felt that due to this particular situation, there was a lack of right atrial tissue. Thus, the neo-pulmonary venous channel was done with an augmentation patch.
In 1 case (patient 4) with associated cor triatriatum, a Mustard procedure was used, since a Senning was deemed unfeasible.
The VSD were approached through a right ventriculotomy in 5 patients and the VSD closed (patients 1 and 2) or a LV to aorta rerouting carried out through this approach with a large dacron patch inserted with interrupted pledgeted sutures and/or continuous suture (patients 6, 7 and 8). In patients 3 and 5 the VSD was approached through the right atrium with the classical recommendations [11]. In patient 4, the ventricular septum was intact.
The mitral cleft was closed with simple interrupted mattress sutures and the attempt of tricuspid valvuloplasty done with a simple pledgeted commissural stitch.
The right ventricular outflow tract (RVOT) repair was done in 2 cases with a Rastelli-type RV-PA connection with a 19 mm diameter cryopreserved homograft (one aortic and one pulmonary). In both cases, in spite of trying to avoid crossing the midline with the conduit to avoid anterior compression, it was felt necessary to leave the chest opened and to carry out a delayed sternal closure. The other case allowed a simpler RVOT repair with a monocusp homograft patch.
| Results |
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Patient 1, with situs inversus, having undergone a left-sided Senning, presented with a severe late pulmonary venous pathway stenosis. He underwent a subsequent repair with a patch augmentation through a left thoracotomy under hypothermic circulatory arrest 1 year after initial operation. He is now 10 years after surgery in excellent condition.
All patients were carefully followed by cardiologists with EKG, Holter, echo-doppler and in 2 patients, by catheterization. The follow-up time is from 3 to 122 months, mean 45 months. The EKG showed a persistent and stable sinus rhythm in 6 patients, and junctional rhythm or occasional junctional tachycardia in 2 cases. These 2 patients were put under antiarrhythmic medication.
Of those 2 patients, 1 (patient 2) died suddenly 18 months after repair, 1 week after an extremely satisfactory evaluation of the cardiac function. A sudden arrhythmia was suspected. All other patients are in class 1, without medication (except the antiarrhythmic treatment in 1).
The last echo-doppler evaluation showed in all: (1) normal systemic LV function, (2) valvular anomalies (either AV or outlet), (3) normal RV function with systolic RV pressures estimated between 22 and 50 mmHg (mean 38 mmHg), and (4) the RV-PA homograft patch or conduit functioning properly. LV angiograms, when carried out, showed a normal function ( Fig. 1 ). Angiograms carried out in the right atrium and RV showed normally-functioning Senning construction, RV function, and Rastelli repair ( Fig. 2 ).
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| Discussion |
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In simple TGAs, the late results after atrial switch (either Mustard or Senning) also show a progressive incidence of tricuspid insufficiency and RV failure [5] [8].
These late problems of a systemic RV and tricuspid valve have fully justified the development of arterial switch for simple TGV and support the concept of anatomic correction for corrected transposition, with a potential best hope for longevity [5]. Since the first report of 2 cases undergoing a combined Mustard and Rastelli [3], more cases have been reported [4] [5] [8] and more recently the atrial switch and arterial switch has been done in the absence of PS or PA [6] [7]. Excellent reports have documented the satisfactory hemodynamics and normal LV function after mid-term follow-up.
The atrial switch
The Mustard has been reported as being more frequently used than the Senning procedure. The latter has been our preferred approach (7/8), the only case of Mustard being in a case with associated cor triatriatum where the Senning was not thought to be possible. However, in cases with situs inversus and levocardia or situs solitus with dextrocardia, the right atrial wall is smaller than usual and has led to augmentation patch of the pulmonary venous pathway. Patient 1 developed a severe retraction of xenopericardium used for this purpose and needed a reoperation 9 months after this left-sided Senning with autologous atrial patch plasty, and showed an excellent long-term (10 years) result. If an augmentation patch is necessary PTFE may be the best material.
We have experienced 2 patients with a late postoperative junctional rhythm or episodes of junctional tachycardia after the Senning; a much higher proportion than in our series of Senning in classic transposition. Due to the anterior location of the AV node, we hypothesized that the conductive tissue pathway may be different and carry a higher risk of junctional dysrhythmias but there is no clear answer to this (R. Anderson, personal communication, 1996). It must be mentioned, however, that spontaneous junctional rhythm has been reported to occur in AVD in 4/17 patients [20].
The VSD closure
The VSD closure or reconstruction of the LV to aorta pathway through a right ventriculotomy avoided any case of heart block in our experience, and, as reported in other papers, this approach has decreased considerably the occurrence of this complication and even avoided it completely
[8].
Arterial switch
No special technical comment has been done in the 7 cases reported in the literature
[6]
[7], but it has been done in general with the classic Lecompte maneuver. In 3 of our cases, due to the respective position of the vessels, it was chosen not to do this maneuver and the pulmonary bifurcation was left posterior, with no evidence of obstruction.
The coronaries buttons, as reported by others [6] [7], were implanted without problem as it was supposed by the knowledge of coronary distribution in AVD [21].
Mid-term and late ventricular function
As already reported
[6], the newly systemic anatomic left ventricle exhibits a normal contractility as shown by echocardiogram and angiogram in 2 patients in whom a postoperative cardiac catheterization was done. Together with the absence of complete AV blocks and the satisfactory RV function, these factors will probably result in a better long-term functional class and hopefully, survival.
The tricuspid insufficiency
The tricuspid insufficiency is a serious problem in AVD, and its repair has been reportedly done without great success
[19]. In 1 of our patients it was attempted together with a classical type of repair (patient 2), done 1 year before the double switch. It was unsuccessful, and in the postoperative period elevated PA and LV pressures persisted, allowing the double-switch procedure, since the LV was thus already prepared to sustain systemic conditions. Tricuspid insufficiency regressed postoperatively in all patients as already reported by others
[7]
[8] and is certainly an excellent indication for the anatomic repair.
Potential of the procedure
This relatively new approach appears therefore as potentially optimal in corrected transposition with RV systemic dysfunction and tricuspid insufficiency. Long-term results are still needed before it can be proposed as the best procedure for the vast majority of patients. In addition, in the cases associated with PS undergoing a conduit placement between the anatomic RV and the PAs, it must be stressed that a conduit replacement may be a difficult procedure according to the location of this conduit.
Are there currently recognized contraindications to this approach?
Some anatomic conditions may be a contraindication.
Is age of the patient a problem?
We think that in cases of VSD and PS or PA, a shunt should be carried out when needed and the anatomic correction deferred until a reasonable size Rastelli repair can be done, although some have carried out a REV-type correction without interposition of a conduit, and this could change the age of correction to a younger one
[5].
In cases with VSD and normal pulmonary outflow we think that a PA banding can be carried out if necessary shortly after birth, and complete correction deferred until 3 months of age. We have done this double-switch as a primary operation in 2 babies of 4 and 6 months, and we think it is feasible even earlier. As far as we know this procedure has not been reported at this age in the literature.
Even if long-term results are necessary to delineate more precisely the indications, it must be stressed than in all the 26 cases reported so far in the literature, and in our 8 cases, there was no complete heart block reported and the hospital mortality was much lower than in all the experiences of classical approach reported, so that the short and mid-term results are already better.
We therefore conclude that despite a more demanding procedure and limited recent experience, the double-switch procedure in AVD already achieves better early results than the classical approach. It is likely that it will provide better long-term results. There are few contraindications, and it can be carried out in infancy if necessary. We consider it as an improvement on the previously employed procedures.
| Footnotes |
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| Appendix A. Conference discussion |
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Dr D. Metras: At birth, you mean, with congenital heart block?
Dr Z. Al-Halees: With congenital heart block. The patient comes to you with impaired RV function (systemic ventricular dysfunction) and complete heart block with no intracardiac anomaly. What would you recommend for that patient? In other words, would you advise doing an anatomic correction on that child which of course will require prior preparation of the left ventricle by a pulmonary artery band?
Dr D. Metras: Well, I hadnt thought of that kind of case before preparing this paper, but I think its a difficult problem. If you have no intracardiac disease, the LV pressure should be low, also the anatomic LV. So I think its a bit of a difficult problem. If you want to transform it, you have to prepare the LV plus put a pacemaker in the patient. So I think it needs more reflection than a quick answer in this meeting, but Im not sure it should be incorporated into that kind of program.
Dr T. Ebels (Groningen, The Netherlands): Do you think there is a case for prophylactic pacemaker implantation in these children because of the natural tendency to develop AV block?
Dr D. Metras: Well, a quick answer is no because I guess that we can wait until a spontaneous block occurs and then there is the possibility of implanting an intravenous pacemaker. If you implant myocardial leads, one knows that you will have to one day reoperate on these patients anyway because of fibrosis around the electrode, and implanting a temporary pacing lead on the myocardium, on the epicardium is not a definitive solution, I would say.
Dr J. Stark (London, UK): I have just a very brief comment on the previous question. We recently had Professor Imai from Tokyo present a very large series, I think it was over 35 patients, with this procedure at our course in London. It seems that although in the natural history of corrected transposition late development of heart block has been described, Professor Imai didnt report any late complete heart block. With this procedure, one approaches the VSD through the RV, which is an advantage, as the conduction is on the morphological LV side of the septum. So I would be reluctant to implant a prophylactic pacemaker because of all the problems that are related to pacemakers in children.
Dr C. Brizard (Melbourne, Australia): In the reconstruction of the RVOT, have you ever done a reconstruction without a conduit, I mean direct PA to RV anastomosis?
Dr D. Metras: One of our cases had a simple RVOT reconstruction with a monocusp outflow patch. It was a kind of double-outlet RV. But we havent gone to this direct anastomosis between the RV and the PA. That has been reported, I think, by Imai in his series. I think its a very good approach.
| References |
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