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Eur J Cardiothorac Surg 1999;15:747-752
© 1999 Elsevier Science NL
Marie Lannelongue Hospital, 133, Avenue de la Resistance, 92350 Le Plessis-Robinson, France
Received 10 November 1998; received in revised form 22 February 1999; accepted 11 March 1999.
Corresponding author. Tel.: +33-1-40-94-28-00; fax: +33-1-40-94-55-81
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Abstract |
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 Top Abstract 1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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Key Words: Congenital • Heart septal defects • Ventricular • Double outlet right ventricle
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1. Introduction |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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1.1. Anatomic considerations
In this series, the VSD was defined as non-committed on the basis of both preoperative echocardiographic data and, above all, intraoperative surgical inspection. Non-committed is not an anatomic definition: it was used to define the location of the VSD in cases with DORV in which the VSD was distant from both arterial valves [2]. This subset includes DORV with an atrioventricular (AV) canal type, inlet (muscular) or trabecular VSD. Also, it exits DORV with conoventricular (perimembraneous) VSD distant to the great arteries because of the length of the subarterial conus. In those cases, echocardiography (subcostal view) shows the left ventricular outflow directed to the right ventricle instead of one of the great arteries. To avoid confusion, we suggest to define these defects as non-committed when the distance between the VSD and the arterial valves was at least equal to the matched aortic valve diameter of the particular patient (Fig. 1)
[7].
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2. Methods |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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The median age was 20 months (range: 50 days to 10 years) and the median weight was 8.5 kg (range: 3.5–24 kg). Nine patients (39%) had undergone previous palliation: seven patients had pulmonary artery banding, four associated with coarctation repair, and, two patients had modified Blalock–Taussig shunt. Twenty patients (87%) had at least one associated cardiac anomaly (Table 1).
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In two patients (9%), related to the great artery malposition (anterior aorta), it was easier to tunnelize the VSD to the pulmonary artery; the procedures were then completed by an arterial switch. The VSD was approached through right ventriculotomy in both, and both required infundibular muscle dissection.
During the study period, in two other patients, the biventricular repair was abandoned after surgical intracardiac exploration. Those patients constituted a small subgroup having a restrictive inlet VSD associated with tricuspid attachments crossing the subaortic pathway: one was converted to univentricular repair and the second patient, excluded from Fontan operation criteria because of high pulmonary vascular resistance, was left with his balanced pulmonary artery banding (Fig. 2) .
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3. Results |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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No late mortality occurred. Eight patients (35%, 70% CL: 23–48%) underwent nine reoperations. The cause was subaortic stenosis in six (26%, 70% CL: 16–39%). The median delay between the two operations was 53 months (range: 10–96 months). Three of them had >25 mmHg (maximum) left ventricle to aorta gradient immediately after the repair. In five, the previously described technique of extended septoplasty was successfully performed and in the remaining subaortic fibromuscular resection was enough to relieve the stenosis [10]. In addition, one patient required two reoperations for tricuspid valve repair and right ventricle outflow valvulation. One other patient who developed valve regurgitation underwent aortic valve replacement 6 years after the repair. No mortality occurred in reoperated patients. At last visit, three patients (16%, 70% CL: 7–29%) still require cardiac medication.
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4. Discussion |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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This study emphasises the importance of detailed definition of the intracardiac anatomy. Although in our recent series [11] the location of the VSD did not influence operative outcome, the classification of DORV according to the VSD site introduced by Lev and Bharati remains an essential surgical-anatomic distinction. In our experience, the VSD location was so relevant that it guided the surgical strategy, namely surgical technique and timing for definite repair. In the presence of two adequate, even slightly imbalanced ventricles, the following anatomic criteria helped to determine the surgical management: great artery relationship, the presence or absence of pulmonary stenosis and other major cardiac anomalies.
The term non-committed was first used by Lev et al. in 1972 [2]. In 1975, Zamora, Moller and Edwards employed the term ‘remote’ to define these defects [12]. Van Praagh et al. also used the term ‘uncommitted’ [7]. Both terms were used to describe the presence of a ‘considerable’ distance between the VSD and the outflow tracts supporting the aorta and the pulmonary trunk rather than being anatomical definitions. Thus, this subgroup of DORV included patients with AV canal type VSD, with trabecular VSD (inlet or not), but also patients with conoventricular VSD distant to the arterial valves (Fig. 1). Our data correspond to the series of previous publications on the morphologic features of this anomaly. Van Praagh et al. presented 32 postmortem cases of DORV with mainly conotrunkal malformations [7]. Eleven of them had non-committed VSD, including 10 conoventricular and one muscular VSD. Eighteen specimens in Stellin et al. included 12 perimembraneous VSD with inlet extension, three AV canal type VSD, two perimembraneous juxtapulmonary VSD (classified as surgically non-committed) and one inlet muscular VSD [5]. In this series, the incidence of the need for VSD enlargement and/or conal septal resection in order to construct an unobstructed left ventricle outflow was particularly high (57%). In those patients, the VSD was enlarged anteriorly or antero-caudally, and a large connection between the left ventricle and the baffle tunnel was obtained. Also, infundibular muscle resection, particularly in the presence of a well-developed parietal band creating a potential subaortic stenosis, was almost always required. In addition, with the two patients presenting with type C AV canal type VSD, in nine patients the atrioventricular valve straddling or the abnormal tricuspid valve chordae attachments on the outlet septum were observed. A variety of surgical techniques were performed [8]. In four patients, the abnormal tricuspid chordae were mobilized by tailoring myocardial flaps which secondarily were reimplanted at the edges of the baffle patch; no evidence of postoperative tricuspid valve regurgitation was observed in those patients. In this series, although there was a high rate of AV valve insertion anomalies, the overriding of the later was not observed [13,14]. At repair, the conventional anatomical landmarks of the conduction axis were considered. The sutures were placed rather at the right ventricular aspect of the VSD rim and on the leaflet tissue of the tricuspid valve, when necessary. However, two patients (9%) developed postoperative complete AV block and required permanent pace-maker implantation. Fig. 3 represents the intraoperative transventricular view of a heart with DORV and non-committed conoventricular VSD, before (A) and after (B) baffle construction.
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Although the Fontan operation has been proposed as a solution to the complex forms of this anomaly, it was always our policy to push the indications of biventricular repair as far as possible [16,17]. Since the functional state of patients following the Fontan operation deteriorates with time, we elected to primarily perform the biventricular repair even in the very complex forms [18]. It is an obvious fact that patients who underwent biventricular repair of DORV with non-committed VSD constitute a high risk group for need of reoperation for secondary subaortic stenosis. At the present time, one-quarter of the patients of this series has been reoperated on. We recently reported our experience on subaortic stenosis after biventricular repair for DORV [10]. The reoperation for subaortic stenosis should not only aim to relieve the obstruction, but also to streamline the left ventricle outflow pathway; ‘extended septoplasty’ constituted an adapted technique to treat this complication. We performed this operation in five of this series of patients, with excellent early results. The data allows us to conclude that a certain percentage of patients having DORV and non-committed VSD require a sort of two-stage management: the biventricular repair and the reoperation for the progressively developing subaortic stenosis relief which can be treated by a standardized technique with excellent results.
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Footnotes |
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Appendix A. Conference discussion |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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Dr Belli: Yes, when it is possible to wait. Thus, it is our policy to push the indications of biventricular repair as far as possible. In this series, one-fourth, maybe it will be one-third later, needed reoperation. This could be realized with good results and without mortality. We can conclude that a certain percentage of patients required a two-stage management: the first was the repair and the second was the treatment of the secondary subaortic stenosis. Thus, in our experience, this is preferable to an univentricular management.
Dr Van Son: Are you sure about that? In my experience and that of others, such as the Great Ormond Street group, there are situations in which the short- and long-term survival after a complex biventricular repair, such as for double-outlet right ventricle with a non-committed ventricular septal defect, has certainly been far less optimal than after univentricular palliation. Although the Fontan principle is clearly a palliative one, the incorporation of an often poorly functioning right ventricle with associated potential problems such as subaortic obstruction is also palliative in nature and the wisdom of this biventricular approach can be questioned. Why did you not preferably apply univentricular palliation in the patient cohort with high potential for subaortic obstruction?
Dr Belli: As recently reported, we believe also that the follow-up after Fontan repair in terms of mortality and quality of life deteriorates with time. On the other hand, in almost all patients who had developed or not, subaortic stenosis are growing well with no cardiac medication nor rhythm disturbance.
Dr K. Yamani (Mecca, Saudi Arabia): The question I wanted to ask, how can you avoid the conduction system, injuring the conduction system in this kind of operation?
Dr Belli: At operation or at reoperation?
Dr Yamani: At the operation.
Dr Belli: When the VSD is muscular inlet the risk of AV block is probably higher. But the other subsets of non-committed VSD don't represent a particular risk compared to the other locations of VSD in double-outlet right ventricle.
Dr Yamani: Did you entertain any AV block passed off in this kind of operation?
Dr Belli: In this series, we had only two patients who required pace-maker implantation; one of them died because of low cardiac output and pulmonary complications. The frequency was not so significant compared to the other patients who underwent DORV repair with VSD enlargement.
Dr J. Moll (Lodz, Poland): I found you had tricuspid insufficiency in those patients which had chordae transferred. And I did another technique. I did incision in the patch and put the chordae through the patch. And I found if very useful, with no tricuspid regurgitation. Of course, it's impossible to do it in all patients, but in this case it was very useful.
Dr Belli: We used the technique that you described. We also used other techniques to avoid chordae. In four patients there was no other solution than doing this particular technique. We observed then, at reoperation, two of them with tricuspid chordae in their initial place without any valve regurgitation. So, this allows us to present the technique.
Dr B. Maruszewski (Warsaw, Poland): In the previous sessions, we discussed the specifically good performance of patients with two ventricles after Fontan operations. I asked the question and haven't heard the answer, may I ask you again, what are the exclusion criteria, for these patients from biventricular repair? Which patients would you exclude from this operation with your experience and reoperation rate?
Dr. Belli: We had 25 patients who were on the operating table for biventricular repair. And I believe the presence of components, together, such as inlet restrictive VSD with the tricuspid chordae crossing the outflow, also with the necessity of doing subaortic resection of a parietal band, should be an indication, maybe, to abandon doing the biventricular repair because this becomes a high-risk procedure. However, the patient who had Fontan repair with chordae straddling or abnormal attachments, developed very soon AV valve incompetence, he had the mechanical valve and later, he required a transplantation. So it's a good example to insist to do biventricular repair for these cases.
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References |
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TopAbstract1. Introduction
2. Methods
3. Results
4. Discussion
Appendix A. Conference...References |
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