Eur J Cardiothorac Surg 2001;19:283-289
© 2001 Elsevier Science NL
Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction
R. Prêtrea,
G. Gendrona,
D. Tamisiera,
F. Vernantb,
D. Sidib,
P. Vouhéa
a Service de Chirurgie Cardiaque, Hôpital Necker–Enfants Malades, 149 Rue de Sèvre, Paris, France
b Cardiologie, Hôpital Necker–Enfants Malades, 149 Rue de Sèvre, Paris, France
Received 10 October 2000;
received in revised form 30 December 2000;
accepted 10 January 2001.
Corresponding author. Tel.: +41-12-551111; fax: +41-12-554446
e-mail: rene.pretre{at}chi.usz.ch
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Abstract
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Objective: Assessment of the Lecompte procedure, our repair method of choice in malposition of the great arteries with pulmonary stenosis. Methods: A retrospective analysis of 42 patients (median age at operation, 1.4 years) operated on between 1986 and 1999 for various forms of great artery malposition, ventricular septal defect, and pulmonary stenosis. Relevant associated findings included the insertion of a tricuspid papillary muscle on the conal septum (nine patients), absence of conal septum (six patients), hypoplasia of a side pulmonary artery (four patients), and hypoplasia of the right ventricle (one patient). A preliminary systemico-pulmonary shunt was created in 28 patients and a cavo-pulmonary anastomosis in one patient. At operation, the conal septum (whenever present) was resected (36 patients), the pulmonary bifurcation was usually translocated over the ascending aorta (37 patients), and the main pulmonary artery was enlarged with a patch of pericardium. A monocusp valve was fashioned within the patch in 40 patients. The follow-up information was complete in 32 patients and ranged from 0.4 to 14 years (mean, 5.4±3.2 years). Results: The survival rate at 5 years was 92±5%. Three patients died post-operatively (mortality, 7%) and none during follow-up. The freedom from reoperation was 86±8 and 51±22% at 5 and 10 years, respectively. Six patients were reoperated, all for a pulmonary stenosis. Calcification of the monocusp patch was present in all. Pulmonary stenosis developed in three further patients: one underwent percutaneous dilatation and two are awaiting surgery. No procedural factors thought to have a potential correlation with the development of right ventricular outflow tract stenosis could be individualized on univariate analysis. Conclusions: The Lecompte procedure, which allows early repair of these defects, provides overall good results. The need to reoperate on the right or left ventricular outflow tract seems reduced in comparison with the Rastelli operation, the other alternative.
Key Words: Transposition of the great arteries • Pulmonary stenosis • Rastelli procedure • Lecompte procedure
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1. Introduction
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The Lecompte procedure, initially called ‘réparation à l'étage ventriculaire’ or REV procedure, was proposed for the repair of ventriculo-arterial connection anomalies associated with a ventricular septum defect and a pulmonary obstruction [1–4]. The presumed advantages over the Rastelli operation, the other alternative, were the possibility to perform the repair early, the avoidance of homograft material doomed to require reinterventions, and the reduced occurrence of subaortic obstruction (Table 1) [1–4]. However, it became clear that, although diminished, the need to reoperate an obstruction on the right ventricular outflow tract (RVOT) was not eliminated. Various modifications of the original description have been proposed to reduce the incidence of recurrent RVOT obstruction [5–7], the merit of which has not yet been fully established. Our more recent experience with an extended follow-up is presented to assess the mid-term results of the original Lecompte procedure.
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2. Materials and methods
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2.1. Patients
All patients who underwent a Lecompte procedure between 1986 and 1999 were reviewed. There were 42 children, who presented various forms of ventriculo-arterial connection anomalies, a perimembranous ventricular septal defect, and an obstruction of the pulmonary artery. Twenty-eight children underwent a preliminary systemico-pulmonary shunt and one child had a cavo-pulmonary anastomosis to improve lung perfusion and arterial oxygen saturation. This cavo-pulmonary anastomosis was chosen in the last child because of an associated underdeveloped right ventricle. Subsequently, a one-and-a-half ventricular repair was performed. The clinical characteristics of the children are summarized in Table 2. The age at operation is depicted in Fig. 1. The median age at operation was 1.3 years (range, 0.3–13 years) and the median weight was 9.0 kg (range, 4.3–36 kg).
2.2. Operative technique
The operation was performed according to the original technique described at Laënnec Hospital, with the exception that, since the end of 1991, an augmentation of the length of the pulmonary artery with a piece of ascending aorta was systematically performed. In this procedure, an intracardiac and an extracardiac part can be individualized. The major steps of the intracardiac part (Fig. 2) include the performance of an infundibulotomy high (i.e. cranial) on the right ventricle, the section of the main pulmonary artery at the level of the valve commissures, an extensive resection of the conal septum, the closure of the pulmonary stump, and the construction of a straight connection between the left ventricle and the aortic annulus. The major steps of the extracardiac part (Fig. 3) include an extensive dissection of the pulmonary arteries (beyond pericardial reflection) to obtain maximal elongation, the connection of the main pulmonary artery to the infundibulotomy, and the enlargement of the RVOT (infundibulum and main pulmonary artery) with a patch of pericardium.
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Fig. 2. Critical steps of the intracardiac part of the Lecompte procedure. (a) The infundibulotomy, the posterior malalignment of the interventricular septum, and the conal septum. (b) The angle of resection of the conal septum. The insertion of a Hegar dilator through the pulmonary stump helps spread the conal septum and protects the mitral valve. (c–d) The creation of a septal flap used to transfer supporting elements of the tricuspid valve.
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Fig. 3. Critical steps of the extracardiac part of the Lecompte procedure. The pulmonary bifurcation is translocated anterior to the ascending aorta, which has been shortened by resection of a ring. The ring of aorta is interposed between the infundibulotomy and the main pulmonary artery to compensate the loss of the pulmonary stump (which has been oversewn). A patch of pericardium with a fashioned cusp is used to enlarge the whole RVOT, from the infundibulum up to the pulmonary bifurcation.
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Resection of the conal septum is a crucial step of the Lecompte procedure to obtain a straight and non-restrictive connection between the left ventricle and the aorta. The safest way to perform the resection is by inserting a Hegar dilator in the transected pulmonary artery stump (Fig. 2). The dilator spreads the conal septum and pushed away posterior structures like the anterior leaflet of the mitral valve. The angle of resection, as depicted in Fig. 2b, should be carefully respected to avoid a posterior opening of the heart above or through the pulmonary artery stump. In case of insertion of a papillary muscle or numerous cordae tendinae of the tricuspid valve on the conal septum, as occurred in nine patients, a flap supporting these structures was created and reinserted on the intracardial tunnel (Fig. 2c–e) [2,8]. Further enlargement (after resection of the conal septum) of a previously restrictive ventricular septum was rarely needed. Anterior enlargement was deemed necessary and performed in four patients.
The pulmonary artery was translocated anterior to the ascending aorta (the so-called Lecompte maneuver) whenever the great vessels had an antero-posterior arrangement (37 patients), and left orthotopically whenever they had a side-by-side arrangement (five patients). To prevent posterior compression of the pulmonary bifurcation and tension on the side pulmonary arteries, a definitive risk in case of dilatation of the proximal aorta, the curvature of the proximal aorta was remodeled more posteriorly: the ascending aorta, aortic arch and origin of the arch arteries were dissected free from adventitial attachment to gain mobility, and a generous ring of ascending aorta was resected. The amount of aortic wall that could be resected depended on the length, diameter and elasticity of the ascending aorta. Longer rings could usually be obtained in younger patients because of the enhanced elastic properties of their aortic wall. After mobilization and cross-clamping of the aorta, the ascending aorta was transversally severed. The cross-clamp set at the base of the truncus brachio-cephalicus and a forceps grasping the aortic root were moved together to estimate the length of the resection. The ring of aortic wall (an asymmetrical ring) has, since 1991, been used as an interposition autograft between the infundibulotomy and the main pulmonary artery to make up for the length loss of the pulmonary artery after transection above the valve commissures (Fig. 3). The pulmonary artery was enlarged over its entire length with a patch of heterologous pericardium. A monocusp valve of autologous pericardium was often constructed within the heterologous pericardium prior to the institution of cardiopulmonary bypass. The autologous pericardium was stiffened with a 10 min bath in a 0.625% glutaraldehyde solution. An enlargement plasty of one or both side pulmonary arteries was performed with autologous pericardium (seven patients) or the aortic ring (one patient) in eight patients. Four patients had a stenosis on the implantation of the modified Blalock–Taussig shunt, three had a diffuse hypoplasia of both pulmonary arteries and one patient had a circumferential stenosis at the ductus arteriosus level.
2.3. Follow-up and statistical analysis
Most of the patients underwent regular clinical and echocardiographic controls at our clinic or by their referring physicians; these data were available. Unfortunately, because of political unrest in their country, ten patients could no longer be tracked after 1995. The follow-up was updated during the first trimester of 2000. The median follow-up was 4.5 years (range, 0.4–13 years) and the mean follow-up was 5.4±3.2 years. Reoperation after hospital discharge was performed in six patients, all because of progressive obstruction of the RVOT. One patient underwent dilatation of a side pulmonary artery 3 years after intervention and two patients are presently awaiting surgery. Only reoperated patients were considered as events in the calculation of cumulative freedom from reoperation. Time-related curves were calculated with the Kaplan–Meyer method. A univariate analysis of preoperative and operative factors suspected to play a role in the development of pulmonary stenosis was performed in the 29 initial survivors with complete follow-up. All patients who developed a significant right outflow tract obstruction (including those who are awaiting surgery) were considered in this analysis. The statistical analysis was performed with SPSS 8.0 for Windows software.
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3. Results
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The survival rate at 5 years was 92±5% (Fig. 3). Overall, three patients died, all during the post-operative period. The post-operative mortality was 7%. The causes of death were a patch endocarditis, a septic multiple organ failure and a sudden death. A transient low output syndrome requiring aggressive and prolonged pharmacological support occurred in three patients. Four patients were reoperated in the post-operative period, because of bleeding (three patients) or a cryptogenic peritonitis (one patient). One of these patients was reoperated again for a septic dehiscence of the intracardiac patch and died shortly thereafter of persistent sepsis. One patient who underwent resection of the conal septum and accessory tissues around the ventricular septum defect developed a complete atrio-ventricular block and received a pacemaker. Among the survivors, the median duration-times of intubation and stay in the intensive care unit were 2 (range, 0.2–7) and 4 days (range, 2–33), respectively.
The post-cardiopulmonary bypass pressure ratio between the right and left ventricles (measured during operation) was below 0.5 in 28 (66%) patients. During the post-operative period, the systolic pressure in the right ventricle was estimated by transthoracic echocardiography at a median value of 40 mmHg (range, 25–65 mmHg). Pulmonary regurgitation was described in 36 patients, and was reported as trivial in four, mild in 20, and moderate in 12. More than mild tricuspid valve insufficiency did not occur, including the nine patients who underwent reimplantation of a papillary muscle. On transthoracic echocardiography, two patients showed a subaortic acceleration of blood flow. The mean gradients, estimated by the modified Bernouilly's formula, were 13 and 10 mmHg. The gradient did not increase after 12 and 30 months, respectively.
During follow-up, nine patients developed significant obstruction of the RVOT. Six patients were reoperated on and underwent replacement of the autologous pericardium with a simple patch of heterologous pericardium (four patients) or a valved conduit (two patients). The latter two patients also underwent a patch enlargement of the origin of one pulmonary artery. In all, the area where the monocusp was inserted was heavily calcified. Freedom from reoperation is depicted in Fig. 4. Two further patients are awaiting surgery to relieve a RVOT obstruction and another patient underwent percutaneous dilatation of a side pulmonary artery.
Univariate analysis did not disclose a statistically significant influence of procedural factors in the development of pulmonary stenosis. The outcome of the most notable factors is depicted in Table 3.
At the last follow-up, all but two patients were in New York Heart Association functional class I or II. The last two patients, known for a significant RVOT stenosis, were in functional class III.
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4. Discussion
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4.1. Major findings and limitations of the study
This series confirms that the Lecompte procedure can be performed with low morbidity and mortality, even in the young age groups. It was initially expected that the avoidance of a homograft would result in a reduced need for subsequent reoperations to relieve obstruction of the RVOT, and would consequently confer this procedure an indisputable advantage over the Rastelli operation (Table 1) [3,4]. This series does not provide a straight answer to this assumption. Although the 86% freedom from reoperation at 5 years is better than the usual 50% reported after the Rastelli operation [9], important limitations of this series and major differences in patient populations make a direct comparison, in the least, hazardous. Few patients in our study were evaluated beyond 5 years after surgery (this accounts for the wide confidence intervals noted after 8 years) and only the reoperated patients were counted as events in the cumulative event curves. Dissimilarities in the population profiles between this series and those reporting the results of the Rastelli operation, especially regarding age at operation and intracardiac anatomic findings, also strongly undermine the value of any comparison.
No preoperative or operative variable could be individualized by univariate analysis as a contributing factor for the late development of pulmonary stenosis. Our samples were, however, considerably underpowered to detect anything other than a strong, readily evident influence. It remains possible that older age at repair, large diameter of the ascending aorta, side-by-side arrangement of the great arteries, anterior translocation of the pulmonary bifurcation or the use of a monocusp patch may bear a significant correlation with the development of late stenosis.
4.2. Ventricular outflow tract obstruction
The major objective in the repair of ventriculo-arterial connection anomalies is to obtain an anatomic connection with unrestricted outflow tracts. Attention in the literature has been focused on the development of a RVOT obstruction. The development of an obstruction on the left ventricular outflow tract, although a more uncommon event, might well turn out to be the most serious problem overall, because of the difficulties encountered in correcting the defect. An obstruction requiring reoperation occurred in 12% of the patients after 5 years in Kreutzer's review of the Boston experience [9]. In this series, the development of an obstruction was more frequent when the correction was performed at a young age. The absence of development of significant subaortic obstruction in our series is gratifying, particularly owing to the relatively young age of our patients at repair. Only two patients showed a mild gradient across the prosthetic part of the outflow tract that did not progress over time. Certainly, the resection of the conal septum and the creation of a relatively straight and short channel between the left ventricle and the aorta were crucial factors in avoiding this complication. Further resection of the antero-superior part of the septum, with the risk of insufficient resection when the septum is hypertrophied or of injury to the conducting tissue, was seldom necessary in our experience, while it is a common requirement in the Rastelli operation. The reason for this might also partly lie in the fact that the majority of our children were operated on at a relatively young age, before occurrence of a ventricular septum hypertrophy, which, in turn, further narrows the ventricular septal defect.
The principle of the Lecompte procedure is the creation of a repair similar to that of a tetralogy of Fallot. Pulmonary insufficiency is usually well tolerated in the absence of increased pulmonary resistance, and the growth of the native tissues is sufficient to allow a harmonious development of the RVOT. Our series shows that the need to relieve an obstruction of the RVOT, although reduced in comparison with the Rastelli operation, was not eliminated and seemed to be higher than after the repair of a Fallot tetralogy with a transannular patch [10,11]. The heterotopic position of the pulmonary bifurcation and the use of a pericardial monocusp patch are two factors that may lead to obstruction of the outflow tract. A series of technical considerations are important to avoid compression of the pulmonary bifurcation between the ascending aorta and the sternum. Shortening the ascending aorta (by resecting a piece of aorta) and dissection of the aortic arch (to remodel of the aortic curvature) position the ascending aorta posteriorly, away from the sternum. Dissection of the pulmonary arteries down to the pulmonary hilum increases their length and avoids excessive traction on the side pulmonary arteries when the pulmonary bifurcation is saddled on the ascending aorta. The piece of resected aorta has frequently been interposed between the infundibulotomy and the main pulmonary artery to augment its length. In view of these considerations, the decision to create palliative modified Blalock–Taussig shunts must be carefully evaluated when a Lecompte procedure is ultimately contemplated. The shunts may tremendously increase the blood flow in the ascending aorta, and consequently, induce an important dilatation. They stiffen and, in the worst case, even distort the pulmonary arteries. These modifications of the arterial wall reduce the length gain after dissection and may preclude a tension free translocation of the pulmonary bifurcation over the ascending aorta. Although most of our patients received a modified Blalock–Taussig or a central shunt, we were reluctant to create a second shunt on the other pulmonary artery. We also tended to proceed early with the repair in patients with a surgically created shunt before excessive dilatation of the ascending aorta had occurred.
The rationale for the insertion of a patch with a cusp, besides historical grounds, is derived from observations and logical assumptions. Although we favor a simple, non-valved patch to enlarge the RVOT in Fallot tetralogy and, more recently, have also used such a patch for the reconstruction of the RVOT in truncus arteriosus, we have stuck to the initial technique (in which a pericardial cusp was fashioned in the patch) in most of our patients who underwent a Lecompte procedure. The loss of the elastic capacitance of the intrapericardial pulmonary arteries after removal of the Blalock–Taussig shunts and possibly slight stretching of the side pulmonary arteries consecutive to the anterior translocation of the pulmonary bifurcation, and the frequently impaired diastolic function of the right ventricle have been the major arguments to purposely protect the right ventricle against an acute regurgitation in the first months. While a mild regurgitation was found in practically every patient, a massive regurgitation was not encountered, indicating a good initial function of the fashioned cusp. Gundry and co-workers had similar results immediately after operation in 19 patients with a surgically created monocusp patch. In this series, the cusp was still correctly retaining blood in only 14% of the patients after 2 years [12]. Surprisingly, these authors reported no case of calcification or of stenotic degeneration of the monocusp valve over time. Among our reoperated patients, the monocusp valve was found to be disintegrated and replaced by heavy calcifications. This finding, corroborated by others [13,14], raises suspicion that the fashioned cusp might be initiating the RVOT obstruction in some patients.
The development of a complete and long-lasting atrio-ventricular block has been associated with the resection of a part of a perimembranous ventricular septum defect in order to enlarge the communication between the left ventricle and the intracardiac baffle. The complication occurred in 6% of patients after a Rastelli operation in Kreutzer's report and in 4% (two out of 58 patients) of patients in the initial report of Lecompte [3,9]. The complication occurred in one of our patients, after resection of the conal septum and accessory tissue around the septal defect. One patient in Lecompte's series regained a normal sinus rhythm 1 year after operation and led this author postulate that the mechanism of injury to the conducting tissue was ischemic. If the ischemic origin of the block were to be confirmed, the resection of the conal septum would appear less damaging for the septal perfusion than the resection of the antero-superior part of the septal defect.
4.3. Modifications of the Lecompte procedure
Conservation of the orthotopic position of the pulmonary bifurcation and avoidance of a cusp in the enlarging patch are the most notable modifications that have been proposed [5–7]. Leaving the pulmonary bifurcation in an orthotopic position in cases of antero-posterior position of the great arteries may lead to narrowing of the origin of the right pulmonary artery which slings around a usually large ascending aorta. In order to reduce this potential complication, Metras and co-workers proposed to reduce the stretch of the right pulmonary artery by interposing the ring of ascending aorta between the infundibulotomy and the main pulmonary artery [6]. Interestingly, in this short series, the interposed circular aortic autograft did not produce an hourglass stenosis after significant somatic growth of the patients. Although clear evidence was lacking in their report, these authors speculated that a harmonious growth of the autograft occurred, similar to that seen with the pulmonary autograft after the Ross procedure [15].
The creation of a unidirectional connection between the right ventricle and the pulmonary arteries belongs to the initial description of the Lecompte procedure. Apart from a few cases where a strong indication for a valved connection exists (tricuspid valve insufficiency, reduced right ventricular function, or increased resistance in the pulmonary circulation), the majority of patients presented only relative indications as mentioned above. A few authors have successfully reconstructed the RVOT without a valve in various ventriculo-arterial anomalies [6,16,17]. These operations have been, for the most, performed during the first weeks or months of life, when the elastic properties of the pulmonary arteries are the most favorable, and before severe hypertrophy of the right ventricle has impaired its compliance. The initial results have been encouraging and are expected to parallel those of the early repair of a Fallot tetralogy [18,19]. It is tempting to think that the tissue properties and the adaptation capacities of the young heart (and pulmonary arteries) may result in an improved and long-lasting hemodynamic performance. More information regarding the safety of reconstructing the RVOT without a valvular component and the long-term consequences of this modification are needed.
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5. Conclusions
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The Lecompte procedure, which allows the early repair of defects characterized by malposition of the great arteries, ventricular septum defect and pulmonary stenosis, provides good results and compares favorably with the Rastelli operation, the other alternative. The incidence of left ventricular outflow tract obstruction is negligible in the short- and mid-term. The development of a RVOT obstruction is less frequent than after a Rastelli operation, but more frequent than after a repair of a Fallot tetralogy. Modifications of the Lecompte procedure (orthotopic position of the pulmonary bifurcation and enlargement of the RVOT with a valveless patch), although appealing, need further and longer evaluations.
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Footnotes
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Presented at the 14th Annual Meeting of the European Association for Cardio-thoracic Surgery, Frankfurt, Germany, October 7–11, 2000.
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Top
Abstract
1. Introduction
