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Eur J Cardiothorac Surg 2003;23:525-531
© 2003 Elsevier Science NL
a Department of Cardiovascular Surgery, Hôpital Européen Georges Pompidou, 20, rue Leblanc, 75015 Paris, France
b Department of Anesthesiology, Hôpital Européen Georges Pompidou, 20, rue Leblanc, 75015 Paris, France
c Department of Radiology, Hôpital Européen Georges Pompidou, 20, rue Leblanc, 75015 Paris, France
Received 5 October 2002; received in revised form 27 November 2002; accepted 2 December 2002.
* Corresponding author. Tel.: +33-1-56-09-36-40; fax: +33-1-56-09-22-19
e-mail: sylvain.chauvaud{at}egp.ap-hop-paris.fr
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Abstract |
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 Top Abstract 1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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Key Words: Ebstein's anomaly • Tricuspid valve
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1. Introduction |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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2. Material and methods |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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Tricuspid valve insufficiency (TVI) was assessed by echocardiography and/or angiocardiography in our early experience. Anterior leaflet motion was studied (Fig. 1 ) with echocardiography (172 pts), computerized tomography (20 pts), and magnetic resonance imaging (four pts). TVI was graded 1–4+. It was 1+ in 2% (three pts), 2+ in 12% (23 pts), 3+ in 48% (93 pts) and 4+ in 38% (72 pts). Tricuspid valve stenosis was present in 31 pts (16%).
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Associated anomalies are listed in Table 1: the most frequent was the presence of an atrial septal defect (ASD). Twelve pts (6%) had a previous operation (Table 2). Indications for surgery were functional disability (60%), cyanosis (38%), and rhythm disturbances not improved by medical treatment (45%).
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Mobilization of the anterior leaflet was obtained by detachment of the leaflet from the annulus, from the antero-septal commissure to the junction with the posterior leaflet when present. An overview of the right ventricular chamber was thus obtained. All abnormal muscular adhesions from the leaflet tissue to the right ventricular wall were dissected with scissors as far as possible in the right ventricle (Fig. 2 ). A lateral papillary muscle was individualized when possible.
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Associated valvular techniques were fenestration of the anterior leaflet (four pts), anterior leaflet enlargement (four pts) with a glutaraldehyde autologous pericardial patch. The infundibulum of the RV was plicated externally from the pulmonary annulus to the apex in four pts. The atrial septal defect was always closed except in two pts. Mapping and cryofulguration of the accessory pathways were performed in two pts. Ventricular septal defect (two pts), mitral valve insufficiency (two pts), pulmonary stenosis (one pts) and subaortic stenosis (one pts) were treated during the same operation.
Four patients underwent tricuspid valve replacement by a bioprosthesis. Indications for replacement were: leaflet tissue totally adherent to the ventricular wall (Type D): three cases, partial AV defect with hypoplasia of leaflet tissue: one case.
In all but three pts, the aorta was cross clamped at 28°C. Blood or cold cristalloïd cardioplegia was injected in the aortic root and repeated if necessary every 30 min. Slush ice was placed on the right and left ventricles. Three patients with poor left and right ventricular function were operated on without aortic cross clamping on a beating heart.
A morphological study by electron beam computerized tomography (Imatron C100 and Siemens Evolution) with EKG triggered, 3 mm contiguous axial slices, after contrast injection was performed on 20 pts. The cine study was performed at 12 levels, with 12–20 views per level in short axis view. Measurements of right and left ventricular volumes were performed independently by two examiners, before and after surgery.
Statistical analysis: hospital mortality was defined as death during surgery or within 30 days of surgery. The Kaplan–Meier method was used to calculate the actuarial survival and the probability of remaining free from reoperation. Confidence limits (95%) were calculated. Continuous variables were evaluated with Student's tests, values of P less than 0.05 were considered to be statistically significant. The association of qualitative risk factors with perioperative death was evaluated with 
2 test.
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3. Results |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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The causes of hospital deaths are listed in Table 3. The most frequent cause of operative death was right ventricular failure which associated low cardiac output with no improvement following volume replacement and inotropic support. Systemic acidosis and anuria appeared very early after surgery: 6–8 h. On echocardiography, the left ventricle appeared small, empty and compressed by the RV. The right ventricle was very dilated and hypokinetic.
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Non-lethal perioperative complications were AV dissociation with pace maker implantation (five pts), pericardial effusion (seven pts), thrombosis of the bioprosthesis (one pt).
Information concerning clinical status were obtained by examining patients and reviewing charts during surgical consultation in our own center or through the referring physician. Three patients were lost to follow-up. Mean follow-up was 6.37±5.1 years (y) (0.01–22).
Seven patients died at a mean delay of 2.8±0.7 y (0.36–13). Three patients died of sudden death at home of unknown cause follow a delay of 0.73, 0.36 and 2.2 y. These patients were all doing well up to the time of death. Post mortem examinations were not performed. These sudden deaths were attributed to rhythm disturbances. Other causes of death were ventricular fibrillation (one pt), seizure (one pt), reoperation for tricuspid valve insufficiency and RV failure (one pt) and late deterioration of ventricular contractility (one pt).
The actuarial (Kaplan–Meier) survival rate was 86.6±2.6% at 10 y and 82.2±4.7% at 20 y (Fig. 3 ).
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Sixteen patients were reoperated, followed by a mean delay of 2.6 years (1 day–9.4 y) (Table 4). The reoperation was performed for residual tricuspid insufficiency in 14 pts, stenosis in one pt, and bioprosthesis thrombosis in one pt. Conservative surgery was performed in 12 pts using various associated techniques: extensive mobilization of the anterior leaflet in six pts, plication of the annulus in four pts, papillary muscle transfer in one pt, patch extension of the anterior leaflet in three pts, and a prosthetic ring was implanted in six pts. A BPCS was performed in three pts associated with valvular surgery in two pts and isolated in one pt (the indication being TV stenosis). Two patients underwent valve replacement and one pt underwent a cardiac transplantation. Four patients died following reoperation. The actuarial rate of freedom from reoperation, calculated on the surviving patients, was 88.6±4 % at 10 and 20 y (Fig. 3).
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The study of ventricular volumes demonstrated decreased contractility of the atrialized chamber with a mean ejection fraction of 37±28%. In two of the 20 patients studied, the atrialized chamber was dyskinetic. Ejection fraction of the effective right ventricle was decreased after surgery from 56±10% to 41±12% (P<0.01). Ejection fraction of the left ventricle was increased after surgery from 56±10% to 68±8% (P<0.01).
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4. Discussion |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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The classical conservative surgical technique was elaborated by Hunter [11], Lillhei [12] and Hardy [13] with plication of the atrialized chamber. This technique was refined by Danielson with the association of tricuspid annulus plication [1]. However, in a large series of 324 pts [5], half had undergone tricuspid valve replacement. This could be due to the inability to restore normal leaflet coaptation with the plication of the atrialized chamber as an isolated technique. Similar techniques also report a high rate of valve replacement [14].
Simple conservative surgical techniques were published with good mid-term results in short series [2,15,16]. However, in these reports, patients were not consecutive and failures of the technique were not included in the results.
The goal of reconstructive valvular surgery is to restore a normal surface of coaptation of the leaflet tissue. In Ebstein's anomaly the anterior leaflet is the only effective leaflet capable of reaching the ventricular septum. Subsequently, the surface of coaptation is between the anterior leaflet and the ventricular septum.
Techniques of valve enlargement were developed [17] with some success, and unknown long term results. Repair of the septal leaflet is technically demanding [18].
Mobilization of the anterior leaflet is based on the concept of a restricted anterior leaflet. This functional analysis was assessed by echocardiography. With extensive mobilization, we were able to perform conservative surgery in 98% of patients. Similar results were obtained by others centers [19]. In ten pts of our series, reoperated on for residual tricuspid insufficiency, it was found that mobilization had been minimal and could be improved with success. These patients of our early experience were initially interpreted as technical failures, retrospectively confirming the validity of the concept. Mobilization of the anterior leaflet has been extended to infants when the lesions were complex [20].
The surgical limit of valve repair appears to be Carpentier Type D disease, in which the individualization of the anterior leaflet from the RV wall is not complete. Valvular replacement in such cases appears prudent and effective [5].
Ebstein's anomaly is also a right ventricular disease [21] with most of the operative mortality being due to underestimation of right ventricular contractility. With the introduction of BPCS as an associated technique, operative mortality was reduced and the post-operative course simplified [22]. Decreasing right ventricle preload is an important part of the management of depressed RV contractility [23,24]. Indications for associated BPCS are not clear and are based on clinical data. At the present time we associate BPCS when the right ventricular wall is thin and dilated, when there is a long history of tricuspid valve insufficiency and when the anterior leaflet is widely attached to the ventricular wall. However, isolated BPCS can not resolve the consequences of massive tricuspid insufficiency, as was the case in two of our patients.
The atrialized chamber participates in the evolution of the right ventricular impairment. In some patients, we found that contractility was decreased and an anevrysm could be present with dyskinetic motion. Exclusion of the atrialized chamber can be obtained by resection [25] or plication. Longitudinal plication associates exclusion of the atrialized right ventricle and plication of the tricuspid annulus to normal size. In such cases no deleterious effect could be demonstrated.
Assessment of RV functions (diastolic and systolic) is still difficult irrespective of the method used. Echocardiography could not explore all the segments of a deformed right ventricle. Computed tomography was useful but the duration of the investigation (10–15 min) is long for children. Magnetic resonance imaging is promising and under evaluation.
Reconstructive surgery for severe Ebstein's anomaly requires the association of several surgical techniques corresponding to each individual anatomic and functional aspect. Mobilization of the anterior leaflet is one of them, participating to the increased rate of successful valve reconstruction.
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Footnotes |
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Appendix A. Conference discussion |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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The question is, do you use this addition in all the patients now or do you select the patients, and in this case, what are the criteria to use or not to use the bidirectional cavopulmonary shunt?
Dr Chauvaud: The bidirectional cavopulmonary shunt is not performed in all patients. Selection is made on preoperative echocardiography when there is huge dilatation of the infundibulum or an akinetic ventricular wall. It means that we need investigation of the right ventricular function. During the operation, when we start on bypass, if the right ventricular wall appears very thin, it means that the post operative course will be very difficult and that decreasing the preload of the right ventricle will be mandatory. So indications of BPCS are a combination of several parameters.
Dr C. Mavroudis (Chicago, IL, USA): I'm not sure that we have very many good questions to ask you, because you are, obviously, the last word on this. But let me ask you, what did you do in those 15% or 20% of patients that had some kind of an arrhythmia, either accessory connections or reentry tachycardia? I know you didn't say it and maybe you didn't have time to, but do you approach that problem at the same time, and what kind of energy dissipation do you use?
Dr Chauvaud: We are effective on Wolff-Parkinson-White syndrome, supraventricular tachycardia, and we are totally ineffective on atrial fibrillation. By using this technique, with detachment of the anterior leaflet from the annulus and using longitudinal plication of the right ventricle, we cut most of the accessory pathways which are located on the right side of the heart behind the coronary sinus. At the present time I associate a limited maze procedure of the right atrium, but I don't have the result of this associated technique.
Dr D. Metras (Marseille, France): At the last meeting in Toronto last year on pediatric cardiac surgery and cardiology we heard an enormous series presented by Dr. Danielson from the Mayo Clinic, and he showed in the last series the technique they do that does not include any plication of the atrialized portion of the ventricle, arguing that this plication may interfere with the coronary circulation, do some stenosis or kinking, and may make problems on the right ventricular function. I see that you plicate this chamber, of course, each time. What do you think of this large experience presented by the Mayo Clinic?
Dr Chauvaud: I totally disagree.
Dr Metras: Not with me. I’m not a representative of the Mayo Clinic. I’m just asking a question.
Dr Chauvaud: Indeed there is a risk for coronary circulation with the plication of the atrialized chamber and the tricuspid annulus reduction. I don't want to enter into the details, but insist on the fact that the suture is very superficial, only in the endothelium. We performed isotope investigations of the myocardial vascularization, before and after surgery, and could see that there is no deleterious effect of the plication on the right ventricular function.
Dr. C Schreiber (Munich, Germany): I would like to stress the same aspect as my colleague. We were looking at specimen a few years ago with Professor Anderson and found that it is not always necessary to restore the size of the right ventricular cavity; the right ventricle is often big enough, and we in the German Heart Center Munich have repaired almost 100 hearts now with a much simpler technique, just using the anterior leaflet and creating a monocusp valve, with good results. We do have though a slightly higher incidence of reoperations, but the overall outcome is comparable to your series. We furthermore believe that if you cannot get the valve competent, we would rather go for valve replacement straight away, because we also know from Danielson's group that long-term results after valve replacement are quite favourable.
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References |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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