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J Thorac Cardiovasc Surg 2007;133:215-223
© 2007 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease |
a Heart Institute, University of São Paulo Medical School, São Paulo, Brazil
b Cardiovascular Surgery Division, Beneficencia Portuguesa Hospital, São Paulo, Brazil.
Read at the Eighty-sixth Annual Meeting of The American Association for Thoracic Surgery, Philadelphia, Pa, April 29-May 3, 2006.
Received for publication April 28, 2006; revisions received August 6, 2006; accepted for publication September 6, 2006. * Address for reprints: Jose Pedro da Silva, MD, Alameda dos Arapanes 631, ap 101, bl 3, Indianápolis, São Paulo, SP 04524-001, Brazil. (Email: dasilvajp{at}uol.com.br).
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Abstract |
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METHODS: From November 1993 through August 2005, 40 consecutive patients with Ebstein’s anomaly (mean age, 16.8 ± 12.3 years) underwent a new surgical repair modified from Carpentier’s procedure, the principal details of which are as follows. The anterior and posterior tricuspid valve leaflets are mobilized from their anomalous attachments in the right ventricle, and the free edge of this complex is rotated clockwise to be sutured to the septal border of the anterior leaflet, thus creating a cone the vertex of which remains fixed at the right ventricular apex and the base of which is sutured to the true tricuspid valve annulus level. Additionally, the septal leaflet is incorporated into the cone wall whenever possible, and the atrial septal defect is closed in a valved fashion.
RESULTS: There was 1 (2.5%) hospital death and 1 late death. Early postoperative echocardiograms have shown good right ventricular morphology and reduction in tricuspid regurgitation grade from 3.6 ± 0.5 to 1.2 ± 0.5 (P < .0001). After mean follow-up of 4 years, the functional class (New York Heart Association) improved from 2.6 ± 0.7 to 1.2 ± 0.4 (P < .0001). Two patients required late tricuspid valve re-repair, and there was neither atrioventricular block nor tricuspid valve replacement at any time.
CONCLUSIONS: This surgical technique for Ebstein’s anomaly can be performed with low mortality and morbidity. Early echocardiograms showed significant reduction of tricuspid insufficiency, and the follow-up showed improvement in patients’ clinical status and low incidence of reoperation.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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Ebstein’s anomaly of the tricuspid valve (TV) constitutes approximately 1% of congenital heart defects and has presented an ongoing challenge since the initial repair attempts in 1958.1,2 Danielson and associates’ approach3 has accumulated a large experience in repair of the TV at the level at which it exists in the right ventricle (RV). However, the goal of eliminating tricuspid regurgitation has limited the procedure to a certain subset of anatomic variations, and TV replacement (TVR) has been necessary in 36% to 65% of cases.3-5
Carpentier and colleagues6 carefully described and reported a new technique in 1988. In contrast to the transverse plication of the atrialized chamber described by Danielson and associates,3 they plicated the RV longitudinally and also returned the TV to the anatomically correct level, thus obtaining good right ventricular morphology. The tricuspid annulus was remodeled and reinforced with a prosthetic ring. Carpentier was able to apply his procedure to almost all anatomic presentations of the disease, but hospital mortality was high (14%) in his initial series, and late complications were also important. Quaegebeur and coworkers7 performed a slightly modified operation without the use of a prosthetic ring. They reported no early deaths but still observed a high incidence of moderate and severe TV regurgitation. Beginning in 1989, our group developed and subsequently began to routinely perform a new surgical technique, termed cone reconstruction.8 It uses some principles of the Carpentier technique but reconstructs the TV in a markedly different manner: the cone-shaped valve opens to a central blood flow and closes with full coaptation of leaflets. The objective of this study is to relate in detail the technical aspects of this operation, as well as to assess the early echocardiographic results and the early and late clinical outcomes.
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Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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Then the septal edge of the anterior leaflet is carefully mobilized by freeing its connections to the interventricular septum. Therefore only the normal attatchment of the anterior leaflet to the true tricuspid annulus and the proper subvalvular apparatus are left in place (Figure 1, B). Then the free edge of the posterior leaflet is rotated clockwise and sutured to the anterior leaflet septal edge, forming a new TV resembling a cone (Figure 1, C).
An important technical consideration is management of the septal leaflet. This leaflet is hypoplastic and displaced downward in Ebstein’s anomaly. However, in 22 patients it was sufficiently developed to take part in the newly constructed TV. Therefore it was extensively mobilized by taking down its proximal edge and releasing the adhesions from the ventricular septum. When it is too short to reach the true tricuspid annulus, longitudinal elongation is done by plicating its proximal edge toward the center.
On completion of these preparations, the anterior edge of the septal leaflet is sutured to the septal edge of the anterior leaflet (Figure 2, A). Anchoring the free edge of the posterior leaflet to the other side creates a wider cone (Figure 2, B). In some cases, a short septal leaflet can be completed with the posterior leaflet tissues, which, on rotation, will form its proximal portion. In a few cases, the septal leaflet is represented by only a fibrous tissue ridge that goes from the membranous septum to the right ventricular apex. It still can be useful as an attachment for the posterior TV leaflet. After this, the atrialized RV is longitudinally plicated to exclude its thin part (used in about 80% of cases). Endocardial placement of this suture avoids damage to the coronary arteries.
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Finally, regarding the issue of atrial septal defect (ASD) closure, the capacity to shunt right to left must be preserved postoperatively. The foramen ovale is thus closed in a valved fashion, usually with a single stitch (Figure 1, C and D), but the fossa ovale must be opened along its superior oblique margin if the atrial septum is found to be intact during the operation. An ostium secundum ASD, if present, is closed with a valved patch technique.
Additional surgical procedures were performed to repair the associated heart anomalies: 1 ventricular septal defect closure, 1 mitral valve repair, 3 enlargements of the right ventricular outflow tract with a monocuspid bovine pericardial graft, and 9 cases of surgical section of accessory conduction pathways.
Echocardiography
The echocardiograms of all patients obtained preoperatively and at hospital discharge were reviewed. The anteroposterior diameter of the true tricuspid annulus, defined as the junction between the right atrium and the RV, was measured in a 4-chamber view. The TV performance was revised for stenosis, insufficiency, or both, with the insufficiency grade classified as 1 to 4 according to the method of Suzuki and coworkers.9
Follow-up Clinical Assessment
All patients were followed over a period of 3 to 143 months (mean, 49 months). The recent clinical condition of all patients was obtained by means of outpatient evaluation or telephone interview. The heart failure functional class, assessed by using the NYHA scale, and important medical events were recorded.
Statistics
The numeric data are expressed as means and standard deviations. Statistical analysis was performed with the Graphpad Prism software (version 4.0; Graphpad Software, Inc, San Diego, Calif). Preoperative and postoperative changes in functional class and grades of tricuspid insufficiency were analyzed by using the Wilcoxon signed-rank test, and the change in TV annulus size was analyzed by using the Student paired t test.
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Results |
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The single hospital death (2.5%) in this series occurred as a result of biventricular hypoxic cardiomyopathy. Although the TV repair was effective, low cardiac output was the cause of death on the fourth postoperative day.
The preoperative and early postoperative echocardiographic results, as well as the preoperative and recent functional class, are shown in Table 2. There was a reduction in tricuspid annulus diameter postoperatively because of surgical plication, which did not result in definitive TV stenosis. The 2 patients who presented with early transvalvular peak gradients of 8.0 and 11.7 mm Hg had these gradients decreased to 5.0 and 7.5 mm Hg, respectively, on late echocardiographic studies. The echocardiogram of patient 32 (Figure 3), a 4-year-old girl, besides indicating good positioning of the TV and restoration of the normal right ventricular morphology, also shows some growth of the tricuspid annulus, the anteroposterior diameter of which went from 14.9 to 17.4 mm in 1 year.
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Of the 9 patients with Wolff–Parkinson–White syndrome who had surgical division of the accessory AV conduction pathway, 8 were successful. The failed case (in the twelfth patient) was submitted to repeat catheter ablation attempts afterward. Eventually, the patient underwent reoperation because she also had TV regurgitation.
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Discussion |
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TopAbstractIntroductionPatients and MethodsResultsDiscussionReferences |
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of bringing the TV leaflets to the true tricuspid annulus level and longitudinal plication of the atrialized RV as necessary to restore the right ventricular volume and morphology in Ebstein’s anomaly repair. Nevertheless, it differs markedly in terms of surgical details, as well as the resulting TV geometry and function. Carpentier and colleagues6 detach the dislocated TV from the interior of the RV, bring it to the normal level of the tricuspid annulus, and rotate it partially only to reach the septal tricuspid region. Therefore reimplantation is to proceed in a monocuspid format, thus generating an off-center diastolic blood flow. In contrast, the cone reconstruction proceeds through a complete clockwise rotation of the lateral margin of the TV megaleaflet while preserving its right ventricular apex attachments. Furthermore, the entire circumference of this cone base (which might or might not include a contribution from the septal leaflet, depending on its state of development) is sutured to the normal level of the tricuspid annulus. Unlike with the method of Carpentier and colleagues,6 this includes the septal region, resulting in central diastolic blood flow and restoration of the septal leaflet rule on the TV closure mechanism. AV block, a complication not observed in this series of patients, is avoided by the use of very superficial sutures near the AV node area. Of note, this technique might reduce the tricuspid annulus to a smaller size than the mitral annulus, raising concerns about TV stenosis; however, to date, that problem has not existed. Although mild early TV stenosis occurred in 2 patients, it reversed on follow-up echocardiographic studies. The inclusion of the septal leaflet to enlarge the newly reconstructed TV seems to add in the TV stenosis prevention, particularly in adult patients. The rotation of cordless posterior leaflet tissue to be fitted to the hypoplastic septal leaflet, completing its proximal aspect, is a helpful maneuver to obtain a bigger TV.
As in the series of Quaegebeur and coworkers,7 we did not use an annuloplasty ring device. That seems to be important in children because a permanent fixation of an already diminished tricuspid annulus could result in late stenosis. Figure 3 shows an example of TV annulus growth while keeping a good coaptation of TV leaflets and, consequently, no regurgitation. Additionally, systolic constriction of the tricuspid annulus was seen at the same echocardiogram. This annular flexibility, which might contribute to the valve opening and closing mechanism, was observed thanks to echocardiographic markers from interrupted suture knots placed at the true tricuspid annulus. The possibility of tricuspid annulus growth and flexibility, good midterm clinical outcomes, and few reoperations in this series of patients stand against routine use of the tricuspid ring with this procedure but do not rule out its need in older patients, especially in those with friable valvular tissues.
In operations described by Carpentier and colleagues6 and Quaegebeur and associates,7 a monocuspid mechanism of closure is the result, and their follow-up studies documented a substantial incidence of TV regurgitation.7,10 It is hoped that the full circumferential attachment to the TV annulus described in this procedure, providing a leaflet-to-leaflet coaptation, will reduce that incidence of regurgitation, but it has to be confirmed by late anatomic and functional studies.
It is reassuring that no AV block occurred in any of the patients. This coincides with the results of Wu and Huang’s recent series.11,12 They used fresh autologous pericardium in 80% of their patients to reconstruct the septal portion of the TV, which they suture a little below the septal annulus level. As in this series, no occurrence of AV block was observed after suturing at the septal portion, although they also touched on the importance of placing superficial sutures in this area.
Ullmann and associates13 reported a technique using the septal tricuspid leaflet, which had its proximal edge mobilized and sutured to the septal tricuspid annulus area. They also did not have any cases of heart block. It should be emphasized, however, that their approach did not include plication of the right ventricular atrialized portion or reduction of the tricuspid annulus, and as they themselves have pointed out, that technique is limited to patients with more favorable anatomy, with replacement of the TV being necessary in 5 of 29 patients. Besides being different, this operation was not restricted to only patients with well-developed septal leaflets, which represent a small portion of the wide anatomic spectrum of Ebstein’s anomaly. Actually, it was applied to all anatomic variations presented in 40 consecutive patients with Ebstein’s anomaly. In 22 of them, the septal leaflet was incorporated in the reconstructed valve, and in 18 patients the septal leaflet was disregarded, but the idea of making a cone was kept in all patients.
In general, the surgical technique to approach the ASD in Ebstein’s anomaly has not been a major concern to authors performing only routine closure. In this cohort a valved interatrial communication was always provided, allowing only right-to-left blood shunting when the atrial pressure becomes greater in the right side. This has proved useful in the early postoperative course of 5 patients with important right ventricular dysfunction, who were nevertheless able to maintain good systemic cardiac output, albeit with mild oxygen desaturation that got better once the RV improved. Chauvaud14 has already reported improvement in results with the use of a bidirectional cavopulmonary shunt used as an adjunctive procedure to Carpentier’s operation in patients with severe right ventricular dysfunction. Lately, Chauvaud and colleagues15 have used that technique in 36% of patients, with the goal of decreasing the right ventricular preload in cases of severely depressed right ventricular contractility. With that procedure, they had a significant reduction in mortality caused by right ventricular failure. Alternatively, in this series a unidirectional ASD was left, aiming to decompress the RV and to increase left ventricular preload by means of temporary right-to-left shunting. Volume, inotropic drugs, and vasodilators were used to improve the right ventricular performance and consequently increase the pulmonary perfusion. The cavopulmonary shunt was a strategy reserved only as a next-step procedure for cases of low right-sided cardiac output insufficient to maintain reasonable systemic oxygen saturation, which did not happen in any patient. It is necessary to point out, though, that the 2 series might not be comparable because in this series the patients were younger and presented with lower mean cardiothoracic ratios than in the series of Chauvaud and colleagues.15
The Danielson operation, despite some technical modifications, remains highly associated with the need for TVR. Kiziltan and coworkers4 reviewed their series of 323 patients with Ebstein’s anomaly, with TVR performed in 158 (48.9%) patients. In regard to long-term results, they found that the freedom from bioprosthesis replacement was 97.5% ± 1.9% after 5 years and 80.6% ± 7.6% after 10 years. They also found no statistically significant difference at 10 and 12 years in freedom from reoperation after TVR compared with freedom from reoperation after TV repair. These good results, according to the authors, might be related to the large size of bioprosthesis that can be implanted relative to patient somatic size and to the normally low right ventricular systolic pressure in patients after Ebstein’s anomaly repair. However, these results are for a limited period and do not rule out the ultimate need for tricuspid prosthesis replacement and therefore do not decrease the importance of creating an efficient and durable TV repair operation.
The indications for surgical intervention in patients with Ebstein’s anomaly remain controversial in asymptomatic patients, although the natural history of the disease is a relentless progression to congestive heart failure, arrhythmias, or both16,17 in the majority of patients not undergoing operations. Mortality for these late-stage complications is high.18,19 It seems also to be true that surgical treatment at late stages has less chance of reversing the ravages of the disease completely. That might be the case in 1 early death and another late progression to heart failure in this series. These events seem to be related to the state of the 2 patients who had left ventricular myocardiopathy preoperatively rather than to the surgical technique. This further reinforces the notion that surgical intervention should come earlier, before deterioration of right and left ventricular function.
In conclusion, this surgical technique that reconstructs the TV in a cone shape, which results in a central flow through the tricuspid orifice and a full coaptation of the leaflets, can be performed with low mortality and morbidity. Early echocardiography showed significant reduction in tricuspid insufficiency, and the follow-up showed clinical improvement in the majority of patients, low incidence of reoperations, and no need for TVR. Further studies and longer follow-up are required to evaluate the behavior of the TV and RV after this procedure.
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