Da Silva s cone repair for Ebstein s anomaly: effect on right ventricular size and function

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1 European Journal of Cardio-Thoracic Surgery Advance Access published December 21, 2014 European Journal of Cardio-Thoracic Surgery (2014) 1 6 doi: /ejcts/ezu472 ORIGINAL ARTICLE Cite this article as: Lange R, Burri M, Eschenbach LK, Badiu CC, da Silva JP, Nagdyman N et al. Da Silva s cone repair for Ebstein s anomaly: effect on right ventricular size and function. Eur J Cardiothorac Surg 2014; doi: /ejcts/ezu472. Da Silva s cone repair for Ebstein s anomaly: effect on right ventricular size and function Rüdiger Lange a,b, *, Melchior Burri a, Lena Katharina Eschenbach a, Catalin Constantin Badiu a, José Pedro da Silva c, Nicole Nagdyman d, Sohrab Fratz d, Jürgen Hörer a, Andreas Kühn d, Christian Schreiber a and Manfred Otto Vogt d CONGENITAL a Department of Cardiovascular Surgery, German Heart Centre Munich at the Technical University Munich, Munich, Germany b DZHK (German Center for Cardiovascular Research) partner site Munich Heart Alliance, Munich, Germany c Cardiovascular Surgery Division, Hospital Beneficencia Portuguesa de São Paulo Escola Paulista de Medicina UNIFESP, São Paulo, Brazil d Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich at the Technical University Munich, Munich, Germany * Corresponding author. Department of Cardiovascular Surgery, German Heart Centre Munich at the Technical University Munich, Lazarettstraße 36, D Munich, Germany. Tel: ; fax: ; lange@dhm.mhn.de (R. Lange). Received 26 May 2014; received in revised form 23 September 2014; accepted 24 October 2014 Abstract OBJECTIVES: Da Silva s cone repair is a novel technique for surgical reconstruction of the tricuspid valve and the right ventricle (RV) in Ebstein s anomaly. The technique consists of extensive leaflet mobilization, longitudinal plication of the atrialized ventricle and coneshaped reconstruction of the tricuspid valve, allowing for leaflet-to-leaflet coaptation. We evaluated the influence of Da Silva s cone repair on tricuspid competency, right ventricular size and function. METHODS: From February 2010 until July 2013, 20 patients (median age 30.0 years, range years) underwent Da Silva s cone repair. A 4- to 6-mm interatrial communication was left in all patients. Echocardiographic studies and magnetic resonance imaging (MRI) before and after the repair were evaluated. RESULTS: Median follow-up was 11 (0.5 36) months. There were 2 early deaths and no late death. Echocardiography at follow-up revealed mild or absent tricuspid regurgitation in 16 patients. Two patients showed moderate tricuspid insufficiency. MRI studies showed that the mean functional RV end-diastolic volume decreased after surgery ( pre 334 [ ] ml; post 175 [ ] ml, P < 0.001). The mean RV ejection fraction decreased (pre 47 ± 10%; post 35 ± 13%, P = 0.001), and the mean antegrade net stroke volume of the RV increased (pre 65 ± 28 ml; post 75 ± 30 ml, P = 0.057). CONCLUSIONS: Da Silva s cone repair for Ebstein s anomaly creates excellent valve function in all patients. Consecutively, the size of the RV decreases and the antegrade net stroke volume increases 6 months after the operation. Keywords: Tricuspid valve Ventricle right Congenital heart disease valve Surgery techniques Magnetic resonance imaging INTRODUCTION Roughly, Ebstein s anomaly is characterized by a downward displacement of the hinge point of the septal and posterior leaflets towards the apex of the right ventricle (RV), dilatation of the tricuspid annulus and atrialization of the RV [1]. Various surgical methods have been described for repair. In the initial attempts, the common goal was to bring the attachments of the posterior and the septal leaflets to the level of the true annulus, thereby plicating the atrialized portion of the RV in a transverse fashion to improve ventricular function [2 4]. Later, Carpentier was the first to propose detaching the leaflets and re-attaching them at the true annulus Presented at the 28th Annual Meeting of the European Association for Cardio- Thoracic Surgery, Milan, Italy, October and plicating the RV in a longitudinal axis [5]. However, Carpentier s technique had no solution for the septal leaflet, which was later reconstructed with pericardial tissue by others [6, 7]. Da Silva s cone repair uses a different approach in that it centres the reconstruction on the downward displacement of the posterior and the septal leaflet. In his technique, the leaflets are partially detached and re-attached to the true tricuspid annulus. The cone shape is created by connecting the leaflets to the longitudinal axis, which has not been described for any valve reconstruction procedure before. Thus, the septal leaflet can be incorporated in the repair and the leaflets later cover 360 of the annulus. A longitudinal plication of the atrialized ventricle and a horizontal plication of the annulus complete the procedure [8]. Although the effects of this surgical technique have been evaluated with respect to tricuspid valve competency [8 12], little is The Author Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

2 2 R. Lange et al. / European Journal of Cardio-Thoracic Surgery known about the impact of Da Silva s cone repair on RV remodelling. In patients with Ebstein s anomaly, the functional RV volumes, that is, the volumes beneath the displaced tricuspid valve, have been shown to be 2-fold enlarged in comparison with healthy individuals [13]. Da Silva s cone repair affects functional RV size in different ways: the addition of the former atrialized part increases functional RV volume, whereas the plication and the post repair reduced volume overload decreases functional RV volume. We aimed to investigate the net effect of Da Silva s cone repair on functional RV size and function. MATERIALS AND METHODS Between February 2010 and July 2013, 25 consecutive patients underwent surgical treatment for Ebstein s anomaly at the German Heart Centre Munich. Five of these patients underwent procedures other than Da Silva s cone repair and were excluded: In 2 patients with a mild form of Ebstein s anomalyandasufficientamountof mobile leaflet, annuloplasty was performed by closure of the gap between the anterior and the posterior leaflets and implantation of a prosthetic ring. Two further patients had undergone previous tricuspid monocusp valve repair: in 1 of them, an anterior leaflet prolapse was treated with chordal replacement and the other underwent valve replacement. One symptomatic neonate underwent a shunt procedure followed by partial cavopulmonary anastomosis, without Table 1: Patient characteristics with previous surgeries and interventions Sex (M/F) 8/12 Mean age at surgery (years) 34.2 ± 17.8 ( ) Mean weight at surgery (kg) 68.1 ± 17.4 Mean CTR 0.58 ± 0.08 Previous interventions WPW Syndrome 4 Ablation 6 Previous surgeries ASD closure 5 VSD closure 1 TVR 3 PAB 1 ASD: atrial septal defect; CTR: cardiothoracic ratio; PAB: pulmonary artery banding; TVR: tricuspid valve repair; VSD: ventricular septal defect; WPW: Wolff Parkinson White. valve repair. Patient characteristics of the remaining 20 patients are tabulated in Table 1. Approval of the institutional review board of the Technical University Munich was obtained. All late survivors have been followed up on a regular basis (mean follow-up 15.4 ± 12.6 months, median 11 months, range months). Surgical method The cone reconstruction technique was performed strictly according to the description given by da Silva et al. [8]. Briefly, the anterior leaflet was partially detached on one end about 1 2 cm cranial to the membranous septum and on the other end 1 2 cm before the true or assumed anterior/posterior junction. The septal and the posterior leaflets were completely detached and extensively mobilized into the RV cavum in order to generate a tension-free re-attachment to the true tricuspid annulus. The atrialized portion of the RV was plicated in a longitudinal fashion. The valve ring was plicated in a horizontal fashion as to create tension-free re-attachment of the leaflets. The cone was completed by longitudinal connection of the leaflets towards the apex of the RV. None of the patients received a prosthetic ring nor has any additional modification of the original cone procedure been performed. No intraoperative maze procedure or ablation for accessory conduction pathways was performed. In all patients, an interatrial communication of 4 6 mm diameter was either left or newly created. Mean cardiopulmonary bypass time was 140 ± 29 min and mean cross-clamp time was 107 ± 15 min. Echocardiography Echocardiography was routinely performed preoperatively, intraoperatively and during follow-up. Tricuspid regurgitation (TR) was categorized by the width and length of the insufficiency jet into four groups: none, trivial or mild, moderate and severe. All echocardiographic evaluations were done by two independent experienced investigators (Andreas Kühn and Manfred Otto Vogt). Magnetic resonance imaging Magnetic resonance imaging (MRI) was performed prior to the operation and postoperatively after a mean time of 194 ± 57 days using a standard cardiac 1.5-T scanner (MAGNETOM Avanto, Siemens Healthcare, Erlangen, Germany). Ventricular volumes were Figure 1: Magnetic resonance imaging measurement of the functional right ventricular end-diastolic volume (asterisks/yellow) before (A) and after (B) Da Silva s cone repair in a patient with Ebstein s anomaly.

3 R. Lange et al. / European Journal of Cardio-Thoracic Surgery 3 measured using axial slices (retrospective ECG trigger, slice thickness 4.5, 6 or 8 mm depending on body weight, 25 phases/cardiac cycle, 1 slice per 8 12 s breath-hold and acquisition matrix ) and the standard analysis software (Argus, Siemens Healthcare) as previously described [13]. In brief, the phases of both the end diastole and end systole were defined for each ventricle independently. The papillary muscles and trabeculae were considered as part of the myocardium and were excluded from the volume. The borders between the functional right atrium and functional RV were defined by tracing the contours of the tricuspid valve. All contours were traced by the personnel blinded to all other clinical information, as is standard practice in our institution. Thus, functional end-diastolic and functional end-systolic volumes of the RV were obtained (Fig. 1). Ejection fraction and stroke volumes were calculated. Antegrade net stroke volume was measured as flow in the pulmonary artery per stroke. Indexed values were calculated by dividing the values by the body surface area of the patient. Statistical analysis Descriptive statistics are described as frequencies and percentages for categorical variables. Continuous variables are expressed as mean ± standard deviation for normal distribution, or median and range for non-normal distribution. Pre- and postoperative data were compared using the paired t-test for continuous variables and the Wilcoxon signed-rank test for categorical variables. All statistical calculations were performed using the R environment. RESULTS Survival and reoperation Two patients died within the first 30 days: no late mortality was seen during the follow-up period. One patient (51 years) had Figure 2: Tricuspid valve insufficiency before repair and at the last follow-up (last fu). Asterisks denote deceased patients. undergone tricuspid valve repair 15 years prior to Da Silva s cone repair and presented in functional class III. The intraoperative echocardiography after the procedure showed a mild TR. In the postoperative course, the patient showed signs of infection with sepsis. On serial echocardiography, severe TR and severely impaired RV function were detected and interpreted as suture dehiscence caused by infection. The patient required extracorporeal membrane oxygenation (ECMO) support. Tricuspid valve replacement was performed on the 20th postoperative day. Upon reoperation, the repair seemed intact with no obvious sign of endocarditis. Two days later, the patient died from multiorgan failure. The source of infection is unknown. Another patient (61 years) initially presented to NYHA class IV with a reduced RV function. After the repair, she showed no TR. She required ECMO support due to severely impaired RV function and low cardiac output upon the first postoperative day. Additionally, she developed an AV III and needed pacemaker implantation. Although the RV showed signs of recovery, the patient died on the 14th postoperative day from bleeding complications after weaning from ECMO. Two other patients needed ECMO support: in an 11-year-old girl, ECMO support was initiated on the day of operation due to RV impairment. She was successfully weaned from circulatory support on the sixth postoperative day. Two months later, she underwent partial cavopulmonary connection due to persistent RV dysfunction. In a 14.5-year-old girl, ECMO was initiated on the day of operation and was successfully discontinued on the fifth postoperative day after interventional enlargement of the interatrial communication. One patient underwent reoperation for subaortic ventricular septal defect. One patient required pacemaker implantation due to a pre- and postoperatively observed sinus bradycardia. Echocardiography Early postoperatively, none of the patients showed more than mild TR. Ten of 20 patients had an interatrial right-to-left shunt, 4 had a left-to-right shunt and 2 had cross-shunting. Despite the interatrial communication, no shunt was seen in 4 patients. Echocardiographic evaluation at the last follow-up showed a significant reduction in TR (P-value <0.01; Fig. 2). None of the patients exhibited a diastolic mean pressure gradient of more than 5 mmhg between the right atrium and the RV. Six patients showed a residual left-to-right shunt. Four of these patients underwent CONGENITAL Table 2: Right ventricular parameters (mean ± standard deviation) preoperative and 6 months after cone repair (n = 15) Preoperative 6 months after repair P-value frv end-diastolic volume (ml) 334 [ ] 175 [ ] <0.001 frv end-diastolic volume index (ml/m 2 ) 191 ± ± 41 <0.001 frv end-systolic volume (ml) 178 [60 440] 123 [57 329] frv end-systolic volume index (ml/m 2 ) 106 ± ± RV ejection fraction (%) 47 ± ± RV SV (ml) 146 ± ± 22 <0.001 RV SV index (ml/m 2 ) 87 ± ± RV antegrade net SV (ml) 65 ± ± RV antegrade net SV index (ml/m 2 ) 38 ± ± frv: functional right ventricle; RV: right ventricle; SV: stroke volume.

4 4 R. Lange et al. / European Journal of Cardio-Thoracic Surgery interventional closure of the interatrial communication at 6 (n = 2), 14 (n = 1) and 24 months (n = 1) postoperatively due to desaturation during exercise performance. No shunt was detected in the remaining 12 patients during the follow-up. Magnetic resonance imaging Pre- and postoperative MRI examinations have been obtained from 15 patients (Table 2). The mean functional, indexed RV enddiastolic volume (RV-EDV) decreased after Da Silva s cone repair (pre 191 ± 62 ml; post 123 ± 41 ml, P =<0.001; Fig. 3A). The mean functional, end-systolic volume of the RV (RV-ESV) also decreased (pre 178 [60 440] ml; post 123 [57 329] ml, P = 0.048). Additionally, the RV ejection fraction and the mean stroke volume decreased (pre47±10;post 35±13; P = 0.001, Fig. 3B). The mean antegrade net stroke volume increased (pre 65 ± 28 ml; post 75 ± 30 ml, P = 0.057, Fig. 3C). DISCUSSION In the present study, Da Silva s cone repair for Ebstein s anomaly yielded mild or less than mild TR in 16 of 18 patients at follow-up. Two patients showed moderate regurgitation. RV size decreased and antegrade net stroke volume increased. This is most likely attributed to the key features of Da Silva s cone repair, which covers 360 of the valvular annulus with leaflet tissue, and includes a longitudinal plication of the atrialized ventricle. The observed relatively low rate of residual TR after the cone repair is in line with other studies [9 11]. Vogel et al. applied the cone repair in 19 patients and observed an 85% reduction in TR measured by the width of the vena contracta. This effect was persistent up to 15 months. In addition, the present study also demonstrates that the cone repair may restore valvular competency even in patients who had previously undergone another technique for tricuspid valve repair. A similar experience was reported by Dearani et al.[14]. Data on RV size and function after repair of Ebstein s anomaly are scarce. A previous study by Kühn et al. [15] has shown that, after tricuspid repair without plication, RV size decreases and cardiac output increases. Up to now, no such data exist on the Da Silva s cone technique with plication of the RV. In the present study, the preoperative functional RV was enlarged with a mean indexed RV-EDV of 191 ml/m 2 compared with 75 ml/m 2 in healthy individuals [16]. Six months after repair, the functional RV size had fallen to a mean of 123 ml/m 2. Hence, the inclusion of the atrialized ventricle into the functional RV, the plication procedure and the volume load reduction lead to a net reduction in functional RV volume. As depicted in Figure 2, RV shape also changed: the atrialized part of the ventricle was eliminated, and the functional ventricle looked like a normal RV. As a consequence, Da Silva s cone repair results in an increase in the antegrade net stroke volume. The mean indexed antegrade net stroke volume rose from 37.7 ml/m 2 preoperatively to 43.7 ml/m 2 postoperatively. However, it did not reach the antegrade net stroke volume of a healthy individual (50 ml/m 2 )[17]. This might be attributed to the reduced contractile dysfunction of the formerly atrialized RV wall, which is now included in the functional RV. In addition, myocardial scar tissue due to extensive leaflet mobilization and the plication may prevent complete normalization of RV stroke volume, at least within the first 6 months after repair. Figure 3: Magnetic resonance imaging generated volumetric parameters before and 6 months after cone repair. (A) Functional right ventricular enddiastolic volume; (B) right ventricular ejection fraction; (C) antegrade net stroke volume. EDV: functional end-diastolic volume; EF: ejection fraction; SD: standard deviation; SVI: stroke volume index; RV: right ventricle. Cardiomegaly and impaired RV function are the major concerns in patients presenting with Ebstein s anomaly. Both affect morbidity and mortality after repair [18]. We regard the early postoperative phase after the Cone repair as a critical time period, beginning with

5 R. Lange et al. / European Journal of Cardio-Thoracic Surgery 5 weaning from the heart lung machine. With a competent tricuspid valve, the regurgitation volume and in consequence, the RV stroke volume and RV ejection fraction decrease. Therefore, the risk for RV failure is high, which accentuates the importance of early RV unloading. A cavopulmonary anastomosis is used by most groups when facing RV failure after surgery for Ebstein s anomaly [12, 19, 20]. As long-term consequence and impact on exercise capacity in adulthood are poorly investigated, we try to avoid this procedure at initial operation. Instead, temporary ECMO support may be used to bridge the patient and the decision for a cavopulmonary connection deferred. This approach is based on the assumption that RV failure is temporary. We think that postoperative RV failure often can be avoided by leaving a limited residual atrial communication. Upon follow-up, this communication had no negative effect at rest. However, during exercise, some relevant right-to-left shunt was observed, leading to relevant desaturation. Therefore, 4 of our patients had an interventional closure of the communication. In these patients, the danger of paradoxical embolization [21] should be considered. The haemodynamic changes 6 months after Da Silva s cone repair are promising. The mid-term results show low rates of reoperations [10]. Furthermore, we found no anatomical contraindication for this technique. While other centres favour bioprosthetic replacement in older patients [22], we think that repair still can be performed, if RV function does not severely deteriorate. It is, however, our feeling that the 61-year-old patient who died in our study group despite perfect valve performance may have been too old for the procedure. We have shown previously that early timing of intervention in Ebstein s anomaly may be crucial for the surgical results [18]. The study has several limitations: the patient number in this study is small and there were no infants and neonates in the cohort. Follow-up time is also short and the long-term durability of the observed changes remains to be determined. In addition, the study did not include exercise testing and evaluation of quality of life. CONCLUSION Da Silva s cone repair is a novel technique for the surgical reconstruction of Ebstein s anomaly. It has been shown by us and others that this technique yields excellent valve function. The present study adds to the knowledge about Da Silva s cone repair as it demonstrates a decrease of functional RV-EDV and an improvement of the antegrade net stroke volume of the RV 6 months after the operation. Funding None declared. Conflict of interest: none declared. REFERENCES [1] Schreiber C, Cook A, Ho SY, Augustin N, Anderson RH. Morphologic spectrum of Ebstein s malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg 1999;117: [2] Hunter SW, Lillehei CW. Ebstein s malformation of the tricuspid valve study of a case together with suggestion of a new form of surgical therapy. CHEST 1958;33: [3] Hardy KL, May IA, Webster CA, Kimball KG. Ebstein s anomaly: a functional concept and successful definitive repair. J Thorac Cardiovasc Surg 1964; 48: [4] Danielson GK, Maloney JD, Devloo RA. Surgical repair of Ebstein s anomaly. Mayo Clin Proc 1979;54: [5] Carpentier A, Chauvaud S, Mace L, Relland J, Mihaileanu S, Marino JP et al. A new reconstructive operation for Ebstein s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96: [6] Sha JM, Yan ZY, Zhu ZY, Tan L, Zheng L, Shen YH et al. Early and midterm results of repair of Ebstein s anomaly with autologous pericardium. Thorac Cardiovasc Surg 2011;59: [7] Wu Q, Huang Z. A new procedure for Ebstein s anomaly. Ann Thorac Surg 2004;77: [8] da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM et al. The cone reconstruction of the tricuspid valve in Ebstein s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007; 133: [9] Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Mayer JE et al. Ebstein s malformation of the tricuspid valve: short-term outcomes of the cone procedure versus conventional surgery. Congenit Heart Dis 2012;7: [10] Silva JP, Silva L da F, Moreira LF, Lopez LM, Franchi SM, Lianza AC et al. Cone reconstruction in Ebstein s anomaly repair: early and long-term results. Arq Bras Cardiol 2011;97: [11] Dearani JA, Said SM, O Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein s malformation: lessons learned with cone reconstruction. Ann Thorac Surg 2013;95: [12] Liu J, Qiu L, Zhu Z, Chen H, Hong H. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg 2011;141: [13] Fratz S, Janello C, Müller D, Seligmann M, Meierhofer C, Schuster T et al. The functional right ventricle and tricuspid regurgitation in Ebstein s anomaly. Int J Cardiol 2013;167: [14] Dearani JA, Said SM, Burkhart HM, Pike RB, O Leary PW, Cetta F. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. Ann Thorac Surg 2013;96: [15] Kühn A, De Pasquale Meyer G, Müller J, Petzuch K, Fratz S, Röhlig C et al. Tricuspid valve surgery improves cardiac output and exercise performance in patients with ebstein s anomaly. Int J Cardiol 2013;166: [16] Lorenz CH, Walker ES, Morgan VL, Klein SS, Graham TP. Normal human right and left ventricular mass, systolic function, and gender differences by cine magnetic resonance imaging. J Cardiovasc Magn Reson 1999;1:7 21. [17] Grothues F, Moon JC, Bellenger NG, Smith GS, Klein HU, Pennell DJ. Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular magnetic resonance. Am Heart J 2004;147: [18] Badiu CC, Schreiber C, Horer J, Ruzicka DJ, Wottke M, Cleuziou J et al. Early timing of surgical intervention in patients with Ebstein s anomaly predicts superior long-term outcome. Eur J Cardiothorac Surg 2010;37: [19] Malhotra SP, Petrossian E, Reddy VM, Qiu M, Maeda K, Suleman S et al. Selective right ventricular unloading and novel technical concepts in Ebstein s anomaly. Ann Thorac Surg 2009;88: [20] Chauvaud SM, Hernigou AC, Mousseaux ER, Sidi D, Hebert JL. Ventricular volumes in Ebstein s anomaly: X-ray multislice computed tomography before and after repair. Ann Thorac Surg 2006;81: [21] Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Ammash NM, Dearani JA. Increased risk of possible paradoxical embolic events in adults with Ebstein anomaly and severe tricuspid regurgitation. Congenit Heart Dis 2014;9:30 7. [22] Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, Dearani JA. Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement. J Am Coll Cardiol 2012;59: APPENDIX. CONFERENCE DISCUSSION Scan to your mobile or go to to search for the presentation on the EACTS library Dr C. Pizarro (Wilmington, DE, USA): You and your colleagues have presented their experience with Da Silva s cone reconstruction for Ebstein s anomaly in a CONGENITAL

6 6 R. Lange et al. / European Journal of Cardio-Thoracic Surgery cohort of 20 patients, aiming to characterise the net effect of the procedure on the right ventricular size and function. During a median follow-up of 11 months, echocardiography showed a significant reduction in right ventricular end-diastolic volume. In addition, right ventricular ejection fraction decreased by 12% and the mean antegrade stroke volume increased by about 10%; two patients in the cohort died, for a total mortality of about 10% in association with important RV failure. This leads me to my first question: Do you think that the evidence of decreased right ventricular ejection fraction can be related to the elimination of the actual volume load on the right ventricle, therefore eliminating the stimulus for the usually seen hyperdynamic contractility in cases of severe AV valve regurgitation? Or do you think that there are any other factors that might have played a role in the mortality observed in this series? Dr Lange: As to the first question regarding the right ventricular volume, I think you see two effects on the volume: One effect is that by plication of the atrialized portion, of course, you take volume away; on the other hand, you also transpose the valve area further to the atrium, so you should also gain some volume. So I think the net effect of the right ventricular volume is not consistent in all patients. In all patients it was significant in the end, but you also see in the curve that in some patients it also increases the volume. So I think it has two sides to it. And your second question was regarding the mortality. I think what we learned, especially from one of our patients, who was a 60-year-old lady, she had actually the best result ever, she had no regurgitation whatsoever, and she went to the intensive care unit and her condition worsened gradually, she survived for four weeks or so, but she gradually went into multi-organ failure. We could not save her. In the end she had ECMO, but it was impossible to save her, although she had a good result. I think the earlier you can do this operation the better it is. And also, it is not more difficult, I think it s almost easier in children. Dr Pizarro: So given the fact that you had an excellent surgical result, yet you know there was important ventricular dysfunction, do you think that then the selection process, in terms of which patients are the candidates for this procedure, is where the explanation lies? Dr Lange: I m convinced of this, yes. Dr Pizarro: Now, I found interesting that you chose ECMO support to help, or attempt to help, for these two patients. Do you think that they could have been better served by closing the atrial septal communication and providing some form of RVAD support, which will give you probably a longer duration of support with lesser complications? Dr Lange: This could have been another strategy. Intentionally we leave the intra-atrial communication, because we think that this might be the reason why we needed a Glenn procedure in only one patient. But as you said, the other strategy might have worked too. Dr Pizarro: Lastly, have you had the opportunity with your echocardiography colleagues to investigate other indices of measure of ventricular function like TAPC, and do you think that this could be particularly useful, or not necessarily given the fact that you obviously are instrumenting the tricuspid valve? Dr Lange: No, there has been extensive workup on echocardiography. You know cardiologists love echocardiography, and as to my knowledge, they re also preparing a paper on this; but I have not seen all the data yet. Dr Pizarro: There is important data regarding volumetric studies in patients with Tetralogy of Fallot and important volume load due to pulmonary insufficiency. Do you think that those are principles that could be potentially applied to the criteria for selection as to when the timing of this particular surgery should be done? And have you explored such development of thresholds to develop that, do you think there s a completely different matter? Dr Lange: That s a good suggestion. We have not explored this, but this might help in the selection process, I agree. Dr O. Jaber (Leeds, United Kingdom): My first question is about atrial reduction. Do you do any atrial reduction? And the second question: Any arrhythmia procedures? Dr Lange: Very good questions. The first question I think I answered. We do a plication of the atrialized portion. Or do you mean a reduction of the atrium itself? No, we did not do a reduction of the atrium. And we also, in this cohort, in contrast to other groups, we did not do any maze procedures so far; but we have been considering it at least for the elder patients. It s a very good question. Dr J. Da Silva (Sao Paulo, Brazil): I want to tell that I have about 106 patients now in Sao Paulo, and we had done a study, on the first 94 consecutive Ebstein s anomaly patients, comparing the survival of patients according to the age at the cone procedure by Kaplan-Meier survival estimate. This study showed that there was no cardiac-related mortality in the group of patients younger than 12 years of age either in the hospital period or on the long-term follow-up. This result was significantly superior as compared with the result achieved in patients older than 12 years. Like us, you had the worst results associated with older age at the operation. These two findings suggest that younger age at operation is an important factor in the result improvement. The question is: Would you consider replacing the tricuspid valve at first in patients older than 50 years with RV dysfunction? Dr Lange: This is actually a difficult question because I m not sure whether replacement of the valve would really yield better results in older patients. I think the question is: Do you address the Ebstein in really old, like in 60-year-old and beyond patients at all? However, whether to do a cone procedure or to do a replacement, I m not sure whether the risk is so much different actually. But this is pure speculation. I don t know.