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Ann Thorac Surg 2009;88:1975-1981. doi:10.1016/j.athoracsur.2009.07.019
© 2009 The Society of Thoracic Surgeons
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Sunil P. Malhotra
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Right arrow Congenital - cyanotic

Original Articles: Pediatric Cardiac

Selective Right Ventricular Unloading and Novel Technical Concepts in Ebstein's Anomaly

Sunil P. Malhotra, MD*, Ed Petrossian, MD, V. Mohan Reddy, MD, Mary Qiu, BS, Katsushide Maeda, MD, Sam Suleman, BS, Malcolm MacDonald, MD, Olaf Reinhartz, MD, Frank L. Hanley, MD

Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Stanford, California

Accepted for publication July 2, 2009.

* Address correspondence to Dr Malhotra, Congenital Heart Center at the University of Florida, PO Box 100296, Gainesville, FL 32610-0296 (Email: spmalhotra{at}peds.ufl.edu).

Presented at the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.


   Abstract
Top
 Abstract
Introduction
 Material and Methods
 Results
 Comment
 Discussion
 References
 
Background: Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG).

Methods: Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria.

Results: No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57).

Conclusions: Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.


   Introduction
Top
 Abstract
 Introduction
Material and Methods
 Results
 Comment
 Discussion
 References
 
Ebstein's anomaly is a complex malformation of both the tricuspid valve and the right ventricle (RV) that exhibits characteristic lesions of variable severity. Tricuspid valve function is affected by failure of delamination of the valve leaflets, anterior leaflet tethering, annular dilatation, and apical displacement of the tricuspid valve annulus [1]. This downward displacement of the attachments of the septal and posterior leaflets also effectively reduces the functional mass of the RV. RV function is further impaired by the histologically abnormal myocardium associated with Ebstein's anomaly [2].

Clinical manifestations of Ebstein's anomaly stem from the severity of the component anatomic lesions. Functional limitations typically present as a result of progressive RV dysfunction and ventricular interdependent effects impairing left ventricular function. Cyanosis develops as a result of right-to-left shunting through an atrial communication. Massive right atrial dilation contributes to the development of recurrent atrial dysrhythmias.

Surgical therapy for Ebstein's anomaly should restore tricuspid valve competence and address the inadequacy of the inefficient RV. As the surgical experience with Ebstein's anomaly has accumulated, the approach has evolved from isolated tricuspid valve replacement [3] and repair [4] to tricuspid repair in conjunction with RV reconstructive techniques [5, 6]. The inferior wall of the RV is often thinned and dyskinetic. Suture plication of this segment excludes the nonfunctional portion of the RV, potentially improving efficiency of the right heart. All of the existing major strategies for repair of the Ebstein's valve described in the literature have in common several similar principles: restoring the functional valve orifice to the anatomic right atrioventricular junction, detachment and reimplantation of the major leaflets, and complete plication of the atrialized portion of the RV [5–7].

The bidirectional Glenn (BDG) has been used to improve RV energetics in a variety of lesions that result in borderline RV function [8–10]. The "one and a half ventricle" strategy uses the superior cavopulmonary connection to unload the myopathic RV, reducing RV work during systole. In Ebstein's anomaly, this approach has been advocated to reduce the preload on a dilated, poorly functioning RV [11, 12]. In patients with significant functional limitations, the BDG can relieve the "pancake effect" of a massively dilated RV on the left ventricle, thus improving biventricular function [13]. An additional benefit we have realized is that the unloaded RV is amenable to an aggressive tricuspid valve repair strategy.

In patients who require very aggressive valvuloplasty techniques that reduce the functional orifice of the tricuspid valve, RV unloading permits an otherwise borderline valve opening to be functionally adequate, avoiding the need for prosthetic tricuspid valve replacement. Use of the BDG serves to reduce both the risk of postrepair tricuspid stenosis and potential progression of residual tricuspid regurgitation (TR).

This report summarizes our experience with the surgical management of Ebstein's anomaly outside of the neonatal period. The study focuses on two major points: First, we describe the use of valve reconstructive techniques that differ substantially from those in the literature:

1 A "play it where it lies" approach to the tricuspid valve in which the reconstruction is performed at the functional orifice instead of moving the valve to the anatomic tricuspid annulus;
2 Avoidance of detachment and reimplantation of valve leaflets; and
3 A limited plication performed only at the level of the displaced valve rather than complete plication of the entire atrialized RV.

Second, we describe specific physiologic and anatomic criteria for selective use of the BDG in conjunction with repair of Ebstein's anomaly.


   Material and Methods
Top
 Abstract
 Introduction
 Material and Methods
Results
 Comment
 Discussion
 References
 
This study was performed in accordance with an accepted protocol from the Stanford University Institutional Review Board. Individual patient consent was waived because of the retrospective nature of the study.

Patient Characteristics
Between September 1993 and September 2008, 57 consecutive patients outside of the neonatal period underwent operations for Ebstein's anomaly. A retrospective review of patient clinical records, operative records, diagnostic reports, and outpatient clinic records was performed.

The diagnosis of severe Ebstein's anomaly of the tricuspid valve was established by echocardiography in all patients. Echocardiography was used to characterize the degree of apical displacement of the tricuspid annulus, the severity and nature of TR, and the degree of mobility of the anterior leaflet. TR was classified on a scale of 1 to 4 (1, trace; 2, mild; 3, moderate, and 4, severe). Echocardiography also was used to assess right and left ventricular function and to identify any atrial level shunts.

The most recent cardiology visit and echocardiogram by the pediatric cardiologist ranged from 3 months to 6 years. Follow-up information was obtained in all patients; however, access to the most current follow-up data was limited to 65% of patients because the surgeons changed institutional affiliation during the study period. Functional status, degree of cyanosis, grade of TR, and systolic function were among the findings assessed at follow-up.

The median patient age at operation was 8.1 years (range, 7 months to 40.4 years). Patients requiring surgical intervention as a neonate were excluded. The age distribution is displayed in Figure 1. There were 28 males and 29 females. Clinical status at presentation was exercise intolerance in 40, cyanosis in 26, RV failure in 18, and atrial dysrhythmias in 8. TR was moderate or severe in 50 patients (87.7%). An atrial septal defect or patent foramen ovale was present in 46 (80.7%).


Figure 1
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  		Fig 1. Graph shows age distribution of patients at time of operation.

 
Operative Technique
A standardized approach using median sternotomy, cardiopulmonary bypass with mild or moderate hypothermia, bicaval cannulation, aortic cross-clamping, and induction of cardioplegia-induced arrest is followed. The tricuspid valve is exposed through a right atriotomy, which extends from the appendage to the inferior vena caval junction. If present, intraatrial communications are closed by primary suture closure or with a patch of glutaraldehyde-treated autologous pericardium. Routinely, at the end of the procedure, wide strips of right atrial free wall on each side of the right atrial incision are removed before atrial closure, achieving a significant right atrial reduction plasty.

Approaches to the Tricuspid Valve
A number of well-known technical approaches to the Ebstein's tricuspid valve have been reported. We have not followed any of these. We believe there are several important concepts that have not been emphasized in the published reports that have an important influence on our method of tricuspid valve repair:

1 The detrimental effects of a very large tricuspid annulus—The functional opening of the tricuspid valve is markedly enlarged in Ebstein's anomaly due to the leaflet displacement. As the displacement becomes more severe, the annular dimension progressively enlarges (Fig 2). As a result, significant components of the valve cross-sectional area can be obliterated, as necessary, to achieve valve competence. The tricuspid valve cross-sectional diameter may be as large as 6 cm in severely displaced valves. A 70-kg adult requires a valve diameter of only 2.5 cm or less. We believe that "excess" valve diameter after repair increases the chances of both early and late valve instability and has no counterbalancing benefits. Thus, our approach is to reduce the valve opening to approximately 2.5 cm in the 70-kg patient (or indexed appropriately for smaller patients). This is achieved by using a number of technical maneuvers in various combinations as needed. One of these maneuvers, the limited plication of the atrialized RV, is described in point 2. The other maneuvers are listed in point 4.
2 The goal of plication of the atrialized RV—Various technical approaches and general philosophies relating to the atrialized portion of the RV have been described. Our approach and philosophy differs from these. We believe that the plication maneuver is useful only as a component of the valve repair; thus, we do not plicate the entire atrialized portion all the way to the anatomic tricuspid annulus. Complete plication runs the risk of distorting major coronary arteries, or at the very least creates myocardial ischemia simply from tissue compression. Rather, we focus only on the portion of the atrialized RV closest to the RV apex, at the point of maximal displacement, essentially where the displaced posterior leaflet, if present, would be positioned (Fig 3). We aggressively perform plication in this region using one, or at most, 2 separate heavy (2-0) polypropylene pledgetted mattress sutures placed internally within the RV. Particular care is taken to avoid occlusion or distortion of all coronary arteries running on the surface of the RV with these sutures. We specifically avoid extending the plication towards the anatomic tricuspid annulus (atrioventricular groove) out of concerns for distorting the main right coronary artery or its major branches; thus, we perform neither a classic radial plication (Danielson) nor a complete vertical plication (Carpentier). The 1 or 2 plication sutures we do place are oriented similarly to the vertical plication technique. The resulting plication essentially coapts the most apical aspect of the anterior leaflet with the apical aspect of the septal leaflet, in essence achieving a functional bicuspid valve. This plication maneuver as described may reduce the orifice size by as much as 50%.
An important point is that the resulting functional valve orifice, although markedly reduced in size, is not moved from its displaced position. Moreover, leaflet detachment and reimplantation is never performed. A number of additional valve reconstructive maneuvers are almost always used, in various combinations, to achieve the best result with respect to valve competence. These are described in point 4. Most of these maneuvers further reduce the opening of the valve (see subsequent discussion of BDG).
3 Selective use of the BDG—We use the BDG in two separate and independent circumstances. The first is physiologic. Cyanosis at rest is a marker for an inadequate RV pump. We typically perform a BDG in these patients as part of the overall operation. If the patient is fully saturated at rest but becomes cyanotic with exercise, this is a relative marker of an inadequate RV pump, and we will have a low threshold for placing a BDG. Typically, we will separate the patient from cardiopulmonary bypass after valve repair and monitor right and left atrial pressure. If the right atrial pressure exceeds 1.5 times the left atrial pressure under these relatively unstressed conditions of an open chest in an anesthetized patient, we will perform a BDG. If the patient presents with an intact atrial septum or an atrial septal defect with left-to-right shunting, a BDG is not performed.
The second circumstance for placing a BDG is anatomic and relates to the ultimate size of the functional tricuspid annulus after repair. If it is necessary to make the functional tricuspid orifice substantially less than 2.5 cm (in a 70-kg patient) to achieve a competent valve, we will assess inflow velocity across the tricuspid after separation from cardiopulmonary bypass using transesophageal echocardiography. If obstruction is demonstrated, a BDG is placed. We acknowledge that many of the maneuvers used to make a regurgitant valve competent involve reducing the valve opening. This option for BDG use frees us to aggressively reduce the functional valve orifice as much as necessary to achieve a stable, competent valve repair.
4 Other maneuvers on the tricuspid valve—In addition to the modified plication of the atrialized RV, other maneuvers may be used in varying combinations as needed to further reduce the valve size and address the regurgitation. These include closure of primary commissures, closure of "secondary commissures" (those within an individual valve leaflet, usually the large anterior leaflet), true anatomic annuloplasty for that component of the valve (anterior leaflet) that is not displaced, primary or patch closure of leaflet fenestrations, and placement of a polytetrafluoroethylene diaphragm in the position of the displaced septal leaflet (acting as a nonmoving buttress for the anterior leaflet to close against) in those cases where the coaptation surface is limited by the combination of insufficient anterior leaflet and absent septal leaflet tissue.


Figure 2
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  		Fig 2. Changes in tricuspid annular dimension with progression of rotational tricuspid valve displacement from normal (left panel) to moderate (middle panel) to severe (right panel). The dotted line indicates functional tricuspid placement with increasing severity of Ebstein's malformation.

 

Figure 3
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  		Fig 3. The "play it where it lies" approach involves limited plication of the tricuspid valve. Points A and B are approximated with 1 or 2 mattress sutures at the level of the native valve, not to the level of the true tricuspid annulus. This results in approximating the apical aspects of the septal and anterior leaflets, effectively creating a bicuspid valve.

 
Surgical Procedures
All 57 patients underwent an initial operation for Ebstein's anomaly. Component procedures are summarized in Table 1. A valve-sparing Ebstein's anomaly repair was performed in 54 patients; of these, valvuloplasty was performed in conjunction with plication of the atrialized RV in 50. Prosthetic tricuspid valve replacement was performed in 3 patients (5.4%) at the initial operation, and a BDG was performed in 31 (55%). Atrial septal defects were closed in all 46 patients. Concomitant procedures at time of initial repair were performed in 13 patients and are reported in Table 2.


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  		Table 1 Surgical Procedures for Ebstein's Anomaly Repair
 

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  		Table 2 Concomitant Procedures Performed at Initial Ebstein's Anomaly Repair
 
Statistical Analysis
Statistical analysis was performed with Prism 5.0 software (GraphPad Inc, San Diego, CA). Data are described as median with ranges or mean with standard deviation. Serial data are compared between groups using the t test. Freedom from tricuspid valve replacement was determined by actuarial Kaplan-Meier analysis. Values of p < 0.05 were considered statistically significant.


   Results
Top
 Abstract
 Introduction
 Material and Methods
 Results
Comment
 Discussion
 References
 
No early or late deaths occurred. Early reoperation was required in 2 patients. One patient required pacemaker placement for atrioventricular nodal block and 1 patient required placement of an implantable cardioverter-defibrillator device for recurrent ventricular arrhythmias. At follow-up echocardiography, RV systolic function was normal in 52 patients, mildly reduced in 3, and moderately reduced in 2 patients.

Outcomes of Tricuspid Valve Repair
At initial operation, 54 of 57 patients (94.7%) underwent a valve-sparing Ebstein's anomaly repair, and 4 (7.4%) required reoperation for recurrent TR. Two patients underwent successful repeat repair at 7 months and 4.1 years after the initial operation, and both had mild TR at follow-up. Two patients required prosthetic valve replacement at 1.5 and 5.6 years after tricuspid repair.

Figure 4 demonstrates the TR echocardiographic score preoperatively and at the postoperative follow-up. At a median follow-up of 30 months, 46 of 56 (85.1%) had mild or trivial TR, 6 had moderate TR, and 2 had severe TR. No patients had evidence of tricuspid stenosis. At 4 years, freedom from prosthetic tricuspid valve replacement was 92% (Fig 5).


Figure 4
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  		Fig 4. Severity of tricuspid regurgitation is shown before and after repair.

 

Figure 5
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  		Fig 5. Curve shows freedom from tricuspid valve prosthesis. The 4-year freedom from tricuspid replacement was 92%.

 
Functional Outcomes
All patients demonstrated symptomatic improvement at follow-up. The mean New York Heart Association (NYHA) functional class improved from 2.3 ± 0.5 preoperatively to 1.0 ± 0.2 at follow-up (p = 0.0002; Fig 6), and all patients were in NYHA class I or II at follow-up. Preoperatively, 26 patients (45.6%) had cyanosis at rest or with exertion; postoperatively, 1 patient remained cyanotic. The mean oxygen saturation improved significantly with repair, from 92.7 ± 6.0 preoperatively to 97.7 ± 2.4 at follow-up (p = 0.005).


Figure 6
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  		Fig 6. New York Heart Association functional class status improved from 2.3 ± 0.5 preoperatively to 1.0 ± 0.2 at follow-up (p = 0.0002).

 
Outcomes of "One and a Half Ventricle" Repairs
A partial biventricular repair with construction of BDG was performed in 31 patients. The patients receiving a BDG had lower oxygen saturations (89.5% ± 5.9% vs 96.2% ± 3.9%, p = 0.01) and worse NYHA functional class (2.5 ± 0.6 vs 2.16 ± 0.4, p = 0.0025) than those who did not require the BDG. The difference in preoperative TR severity between patients who did or did not undergo BDG was not significant (3.3 ± 0.7 vs 3.4 ± 0.5, p = 0.53).

There were no BDG-related complications, including chylothorax or pulmonary arteriovenous malformations causing desaturation. Initial tricuspid replacement was performed in 2 of 31 patients (6.4%) requiring BDG. By unloading the RV, use of the BDG permitted aggressive valvuloplasty measures. One of the 29 BDG patients (3.7%) who underwent a valve-sparing Ebstein's repair required subsequent tricuspid valve replacement.

Patients undergoing the BDG accounted for the greatest improvement in oxygen saturation. At follow-up, the mean saturation was 96.9% ± 3.0% among those who received the BDG compared with 89.5% ± 5.9% preoperatively (p = 0.003). NYHA functional status in this cohort improved from 2.5 ± 0.6 to 1.0 ± 0.2 (p = 0.0002).


   Comment
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
Discussion
 References
 
Long-term prognosis for Ebstein's anomaly is predicated on tricuspid valve competence and RV function. We believe our approach to this complex lesion is applicable to the entire spectrum of anatomy and physiology of non-neonatal Ebstein's anomaly. Our surgical management has focused on preserving the native tricuspid valve and maximizing RV function. The outcomes from our 15-year experience with Ebstein's anomaly reflect the goals of improving functional status, eliminating cyanosis, and minimizing the need for reoperations.

Avoiding prosthetic tricuspid valve replacement is a major guiding force in our philosophy of the surgical management of Ebstein's anomaly. Prosthetic tricuspid valve replacement in the pediatric population is certainly problematic, with issues of somatic growth and subsequent need for reoperation, thromboembolic complications, risk of endocarditis, and the effect on long-term RV function. In a 15-year experience, a valve-sparing repair was achieved in 54 of 57 patients at the initial operation. The overall rate of prosthetic replacement at either primary or subsequent operation was 8.8%.

The valvuloplasty techniques are reproducible and based on the basic principles of valve repair and RV remodeling. As illustrated in Figure 2, the true annulus is smaller than the displaced functional annulus. Consequently, plication using the technique described here effectively reduces the tricuspid annulus and also accomplishes valvuloplasty. Concerns exist about more traditional plication, and we agree with these concerns. In an extensive experience of 539 patients undergoing operations for Ebstein's anomaly, Brown and colleagues [14] reported that RV plication is no longer routinely performed due to the development of intractable ventricular arrhythmias. Using the modified plication techniques described here, we have not encountered any cases of acute coronary insufficiency and one case of ventricular arrhythmias requiring placement of an internal defibrillator after ventriculoplasty.

Our approach to the Ebstein's tricuspid repair relies on the redundant nature of the anterior leaflet to create almost all of the coaptation surface area, the use of plication as a form of "annuloplasty" and "valvuloplasty," and aggressive reduction in the functional valve orifice using the various techniques described. Detachment of the anterior leaflet from the tricuspid annulus, described in several Ebstein repairs, including the Carpentier technique and, more recently, the "cone" technique, has never been necessary in our experience [6, 7, 15]. In instances where the annulus required significant downsizing, the cavopulmonary connection has been used successfully to prevent tricuspid stenosis by reducing tricuspid valve inflow.

The BDG has been a valuable adjunct for the surgical management of Ebstein's anomaly. We have followed a selective protocol in which the BDG is added in all patients with resting cyanosis and in selected patients with effort-induced cyanosis. In these patients, the BDG serves to off-load a marginal RV that has required considerable right-to-left shunting. Intraoperatively, the BDG may be used if it is judged that the extensive valvuloplasty measures required would create functional tricuspid stenosis.

In addition to facilitating significant annular reduction during tricuspid repair, the volume load reduction from BDG alone can lead to reduction in severity of TR. Three cyanotic patients in our early experience underwent BDG without valvuloplasty and had mild TR that was found to be trivial at follow-up. This observation of a reduction in TR with a cavopulmonary connection alone was previously reported by Marianeschi and colleagues [16].

In cyanotic patients with significant right-to-left shunting, the BDG reduces the volume load on the dysfunctional RV. The reduced volume along with improved RV efficiency resulting from RV plication permits safe closure of the intraatrial communication in these patients.

Our approach contrasts with other groups who have reserved the BDG for only the most severe forms of Ebstein's anomaly. Quinonez and colleagues [17] described using the BDG in 14 critically ill patients with severe RV dysfunction or dilation in which 11 required valve replacements. Chauvaud and colleagues [11] described improved survival when BDG was performed in a cohort of high-risk Ebstein's anomaly patients. Our experience demonstrates the value of the selective use of the BDG for both durable valve repair and improved RV performance, leading to the resolution of cyanosis.

This study had several limitations. Its retrospective nature prevents a uniform approach to echocardiographic interpretation. This is especially noted in the evaluation of RV function, which is challenging to accurately gauge by echocardiography. Future studies should ideally incorporate magnetic resonance imaging, which is a more reliable tool in the evaluation of RV function. Moreover, the follow-up was limited to the short-term and medium-term. Although no trends have emerged to cause concern for long-term outcomes, longer cross-sectional follow-up will be necessary to assess the durability of this approach in the long-term. Finally, exercise testing is necessary to gain an accurate assessment of postrepair improvements in functional status.

In conclusion, we have described a surgical approach to Ebstein's anomaly that incorporates unique methods of valvuloplasty, ventricular remodeling, and ventricular unloading. Our management strategy for these challenging patients has yielded a high rate of native tricuspid valve preservation, a low incidence of recurrent TR, favorable functional status and RV function, and marked resolution of cyanosis.


   Discussion
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 Discussion
References
 
DR JOSEPH A. DEARANI (Rochester, MN): Thank you, President Chitwood, Secretary Wood. I have no disclosures. Dr Malhotra and colleagues, your findings are consistent with the literature and with my own experience that the bidirectional Glenn is a helpful adjunct when treating Ebstein's anomaly, particularly when the right ventricle is dilated and dysfunctional. In contrast to many others who have employed the bidirectional Glenn routinely in Ebstein's, you have utilized a selective strategy and have applied it in typical Frank Hanley fashion—thoughtful, rational, and protocolized. Your physiologic and anatomic indications will be helpful in guiding surgeons to know when it is important to use the bidirectional Glenn.

My first comment and question has to do with the adult patient. There is concern about the earlier development of venous collaterals and pulmonary AV [arteriovenous] fistula in older patients with a bidirectional Glenn. How many older patients with Ebstein's anomaly received a bidirectional Glenn and what are your indications for using the Glenn outside of the pediatric population? This is a practical issue since many patients with Ebstein's anomaly undergo their first or repeat operation in adulthood when RV function is often significantly depressed.

My second question has to do with RV [right ventricular] function. Echocardiography is a poor method at accurate determination of RV function. I would challenge your findings in the manuscript that postoperative RV systolic function was "normal" in more than 90% of your patients. This would be uncommon in this patient population, whose primary problem is a right ventricular myopathy to begin with. What would be helpful would be a comparative analysis of postoperative RV function in patients with and without the Glenn. Obviously, MRI [magnetic resonance imaging] would be a better method to assess RV function, but could you comment on the differences you may have noted between these two groups?

Finally, it has been shown that New York Heart functional class correlates poorly with exercise capacity, particularly in young patients. Do you have any exercise data, and, if so, is there a performance benefit in one group over the other?

Congratulations to the authors for an important contribution, and thank you to the Society for the privilege of discussing it.

DR MALHOTRA: Thank you for your kind comments, Dr Dearani. Your contributions to this field are tremendous and greatly appreciated by all of us that deal with this challenging group of patients. Regarding the adult population, in reviewing the data, approximately half of the adults underwent a Glenn and the conditions or criteria were unchanged regardless of patient age. We have not seen development of pulmonary AV collaterals. This is likely due to the fact that this is a pulsatile Glenn with antegrade flow through the pulmonary artery from the right ventricle. But we do not change the criteria for adults to children, it is the same criteria, and we have not seen any untoward events following that.

Regarding RV function, I agree wholeheartedly with your statement about RV function assessed by echo as being suboptimal. It would be a very valuable adjunct to use MRI to look at the function, because I think, as I stated, echo is a poor indicator of RV function.

Regarding New York Class Association vs exercise testing, that is a very salient point. Exercise testing is the gold standard for performance status in these patients, and we are currently beginning to look at exercise improvement in patients after an Ebstein's repair, but no conclusions can be made until after that study is completed.

Thank you again.


   References
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 Discussion
 References
 

  1. Zuberbuhler JR, Allwork SP, Anderson RH. The spectrum of Ebstein's anomaly of the tricuspid valve J Thorac Cardiovasc Surg 1979;77:202-211.[Abstract]
  2. Anderson KR, Lie JT. The right ventricular myocardium in Ebstein's anomaly: a morphometric histopathologic study Mayo Clinic Proc 1979;54:181-184.[Medline]
  3. Barnard CN, Schirire V. Surgical correction of Ebstein's malformation with prosthetic tricuspid valve Surgery 1963;54:302-308.[Medline]
  4. Hardy KL, May IA, Webster CA, Kimball KG. Ebstein's anomaly: a functional concept and successful definitive repair J Thorac Cardiovasc Surg 1964;48:927-940.[Medline]
  5. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC. Operative treatment of Ebstein's anomaly J Thorac Cardiovasc Surg 1992;104:1195-1200.[Abstract]
  6. Da Silva JP, Baumgratz JF da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007;133:215-223.[Abstract/Free Full Text]
  7. Carpentier AC, Chavaud S, Mace L. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve J Thorac Cardiovasc Surg 1988;96:92-101.[Abstract]
  8. Mavroudis C, Backer CL, Kohr LM, et al. Bidirectional Glenn shunt in association with congenital heart repairs: the 1-1/2 ventricle repair Ann Thorac Surg 1999;68:976-982.[Abstract/Free Full Text]
  9. Chowdhury UK, Airan B, Sharma R, et al. One and a half ventricle repair with pulsatile bi-directional Glenn: results and guidelines for patient selection Ann Thorac Surg 2001;71:1995-2002.[Abstract/Free Full Text]
  10. Reddy VM, McElhinney DB, Silverman NH, et al. Partial biventricular repair for complex congenital heart defects: an intermediate option for complicated anatomy or functionally borderline right complex heart J Thorac Cardiovasc Surg 1998;116:21-27.[Abstract/Free Full Text]
  11. Chauvaud S, Fuzellier JF, Berrebi A, et al. Bi-directional cavopulmonary shunt associated with ventriculo and valvuloplasty in Ebstein's anomaly: benefits in high risk patients Eur J Cardiothorac Surg 1998;13:514-519.[Abstract/Free Full Text]
  12. Corno A, Chassot P, Payot M, Sekarski N, Tozzi P, von Segesser LK. Ebstein's anomaly: one and a half repair Swiss Med Wkly 2002;132:485-488.[Medline]
  13. Dearani JA, O'Leary PW, Danielson GK. Surgical treatment of Ebstein's malformation: state of the art in 2006 Cardiol Young 2006;16(suppl 3):12-20.[Medline]
  14. Brown ML, Dearani JA, Danielson GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly J Thorac Cardiovasc Surg 2008;135:1120-1136.[Abstract/Free Full Text]
  15. Quaegabeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique J Am Coll Cardiol 1991;17:722-728.[Abstract]
  16. Marianeschi SM, McElhinney DB, Reddy VM, et al. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading Ann Thorac Surg 1998;66:1546-1550.[Abstract/Free Full Text]
  17. Quinonez LG, Dearani JA, Puga FJ, et al. Results of the 1.5 ventricle repair for Ebstein anomaly for the failing right ventricle J Thorac Cardiovasc Surg 2007;133:1303-1310.[Abstract/Free Full Text]



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Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair
J. Thorac. Cardiovasc. Surg., May 1, 2011; 141(5): 1178 - 1183.
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T. P. Graham Jr
The Year in Congenital Heart Disease
J. Am. Coll. Cardiol., January 11, 2011; 57(2): 210 - 218.
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