Tricuspid Annuloplasty and Ventricular Plication for Ebstein’s Malformation Florentino J. Vargas, MD, Gustavo Mengo, MD, Miguel A. Granja, MD, Jorge A. Gentile, MD, Maria E. Rannzini, MD, and Juan C. Vazquez, MD Unit of Pediatric Cardiovascular Surgery, Hospital Italiano, Buenos Aires, Argentina Background. Seven patients with the diagnosis of Ebstein’s malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present. Methods. Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described. Results. The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures. Conclusions. This technique seems applicable to most forms of Ebstein’s malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface. (Ann Thorac Surg 1998;65:1755–7) © 1998 by The Society of Thoracic Surgeons C treatment of arrhythmia were not indicated in this series. Preoperative echocardiographic assessment displayed an adequate excursion of the anterior leaflet in all but 2 patients. onservative procedures should prevail, when possible, for repair of Ebstein’s malformation of the tricuspid valve, especially for patients in the pediatric age group. Annuloplasty techniques associated with either transverse or longitudinal plication of the atrialized right ventricle have been used [1–5]. Prosthetic rings have also been used when necessary [4]. Regardless of the method used, feasibility for repair would depend on the presence of an anterior leaflet of adequate size. Septal and posterior leaflets have no importance for repair, and are frequently absent or severely dysplastic. We here report on seven patients in whom successful tricuspid annuloplasty, associated with a longitudinal plication of the atrialized portion of the right ventricle, was performed. Material and Methods Since 1987, 7 patients with the diagnosis of Ebstein’s malformation of the tricuspid valve have been admitted for surgical repair. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, in New York Heart Association functional class II to III, with severe tricuspid regurgitation. None had previous episodes of thromboembolism. Two patients had histories of episodes of a nonspecific type of supraventricular tachycardia. Procedures for Accepted for publication Feb 6, 1998. Address reprint requests to Dr Vargas, Pediatric Cardiac Surgery, Hospital Italiano, San Martin 1353, 1828 Banfield, Buenos Aires, Argentina. © 1998 by The Society of Thoracic Surgeons Published by Elsevier Science Inc Surgical Technique On cardiopulmonary bypass temperature was lowered to 28°C and the aorta cross-clamped after cardioplegia was administered (Fig 1). A previous external inspection of the atrialized portion of the right ventricle was made to rule out the presence of a coronary artery branch. Through a right atriotomy running parallel to the atrioventricular sulcus, the degree of mobility of the anterior leaflet was assessed by injecting cold saline solution under pressure into the ventricular cavity with a bulb syringe. If leaflet excursion seemed adequate, a 4-0 polypropylene pledgeted suture was placed at the anterior-posterior commissure. When the posterior leaflet was absent, and therefore no such commissure was identified, this suture was passed through the corresponding part of the anterior leaflet only. From here, this suture was passed through the septal leaflet tissue (or its remnants if hypoplastic) at the level of the posteriorseptal commissure. Another piece of Dacron pledget was then placed at this point for reinforcement, and the suture was tied up. An almost monocuspid valve provided by the anterior leaflet was created. The resulting annulus size was calibrated with the valvular probe that 0003-4975/98/$19.00 PII S0003-4975(98)00290-2 1756 VARGAS ET AL EBSTEIN’S MALFORMATION Ann Thorac Surg 1998;65:1755–7 Fig 1. Surgical technique. (A) Through a right atriotomy, the abnormally implanted tricuspid valve, together with a zone of thin atrialized ventricular wall (shaded area), is exposed. (B) A pledgeted suture that runs from the anterior-posterior commissure to the posterior-septal commissure (or a septal leaflet remnant) produces the annuloplasty by approximating both points. A monocusp valve is fashioned out of the anterior leaflet. (C) A cul-de-sac of thin atrialized ventricular wall is created. It is then plicated with a continuous double running suture. A single suture is used for both annuloplasty and plication. (D) Both monocusp valve and plicature of the atrialized ventricle are seen. Plication runs longitudinally from the initial annuloplasty site (pledget) to the true tricuspid annulus. Conduction system lies away, anterior to the coronary sinus, in its normal position. (A 5 anterior leaflet; B 5 conduction system; CS 5 coronary sinus; P 5 posterior leaflet; S 5 septal leaflet.) corresponded to the predicted tricuspid valve annulus for the patient’s age and height [6] to minimize the risk of creating iatrogenic stenosis. Valve competence was then tested by saline solution injection into the right ventricle under pressure. At this point, most of the atrialized noncontractile portion of the right ventricle became a cul-de-sac, longitudinally oriented between the previously mentioned suture and the true tricuspid annulus. This area was then plicated by using both ends of the suture, with a double continuous running suture ending at the level of the tricuspid annulus. Care was taken to maintain this plication suture within the intramural tissue of this thin-walled chamber. After the valve was tested again, the atrial septal defect was closed, the right atriotomy was closed, and the aorta was unclamped. At operation, the posterior tricuspid valve leaflet was found absent in 4 patients, and the septal leaflet was underdeveloped in 3 and was represented only by a fibrous remnant. Operation was performed in all, using the technique described. To obtain adequate mobilization of the anterior leaflet, resection of several fibrous bands in its ventricular surface was necessary in 2 patients. Results The postoperative course was uneventful. Mean follow-up for the series is 4.3 years (range 1 to 10 years). All patients are in New York Heart Association functional class I, free of medication and have no clinical evidence of tricuspid incompetence or arrhythmias. Twodimensional echocardiographic and color Doppler echocardiographic studies have shown absence of either valve incompetence or stenosis. Adequate coaptation of the anterior leaflet with the septal leaflet or its remnant was the common finding for all. It was always easy to identify the refringent area of plicature of the atrialized ventricular wall opposite to the septum. (Fig 2). Comment Most patients with Ebstein’s malformation of the tricuspid valve can now be treated with reconstructive proce- Ann Thorac Surg 1998;65:1755–7 Fig 2. Postoperative two-dimensional echocardiogram. A monocusp valve provided by the anterior leaflet is shown both opened (A) and in closed position (B). Adequate leaflet coaptation with the septum (septal leaflet remnant) is displayed. The area of plicature is seen opposite to the septum (arrow). (AP 5 area of plicature; LV 5 left ventricle; RV 5 right ventricle.) dures [1–5]. Valve replacement should probably be confined to a small group of patients in whom the anterior tricuspid valve leaflet cannot be mobilized enough to meet the septal structures. The largest experience in this regard has been reported by the Mayo Clinic. Danielson and colleagues [2, 3] have standardized the use of a combined annuloplasty with transverse plication of the atrialized chamber, reporting large series and excellent results. Carpentier and associates [4] reported successful repair even for patients in whom the functional right ventricle was minute and the VARGAS ET AL EBSTEIN’S MALFORMATION 1757 tricuspid valve extremely dysmorphic. In their experience, plication of the atrialized portion of the right ventricle was performed along the longitudinal axis of the heart. Both anterior and posterior leaflets were detached and repositioned at the level of the tricuspid annulus, leaving the area of plication included within the right ventricular chamber. The creation of a larger ventricular chamber was proposed as an advantage of this repair [4]. A valve ring annuloplasty was an additional variant included in the procedure. This repair has also been advocated by Quaegebeur and colleagues [5], but without a valvuloplasty valve ring as part of the repair. In our view, no definitive rationale supports a clear advantage of using a longitudinal versus a transverse plicature of the atrialized ventricle. In both, a part of the thin-walled chamber is excluded. It is also unclear whether attempts to restore the shape of the right ventricle by including this plicated area beyond the translocated valve (ie, within the right ventricle), as proposed by Carpentier and associates [4], would be of fundamental importance. We believe it is doubtful that this plicated area of thin muscle could be of significance in right ventricular performance postoperatively. Perhaps a successful result will rely mainly on an effective treatment of tricuspid valve regurgitation rather than on the type of plicature used for repair. The technique we have used in our patients seems to fulfill both requirements by performing a successful tricuspid annuloplasty together with the exclusion of a part of the atrialized ventricular wall with a plicature. We found it very reproducible with predictable results and without postoperative arrhythmias. Both annuloplasty and plication can be performed by using a single suture. The long-term results obtained have encouraged us to continue using this procedure for repair of Ebstein’s malformation of the tricuspid valve. References 1. 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– 40. 2. Danielson GK, Maloney JD, Devloo RAE. Surgical repair of Ebstein’s anomaly. Mayo Clin Proc 1979;54:185–92. 3. Danielson GK, Fuster V. Surgical repair of Ebstein’s anomaly. Ann Surg 1982;196:499 –504. 4. Carpentier A, Chauvaud S, Mace L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101. 5. Quaegebeur 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– 8. 6. Shultz DM, Giordano DA. Hearts of infants and children. Arch Pathol 1962;74:464–71.