Long-term follow-up after repair of Ebstein's anomaly.

OBJECTIVE: We studied the long-term results of vertical plication repair of Ebstein's anomaly according to Carpentier. METHODS: Between 1988 and 2007, 28 patients (mean age 28.8+/-15.7 years, range 4-58 years) underwent vertical plication repair of Ebstein's anomaly. At operation the anomaly was classified according to Carpentier. In three patients (11%) a cavopulmonary shunt was added at the repair on the indication of impaired right ventricular function. RESULTS: There was no operative mortality. Early mortality was 3.6% (one patient). Actuarial survival and actuarial freedom from reoperation at 19 years were 96% (95% CI; 96-97%) and 72% (95% CI; 53-92%), respectively. Six patients required reoperation, with a successful re-repair in three patients. Mean duration of follow-up was 10.7+/-6.5 years. One year postoperatively, tricuspid incompetence had decreased significantly (p<0.001), as had New York Heart Association (NYHA) functional class (p<0.001). In addition, exercise tolerance had increased (70+/-19% to 92+/-9% of predicted values, p<0.05). Both tricuspid function and NYHA functional class remained essentially unchanged at the end of follow-up, indicating durable haemodynamic and functional results. CONCLUSION: This study demonstrates favourable long-term results following vertical plication repair of Ebstein's anomaly with low mortality, acceptable morbidity and good haemodynamic and functional results.     

Ebstein's anomaly: repair based on functional analysis.

OBJECTIVE: 'Classical' repair of Ebstein's anomaly is usually performed with transverse plication of the atrialized chamber. However, the anterior leaflet has restricted motion which is an important factor of the tricuspid valve insufficiency. We studied the long term results of mobilization of the anterior leaflet associated with longitudinal plication of the right ventricule. METHODS: From 1980 to July 2002, 191 patients (mean age 24.4+/-15 years (1-65)) were operated on. Anterior leaflet function was assessed on pre-op echocardiography and on surgical examination. Conservative surgery was possible in 187 patients (98%) and included mobilization of the anterior leaflet, longitudinal plication of the right ventricle and prosthetic annuloplasty in adults. Bidirectional cavo-pulmonary shunt was associated in 60 patients. Four patients had valve replacement. RESULTS: Hospital mortality occurred in 18 patients: 9% (95%CL: 6-15%) due to right ventricle (RV) failure in nine patients. Mean follow-up was 6.4 years (0.07-22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was 1 or 2+ in 80% of the cases. Reoperation occurred in 8% (16 patients). A successful second repair was obtained in ten patients. Electron beam computerized tomography (20 patients) demonstrated improved left ventricle ejection fraction 56-66% (P<0.05). Supraventricular tachycardia and pre-excitation syndromes were reduced from 23 to 5%. CONCLUSION: Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.     

Surgical management of Ebstein's anomaly in the adult

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.     

Enlarged coronary sinus thrombosis after repair of Ebstein's anomaly

A 58-year-old woman was referred to our hospital with progressively increasing breathlessness. She reported a history of bioprosthetic valve implantation for tricuspid valve replacement and direct closure of an atrial septal defect for Ebstein's anomaly, 31 years before presentation. Transthoracic echocardiography revealed prosthetic valve failure, an enlarged coronary sinus, and severe mitral regurgitation. Computed tomography revealed a giant coronary sinus with thrombosis and persistent left superior vena cava. She underwent successful mitral and tricuspid valve replacement; however, severe hemodynamic deterioration necessitated mechanical ventilatory support with extracorporeal membrane oxygenation.     

Surgical treatment of Ebstein's anomaly: valve replacement and reconstruction

Because of the relative infrequency of Ebstein's anomaly the type of effective surgical treatment remains controversial. Various authors have reported different results depending upon the type of surgical treatment involved. In TYIH between 1974-1987, 30 cases with Ebstein's anomaly have been operated upon; 12 cases underwent surgical repair and in the remaining 18 cases a tricuspid valve replacement was performed. Four out of the 12 reconstruction cases remained on cardiopulmonary by-pass (CPB) because of the failure of the right ventricle and artificial valves were implanted in these cases. One of these patients died. In all, 3 patients died as a result of annuloplasty and the postoperative courses of these patients were eventful. Among the remaining 18 patients with artificial valve replacement only one patient died and the remainder of the tricuspid valve replacement group had an uneventful postoperative period.     

Ebstein's anomaly of the tricuspid valve: the cone repair

Ebstein's anomaly is a cardiac malformation affecting the tricuspid valve and right ventricle with a wide range of anatomic and pathophysiologic presentations. The cone repair technique is described in detail, as applied to several types of this complex congenital heart disease. The importance of extensive mobilization of displaced and tethered tricuspid leaflets to permit a good leaflet-to-leaflet coaptation after the cone construction is highlighted. This technique was performed in 100 patients with a hospital mortality rate of 3.0%, good clinical outcome, and no need for tricuspid valve replacement. Echocardiograph results showed good anatomic and functional tricuspid valves at immediate and long-term postoperative follow-up.     

Cardiac arrhythmia in patients undergoing surgical repair of Ebstein's anomaly

BACKGROUND: Arrhythmias remain an unsolved problem in Ebstein's anomaly. The aim of this study was to investigate the evolution of arrhythmias after surgical repair. METHODS: Forty-five patients with Ebstein's anomaly and arrhythmias were studied. Mean age was 33 +/- 15 years. Twenty-four patients (53%) had paroxysmal supraventricular tachycardia, 12 (27%) had atrial fibrillation or flutter, 8 (18%) had ventricular preexcitation (Wolff-Parkinson-White syndrome), and 1 (2%) had a nonsustained ventricular tachycardia. Surgical technique included detachment of the tricuspid anterior leaflet and suture on the atrioventricular annulus associated with right ventricular longitudinal plication. RESULTS: There were four hospital deaths (9%). A pacemaker was implanted early after operation in 5 patients (11%). During a mean follow-up of 57 +/- 50 months (range, 4 to 226 months), there were six additional deaths, three of which were sudden. Two patients were lost to follow-up. Of the 33 surviving patients, 8 (24%) continued to have symptomatic arrhythmias, and 15 (45%) were in permanent sinus rhythm. Of the 24 patients with preoperative paroxysmal supraventricular tachycardia and the 12 with atrial fibrillation or flutter preoperatively, 9 and 2 of the survivors, respectively, have had no further episodes of arrhythmia. The incidence of arrhythmia with or without symptoms was reduced to 39% (13/33) of the surviving patients. CONCLUSIONS: Arrhythmia is not totally abolished after operation. However, patients with Ebstein's anomaly and arrhythmia show substantial improvement after conservative surgical intervention.     

Operative treatment of Ebstein's anomaly.

From April 1972 to February 12, 1991, 189 patients with Ebstein's anomaly underwent repair. Ages ranged from 11 months to 64 years (median 16 years, mean 19.1 years). In 58.2%, tricuspid valve reconstruction was possible, and in 36.5%, a prosthetic valve, usually a bioprosthesis, was inserted. In 5.3%, a modified Fontan or other procedure was performed. There were 12 hospital deaths (6.3%). All 28 patients who had accessory conduction pathways (Wolff-Parkinson-White syndrome) underwent successful ablation of the pathways as part of the operative treatment. Follow-up was obtained in 151 (85.3%) patients. Of those patients followed up more than 1 year after operation, 92.9% were in New York Heart Association class I or II. There were 10 late deaths: seven cardiac (four sudden), two noncardiac, and one of an unknown cause. Postoperative Doppler echocardiographic assessment showed the atrial septum was intact in all patients and tricuspid valve function was good to excellent in most patients. Four of the 110 patients (3.6%) who underwent valve reconstruction required reoperation 1.4 to 14.1 years later. Postoperative reduction in heart size was usual, atrial arrhythmias were reduced, and late postoperative exercise testing showed a significant improvement in performance: Maximal oxygen consumption increased from a mean of 47% of predicted value before the operation to a mean of 72% after the operation. Nine patients had a total of 12 successful pregnancies with deliveries of normal children.     

Posterior annular plication: tricuspid valve repair in Ebstein's anomaly

BACKGROUND: Nonneonatal Ebstein's anomaly is a rare congenital anomaly for which numerous operative techniques have been described to repair the aberrant tricuspid valve, exclude the atrialized right ventricle, and address right heart dysfunction. We reviewed short-term outcomes in surgical patients treated by a simplified operative technique. METHODS: Our operative approach to Ebstein's anomaly is approximation of the anteroposterior commissure to the remnant septal leaflet with closure of the cul-de-sac longitudinally. Plication of the atrialized right ventricle (1 patient) and a bidirectional cavopulmonary connection (2 patients) were performed only if necessary. All patients were followed postoperatively by their cardiac surgeon and cardiologist. RESULTS: Seven patients with a mean age of 39 years (range, 3.6 to 63.8 years) underwent repair. Preoperatively all patients had 4+ tricuspid valve regurgitation and were New York Heart Association class III. Mean postoperative hospital stay was 7 +/- 2 days (range, 4 to 11 days). Mean follow-up is 42 +/- 18 months (range, 7 to 58 months). At last follow-up 5 patients are New York Heart Association class I and average tricuspid valve regurgitation is mild. CONCLUSIONS: Plication of the posterior annulus without plication of the atrialized right ventricle, resiting the tricuspid valve, or performing prophylactic cavopulmonary connection appears to be a reasonable operative approach to nonneonatal Ebstein's anomaly. Long-term follow-up of this cohort is necessary to determine the durability of such a surgical approach.     

Surgical treatment of Ebstein's anomaly. Early and late results in seven consecutive cases.

The surgical treatment of Ebstein's anomaly is discussed on the basis of our experience with 7 patients who underwent operation at this clinic during the last ten years. The age of the patients (2 males, 5 females) ranged from 17 to 47 years (mean 27 years) at the time of operation. Two patients were in functional class II and five in classes III or IV (N.Y.H.A.). The Wolff-Parkinson-White syndrome was concomitant in 2/7 cases and patent foramen ovale or atrial septal defect (ASD) with right-to-left shunt was present in 6/7 cases. The valvular anomaly was typical in 5 patients and stenosis of displaced tricuspid leaflets into right ventricular outflow region was detected in two patients. Tricuspid valve replacement (TVR) with disc valve prosthesis was performed in 5 patients (Bj?rk-Shiley model in 4 cases, Kay-Shiley model in one) and tricuspid valve commissurotomy in two patients. All six patients with interatrial communication underwent closure of this communication simultaneously. One patient had concomitant epicardial mapping and attempted division of the anomalous conduction pathways was unsuccessful. This patient died on the 14th postoperative day from ventricular fibrillation. No other early or late mortality was recorded. Life-long anticoagulation therapy was introduced in all 5 patients with TVR. Thrombotic malfunction of the prosthetic valves necessitated re-operation and new prosthetic valve replacement in two patients 15 and 16 months after the first TVR. One of these patients had recurrent prosthetic thrombosis twice which was successfully treated with streptokinase. The other patient's prosthesis is functioning well 18 months after re-operation. All 6 long-term survivors were improved and remained in good condition, in functional class I, 1, 3, 3, 8, 9, and 10 years, respectively, after operation.     

Restoring anterior leaflet continuity: the Spinnaker repair of Ebstein's anomaly

Several repair techniques for Ebstein's anomaly include aggressive mobilization of the anterior leaflet and the construction of a monobicuspid valve coapting against the interventricular septum. This can prove difficult when leaflet tissue is of poor quality or several fenestrations reduce leaflet surface. We present a simple and highly reproducible technique for restoring tissue continuity of the anterior leaflet that could represent a possible addendum to the existing array of surgical solutions. Two surgical cases are described. Both patients showed good valve function and trivial tricuspid regurgitation at short-term follow-up.     

Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly.

OBJECTIVE: Ebstein's anomaly is the most common pathology associated with the accessory conduction pathways. METHODS: From January 1990 to August 1999 48 patients underwent surgical repair of Ebstein's anomaly by various plastic techniques. The pathways were identified and characterized at preoperative electrophysiologic mapping in 17 (34.5%) patients. There were seven males and ten females. The patients age ranged 6-35 years (mean 12.7+/-2.1 years). Five patients were in NYHA class II and were in 12 in NYHA class III. The cardiothoracic ratio ranged from 0.59 to 0.69 (mean 0.65+/-0.08). Tachycardia was present in 15 patients with Wolf-Parkinson-White (WPW) syndrome. Atrial septal defect was present in 12 patients (70. 6%). Accessory conduction pathways were in the right posterior septal area in seven patients, in right posterior septal area and free wall in seven patients and in right free wall in three. The pathways were successfully ablated in all patients during cardiopulmonary bypass. RESULTS: There were no deaths, no relapses. Follow-up ranged from 4 months to 7 years (mean 4.7+/-1.1 years). There were no late deaths, but one patient required successful tricuspid valve replacement because of severe tricuspid insufficiency. At follow-up 57.8% of patients were in NYHA class I. CONCLUSIONS: Preoperative electrophysiologic study allows to identify the accessory conduction pathways in patients with Ebstein's anomaly. The combined approach of tricuspid valve repair and surgical ablation of accessory atrioventricular connections has been proved safe and effective. This operative procedure allows to improve functional results in patients with Ebstein's malformation and concomitant accessory conduction pathways.     

Oblique plication for repair of the atrialized ventricle and tricuspid incompetence of Ebstein's anomaly.

An 8-year-old girl who weighed 42 kg presented Ebstein's anomaly with severe tricuspid incompetence, and mild systemic cyanosis during exercise. A new reconstructive procedure for this complex anomaly was used. Oblique transference of the displaced posterior leaflet was performed, which resulted in plication of the atrialized ventricle and reduction in the tricuspid annular diameter. This procedure requires neither detachment nor closure of the tricuspid valve.