Summary
Background
Ebstein’s anomaly is a complex malformation. Justification of a procedure in mildly symptomatic adults is debatable: repair techniques are demanding and valve replacement is associated with poorer outcome.
Objectives
We report our initial experience with the cone procedure versus medical follow-up.
Methods
Patients aged ≥ 15 years with Ebstein’s anomaly were enrolled during 2007–2014. The cone procedure was performed in consecutive patients with severe tricuspid regurgitation (TR); those with less severe disease did not undergo surgery, although some underwent percutaneous catheter atrial septal defect closure.
Results
The cone procedure was performed in 20 patients (mean age 34.3 ± 14.4 years; TR grade 3.3 ± 0.7) because of impaired functional capacity: six New York Heart Association (NYHA) class II, 14 class III. No surgical patient died during a mean (range) follow-up of 2.8 (0.5–5.0) years. One patient required subsequent repair for suture dehiscence 6 months postoperatively. All patients presented with mild or less TR at last echocardiographic follow-up. NYHA functional class was significantly improved at follow-up ( P < 0.0001): 16 NYHA class I and four class II. Of 24 non-surgical patients (mean age 37.3 ± 16.9 years; TR grade 2.2 ± 0.8), seven underwent percutaneous ASD closure. During a mean (range) follow-up of 4.8 (3.6–5.0) years, two patients died: one sudden death and one stroke.
Conclusion
The cone repair of the tricuspid valve in adults with Ebstein’s anomaly provided excellent mid-term results and significantly improved functional status. This procedure might be considered even in mildly symptomatic patients in the presence of severe valve regurgitation.
Résumé
L’anomalie d’Ebstein est une malformation complexe. L’indication opératoire chez un patient peu symptomatique est débattue, la réparation de la valve est requise car le remplacement valvulaire est associé à de mauvais résultats.
Objectifs
Nous rapportons notre expérience initiale de chirurgie de cône ainsi que le suivi médical.
Méthodes
Les patients de plus de 15 ans furent inclus entre 2007 et 2014. La chirurgie de cône fut indiquée chez les patients avec fuite tricuspide sévère, ceux ayant une forme moindre de la maladie ne furent pas opérés, certains eurent une fermeture percutanée de la communication interauriculaire.
Résultats
La chirurgie de cône fut réalisée chez 20 patients (âge moyen de 34,3 ± 14,4 ans ; fuite tricuspide de grade 3,3 ± 0,7) en raison de leur symptomatologie fonctionnelle : 6 étaient en NYHA classe II, 14 en classe III. Il n’y a eu aucun décès de patients opérés durant in suivi de 2,8 (0,5–5,0) ans. Un patient a dû être réopéré 6 mois après pour lâchage de suture. La fuite tricuspide était de faible grade chez tous les patients opérés. La classe fonctionnelle NYHA a été améliorée de façon significative ( p < 0,0001) : 16 étaient en NYHA classe II, 4 en classe III. Parmi les 24 patients non opérés (âge médian de 37,3 ± 16,9 ans ; fuite tricuspide de grade 2,2 0,8), sept patients eurent une fermeture percutanée de la communication interauriculaire. Durant un suivi moyen de 4,8 (3,6–5,0) ans, deux patients décédèrent : 1 mort subite et 1 accident vasculaire cérébral.
Conclusion
La chirurgie de cône de reconstruction valvulaire tricuspide chez des patients adultes avec anomalie d’Ebstein donne d’excellent résultats à moyen terme et améliore significativement le statut fonctionnel. Cette chirurgie peut être discutée chez des patients peu symptomatiques avec des fuites tricuspides sévères.
Background
Ebstein’s anomaly was described for the first time by Wilhelm Ebstein in 1866 and accounts for less than 1% of congenital heart disease (approximately 1:200,000 births) . This anomaly has always been difficult to classify, because of a specific pathophysiology involving tricuspid valve regurgitation and a primary abnormality in the development of the right ventricle, frequently associated with a right-to-left shunt at atrial level . Another characteristic of this disease is the wide variety of its types and ages of detection, related to a wide spectrum of severity of the anatomical abnormality, ranging from minimal apposition of the septal leaflet of the tricuspid valve to a quasi-absence of functional right ventricle.
The therapeutic approach to Ebstein’s anomaly in paediatric cardiology is still debated. Surgery has had a central place – since the 1970s – for most congenital heart defects and allows – for a significant proportion of patients – a full recovery with good long-term prognosis. However, the management of Ebstein’s anomaly, particularly in adults, is still the source of much debate on the appropriateness of an invasive procedure for many reasons. First, the surgical repair to directly correct the valvular abnormality – the tricuspid plasty – is technically challenging. Second, Ebstein’s disease can be very well tolerated despite severe anatomical lesions. Third, effective tools to stratify the patient’s prognosis, and predict the occurrence of right heart failure are still lacking.
Several surgical techniques have been developed. The first ones consisted of valve replacement by mechanical prosthesis then bioprothesis . The first valve repair was simply forming a ‘mono-cusp’ valve by associating a transverse plication of the atrialized ventricle to a posterior annuloplasty . Carpentier et al. published, in 1988, an innovative technique based on the complete detachment of the proximal insertion of the anterior leaflet associated with the most effective ‘surgical delamination’ possible and the re-implantation at the anatomic tricuspid annulus level . A longitudinal plication of atrialized ventricle, a prosthetic ring and, eventually, a superior cavopulmonary anastomosis were associated for most of the patients. This surgery was guided by preoperative ultrasound assessment of the state of the anterior leaflet in a specific detailed classification . Until 2007, the Mayo Clinic team continued to use this technique. Also, after stopping the original technique, they preferred a functional reconstruction technique using anterior leaflet with annuloplasty, moving the anterior pillar and making a ‘mono-cusp’ valve. The proportion of tricuspid valvuloplasties performed in this group remained below 30% . An elective resection of atrialized ventricle was preferred to the systematic plication, with a conservative attitude when the atrialized ventricle showed residual contractility.
The difficulty in Ebstein’s anomaly is the lack of valvular tissue and the importance of defects of the sub-valvular apparatus, making anatomic repair in three leaflets illusory. Since the late 1990s, introduced by da Silva et al. , there has been a new surgical repair technique called ‘cone reconstruction’ that brings several important innovations. The first is to replace the tricuspid annulus in its anatomical position; the second is to repair the valve with two sheets, opening in the right ventricle in an axis more ‘physiologically’ directed to the apex . Since the publication of the paper by da Silva et al. in 2008, we opted for this new technique for all types of Ebstein’s patients of any age. In our current practice, the original ‘cone’ technique was modified, particularly in the adult population, including the use of prosthetic annulus and/or leaflet patch augmentation.
The aims of this study were to evaluate the technique of modified ‘cone’ reconstruction of the tricuspid valve in patients aged 15 years or older with Ebstein’s anomaly and to compare their outcomes to those of patients who had received only medical follow-up, including some who benefited from percutaneous closure of their atrial septal defect (ASD) by cardiac catheterization.
Methods
During 2007–2014, consecutive patients with Ebstein’s disease underwent ‘cone’ reconstruction. Surgical patients were compared with those who were not proposed for surgical procedure, some of whom underwent percutaneous ASD closure.
Medical history prior to inclusion was collected through analysis of medical records and interviews of the patients. The following symptoms were exhaustively sought: dyspnoea, cyanosis, weakness, palpitations, angina pectoris and episodes of faintness or syncope. Dyspnoea was graded according to the New York Heart Association (NYHA) classification into 4 grades. Saturation at rest was systematically recorded at inclusion. Ongoing medical treatments were collected.
A 12-lead electrocardiogram was recorded in all patients at inclusion and at every follow-up visit, searching for: sinus rhythm or not, right bundle branch block, pre-excitation and first-degree atrioventricular block. Chest radiography was available for most patients (except for five patients in the medical group) and was used to measure cardio-thoracic index. Echocardiography was available in all patients. The degree of tricuspid regurgitation (TR) was graded into four classes , and the existence of an atrial shunt and its type (ASD or patent foramen ovale) were sought, as well as any association with other congenital heart defects. Accurate and reproducible data for assessing right ventricular function were only available for a limited number of patients so were not used for this study. All surgical patients also underwent a transoesophageal echocardiography before leaving the operating room and a transthoracic echocardiography prior to hospital discharge, to evaluate surgical results and track complications.
The modified ‘cone’ reconstruction tricuspid valve repair technique
The repair consisted, firstly, of a basal detachment of anterior and posterior leaflets. The access thus created allowed optimal surgical ‘delamination’ of both leaflets, of the septal leaflet when it existed, and also allowed the release of the sub-valvular apparatus, especially working on the anteromedial papillary muscle. It is essential to keep the distal attachments of the leaflets to the ventricle, but it is necessary to reduce their number and to extend them ( Fig. 1 A).
After this initial preparation phase, the ‘atrialized’ intermediate chamber was plicated by means of continuous monofilament sutures. This plication, as well as the annular plication, was performed in a limited manner by the insertion of two or three polyester sutures at the level of the caudal segment of the tricuspid annulus. Then, the free end of the posterior leaflet was rotated in a clockwise direction to be sutured to the free end of the anterior leaflet, forming a cone. The modification essentially aimed to preserve the septal commissure, not to result in an atrioventricular valve without any commissure and, on the other hand, to avoid extensive plication of the atrialized segment, particularly the annulus. Meticulous attention was paid to avoid coronary artery distortion. The posterior leaflet was slid and fixed to the septum to form a septal leaflet. The septal leaflet, when it had retained a certain mobility, was used to expand the cone, using a suture zone between the septal and posterior leaflets. The latter was then enlarged by means of an autologous pericardial patch in 14 at the beginning of the present experience. This patch formed part of the cone ( Fig. 1 B). The prosthetic ring (Uniring ® , Peters Surgical, Bobigny, France) was used in 16 patients in the current study. The prosthetic ring was used mainly to support leaflet repair sutures. The size of the annulus was adjusted to reduce the posterior annulus without any particular size target allowed by the specificity of the device, with the number of stitches varying between 10 and 14. Only four patients underwent repair without an additional patch or ring. In three patients, leaflet coaptation was optimized by the addition of Sebening stitches . Finally, the atrial septal defect was closed.
The percutaneous ASD closure technique
In the medical group, some patient benefited from an ASD catheter closure, which involved the implantation of a self-expandable prosthesis of variable size, usually of the Amplatzer type, by femoral venous access, on the edges of the ASD . The principal indication for ASD closure was cyanosis due to right-to-left shunt, particularly during exercise, which limited functional capacity.
Postoperative data collection and follow-up
All events during hospitalization were collected exhaustively. The types of prosthetic equipment used were noted. In the postoperative period, the following data were collected: duration of stay in the intensive care unit, time to extubation, duration of catecholamine therapy, occurrence of infectious events related to surgery or resuscitation and occurrence of other complications. Indications for immediate surgical reintervention were the occurrence of severe bleeding, the presence of a residual TR greater than grade II and the occurrence of surgical site infection. The occurrence of arrhythmias or conduction disorders after surgery was investigated. The need for peri- or postoperative transfusion was noted.
We paid particular attention to the data permitting comparison with previous studies, i.e. mortality and morbidity, supraventricular arrhythmias, heart failure and the increase in functional status after the procedure. Event rates associated with the disease were assessed using a composite criteria consisting of recurrence of supraventricular tachycardia or occurrence ≥ 2 months after procedure, cardiac insufficiency, endocarditis, stroke or sudden death.
Forty of 44 patients completed a telephone survey (two patients with mental retardation were helped with for this). Patients were asked about the same types of symptoms as those sought during the first interview. In case of death, questioning of relatives or doctors allowed us to ascertain its causes and circumstances.
Patients were asked about the need for hospitalization for whatever reason, focusing on heart failure, arrhythmias and the use of rhythmic procedures. Information on the need for surgery (first time or reoperation) and its indication was collected.
Two questions derived from a standardized and validated test of quality of life seeking a possible global subjective functional benefit were asked: ‘Since the surgery, do you feel: much better, a little better, unchanged, at bit worse or much worse than before?’ and ‘If you were to give your opinion on the support around the intervention, would you be: very satisfied, quite satisfied, not really satisfied or not satisfied at all?’.
Statistical analysis
Data are expressed as counts and percentages for categorical variables and means for continuous variables. StatView ® 4.0 and Excel 5.0 softwares were used. Differences between groups were analysed by the Chi 2 test, the paired t -test, the Fisher least significant difference test and analysis of variance. P values < 0.05 were considered statistically significant.
Results
Patients characteristics
The mean ± SD (range) ages at inclusion were 34.3 ± 14.4 (17–60) years in the surgical group and 37.3 ± 16.9 (15–75) years in the medical group ( P = 0.55) and, overall, 28 (64%) were women ( Table 1 ). The surgical patients had significantly worse NYHA functional status than patients in the medical group ( P = 0.0023).
| Surgical group ( n = 20) | Medical group ( n = 24) | P | Catheter subgroup ( n = 7) | |
|---|---|---|---|---|
| Age (years) | 34.3 ± 14.4 | 37.3 ± 16.9 | 0.55 | 48.2 ± 16.2 |
| Women | 13 (65) | 15 (63) | NS | 4 (57) |
| NYHA class | 2.4 ± 0.6 | 1.7 ± 0.7 | 0.0023 | 2.0 ± 0.8 |
| I | 0 | 9 (38) | – | 2 (29) |
| II | 6 (30) | 13 (54) | – | 3 (43) |
| III | 14 (70) | 2 (8) | – | 2 (29) |
| Other symptoms | ||||
| Cyanosis | 7 (35) | 6 (25) | NS | 3 (43) |
| Asthenia | 6 (30) | 4 (17) | NS | 1 (14) |
| Palpitations | 12 (60) | 10 (42) | NS | 1 (14) |
| Angina pectoris | 0 | 1 (4) | NS | 0 |
| Faintness/syncope | 2 (10) | 1 (4) | NS | 1 (14) |
| Saturation at rest (%), mean | 95.8 | 94 | NS | 90.5 |
| Medical history of SVT | ||||
| Wolff Parkinson White | 3 (15) | 3 (13) | NS | 1 (14) |
| Atrial | 7 (35) | 6 (25) | NS | 4 (57) |
| Medical history of rhythmic procedure | 2 (10) | 6 (25) | NS | 3 (43) |
| Electrical cardioversion | 1 (5) | 3 (13) | 2 (3) | |
| Radiofrequency | 1 (5) | 4 (17) a | 1 (1) | |
| Medical history of cardiac insufficiency | 1 (5) | 1 (4) | NS | 0 |
| Medical history of stroke | 2 (10) | 2 (8) | NS | 1 (14) |
| Electrocardiogram | ||||
| Sinus rhythm | 19 (95) | 23 (96) | NS | 6 (86) |
| Right bundle branch block | 18 (90) | 19 (79) | NS | 6 (86) |
| First-degree atrioventricular block | 3 (15) | 5 (21) | NS | 3 (43) |
| Pre-excitation | 3 (15) | 1 (4) | NS | 0 |
| Cardio-thoracic index, mean | 0.58 | 0.59 | NS | 0.6 |
| Haematocrit (%), mean | 45.6 | 50.7 | 0.02 | 53.7 |
| Tricuspid regurgitation grade | 3.5 ± 0.7 | 2.3 ± 0.8 | < 0.0001 | 2.6 ± 0.6 |
| I | 0 | 3 (13) | – | 0 |
| II | 2 (10) | 13 (54) | – | 3 (43) |
| III | 7 (35) | 7 (29) | – | 4 (57) |
| IV | 11 (55) | 1 (4) | – | 0 |
| Atrial shunt | 13 (65) | 17 (71) | NS | 7 (100) |
| Other cardiac congenital anomaly | 5 (25) | 3 (13) | NS | 0 |
| Medical treatment | 14 (70) | 12 (50) | NS | 5 (71) |
| Diuretics | 4 (20) | 2 (8) | NS | 0 |
| Anticoagulants (VKA or similar) | 7 (35) | 8 (33) | NS | 5 (71) |
| Antiarrhythmic drugs | 6 (30) | 7 (29) | NS | 4 (57) |
| Beta-blocker, ACEI | 6 (30) | 6 (25) | NS | 3 (43) |
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
