[Abstract] Objective To review and compare the various surgical methods for congenital tricuspid valve subluxation malformation (Ebstein), and to propose a more reasonable surgical approach and achieve better therapeutic results for the clinical treatment of Ebstein. Methods The clinical data of 115 children with Ebstein admitted to the Department of Thoracic Surgery at Shanghai Children’s Medical Center between January 2006 and December 2014 were statistically analyzed: age at surgery ranged from 3 months to 16 years and 1 month, mean (4,9±4,3) years, weight ranged from 5,5 to 59,2 kg, mean (18,4±12,2) kg; 104 cases underwent tricuspid valvuloplasty (39 of which underwent additional BCPS), 9 cases of palliative surgery, and 2 cases of tricuspid valve replacement. There were 2 in-hospital deaths, with a mortality rate of 1.7%. The average postoperative follow-up of 113 children was 32,7 months (4 to 88 months), and the results of tricuspid regurgitation after cardiac ultrasound showed that 31 of the 39 children who underwent BCPS had mild regurgitation and 8 had moderate regurgitation, and 35 of the 64 children who did not undergo BCPS had mild, 21 had moderate, and 8 had severe regurgitation. Conclusion Tricuspid valvuloplasty is effective as an important treatment for Ebstein malformation; for younger children, the incidence of moderate to severe tricuspid regurgitation and right heart failure after simple cone reconstruction is high, and 1½ventricular repair should be performed as a routine additional procedure; according to the child’s age, tricuspid anatomic morphology, degree of regurgitation, and intraoperative hemodynamics, individualized The selection of the appropriate surgical plan can effectively reduce the operative mortality of the child.
Ebstein anomaly, also known as Ebstein syndrome, was first discovered by the German scholar Ebstein in 1866 during an autopsy, and refers to a partial or complete downward shift of the tricuspid valve annulus into the right ventricular cavity, accompanied by malformations of the tricuspid valve and structural changes of the right ventricle. Ebstein anomaly is a relatively rare group of congenital cardiovascular anomalies, accounting for 0.5% to 1.0% of the total incidence of congenital heart disease1.
Since 2004, when da Silva et al2 first reported tricuspid tapered reconstruction, its use in the treatment of Ebstein anomalies has become increasingly widespread. Typical tapered reconstructions include complete tricuspid valve transposition, rotation of the posterior leaflet to join the proximal septum; longitudinal folding of the atrialized right ventricle; and annular reduction of the annulus. Although there are many procedures available regarding the correction of tricuspid valve malformations, multicenter statistical results show that the tricuspid valve after tapered reconstruction is the closest to a normal valve, both functionally and anatomically.
Bidirectional cavopulmonary shunt (BCPS) or 1½ ventricular repair is mostly used in patients with severe Ebstein anomalies. In patients with decreased left heart function due to right heart failure and dilatation, BCPS reduces right ventricular preload while increasing left ventricular preload and promoting recovery of biventricular function. In addition, decreased right ventricular load reduces blood flow through the tricuspid valve and stabilizes the annularly constricted tricuspid annulus, thereby reducing the likelihood of distant tricuspid valve replacement.
This article retrospectively examines the use of conical reconstruction with or without 1½ ventricular repair for Ebstein’s anomaly at our center in recent years.
1. Data and Methods
1.1 Case data
From January 2006 to December 2014, a total of 115 children with Ebstein malformation underwent surgical treatment at our center. age at surgery ranged from 3 months to 16 years and 1 month, with an average of (4,9±4,3) years, weight ranged from 5,5 to 59,2 kg, with an average of (18,4±12,2) kg. all patients underwent preoperative cardiac ultrasound examination, including the degree of tricuspid valve inferior migration, valvular All patients underwent preoperative cardiac ultrasound examination, including the degree of tricuspid inferior regurgitation, valve regurgitation, right and left heart function, and the presence of shunts at the atrial level.
All patients were followed up for an average of 32,7 months (4 to 88 months) after discharge, mainly for clinical manifestations, NYHA cardiac function classification, degree of cyanosis, and tricuspid regurgitation, etc. All follow-up information was recorded in the clinic.
Table 1: Basic information of patients before surgery
Basic information
Numerical value
Age, months (median)
57, 1 (3-193)
Tricuspid valvuloplasty without total cavopulmonary anastomosis
44, 3 (3-181)
Tricuspid valvuloplasty with total cavopulmonary anastomosis
80, 0 (11-193)
Glenn procedure + ASD enlargement + tricuspid valve closure
47, 9 (20-63)
Glenn procedure + ASD enlargement + pulmonary artery annuloplasty
10, 9 (6-16)
Fontan procedure
19, 2 (12-25)
Tricuspid valve replacement
128, 0 (118-137)
Clinical presentation
Cyanosis
84 (73,0%)
Right ventricular dilatation
79 (68, 7%)
NYHA class III
73 (63, 5%)
Indication for total cavopulmonary anastomosis
Severe right ventricular dilatation
31
Significant septal valve abnormality
13
Unstable circulation after extracorporeal arrest
2
Follow-up time
32, 7 (4-88)
Cone reconstruction without total cavopulmonary anastomosis
30, 1 (4-71)
Tapered reconstruction with total cavopulmonary anastomosis
38, 0 (5-88)
2. Surgical approach
Tricuspid valve tapered reconstruction surgery: the first step of valvuloplasty: fully free and remove the abnormal tissue between the tricuspid valve and the ventricular wall, including the abnormal papillary muscle and endocardial muscle bundle, so that the valve leaflet movement is not restricted, keeping each leaflet directly connected to the distal ventricular wall only through the normal papillary muscle or myocardium; free the anterior and posterior leaflets of the tricuspid valve from the proximal end of the atrioventricular junction; make an incision 25px in front of the anterior septal junction toward the septum The septal leaflets are freed by making an incision counterclockwise at 25 px in front of the anterior septal junction; the interrupted sutures are used to close the spacing of the leaflets and bring them together; the second step is conical valve reconstruction: all the useful leaflet tissue is rotated in a 360° conical shape around the true tricuspid annulus to create a conical valve; the third step is tricuspid annulus reduction: generally, patients with tricuspid inferior displacement deformity are accompanied by tricuspid valve enlargement to varying degrees, and an appropriate size autologous pericardial strip is used from the anterior The third step is tricuspid annuloplasty: in general, patients with tricuspid valve subluxation with varying degrees of tricuspid valve enlargement are treated with an appropriate size autologous pericardial strip fixed clockwise from the septal junction to the posterior septal junction in the coronary sinus to annul the tricuspid valve annulus.
In addition to the above routine surgical operations, our center has also summarized some surgical experiences: 1) the size of the reconstructed tricuspid annulus should be basically the same as that of the mitral annulus; 2) for children with obvious tricuspid septal abnormalities, fresh autologous pericardial patches are used to expand the septal valve and then sutured to the posterior valve; 3) interrupted sutures are used to suture the septal valve to the new annulus, and continuous sutures are used for the rest of the valve; 4) folding (5) additional BCPS in children with severe disease; (6) all children with atrial shunts should have their septal defects or foramen ovale closed.
3. Treatment results
In this study, a total of 104 children with Ebstein malformation underwent tricuspid valve cone reconstruction with a mean age of 62,5 months (3 to 193 months), of which 20 had BCPS added with a mean age of 80,0 months (11 to 193 months).
The mean aortic block time was 64±13 min, the mean extracorporeal circulation time was 102±24 min, and 2 (1,7%) cases of in-hospital death occurred, the cause of death was final death due to heart pump failure after tricuspid valvuloplasty in one case; the other case was electrolyte disturbance due to renal failure after palliation eventually leading to severe heart failure. No intermediate or long-term deaths occurred. 25 patients had permanent I° AV block. Postoperatively, 12 children opted for delayed chest closure due to myocardial edema (mean 2,8 d). Preoperative, early postoperative, and distant postoperative tricuspid regurgitation and cardiac function are shown in Table 2. 33 cases of moderate and 71 cases of severe tricuspid valve insufficiency before surgery; early postoperative cardiac echocardiographic follow-up showed 65 cases of mild tricuspid regurgitation, 30 cases of moderate regurgitation, and 8 cases of severe regurgitation (p<0,01 compared with preoperative cardiac echocardiographic findings); intermediate and distant follow-up showed that 39 children who had bcps added ( The medium and long-term follow-up results showed that 31 of the 39 children who had bcps (mean 30,1 months postoperatively) had mild reflux and 8 had moderate reflux, and 31 of the 64 children who did not have bcps (mean 38,0 months postoperatively) had mild reflux, 25 had moderate reflux, and 8 had severe reflux. All children with additional bcps had preoperative cardiac ultrasound suggestive of severe right ventricular dilatation. < span="">
Table 2: Preoperative, early postoperative, and mid- and long-term cardiac echocardiographic follow-up results
Preoperative
Early postoperative
Postoperative distant
TR
LEVF
RVEF
Atrialized ventricular ratio
TR
LEVF
RVEF
TR
LEVF
RVEF
Light
medium
Severe
Normal
Abnormal
Normal
Abnormal
<50%
>50%
Light
Moderate
severe
Normal
Abnormal
Normal
Abnormal
Light
Moderate
Heavy
Normal
Abnormal
Normal
abnormal
No BCPS(n=65)
0
23
42
47
18
58
7
25
40
34
22
8
64
0
59
5
31
25
8
64
0
59
5
Companion BCPS (n=39)
0
10
29
34
15
36
3
8
31
31
8
0
39
0
39
0
31
8
0
39
0
39
0
P-value
0, 05
0, 02
The left heart ejection fraction was decreased in 33 children before surgery, and normalized in all children after surgery. There was no significant difference between the BCPS and non-BCPS groups in terms of cardiac function grade at follow-up.
The arterial oxygen saturation reached 96-100% in 39 children in the BCPS group at postoperative follow-up. There were no postoperative superior vena cava complications or pulmonary arteriovenous fistulas. 7 children had facial swelling at follow-up, but there were no other effects.
4. Discussion
In this study, tricuspid valve cone reconstruction was performed in children with Ebstein malformation, and early postoperative echocardiography showed significant improvement in tricuspid regurgitation, but in children with severe preoperative right ventricular enlargement, midterm echocardiographic follow-up of tricuspid regurgitation after cone reconstruction alone was not very satisfactory. This is not consistent with the results of the study by da Silva et al. This may be related to the presence of severe morphological abnormalities of the tricuspid valve itself (not just a subluxation of the tricuspid position) in the children in this study; another reason may also be related to ethnicity, especially considering the higher incidence of right heart system abnormalities in the Asian population.
Another issue of concern is atrialized ventricular folding. In younger children, conventional atrialized ventricular folding and annuloplasty may result in relative stenosis of the tricuspid valve orifice, as well as compression or even distortion of the right main coronary artery, leading to postoperative underperfusion and eventual right heart insufficiency. In recent years, a variety of modified atrialized ventricular folding procedures as well as valvuloplasty have been used with good clinical results4-7: 1) expansion of the valve leaflets using autologous pericardium to increase the valve height; 2) expansion of the cone valve using a triangular patch to avoid tricuspid stenosis; 3) modified Sebening suture to join the intact papillary muscle head end at the base of the right ventricular free wall to the septal valve attached to the corresponding septal The papillary muscle head end is joined to the head end of the corresponding septal valve, or the right ventricular wall is joined directly to the septum to reduce the enlarged right ventricular cavity. In this study, we folded part of the atrialized ventricle (not all atrialized ventricles were folded over the tricuspid annulus) and performed a partial annuloplasty from the anterior-posterior junction to the posterior septal junction to reduce the pressure on the tricuspid annulus while facilitating the maintenance of the conical valve shape over time. Only 1 child had ventricular arrhythmias due to electrolyte disturbances after surgery, and no cases of acute coronary artery insufficiency occurred.
There is an ongoing debate as to whether biventricular repair in children with Ebstein malformation is superior to single ventricle repair. From an anatomical point of view, biventricular repair is clearly superior to single-ventricle repair. However, in complex malformations, biventricular repair also has a significantly higher early and intermediate mortality rate than single ventricle repair. Therefore, it should not be assumed that biventricular repair is better than single ventricle repair. Because of the lack of follow-up studies of large numbers of cases with intermediate and long-term prognosis in the surgical management of Ebstein’s malformation, information to guide the choice of surgical strategy is still very limited8.
Van Arsdell et al. showed that Ebstein’s anomaly is probably the most utilized precardiac disease for which BCPS is an add-on procedure.8 The results of our center showed that nearly 40% of children with cone reconstruction underwent BCPS. This is due to the fact that in cases of less than ideal right ventricular conditions, Ebstein’s anomaly plus 1½ventricular repair or BCPS reduces right ventricular preload, improves left ventricular preload, and promotes left heart development while achieving anatomical and functional balance and reducing operative mortality.
Another major advantage of BCPS is that it facilitates the maintenance of good postoperative tricuspid valve function. marianeschi et al. concluded that BCPS is effective in reducing tricuspid regurgitation and decreasing reoperation rates.9 Our findings support this assertion. In the present study, the improvement of postoperative tricuspid regurgitation was significantly better in the BCPS group than in the non-BCPS group. This may be due to the fact that tapered reconstruction + annulus annuloplasty can lead to postoperative tricuspid valve relative stenosis, whereas the addition of BCPS can shunt part of the vena cava reflux and reduce right heart pressure, effectively avoiding the relative stenosis caused by surgery and thus improving postoperative tricuspid regurgitation. In addition, in children with suboptimal tricuspid anatomy, such as severe septal malformation, it is more difficult to reconstruct the taper and maintain the postoperative morphology.
There is still a lack of consensus on the indications for the addition of BCPS for Ebstein’s anomaly. Quinonez et al. suggested that BCPS should be performed in children with preoperative right heart insufficiency who are critically ill.11 Malhotra et al. suggested that BCPS should be performed in children with preoperative cyanosis (in quiet or after activity).12 In the present study, we performed BCPS with intraoperative reference to the septal valve after cone reconstruction. We chose whether to add BCPS intraoperatively with reference to the tricuspid valve condition and right ventricular function as reflected by esophageal ultrasound after cone reconstruction. In our experience, a severely dilated right ventricle (atrialized ventricle >50% of the right ventricle, right ventricular outflow tract obstruction due to anterior tricuspid valve abnormality), significant tricuspid valve morphology, and hemodynamic instability after stopping extracorporeal circulation are all high-risk factors for tricuspid valve reoperation and right heart failure. In the above children, regardless of the presence of right heart dysfunction or severe cyanosis, we recommend the addition of BCPS to keep TR below moderate and thus prevent postoperative right heart failure.
It is well known that upper ventricular circulatory system complications, collateral venous or pulmonary arteriovenous fistulas may occur after BCPS. numata et al. found that 1½ ventricular repair did not provide more effective circulatory volume as well as better cardiac function compared to Fontan.13 However, only 7 children in the BCPS group in our follow-up had facial swelling and no other significant symptoms. It is worth noting that 1½ventricular repair has been used in the treatment of many complex malformations with good intermediate and long-term follow-up results. This is consistent with our findings, and we believe that all patients with Ebstein malformation in childhood have the best indication for BCPS. In terms of long-term outcome, radical biventricular repair may still be considered in the future if the right ventricular condition is well developed at follow-up after BCPS.
In conclusion, although tapered reconstruction is an anatomical correction, our results show that tapered reconstruction alone cannot achieve therapeutic results if the child’s condition cannot tolerate the hemodynamic changes associated with normal anatomy. In younger children with Ebstein’s malformation, the incidence of reoperation and right heart failure after tapered reconstruction alone is high, and 1½ventricular repair should be performed as a routine additional procedure.