ATS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Masahiro Koh
Toshikatsu Yagihara
Hideki Uemura
Ikuo Hagino
Toru Ishizaka
Soichiro Kitamura
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Koh, M.
Right arrow Articles by Kitamura, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Koh, M.
Right arrow Articles by Kitamura, S.
Related Collections
Right arrow Congenital - cyanotic

Ann Thorac Surg 2006;81:1808-1816
© 2006 The Society of Thoracic Surgeons

Original article: Cardiovascular

Biventricular Repair for Right Atrial Isomerism

Masahiro Koh, MD a , Toshikatsu Yagihara, MD a , * , Hideki Uemura, MD b , Koji Kagisaki, MD a , Ikuo Hagino, MD a , Toru Ishizaka, MD a , Soichiro Kitamura, MD a

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Cardiothoracic Surgery, Royal Brompton Hospital, London, United Kingdom

Accepted for publication December 1, 2005.

* Address correspondence to Dr Yagihara, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan (Email: yagihara{at}hsp.ncvc.go.jp).

Presented at the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24–26, 2005.


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
BACKGROUND: Biventricular repair is often difficult to accomplish in patients with right atrial isomerism because of complex anomalous structures.

METHODS: Ten patients with right atrial isomerism underwent biventricular repair. Their ages ranged from 15 months to 21 years. The follow-up period ranged from 1 month to 21 years. The Fontan procedure was unsuitable in 7 patients. The atrioventricular valves were separated in 5 patients and common in 5. One patient who had severe right-sided atrioventricular valvular regurgitation required concomitant prosthetic valve replacement. Another patient with a hypoplastic intraventricular septum underwent ventricular septation. Nine patients had two balanced ventricles. A ventricular septal defect was enlarged for rerouting in 3 patients. All patients had anomalous venoatrial connections and required intra-atrial baffle rerouting. One with major aortopulmonary collateral arteries underwent staged unifocalization. Three had extracardiac conduit repair. The outcomes were compared with 97 patients who underwent the Fontan procedure.

RESULTS: There were 3 early deaths and 1 late death. Six survivors are in New York Heart Association functional class I or II. Two are free from medications. Two required reoperation owing to infection or prosthetic valve failure. At 1 year, cardiac index and systemic venous pressure were 3.2 ± 0.9 L · min–1 · m–2 and 6.6 ± 1.6 mm Hg, respectively. There were no significant differences in survival, freedom from arrhythmia, freedom from reoperation, or exercise tolerance between biventricular repair and the Fontan procedure.

CONCLUSIONS: Biventricular repair provided good long-term outcomes in patients with right atrial isomerism. In selected circumstances, biventricular repair is an acceptable alternative to the Fontan procedure.


   Introduction
Top
 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 Discussion
 References
 
Biventricular repair (BVR) is often difficult to accomplish in patients with right atrial isomerism because of complex anomalous structures [1]. Right atrial isomerism is generally associated with anomalous venoatrial connections, a common atrioventricular valve, unbalanced ventricular structures, and pulmonary outflow tract obstruction [2–4]. Biventricular repair was used frequently in the past, because of suboptimal outcome of the Fontan procedure [5–7]. With recent modifications to the Fontan strategy, the outcome of the Fontan procedure has improved, and BVR for right atrial isomerism has become infrequent.

In this retrospective study, we compared our experience with BVR in 10 patients with right atrial isomerism and our experience with the Fontan procedure in 97 patients. Our goal was to clarify the long-term outcome and to determine the current indications for BVR in right atrial isomerism.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 Discussion
 References
 
Patients' Profiles
We defined right atrial isomerism as having bilaterally right pattern of lung, bronchus, and appendage, in particular the internal features of atrial appendages [2]. We retrospectively reviewed 107 patients with right atrial isomerism who underwent definitive repair between 1981 and 2004, BVR in 10 and the Fontan procedure in 97 (Fig 1). The Institutional Review Board approved this study.


Figure 1
View larger version (19K):
[in this window]
[in a new window]
  		Fig 1. Number of cases of definitive repair for right atrial isomerism. (Open bars = survived; shaded bars = hospital death; pt = patient.)

 
The ages at BVR ranged from 15 months to 21 years old. Seven were male and 3 were female. The morphologic findings are summarized in Figure 2. All patients had a common atrium, but only 1 patient had a relatively normal pattern of venous connections in which the systemic and the pulmonary venous connections were completely separated (patient 1). Including this patient, 3 had atrioventricular concordance-like venous connections, in which the majority of the systemic veins connected to the atrial side ipsilateral to the morphologic right ventricle (RV) and the pulmonary veins connected to the other atrial side ipsilateral to the morphologic left ventricle (LV) (patients 1, 2, and 3). The remaining 7 had complex venous connections. The pulmonary veins were abnormally connected through an extracardiac pathway in 4. Of note, 1 patient had no common pulmonary venous chamber behind the atrium (patient 10). Nine patients had two balanced ventricles. One had a hypoplastic intraventricular septum but both apical components were present (patient 6). The atrioventricular valves were separated in 5 and common in 5. All common atrioventricular valves were classified as Rastelli type C. One had severe regurgitation through a right-sided atrioventricular valve (patient 5) and 2 had moderate regurgitation through a common atrioventricular valve (patients 2 and 10). Regarding ventriculoarterial connection, a solitary outlet of the aorta arising from the morphologic RV with pulmonary atresia in 3, ventriculoarterial discordance with pulmonary stenosis in 1, double outlet right ventricle with pulmonary stenosis in 5 and double outlet right ventricle without pulmonary stenosis in 1. Associated anomalies were multiple muscular-type ventricular septal defects in 1 (patient 2), major aortopulmonary collateral arteries with abnormal arborization of the pulmonary arteries in 1 (patient 3), and hypoplasia of the pulmonary arteries in 2 (patients 6 and 10).


Figure 2
View larger version (40K):
[in this window]
[in a new window]
  		Fig 2. Patients' clinical data. (Ao from RV = solitary outlet of the aorta arising from the right ventricle; AV valve = atrioventricular valve; 2AVV = separated atrioventricular valves; CAVV = common atrioventricular valve; CAVVR = common atrioventricular valvular regurgitation; DORV = double outlet right ventricle; IVC = inferior vena cava; IVS = interventricular septum; MAPCAs = major aortopulmonary collateral arteries; PA = pulmonary atresia or pulmonary artery; PAP = pulmonary arterial pressure; PS = pulmonary stenosis; PV connection = pulmonary venous connection; PVR = pulmonary vascular resistance; P & W = peel and wrap; SP shunt = systemic-pulmonary artery shunt; SVC = superior vena cava; TAPVC = total anomalous pulmonary venous connection; TR = tricuspid valvular regurgitation [right-sided AV valvular regurgitation]; UF = unifocalization; VA connection = ventriculoarterial connection; VSD = ventricular septal defect.)

 
Previous Palliation
Five patients had previous palliative procedures including systemic-pulmonary artery shunting in 4, Glenn anastomosis in 1, and repair of an extracardiac-type totally anomalous pulmonary venous connection in 1.

Indication for the Fontan Procedure
We deemed that the Fontan procedure was absolutely contraindicated in 7. The major reason was elevated pulmonary vascular resistance in 6 and poor pulmonary vascular segments combined with major aortopulmonary collateral arteries in 1. One patient with severe pulmonary obstructive disease was in critically ill condition preoperatively, requiring mechanical ventilation and inotropic support (patient 8). The Fontan procedure was considered a possible option in 3 (patients 2, 4, and 5).

Preparative Procedures for BVR
Two patients underwent systemic-pulmonary artery shunting before BVR for the purpose of increasing the ventricular volume to facilitate intraventricular rerouting (patients 2 and 4). One previously had conventional Glenn anastomosis and developed hypoplasia of the right pulmonary arteries and abundant collateral arteries to the right lung (patient 6). We performed a takedown of the Glenn anastomosis and systemic-pulmonary artery shunting through a median approach, followed by surgical division of the developed collateral arteries through a lateral thoracotomy, what we call the "peel and wrap" procedure [8, 9]. Two patients underwent staged reconstruction of the pulmonary arteries in preparation for future construction of the confluence of the pulmonary arteries at the time of BVR (patients 3 and 10). One with major aortopulmonary collateral arteries underwent staged unifocalization of the pulmonary arteries, which we reported in detail previously (patient 3) [10–12]. One with hypoplasia of the pulmonary arteries underwent intrapulmonary reconstruction of the pulmonary arteries using a heterologous pericardial roll through a lateral thoracotomy (patient 10) [13].

Operative Techniques
All patients were approached through a median sternotomy. Hypothermic extracorporeal circulation with cold antegrade crystalloid cardioplegia was used. A summary of the operative techniques is shown in Figure 3.


Figure 3
View larger version (48K):
[in this window]
[in a new window]
  		Fig 3. Operative techniques and outcomes. (2-patch = two-patch repair; AT = atrial tachyarrhythmia; AV valve = atrioventricular valve; ECP = endocardial cushion prosthesis; ECR = extracardiac conduit repair; IE = infective endocarditis; MR = mitral valvular regurgitation (left-sided AV valvular regurgitation); NYHA = New York Heart Association functional class; PA = pulmonary artery; PVF = prosthetic valve failure; Reop = reoperation; RV = right ventricle; TAP = transannular patching; TAPVC = total anomalous pulmonary venous connection; TVR = tricuspid valve replacement; VSD = ventricular septal defect.)

 
Atrial septation
The atrial chambers were separated with a simple patch in 6 patients. Complex intra-atrial redirection of blood was required in the remaining 4 patients using a carefully designed baffle of glutaraldehyde-treated heterologous pericardium, as used in the Mustard procedure. With this maneuver, the systemic veins were promptly drained to the morphologic RV and the pulmonary veins to the LV in 3 patients (patients 4, 6, and 10). Therefore, these procedures might be categorized as a double switch operation for atrioventricular discordance with pulmonary valve stenosis or atresia [14]. In another patient, with a ventriculoarterial discordance-like venous connection, the systemic veins were redirected to the LV and the pulmonary veins to the RV from which the aorta originated (patient 5). Therefore, this was a functional BVR, in which the morphologic RV was used to support the systemic circulation [15]. Of the 3 patients with untreated extracardiac type totally anomalous pulmonary venous connection, 2 underwent anastomosis of the common pulmonary venous chamber and the atrium and ligation of the vertical vein (patients 7 and 9), and the third, without a common pulmonary venous chamber, underwent cutback enlargement of the stenotic vertical vein orifice (patient 10).

Atrioventricular valve repair
Three of 5 with a common atrioventricular valve underwent two-patch repair (patients 2, 9, and 10), and the remaining 2 had endocardial cushion prosthesis repair (patients 1 and 6) [6]. In 1 patient who had functional BVR, the right-sided atrioventricular valve that was supporting the systemic circulation was incompetent and was replaced with a mechanical valve (patient 5) [16].

Intraventricular rerouting or septation
An intraventricular channel was constructed between the morphologic LV and the aortic orifice using glutaraldehyde-treated heterologous pericardium in 5 patients with double outlet right ventricle and 3 that had a solitary outlet of the aorta arising form the RV with pulmonary atresia. To obtain an unobstructed channel, the ventricular septal defect was enlarged in 3 patients (patients 1, 2, and 7). In 1 patient who had a hypoplastic septum, septation and rerouting using an ePTFE (expanded polytetrafluoroethylene) patch was performed (patient 6).

Pulmonary outflow tract reconstruction
In 2 patients who previously had staged reconstruction of the pulmonary arteries using a heterologous pericardial roll, the confluence of the pulmonary arteries was constructed using another heterologous pericardial roll (patients 3 and 10) [13]. The pulmonary outflow tract was reconstructed using a handmade tri-leaflet conduit (what we call a "valved pericardial roll") in 4 patients (patients 3, 6, 7, and 10) [17]. An RV-pulmonary artery connection using interposition of autologous pericardium was accomplished in 1 (patient 4), transannular patching in 2 (patients 8 and 9), and pulmonary valvotomy with or without resection of the infundibular muscle in 2 (patients 2 and 5). One patient had an intact pulmonary valve and required no intervention in the pulmonary outflow tract (patient 1).

Comparison With the Fontan Procedure
The outcomes of BVR were compared with those of 97 patients with right atrial isomerism who underwent the Fontan procedure during the same period. All surviving patients in each group had periodic follow-up at our institution. Follow-ups ranged from 1 month to 21.7 years with a median of 8.6 years for BVR, and from 1 month to 16.8 years with a median of 3.9 years for the Fontan procedure. Routine postoperative catheterization studies were carried out in most cases at approximately 1 and 10 years after the operation, and catheterization data were compared between the two groups. Exercise tolerance was evaluated with a treadmill test and also compared between the two groups.

Data Analysis
Data are presented as the mean ± SD. The cumulative survival estimates were made by the Kaplan-Meier method. The log-rank test was applied for comparison between time-related variables. Differences of continuous variables were assessed by Student's unpaired t test. Values of p less than 0.05 were considered significant. All statistical tests were conducted with JMP 5.1.1 software (SAS Institute, Cary, North Carolina).


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 Discussion
 References
 
Survival
There were 3 early deaths and 1 late death. The main cause of early deaths was ventricular failure, which was complicated by residual left-sided atrioventricular valvular regurgitation (patients 2 and 8), residual muscular ventricular septal defects (patient 2), and pulmonary hypertension (patient 10). Seven patients survived BVR. In 1 (patient 4), an intra-atrial baffle infection developed and required exchange of the baffle 1 month after BVR; she survived the reoperation but died 1 year later of pneumonia. Survival after BVR, including 3 early deaths, was 70.0% ± 14.9% at 1 year, 60.0% ± 15.5% at 5 years, 60.0% ± 15.5% at 10 years, and 60.0% ± 15.5% at 15 years (Fig 4A). Currently, 6 patients are doing well. Four patients are in New York Heart Association (NYHA) functional class I (patients 1, 3, 5, and 7), and 2 of them are free from medication (patients 3 and 7). Two patients are in NYHA class II, 1 patient who underwent ventricular septation (patient 6) and 1 who developed left atrioventricular valvular regurgitation and atrial tachyarrhythmia (patient 9). All patients have resumed their routine activities.


Figure 4
View larger version (17K):
[in this window]
[in a new window]
  		Fig 4. Kaplan-Meier freedom-from-event curves. The solid lines represent patients with biventricular repair (BVR), and the dotted lines represent patients with the Fontan procedure. (A) Overall survival. (B) Freedom from arrhythmia. (C) Freedom from reoperation.

 
During the same period, 97 patients underwent the Fontan procedure. There were 24 early and 8 late deaths. The causes of early deaths were low output syndrome or Fontan failure in 11, mediastinitis in 4, airway or esophageal bleeding in 3, graft thrombosis in 2, arrhythmia in 2, pneumonia in 1, and pulmonary venous obstruction after totally anomalous pulmonary venous connection repair in 1.The causes of the late deaths were heart failure combined with a regurgitant common atrioventricular valve in 4, arrhythmia in 3, and airway obstruction in 1. The overall survival was 71.9% ± 4.6% at 1 year, 67.1% ± 4.9% at 5 years, 65.2% ± 5.1% at 10 years, and 65.2% ± 5.1% at 15 years. There was no significant difference in survival between the two groups (p = 0.83).

Arrhythmia
Atrial tachyarrhythmias developed in 2 of 7 BVR survivors. One patient suffered from persistent atrial tachyarrhythmia immediately after the operation and was medically controlled (patient 9). Another patient (patient 5) had atrial tachyarrhythmia 13 years after BVR. Freedom from arrhythmia in the BVR survivors was 85.7% ± 14.3% at 1, 5, and 10 years, and 57.4% ± 24.9% at 15 years (Fig 4B). Thirteen of 73 Fontan survivors had arrhythmia. Freedom from arrhythmia in the Fontan survivors was 94.0% ± 6.0% at 1 year, 81.5% ± 5.1% at 5 years, 78.2% ± 5.9% at 10 years, and 73.9% ± 7.0% at 15 years. There was no significant difference between the two groups (p = 0.82).

Reoperations
Of the BVR survivors, 1 patient required intra-atrial baffle exchange during the early postoperative period, as described above. Another patient (patient 5), who received a mechanical valve for replacement of the right-sided atrioventricular valve, required reoperation because of prosthetic valve failure 8 years later. Of the Fontan survivors, 5 required reoperation: replacement of the common atrioventricular valve in 2, conversion to extracardiac grafting in 1, construction of the confluence of the pulmonary arteries in 1, and reconstruction of the pulmonary artery obstruction in 1. Again, there was no significant difference between BVR and the Fontan procedure (p = 0.41; Fig 4C).

Catheterization Data
Seven BVR and 63 Fontan survivors underwent catheterization 1 year after the definitive repair (Fig 5). Cardiac index was significantly greater (p = 0.032) and systemic venous pressure was significantly lower (p = 0.005) in the BVR patients compared with the Fontan patients. Similarly, catheterization data 10 years after the definitive repair were compared between 3 BVR and 21 Fontan survivors. Cardiac index was significantly greater in the BVR patients (p = 0.042).


Figure 5
View larger version (25K):
[in this window]
[in a new window]
  		Fig 5. Comparison of catheterization data. The empty circles represent patients with the Fontan procedure, and the full circles represent patients with biventricular repair (BVR). (A) One year after the surgery. (B) Ten years after the surgery. Bars represent mean ± SD.

 
Exercise Tolerance
Five BVR and 27 Fontan survivors underwent exercise testing. There was no significant difference in the anaerobic threshold and oxygen uptake between the two groups (Fig 6).


Figure 6
View larger version (21K):
[in this window]
[in a new window]
  		Fig 6. Comparison of exercise tolerance. The empty circles represent patients with the Fontan procedure, and the full circles represent patients with biventricular repair. Bars represent mean ± SD. (VO2= oxygen consumption.)

 

   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
Discussion
 References
 
To accomplish BVR, two balanced ventricles are required, each with an adequate volume and function [18]. Separated atrioventricular valves and separated venoatrial connections are also preferable for BVR. In general, the majority of patients with right atrial isomerism have multiple anomalous structures that are unfavorable for BVR [1–4].

Thus far, we have performed 107 definitive repairs for right atrial isomersim, BVR in 10 and the Fontan procedure in 97. Morphologic findings in these 107 patients showed that 12 (11.2%) had two balanced ventricles (Fig 7). Only 4 (3.8%) had two balanced ventricles with separated atrioventricular valves and we selected BVR for these 4 patients. There was no patient with an ideal structure for BVR that included two balanced ventricles, separated atrioventricular valves, and separated venoatrial connections.


Figure 7
View larger version (20K):
[in this window]
[in a new window]
  		Fig 7. Venn diagram depicting associated anomalous structures in right atrial isomerism. Numbers without underlining represent biventricular repair (BVR), and numbers with underlining represent the Fontan procedure. (2AVV = separated atrioventricular valves.)

 
We promoted BVR aggressively from the 1980s to the early 1990s, since at that time the short-term use of the Fontan procedure was considered unsatisfactory (Fig 1) [5–7]. Particularly in patients with right atrial isomerism, unfavorable factors for the Fontan strategy are common, including anomalous venoatrial connections often accompanied by obstruction of the pulmonary venous drainage and hypoplasia of the pulmonary arteries [19, 20]. However, recent modifications of the surgical technique have improved the outcome of the Fontan procedure [21, 22]. A staged strategy using the bidirectional Glenn procedure, the concept of total cavopulmonary connection, creation of a fenestration, and techniques eliminating the use of cardiopulmonary bypass have been helpful to improve the short-term outcome of the Fontan procedure [23–25]. In our own experience, there were 24 early deaths in 97 patients who had the Fontan procedure and the overall operative mortality was 24.7%. In the latter half of the series since 1995, however, mortality decreased to 13.0% (6 of 46). The modern Fontan strategy is advantageous in terms of short-time survival in comparison with the more complex BVR.

It has not yet been determined that BVR is always preferable to the Fontan operation [26]. Biventricular repair has the potential advantage of maintaining normal biventricular physiology, which theoretically leads to better exercise tolerance, lower systemic venous pressure, and potentially improved long-term outcome [27, 28]. In contrast, analysis of long-term outcome of the Fontan procedure shows that univentricular physiology is obviously palliative [29].

Our data revealed that the long-term outcome of BVR was equivalent to the outcome of the Fontan procedure in terms of survival, arrhythmia, and reoperation. Catheterization showed that BVR provided better hemodynamics with lower systemic venous pressure than the Fontan procedure. We could not find any advantage of BVR in terms of exercise tolerance. Possible reasons for impaired exercise tolerance are residual atrioventricular valvular regurgitation or advanced age at the time of the definitive repair [27, 28]. The present retrospective study is clearly inconclusive, as there were a small number of patients and each group had a different background. However, it is notable that the majority of the BVR patients in this study were contraindicated for the Fontan strategy. Biventricular repair was their only definitive surgical option, in spite of the presence of several unfavorable structures for BVR.

In general, the strategy for BVR often conflicts with the Fontan strategy. When the ventricular volume is too small to function as two ventricles, and to create an unobstructed intraventricular channel, systemic-pulmonary artery shunting is a possible preparative procedure for the purpose of increasing ventricular volume. However, it might result in deterioration of atrioventricular valve function, progression of pulmonary vascular obstructive disease, or distortion of the pulmonary arteries, which are undesirable situations for the Fontan procedure [30].

Currently, when we encounter infants with right atrial isomerism, we consider the majority Fontan candidates because of their anomalous structures. Palliative procedures including surgery and catheter intervention are planned with special care not to deviate from the Fontan strategy. The Fontan strategy has been applied to an increasing number of patients. Actually, we have not had a candidate suitable for BVR since 1998 (Fig 1). However, there might be situations in which BVR is the only definitive option because of unsuitable factors for the Fontan strategy. We believe that BVR is still worth considering as an option for definitive repair particularly in patients who have two balanced ventricles and deviate from the Fontan strategy. In the event that a patient has ideal structures for both BVR and the Fontan procedure, which is extremely rare, we recommend BVR as the method for definitive repair.

In conclusion, although rather extensive, BVR provided equivalent long-term outcomes compared with the Fontan procedure in patients with right atrial isomerism. In selected circumstances with suitable morphologic features and particularly with advanced pulmonary vascular obstructive disease, BVR should still be considered for definitive repair in patients with right atrial isomerism.


   Discussion
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
References
 
DR CHRISTO I. TCHERVENKOV (Montreal, PQ, Canada): I very much enjoyed your presentation. This is obviously a very difficult group of patients if one considers biventricular repair.

I would like for you to elucidate a few additional factors that may be of importance in these patients. You didn't mention anything about the ventriculoarterial connections, and obviously that may be of importance if you have a double outlet right ventricle, how you achieve the biventricular repair.

The second issue is, that when we consider biventricular repair the best scenario, if you can connect the morphologic left ventricle to the systemic circulation. So I would be interested in the outcome of patients with a systemic right ventricle when compared with patients with systemic left ventricle.

And finally, what do you think might be the role of ventricular septation in these patients? It appears that 1 of the 7 patients with a single morphologic right ventricle had a ventricular septation. How did you make that decision in that patient? The first question is, what were the ventriculoarterial connections? How many patients had double outlet right ventricle or transposition?

DR KOH: In 7 patients of this series, 4 patients had a double outlet right ventricle or ventriculoarterial discordance with pulmonary stenosis, 2 patients had solitary outlet of the aorta arising from the right ventricle, and 1 patient had double outlet right ventricle with intact pulmonary valve.

DR TCHERVENKOV: The second question is, how did the patient have the morphologic left ventricle as a systemic pump after the repair compared with the ones that had the morphologic right ventricle?

DR KOH: Only 1 patient underwent functional biventricular repair. Because of the small number of cases, we didn't conduct comparison of these two groups. The patient having functional biventricular received concomitant prosthetic valve replacement of the right-sided atrioventricular valve, which was to be placed in the systemic circulation. He required reoperation owing to prosthetic valve failure 8 years later; however, he is currently doing well and in NYHA functional class 1.

DR TCHERVENKOV: And my final question is regarding the role of ventricular septation. One of the 7 patients seems to have had that.

DR KOH: We chose ventricular septation in 1 patient. This patient had a biventricular component, and the ventricular septum was very hypoplastic. He previously underwent conventional Glenn procedure and developed hypoplasia of the pulmonary arteries. The Fontan strategy seemed to be impossible because of elevated pulmonary vascular resistance. We carried out takedown of the Glenn anastomosis and constuction of systemic-pulmonary artery shunt followed by biventricular repair.

DR TCHERVENKOV: This looks like a common right ventricular morphology, at least the way it's drawn on the picture.

DR KOH: That is not true. This patient is categorized as common ventricle with hypoplastic intraventricular septum.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 

  1. Uemura H, Yagihara T. Surgical treatment in patients with isomeric atrial appendagesIn: Jacoub M, Carpentier AF, editors. Annual of cardiac surgery. 10th ed.. London: Rapid Science; 1997. pp. 105-112.
  2. Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy Ann Thorac Surg 1995;60:561-569.[Abstract/Free Full Text]
  3. Uemura H, Anderson RH, Ho SY, et al. Left ventricular structures in atrioventricular septal defect associated with isomerism of atrial appendages compared with similar features with usual atrial arrangement J Thorac Cardiovasc Surg 1995;110:445-452.[Abstract/Free Full Text]
  4. Uemura H, Ho SY, Anderson RH, Yagihara T. Ventricular morphology and coronary arterial anatomy in hearts with isometric atrial appendages Ann Thorac Surg 1999;67:1403-1411.[Abstract/Free Full Text]
  5. Pacifico AD, Ricchi A, Bargeron Jr LM, et al. Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies Ann Thorac Surg 1988;46:645-651.[Abstract]
  6. Kawashima Y, Matsuda H, Naito Y, Yagihara T, Kadoba K, Matsuki O. Biventricular repair of cardiac isomerism with common atrioventricular canal with the aid of an endocardial cushion prosthesis J Thorac Cardiovasc Surg 1993;106:248-254.[Abstract]
  7. Hirooka K, Yagihara T, Kishimoto H, et al. Biventricular repair in cardiac isomerism. Report of seventeen cases J Thorac Cardiovasc Surg 1995;109:530-535.[Abstract/Free Full Text]
  8. Yoshida M, Yagihara T, Uemura H, Yamashita K, Kawahira Y, Yoshizumi K. A successful biventricular repair in an adult case with "common ventricle" and isomeric atrial appendages previously undergoing the conventional Glenn procedure Jpn J Thorac Cardiovasc Surg 1998;46:1177-1181.[Medline]
  9. Matsuki O, Yagihara T, Kawashima Y. A new surgical approach for preparation of candidates for the Fontan operation with acquired systemic-pulmonary collateral arteries Cardiol Young 1995;5122–12.
  10. Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Staged unifocalization and anatomic repair in a patient with right isomerism Ann Thorac Surg 2001;71:2039-2041.[Abstract/Free Full Text]
  11. Yagihara T, Yamamoto F, Nishigaki K, et al. Unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries J Thorac Cardiovasc Surg 1996;112:392-402.[Abstract/Free Full Text]
  12. Ishizaka T, Yagihara T, Yamamoto F, et al. Results of unifocalization for pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteriespatency of pulmonary vascular segments. Eur J Cardiothorac Surg 1996;10:331-337.[Abstract]
  13. Uemura H, Yagihara T, Kawashima Y, Yamashita K, Kamiya T. Intrapulmonary reconstruction of pulmonary arteries using a heterologous pericardial roll Ann Thorac Surg 1995;59:1464-1469.[Abstract/Free Full Text]
  14. Yagihara T, Kishimoto H, Isobe F, et al. Double switch operation in cardiac anomalies with atrioventricular and ventriculoarterial discordance J Thorac Cardiovasc Surg 1994;107:351-358.[Abstract/Free Full Text]
  15. Koh M, Yagihara T, Uemura H, Kagisaki K, Kitamura S. Functional biventricular repair using left ventricle–pulmonary artery conduit in patients with discordant atrioventricular connections and pulmonary outflow tract obstruction. Does conduit obstruction maintain tricuspid valve function? Eur J Cardiothorac Surg 2004;26:767-772.[Abstract/Free Full Text]
  16. Numata S, Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y, Kitamura S. Replacement of the morphologically tricuspid valve in children with discordant atrioventricular connections J Heart Valve Dis 1999;8:649-654.[Medline]
  17. Koh M, Yagihara T, Uemura H, et al. Long-term outcome of right ventricular outflow tract reconstruction using a handmade tri-leaflet conduit Eur J Cardiothorac Surg 2005;27:807-814.[Abstract/Free Full Text]
  18. Freedom RM, Van Arsdell GS. Biventricular hearts not amenable to biventricular repair Ann Thorac Surg 1998;66:641-643.[Abstract/Free Full Text]
  19. Humes RA, Feldt RH, Porter CJ, Julsrud PR, Puga FJ, Danielson GK. The modified Fontan operation for asplenia and polysplenia syndromes J Thorac Cardiovasc Surg 1988;96:212-218.[Abstract]
  20. Uemura H, Yagihara T, Kawashima Y, et al. What factors affect ventricular performance after a Fontan-type operation? J Thorac Cardiovasc Surg 1995;110:405-415.[Abstract/Free Full Text]
  21. Mainwaring RD, Lamberti JJ. Extracardiac conduit Fontan for children with heterotaxy and functionally single ventricle Cardiol Young 1998;8:479-485.[Medline]
  22. Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome Ann Thorac Surg 2002;74:1967-1978.[Abstract/Free Full Text]
  23. Bridges ND, Jonas RA, Mayer JE, Flanagan MF, Keane JF, Castaneda AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results Circulation 1990;82(Suppl 5):170-176.
  24. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience J Thorac Cardiovasc Surg 1988;96:682-695.[Abstract]
  25. Uemura H, Yagihara T, Yamashita K, Ishizaka T, Yoshizumi K, Kawahira Y. Establishment of total cavopulmonary connection without use of cardiopulmonary bypass Eur J Cardiothorac Surg 1998;13:504-507.
  26. Delius RE, Rademecker MA, de Leval MR, Elliott MJ, Stark J. Is a high-risk biventricular repair always preferable to conversion to a single ventricle repair? J Thorac Cardiovasc Surg 1996;112:1561-1568.[Abstract/Free Full Text]
  27. Ohuchi H, Arakaki Y, Yagihara T, Kamiya T. Cardiopulmonary responses to exercise after repair of the univentricular heart Int J Cardiol 1997;58:17-30.[Medline]
  28. Uemura H, Yagihara T. Ventricular septation in patients with double inlet left ventricleIn: Redington AN, Brawn WJ, Deanfield JE, Anderson RH, editors. The right heart in congenital heart disease. London: Greenwich Medical Media; 1998. pp. 163-167.
  29. Fontan F, Kirklin JW, Fernandez G, et al. Outcome after a "perfect" Fontan operation Circulation 1990;81:1520-1536.[Abstract/Free Full Text]
  30. Shimazaki Y, Kawashima Y, Mori T, Kitamura S, Matsuda H, Yokota K. Ventricular volume characteristics of single ventricle before corrective surgery Am J Cardiol 1980;45:806-810.[Medline]



This article has been cited by other articles:


Home page
 Eur. J. Cardiothorac. Surg.Home page
J. H. Shuhaiber, S. Y. Ho, M. Rigby, and B. Sethia
Current options and outcomes for the management of atrioventricular septal defect
Eur. J. Cardiothorac. Surg., May 1, 2009; 35(5): 891 - 900.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
H.G. Lim, E.A. Bacha, G.R. Marx, A. Marshall, F. Fynn-Thompson, J.E. Mayer, P. Del Nido, and F.A. Pigula
Biventricular repair in patients with heterotaxy syndrome.
J. Thorac. Cardiovasc. Surg., February 1, 2009; 137(2): 371 - 379.e3.
[Abstract] [Full Text] [PDF]

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Masahiro Koh
Toshikatsu Yagihara
Hideki Uemura
Ikuo Hagino
Toru Ishizaka
Soichiro Kitamura
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Koh, M.
Right arrow Articles by Kitamura, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Koh, M.
Right arrow Articles by Kitamura, S.
Related Collections
Right arrow Congenital - cyanotic

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS