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Ann Thorac Surg 2009;87:1475-1483. doi:10.1016/j.athoracsur.2008.12.098
© 2009 The Society of Thoracic Surgeons
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Christopher A. Caldarone
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Right arrow Congenital - cyanotic

Original Articles: Pediatric Cardiac

Superior Vena Cava to Pulmonary Artery Anastomosis as an Adjunct to Biventricular Repair: 38-Year Follow-Up

Siho Kim, MDa, Osman Al-Radi, MDb, Mark K. Friedberg, MDb, Christopher A. Caldarone, MDb, John G. Coles, MDb, Erwin Oechslin, MDc, William G. Williams, MDb, Glen S. Van Arsdell, MDb,*

a Dong-A University Medical Center, Dong-A University, Busan, Korea
b Labatt Family Heart Centre, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
c Toronto Congenital Cardiac Center for Adults, Toronto General Hospital, Toronto, Ontario, Canada

Accepted for publication December 15, 2008.

* Address correspondence to Dr Van Arsdell, Divisions of Cardiovascular Surgery and Cardiology, Hospital for Sick Children Toronto, 555 University Ave, Toronto, Ontario, M5G 1X8, Canada (Email: glen.vanarsdell{at}sickkids.ca).

Presented at the Forty-fourth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.


PEDIATRIC CARDIAC SURGERY: The Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member or an individual non-member subscription to the journal.

 

   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Background: The working hypothesis for a one and a half ventricle repair has been that the benefits of a pulsatile pulmonary circulation may negate some of the late complications of the Fontan procedure. Those benefits are thought to outweigh the downside risk of having retrograde pulsatility in the superior vena cava. We sought to define the long-term fate of this strategy.

Methods: One hundred fourteen patients who underwent a superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair were identified for the years 1965 to 2003. Median follow-up was 92.3 months (range, 1 month to 38 years).

Results: The long-term outcome for operative survivors was 83.4%, 80.1%, and 69.3% at 5, 10, and 20 years, respectively. The survival in the most recent 10 years is 91.8% (p = 0.063). Of the late deaths, 69.6% (16 of 23) were known cardiac deaths or sudden. Patients with chronic right ventricular dysfunction demonstrated the best 10-year survival (91.6%). Of the late survivors, 98.8% of patients are in New York Heart Association class I or II. Arterial O2 saturation increased significantly from before to late after repair. (83.5% to 94.5%, p < 0.001; n = 82). Freedom from new atrial arrhythmia was 92.2% at 20 years. The superior vena cava to pulmonary artery anastomosis was taken down in 3. There was no patient with clinically evident protein-losing enteropathy.

Conclusions: The most common cause of late mortality is cardiac. Atrial and ventricular arrhythmias occur, but no protein-losing enteropathy was identified. The serious complication risk related to pulsatility in the superior vena cava was 2.6%.

Since the 1989 manuscript description of a one and a half (11/2) ventricle repair by Billingsly and coworkers [1], numerous reports have shown successful expansion of the indications for this repair to include a variety of anomalies that feature a functionally or anatomically abnormal subpulmonary ventricle that was judged to be unable to cope with the entire pulmonary circulatory volume [2]. A superior vena cava (SVC) to pulmonary artery (PA) anastomosis, as an adjunct to biventricular repair, allows for subpulmonary ventricular volume unloading, partial ventricular augmentation of pulmonary blood flow, and full systemic saturation while maintaining a theoretic pulmonary/systemic blood flow ratio of 1. The working hypothesis has been that the benefits of a pulsatile pulmonary circulation may negate some of the late complications of the Fontan procedure and that those benefits outweigh the downside risk of having pulsatility constraints to SVC drainage [3, 4].

A number of reports have shown early to midterm outcomes for 11/2 ventricle repairs. In contemporary series, the early physiologic tolerance of reducing the volume load on the marginal subpulmonary ventricle is good. There also appears to be a limited likelihood of pulmonary arteriovenous malformations [5–9]. For patients with the most anatomically or functionally marginal subpulmonary ventricles, a restrictive atrial septal defect (ASD) has been utilized to facilitate the safety of the 11/2 ventricle repair approach. While physiologically helpful, the 11/2 ventricle repair is not considered complete until the ASD is closed.

Present long-term outcome data for 11/2 ventricle repairs are limited. First used at this institution in conjunction with a classic Glenn, the 11/2 ventricle repair has been performed for a period spanning 4 decades. In this report describing outcomes encompassing 38 years, we sought to define the incidence of late outcome complications for patients having an SVC-PA anastomosis as an adjunct to biventricular repair. Clinical outcomes and morbidities of arrhythmia, reoperation, saturation, functional class, and other items are identified.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 Discussion
 References
 
Study Population
A Division of Cardiovascular Surgery database search at the Hospital for Sick Children and Toronto Congenital Cardiac Center for Adults defined 114 patients receiving an SVC-PA anastomosis in association with biventricular repair with or without the use of an atrial level shunt. The search collection period was between September 1965 and November 2003. Our inclusion and exclusion criteria have been previously published [2]. Patients having an SVC-PA anastomosis as palliation alone or as a staging procedure to a Fontan operation were excluded. Approval for this study was obtained from the Hospital for Sick Children Research Ethics Board.

Early and late results were reviewed. The current status of surviving patients was ascertained by direct examination and chart review. Follow-up of hospital survivors who completed the biventricular repair ranged from 1 month to 38 years (median, 92.3 months [111.7 ± 88.7]). The operative ages (SVC-PA anastomosis) ranged from 3 months to 59 years (median, 4 years [12.2 ± 16.4]). There were 58 male patients.

These patients were classified into four groups (Table 1) on the basis of the indications for operation [2]. Table 2 lists the underlying diagnosis for all groups. There were associated anatomic defects requiring multiple repairs in each patient. Four hundred seventy-seven procedures were performed in these 114 patients. Of those, 213 were index operations [10] (meaning the primary operation for a given hospitalization) as assigned by the Risk Sdjustment in Congenital Heart Surgery (RACHS-1) system [11] (Fig 1). Two patients had diagnoses that were not ranked in the RACHS-1 system, who were excluded in Figure 1. Sixty-three patients underwent operations before the SVC-PA anastomosis, and 17 of the 63 (30.0%) had multiple procedures. Ninety-four had associated procedures at the time of SVC-PA anastomosis. Of these, 67 (71.3%) had multiple components to the procedure. Fifty patients had subsequent operations after the SVC-PA anastomosis, and 11 of these (22%) had multiple components to the associated procedure. The time span from the SVC-PA anastomosis to the biventricular repair ranged from 21 days to 361 months (median, 43.1 months [62.3 ± 67.3]).


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  		Table 1 Indications for Operation
 

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  		Table 2 Underlying Primary Diagnosis: Number of Patients and Group
 

Figure 1
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  		Fig 1. The proportions of total operative procedures are shown, categorized by the Risk Adjustment for Congenital Heart Surgery (RACHS-1) system.

 
Surgical Technique
Biventricular repair would normally be defined as complete septation with repair of all intracardiac defects. In this cohort, the SVC-PA anastomosis was performed in conjunction with biventricular repair in 67 patients, as a stage to biventricular repair in 32, and after biventricular repair in 15 patients. Atrial fenestrations were employed for surgical convenience (in the case of the early classic Glenn) or for those with lower functional capacity or size toward the lower limits of physiologic tolerance (n = 31). Refenestration after closing of an ASD was carried out in 2 patients (Table 3). Before 1989, 18 patients underwent thoracotomy to perform the SVC-PA anastomosis.


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  		Table 3 Follow-Up of Patent Foramen Ovale (PFO) and Fenestrated Atrial Septal Defect (ASD)
 
Statistics
The {chi}2 test with cross-table analysis and paired t test were applied for comparing and evaluating the operation results. The cumulative probability of patients' survivals and the hazard rates were estimated by the Kaplan- Meier method and life table. The effects of the era of surgery and patient's groups, on cumulative patient survival were assessed with log-rank tests. If a significant difference was identified, multiple pairwise comparisons were performed to specify between-group differences. Numbers are expressed as mean ± SD. Differences were considered statistically significant when a p value was less than 0.05. Data were analyzed with SPSS statistical software package (SPSS, Chicago, IL).


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 Discussion
 References
 
Early and Late Mortality
Mortality at the time of SVC-PA anastomosis was 6.1%. A total of 23 late deaths occurred after the SVC-PA anastomosis (20.2%). In terms of the causes of late deaths (n = 23), there were 16 (69.6%) cardiac-related deaths including 2 sudden deaths. Three were caused by severe ventricular arrhythmia. Concurrent illness contributed to 4 late deaths (17.4%). The causes of 3 deaths (13.0%) are unknown. Subsequent operation performed after the SVC-PA anastomosis (48 of 114, 42.1% [for example, biventricular repair (n = 32), tricuspid valve replacement, pace maker insertion and others]) was associated with increased the risk of late death (p = 0.001, {chi}2 test with cross table).

Reoperation
Three takedowns of the SVC-PA anastomosis were done 4 months, 10 months, and 2 years after the operation. One had failed medical and surgical management of chylothorax, and another child had an aneurysmal dilatation of the left SVC-PA anastomosis. The aneurysm was 1.8 by 2.2 cm and necessitated resection and ligation 10 months after the repair [12]. The third had facial swelling with symptoms like SVC syndrome.

One patient with a SVC-PA classic Glenn was revised to a bidirectional cavopulmonary connection 7 years after the previous operation because of desaturation pertaining to azygos runoff. No concomitant pulmonary arteriovenous malformation was identified. Including this case, there were 2 delayed creations of an interatrial fenestration during the subsequent procedures (Table 3). The other patient underwent delayed creation of an ASD owing to long-standing high pulmonary pressure and right-side heart failure 2 years after the primary operation. One child with corrected transposition of great arteries underwent a double switch operation accompanying the SVC-PA anastomosis and then received a heart transplantation about 2 years after the biventricular repair because of ventricular failure.

Analysis of Survival and Complications
Figure 2 demonstrates Kaplan-Meier survival curves for the 104 hospital survivors after the SVC-PA anastomosis and biventricular repair with or without an associated ASD (Fig 2A). Era survival curves before and after 1996 are seen in Figure 2B. Three takedowns of the SVC-PA anastomosis were excluded. The overall cumulative survival rates were 83.4%, 80.1%, and 69.3% at 5, 10, and 20 years. The 10-year survival rate of the two eras were 74.9% and 91.8% (p = 0.063).


Figure 2
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  		Fig 2. (A) Kaplan-Meier curve for cumulative survival of the 105 late survivors. (B) Kaplan-Meier curve for cumulative survival according to the operative era. Numbers in parentheses indicate the actual number of patients at a given time point. aThe 9 operative deaths of subsequent operations were included.

 
Long-term survival by operative indication and timing of biventricular repair is shown in Figure 3. Group B with chronic right ventricular dysfunction demonstrated a 10-year survival rate of 91.6%. Most of these had an Ebstein anomaly (Table 2). Group B had improved overall survival as compared with group A.


Figure 3
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  		Fig 3. (A) Kaplan-Meier curve for cumulative survival according to the groups. (B) Kaplan-Meier curve for cumulative survival according to the timing of biventricular repair. Numbers in parentheses indicate the actual number of patients at a given time point. aAnalysis of survival difference between groups by pairwise comparing. bThe 9 operative deaths of subsequent operations were included. (RV = right ventricle; SVC-PA = superior vena cava to pulmonary artery.)

 
Timing of Biventricular Repair
Patients who underwent biventricular repairs after the SVC-PA anastomosis (n = 32) showed relatively poor long-term outcome compared with other groups (p = 0.008, p < 0.001) with 16 late deaths. Of those, 12 patients (75%) underwent biventricular repair before 1996. The survival rates were 51.6% at 10 and 20 years. Most late deaths occurred within 2 years after the biventricular repair (Fig 3B).

Freedom From Long-Term Complications
Figure 4 demonstrates freedom from headache, defined as a headache or a migraine that was noted in the clinical records with or without medication (20 years, 88.9%). Freedom from atrial arrhythmia was 92.2% at 20 years. Freedom from other long-term complications including the takedown of the SVC-PA anastomosis, a chylothrax, a permanent pacemaker insertion, endocarditis, an SVC aneurysm, and heart transplantation was analyzed and is demonstrated in Table 4.


Figure 4
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  		Fig 4. (A) Freedom from a headache. (B) Freedom from an atrial arrhythmia. The operative deaths were included. Numbers in parentheses indicate the actual number of patients at a given time point.

 

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  		Table 4 Freedom From Other Postoperative Complications
 
Atrial Septal Defect
The fate of the surgical ASDs (n = 31), recreated ASDs (n = 2), and patent foramen ovales (n = 17) of thoracotomy cases is shown in Table 3. Echocardiographic review demonstrated that 18 (58.1%) of 31 ASDs left in open surgery had been closed (n = 11) or sponataneously closed (n = 7) in a median follow-up period of 40.2 months. One was closed along with a takedown of the previous SVC-PA anastomosis owing to intractable chylothorax. Of the 18 thoracotomy case patent foramen ovales, 7 were closed surgically at some point (Table 3). The probability of keeping an interatrial shunt from a surgical creation decreased from 45.2% in 5 years to 7.5% in 10 years (follow-up n = 25; Fig 5). There was no incidence of cerebrovascular accidents related to ASDs.


Figure 5
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  		Fig 5. The probability of keeping an interatrial shunt of the fenestrated atrial septal defect (ASD) cases. Numbers in parentheses indicate the actual number of patients at a given time point. a There were 8 follow-up losses.

 
Current Clinical Status
Current clinical problems are shown in Table 5. Several symptoms and signs were related to the SVC-PA anastomosis. Three patients (patients 1, 3, and 6) have intermittent headaches and upper body swelling. An aneurysmal dilatation of the main pulmonary artery and a mild SVC syndrome without headache were also found (patients 2 and 5). No clinically evident protein-losing enteropathy or pulmonary arteriovenous fistula were identified among late survivors.


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  		Table 5 Current Clinical Problems
 
Forty-five (53.6%) and 39 (46.4%) of 82 late survivors do not take cardiac medication or any medication, respectively. Currently 8 of the late survivors require antiarrhythmic medication, 5 of whom had tachyarrhythmia preoperatively. Of those, 4 had Ebstein's anomalies, with the remainder being pulmonary atresia with intact ventricular septum (Table 6).


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  		Table 6 Follow-Up of Arterial O2 Change
 
All late survivors' arterial O2 saturations were examined by a pulse oximetry (Table 6). The arterial O2 saturations increased significantly, compared with the preoperative saturation, in both total patients (n = 82; p < 0.001) and patients who were left with a fenestrated ASD (n = 33); (p < 0.001; Table 6). Seventy-two of the late survivors (85.7%) were in New York Heart Association (NYHA) class I and 11 (13.1%) were judged to be in NYHA class II. One patient was in NYHA class III (1.2%).

Ebstein's Anomaly and Pulmonary Atresia With Intact Ventricular Septum
Twenty-eight Ebstein's anomaly patients underwent a SVC-PA anastomosis as an adjunct to biventricular repair. Of the 28, 25 patients belonged to group B (poor right ventricle function) with the others being in group A (small right ventricle, n = 2) and C (facilitation of repair, n = 1; Table 2). Early (n = 1) and late deaths (n = 2) occurred in group B. All underwent a SVC-PA anastomosis before 1996. Cumulative 5-year and 10-year survival rates are 90.2% in both. Maze operations (n = 3) and cryoablation procedures (n = 6) were also performed in the Ebstein's anomaly patients. One patient underwent catheter ablation owing to newly developed atrial flutter 6 years after biventricular repair. Six patients had insertion of a permanent pacemaker during or after the operation.

Fourteen patients with pulmonary atresia with intact ventricular septum underwent a SVC-PA anastomosis. Twelve patients belonged to group A (small right ventricle), and the others were in group C (facilitation of repair). There were no operative deaths. Late death occurred in 2 patients. Cumulative 5-year and 10-year survival rates are 92.9% and 84.4%, respectively.


   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
Discussion
 References
 
Determining optimal repair strategy in a situation where one ventricle is functionally or anatomically marginal in size remains a challenge. The bias that a biventricular repair is always better than a single ventricle repair has been questioned by the finding that operative mortality for a complex biventricular repair can be higher than a single ventricle repair with similar anatomy [12]. Mortality outside of the operative window may also be higher in complex biventricular repairs, as demonstrated by era-independent late mortality in the Rastelli operation [14, 15]. For marginal left ventricles, a binary decision regarding the adequacy of the left ventricle is made. For marginal right ventricles, there is the opportunity to incrementally volume unload the right ventricle with a SVC-PA connection and a variable-sized ASD. The ASD component can be made to be adjustable so that optimal volume loading of the right ventricle can be adjusted after separation from cardiopulmonary bypass. A small to moderate ASD can later close on its own or be test occluded, and closed if appropriate, in the cardiac catheterization laboratory. As compared with the requirements of a marginal systemic ventricle, the 11/2 ventricle repair, for the subpulmonary ventricle, thus offers the considerable advantage of a variable volume load on the right while largely separating the systemic and pulmonary circulations [1, 2, 5, 16, 17].

The use of the physiologic concept of a SVC-PA anastomosis has been expanded to any lesion with an inadequate right ventricle extending from right ventricular hypoplasia, to right ventricular cardiomyopathy, or to situations where a biventricular repair is facilitated by a 11/2 ventricle repair (Fig 1, Table 2). As such, the anatomy and physiology for reports of 11/2 ventricle repairs is heterogeneous. Data on short-term outcomes has been favorable in the past 10 years. Although much is known of the fate of late biventricular repairs for various lesions (tetralogy of Fallot, atrial switch procedures, and others) [18, 19], and much is known about 20-year follow-up for single ventricle repairs [20–22], little is known about the long-term consequences of a 11/2 ventricle repair in terms of functional physiology.

As such, this report focuses on late functional physiology and the clinical outcomes for patients who survived 11/2 ventricle repairs (with or without the presence of a small ASD). An understanding of the long-term efficacy and consequences can therefore be made.

Late Mortality
Mortality outside of the operative window showed two distinct risk phases, with a higher mortality occurring within the first year and then a continuous but lower risk of mortality occurring throughout follow-up. This finding suggests that decision making regarding candidacy for the 11/2 ventricle repair is important. Once a steady physiologic state is reached, hazard rates for mortality are 5% and 0% at 10 and 20 years, respectively (hazard rate in life table).

Of note, a higher mortality was seen among patients who had an SVC-PA anastomosis palliation that was later followed by an intracardiac repair (Fig 3B). That most of these late deaths occurred within 1 year after the biventricular repair reinforces the importance of patient selection. It also correlates with era, suggesting that changes in patient care strategy may negate some of the outcome effects [13, 23, 24]. In our series, a 11/2 ventricle repair for a moderately dilated right ventricle with moderately reduced ventricular function (group B) was associated with the most favorable outcome. The majority of these patients had Ebstein's anomaly (n = 28; Fig 3A) [2, 6, 7, 25, 26].

Late Morbidity
Late morbidity of single and biventricular repairs has largely been defined by functional status, need for cardiac medication and atrial and or ventricular arrhythmia. Fontan physiology patients can also have protein-losing enteropathy, ascites, liver dysfunction, systemic venous thrombosis, or other complications [20–22, 27–29].

Aarrythmia and other
Freedom from arrhythmia among patients who did not have a preexisting arrhythmia was 92.2% at 20 years. Just under half of the late survivors take cardiac medications. Their functional status is NYHA I in 86%, with 1% being class III. One patient required a cardiac transplant for congenitally corrected transposition of great artery and heart failure.

SVC-related complications
Unique to the 11/2 ventricle repair (as compared with a biventricular or single ventricle repair) is the risk of complications related to reverse pulsatile flow in the SVC. A total of 3 patients (3 of 114, 2.6%) had a serious complication requiring takedown of the SVC (2 of them done for facilitation of biventricular repair). Five (4.4%) had a mild form of upper body swelling that did not require SVC-PA anastomosis revision.

ASD fenenstration
Based on clinical need or surgical strategy, an atrial fenestration was utilized in 33 patients. Overall probability of closure of the fenestrated ASDs or remaining patent foramen ovales increased significantly over time without any noticeable complication (Fig 5). By 10 years of follow-up, only 7.5% remained open. A closed atrial fenestration or closure of the atrial fenestration was associated with higher oxygen saturation. While it seems intuitive that greater use of an ASD with repair might allow for better outcome, our era-independent data demonstrate the opposite. A potential explanation is that the use of an atrial fenestration was a marker for less favorable anatomy that resulted in a poorer outcome.

Caveats of Study Design
This study was designed to provide a descriptive long-term outcome for 11/2 ventricle repairs. Comparative analysis to complex biventricular repairs or Fontan repairs has therefore not been done. The wide age range of anatomy and physiology in the study population and the small number of patients in each subgroup are additional limitations. The present data provide a starting point for future analysis that might include case-match control studies comparing Fontan patients, complex biventricular repair patients, and 11/2 ventricle repair patients.

In conclusion, long-term clinical results of a SVC-PA anastomosis as an adjunct to biventricular repair indicate that the procedure demonstrate ongoing late patient attrition that is cardiac related. More than 86% of patients are in NYHA class I, with an additional 13% in class II. Long-term patient evaluation should include follow-up for supraventricular arrhythmia and potential markers for risk of sudden death. Application of a 11/2 ventricle repair has not resulted in clinically evident protein-losing enteropathy. The complication risk related to the pulsatile SVC-PA connection was 7%. The serious SVC-PA connection risk, defined by need for anastomotic takedown, was 2.6%.


   Discussion
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
References
 
DR JOSEPH M. FORBESS (Dallas, TX): I have one question about the echoes of these patients long-term. Because the thing I've always wondered about with these sorts of patients is, and you've sort of answered it with the protein-losing enteropathy (PLE) issue, is that if their right atrial pressures are high and their atrial dimensions are large, would they be better served with a total cavopulmonary connection? In other words, are they better as a 11/2 ventricle repair if their right atrium resembles that of an atriopulmonary Fontan? Do you have any sense, or were you able to look at echoes of these patients at all to see if their atrial dimensions were abnormally large?

DR KIM: Thank you for your good question and comments, Dr Forbess. First of all, I have to tell you that, in this study, we tried to seek to define long-term fate of this strategy, especially late physiologic outcomes for those who survived 11/2 ventricle repairs. So I didn't follow up the preoperative and postoperative echocardiography data.

Preoperatively, as long as the right ventricles' functional and morphogical status were marginal and fell into our four indications, which I presented, regardless of their pressure and sizes, we selected an SVC-PA anastomosis as an adjunct to a biventricular repair instead of Fontan. Also, postoperatively, some patients presented with clinically significant complications and poor survival outcomes in our series. Maybe some of their atrial pressures and sizes were high and big. I suspect that some of them should have had Fontan rather than this procedure, like you said, sir. But, in term of late complications, our results are satisfactory without any PLE. However, as I mentioned in the conclusions, given that most late death occurred within 1 or 2 years after the operation, patient selection is still an important and cumbersome issue.

DR CARL L. BACKER (Chicago, IL): One question I have, and I may have missed this, but sometimes we're presented with a patient that is only going to be a candidate for a 11/2 ventricle repair if they also have a conduit from the right ventricle to the pulmonary artery. I always feel, and it's similar to the question that Joe asked, that maybe those patients would be better served with a simple cavopulmonary connection and then you can avoid the conduit change in the future. So the question is, how many of these patients who had the Glenn anastomosis also had a conduit from the right ventricle to the pulmonary artery as part of their correction?

DR KIM: Thank you for your question, Dr Backer. This series includes 21 RV-PA conduit procedures, of course, with Glenn anastomosis. Many of them fell into the indication C. Still it's very hard to tell you as to whether which was better option, especially when it comes to comparing to Fontan. Because they have different physiologic consequences after finishing surgical corrections and, actually different surgical histories, I mean, preoperatively, sir. I can give you just the numbers of our surgical results. Well, I'm not sure if this is a good answer for you. We have 9 double-switch patients with 3 late deaths.

DR FORBESS: These are Senning/Rastellis with a Glenn?

DR KIM: Basically, yes. But, in most cases, we performed Mustard operations instead of Senning.

DR FORBESS: And that's all of their SVC returns. Does that also include patients where one SVC might still be hooked to the systemic venous pathway but one cava is hooked directly to the pulmonary arteries, or do you know?

DR KIM: You are talking about double-switch procedures?

DR FORBESS: Yes. For Senning/Rastellis that may have bilateral SVCs, does that mean both SVCs are hooked to the pulmonary arteries or maybe just one?

DR KIM: Most of them belonged to the indication C, in other words, we hooked the SVC to the pulmonary artery to facilitate the biventricular repair.

DR SHUNJI SANO (Okayama, Japan): I have similar experience. But I always wonder whether these patients should have a Fontan or 11/2 ventricular repair, because the Fontan result is so good. And the question is, which operation has much better exercise tolerance? Do you have any data of exercise tolerance in patients with Fontan and the 11/2 ventricular repair?

DR KIM: Thank you for your question, Dr Sano. Unfortunately, I have no data about on the exercise tolerance. Because it was not in our routine check-ups, so only few patients had the results on that. To answer your question of which procedure is better, I can just show the numbers of our surgical and long-term clinical results. As a matter of fact, comparing a 11/2 procedure to Fontan was one of our study purposes in the first place, but soon after we started, it was found that it's impossible. They have different cohorts and many of whom fall into, so called, the grey zone. As Dr Caldarone gave us really a beautiful lecture on Saturday, it's hard to evaluate the surgical results of this group.

Most importantly, if you look at Fontan patients, their surgical histories were pretty simple, such as BT shunt, first, followed by Glenn shunt and then ended up Fontan. But if you go back to our cohort, most patients had various preoperative procedures and also had to have more complicated subsequent operations during or after the 11/2 procedures owing to corrections of their own primary defects. Those make a significant statistical bias in terms of the long-term clinical outcomes. So I think that it's difficult to compare this to Fontan until we have an ideal, prospectively well-designed study population.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 

  1. Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum J Thorac Cardiovasc Surg 1989;97:746-754.[Abstract]
  2. Van Arsdell GS, Williams WG, Maser CM, et al. Superior vena cava to pulmonary artery anastomosis: an adjunct to biventricular repair J Thorac Cardiovasc Surg 1996;112:1143-1149.[Abstract/Free Full Text]
  3. Björk VO, Olin CL, Bjarke BB, Thorén CA. Right atrial-right ventricular anastomosis for correction of tricuspid atresia J Thorac Cardiovasc Surg 1979;77:452-458.[Abstract]
  4. Gentles TL, Keane JF, Jonas RA, Marx GE, Mayer JE. Surgical alternatives to the fontan procedure incorporating a hypoplastic right ventricle Circulation 1994;90:1-6.[Free Full Text]
  5. Muster AJ, Zalles VR, Ilbawi MN, Backer CL, Duffy CE, Mavroudis C. Biventricular repair of hypoplastic right ventricleassisted by pulsatile bidirectional cavopulmonary anastomosis J Thorac Cardiovasc Surg 1993;105:112-119.[Abstract]
  6. Kreutzer C, Mayorquim RC, Kreutzer GO, et al. Experience with one and a half ventricle repair J Thorac Cardiovasc Surg 1999;117:662-668.[Abstract/Free Full Text]
  7. Van Arsdell GS, Williams WG, Freedom RM. A practical approach to 11/2 ventricle repairs Ann Thorac Surg 1998;66:678-680.[Abstract/Free Full Text]
  8. Reddy VM, McElhinney DB, Silvermann NH, Marianeschi SM, Hanley FL. Partial biventricular repair for complex congenital heart defects: an intermediate option for complicated anatomy or functionally borderline right complex heart J Thorac Cardiovasc Surg 1998;116:21-27.[Abstract/Free Full Text]
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