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Ann Thorac Surg 2006;82:146-152
© 2006 The Society of Thoracic Surgeons

Original article: Cardiovascular

Analysis of Surgical Outcome in Complex Double-Outlet Right Ventricle With Heterotaxy Syndrome or Complete Atrioventricular Canal Defect

Koh Takeuchi, MD a , d , * , Francis X. McGowan, Jr, MD b , Emile A. Bacha, MD a , John E. Mayer, Jr, MD a , David Zurakowski, PhD c , Masaki Otaki, MD a , b , c , d , Pedro J. del Nido, MD a

a Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts
b Department of Anesthesiology, Children's Hospital, Harvard Medical School, Boston, Massachusetts
c Department of Biostatistics, Children's Hospital, Harvard Medical School, Boston, Massachusetts
d Department of Cardiovascular Surgery, Saiseikai Kyotofu Hospital, Tokyo, Japan

Accepted for publication February 2, 2006.

* Address correspondence to Dr Takeuchi, Department of Cardiovascular Surgery, National Children's Hospital, Japan, 2-10-1 Ohkura, Setagaya, Tokyo, 157-8535, Japan (Email: koutakeuchi-circ{at}umin.ac.jp).


   Abstract
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 The Society of Thoracic...
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BACKGROUND: Double-outlet right ventricle encompasses a broad spectrum of anomalies. Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle.

METHODS: From January 1992 to May 1999, medical records of 96 patients (50 males, 46 females) who had complex double-outlet right ventricle with heterotaxy and/or complete atrioventricular canal defect were reviewed (median age at initial surgery 3 months). Seventeen patients were neonates requiring surgery. Follow-up ranged from 1 day to 7.4 years (median, 16 months).

RESULTS: Sixty-eight patients had heterotaxy syndrome (27 with total anomalous pulmonary venous connection). Eighty-three had complete atrioventricular canal defect, 22 with moderate to severe atrioventricular valve regurgitation at the time of surgical repair. Eight patients had two-ventricle repair, and 88 patients were considered for single-ventricle management (bidirectional Glenn, 37; Fontan, 44). One patient had heart transplantation after bidirectional Glenn. There were 16 deaths including 10 early (<30 days postoperatively). Overall survival (95% confidence interval) estimated by the Kaplan–Meier method was 89% (83% to 96%) at 1 month, 84% (76% to 91%) at 1 year, and 81% (73% to 89%) at 5 years. Multivariate analysis revealed that neonatal presentation requiring surgery (p < 0.0001), moderate to severe atrioventricular valve regurgitation (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death.

CONCLUSIONS: Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are risk factors for mortality in patients with complex double-outlet right ventricle. Early surgical intervention in symptomatic neonates and infants, including those with pulmonary venous obstruction, may reduce mortality and improve outcome after staged operation.


   Introduction
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Double-outlet right ventricle (DORV) encompasses a broad spectrum of anomalies. Certain anatomic forms of DORV require surgical approaches that are more closely related to those used for management of a single ventricle than to those used for the simple forms of DORV. Examples of these include hypoplastic right ventricle, inlet type of ventricular septal defect or unbalanced atrioventricular (AV) canal defect, and associated anomalies such as heterotaxy syndrome. The majority of patients with complex forms of DORV may ultimately be palliated by the modified Fontan procedure, although the mortality rate in the subgroup with associated heterotaxy syndrome has been reported to be high primarily because of the high incidence of total anomalous pulmonary venous connection (TAPVC) [1–3]. Associated complete AV canal defect (CAVC) has also been considered a risk factor for surgical repair of DORV.

This study was undertaken to assess the significance of associated lesions in complex forms of DORV on early and intermediate term mortality and the role of aggressive early surgical intervention, particularly with the management of TAPVC.


   Material and Methods
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Patients
From January 1992 to May 1999, 96 patients (50 males, 46 females) who had complex DORV with heterotaxy or CAVC defect and were admitted to Children's Hospital, Boston, Massachusetts, for surgery were eligible for entry into the study. Patients were retrospectively identified by review of the cardiac surgical, echocardiography, and cardiac catheterization laboratory databases. Assignment to the asplenia or polysplenia group was done according to the findings at echocardiography using criteria set forth by Van Praagh and colleagues [4]. The medical records were reviewed, and follow-up information was obtained from medical records and contact with referring physicians. Permission for review of the medical records was obtained from the Children's Hospital Institutional Review Board, although patient consent for this clinical study was waived.

Statistical Analysis
The following 21 variables were tested: sex, age at the time of operation, heterotaxy syndrome, CAVC defect, TAPVC, pulmonary venous obstruction, pulmonary stenosis, pulmonary atresia, coarctation of the aorta, hypoplastic aortic arch, AV valve regurgitation (AVVR) and discordance, criss-cross heart, straddling AV valve, double-chambered right ventricle, patent ductus arteriosus, atrial septal defect, subaortic stenosis, persistent left superior vena cava, dextrocardia, and year of operation. A backward likelihood ratio procedure was applied to build the multivariable model. Fisher's exact test was used to compare proportions. Estimated rates of survival were determined using the Kaplan–Meier product-limit method with 95% confidence intervals (CI) calculated by Greenwood's formula (as reported in [5]). The Cox proportional-hazards regression model was used to establish the variables independently associated with mortality, and risk was measured by the adjusted risk ratio [6]. Data were analyzed with the SPSS software package (version 10.1, SPSS Inc, Chicago, IL). All reported probability values are two-tailed.


   Results
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Between January 1992 and May 1999, 96 children (50 males, 46 females) with complex DORV (with heterotaxy or CAVC) were admitted to Children's Hospital, Boston, and underwent surgery. Median follow-up was 16 months (range, 1 day to 7.4 years). As depicted in Table 1, of these 96 children, 68 patients had heterotaxy syndrome, 83 patients had CAVC defect, including 37 patients with unbalanced form of AV canal defect, and 30 patients had TAPVC. Fifty-six patients had both heterotaxy syndrome and CAVC. Among those 56 patients, 26 patients had TAPVC. Twenty-seven patients of 68 patients with heterotaxy syndrome had TAPVC. Table 2 shows associated major anomalies. The pulmonary venous connection was supracardiac in 13 patients, cardiac in 6 patients, infracardiac in 5 patients, and mixed in the remaining 6 patients. All cardiac TAPVC drained into the coronary sinus. Obstructed pulmonary venous drainage was diagnosed clinically in 13 patients (43%). Pulmonary stenosis was found in 46 patients including 31 patients with severe pulmonary stenosis. A total of 15 patients had pulmonary atresia. Aortic coarctation with or without hypoplastic arch was found in 8 infants. Common AVVR was trivial to mild in 42 patients, moderate in 15 patients, and severe in 7 patients. Most children had more than one major associated defect. Atrioventricular discordance and criss-cross AV connections were found in 3 and 7 patients, respectively. Dextrocardia was found in 23 patients. Table 3 lists the minor associated anomalies. All potential risk factors including 19 anatomic variants, age, and year of surgery were statistically tested (Table 4), and only mild or worse AVVR and pulmonary venous obstruction were identified to be risk factors of mortality in anatomical variants.


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  		Table 1. Number of Patients With Major Associated Anomalies
 

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  		Table 2. Major Associated Anomalies
 

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  		Table 3. Minor Associated Anomalies
 

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  		Table 4. Predictors of Mortality
 
Median age at initial surgery was 3 months. Seventeen patients were neonates and symptomatic. Figure 1 depicts the outcome at last follow-up in all 17 neonates. Initial palliation alone was performed in 16 of the 17 neonates, including systemic to pulmonary shunt in 5, first-stage palliation for hypoplastic left heart syndrome (Norwood stage I) in 3 (in 1 neonate TAPVC was repaired concomitantly), and pulmonary artery banding in 1. The indications for surgical intervention and the operative procedures are listed in Table 5. Seven neonates with TAPVC presented with severe obstruction (infracardiac, 2; supracardiac, 3; mixed, 2) and underwent repair with the addition of a systemic to pulmonary shunt with 3 survivors. One neonate with DORV, CAVC, hypoplastic aortic arch, and transposition of the great arteries underwent repair with arterial switch operation, repair of CAVC, and aortic arch repair and died 16 days after surgery of overwhelming sepsis. Another 15 patients were not deemed candidates for two-ventricle repair. Six patients reached the modified Fontan operation, 5 of these patients had a bidirectional Glenn as an intermediate procedure, and 2 patients remained with bidirectional Glenn. Eight deaths including 1 death with two-ventricle repair were found in this subgroup of patients.


Figure 1
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  		Fig 1. Flowchart indicating the surgical management and outcome for the 17 neonates with complex double-outlet right ventricle (DORV). (ASO = arterial switch operation; AV plasty = atrioventricular valve plasty; BDG = bidirectional Glenn; B-T shunt = Blalock-Taussig shunt; PAB = pulmonary artery banding; TAPVC = total anomalous pulmonary venous connection.)

 

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  		Table 5. Operative Risk in Neonates by Indication for Surgery and Procedure
 
Figure 2 depicts the operative procedures and outcome at last follow-up in the 79 children older than 30 days at the time of first intervention. Seven patients older than 30 days underwent attempted biventricular repair. The procedures include intraventricular baffling in 4 (1 death), Rastelli operation in 2 (both alive), and Senning with intracardiac baffling in 1 (alive). Of the seventy-two patients older than 30 days who were not deemed candidates for two-ventricle repair, 38 reached the modified Fontan operation, and 14 of these had a bidirectional Glenn as an intermediate procedure toward the Fontan operation during the follow-up period. Thirty-five patients remained with bidirectional Glenn as an intermediate procedure. One patient underwent orthotopic heart transplantation after the bidirectional Glenn procedure as a result of severe ventricular dilatation and dysfunction. Takedown of the Fontan connection to a bidirectional Glenn was required in 1 patient who remains alive (Fig 2). Common AV valve repair or replacement was performed in 10 patients. Repair of TAPVC by direct connection of the pulmonary venous confluence to the atrium was carried out in 15 patients, including 6 patients with obstructed TAPVC.


Figure 2
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  		Fig 2. Flow chart indicating the surgical management and outcome for the 79 patients older than 30 days of age at initial repair. (AV plasty = atrioventricular valve plasty; AV replace = atrioventricular valve replacement; AVVP = atrioventricular valve plasty; BDG = bidirectional Glenn; B-T shunt = Blalock-Taussig shunt; DORV = double-outlet right ventricle; IVR = intraventricular repair; PA = pulmonary artery; PAB = pulmonary artery banding; TAPVC = total anomalous pulmonary venous connection.)

 
There were 16 deaths in the entire group of 96, including 10 deaths within 30 days of surgery. Eleven of the 16 patients who died during the follow-up period had heterotaxy syndrome; 5 of these had severely obstructed TAPVC. Complete AV canal defect was present in 15 patients who died, including 8 patients with unbalanced AV canal defect. The procedures performed in the 16 nonsurvivors included intraventricular repair with AV valve repair for 1; arterial switch with repair of aortic arch and CAVC in 1; repair of TAPVC and shunt insertion in 4; left pulmonary artery plasty in 1; AV valve repair in 1; systemic-to-pulmonary shunt in 1; bidirectional Glenn in 3; Fontan operation in 3; and Norwood stage I in 1.

Overall survival estimated by the Kaplan–Meier method was 89% (95% CI, 83% to 96%) at 1 month, 84% (95% CI, 76% to 91%) at 1 year, and 81% (95% CI, 73% to 89%) at 5 years. The Kaplan–Meier survival curve is presented in Figure 3. The 21 variables tested for significance in the univariate analysis and multivariate analyses are shown in Table 4. Multivariate analysis using the Cox model revealed that neonatal presentation requiring surgery (p < 0.0001), moderate or severe AVVR (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death. The monthly odds of death were estimated to be more than 13 times higher for neonates requiring surgery compared with patients operated on after 30 days of life (risk ratio, 13.9; 95% CI, 5.3 to 36.5; p < 0.0001). Patients with moderate or severe AVVR at the time of surgery were estimated to be more than four times more likely to die (risk ratio, 4.1; 95% CI, 1.6 to 14.8; p = 0.03). Patients with pulmonary venous obstruction were estimated to be more than four times more likely to die (risk ratio, 4.9; 95% CI, 1.3 to 17.4; p = 0.02). The year of surgery was also found to be a significant predictor inasmuch as more recent surgeries were associated with a better odds of patient survival (risk ratio, 0.7; 95% CI, 0.6 to 0.9; p = 0.007). None of the other variables tested including aortic arch hypoplasia or coarctation, pulmonary stenosis or atresia, subaortic stenosis, and TAPVC was identified to be a risk factor of mortality.


Figure 3
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  		Fig 3. Kaplan–Meier estimated overall survival based on all 96 patients through 5 years of follow-up. Each step in the curve denotes an event. Error bars denote the 95% confidence interval for estimated survival. Numbers shown in parentheses represent the patients who are alive and still being followed.

 

   Comment
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 Abstract
 Introduction
 Material and Methods
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 Comment
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In this study, we found that AVVR, pulmonary venous obstruction, and neonatal presentation, but not TAPVC, are independent risk factors for mortality in patients with complex DORV with heterotaxy and/or CAVC.

Double-outlet right ventricle encompasses a broad spectrum of anatomic variants and associated malformations. Heterotaxy syndrome, which is often associated with TAPVC and CAVC, has been considered a risk factor for surgical repair of DORV. The early and mid-term outcomes for children with functional single ventricle have markedly improved in recent years even in patients with heterotaxy syndrome [3]. Since the introduction of the Fontan procedure in the early 1970s, modifications of surgical techniques, such as total cavopulmonary connection with a lateral tunnel and tunnel fenestration [7], along with improved patient selection and perioperative management [8] have resulted in significant decreases in both early and mid-term mortality. The use of staged reconstruction with an intermediate bidirectional Glenn connection [9] has further reduced overall mortality. We prefer to create bidirectional Glenn connection before the Fontan procedure in most patients who have single-ventricle physiology to reduce postoperative morbidity and mortality. But this is not true for some patients, who can undergo straight Fontan with reasonable risk. In the later series of this study, bidirectional Glenn procedure became a more popular procedure than before. This might be an explanation for the number of straight Fontan patients.

Previous reports have suggested that the association of TAPVC with single ventricle, particularly in patients with heterotaxy syndrome, is a risk factor for poor outcome [10–12]. In a recent report, Gaynor and colleagues [13] suggested that factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure included greater than mild AV regurgitation, hypoplastic pulmonary arteries, and high pulmonary artery pressure after 6 months of age. They postulated that inadequate development of the pulmonary vasculature, especially the pulmonary veins, occurring even in children without clinical evidence of pulmonary venous obstruction, resulted in high early mortality and a continuing risk for late death. Their more recent report suggested an improvement of surgical result for patients with single ventricle and TAPVC [14]. Most of the patients in our study were managed as having single-ventricle physiology, and 68 patients had heterotaxy, with 27 of the 68 (40%) having TAPVC. Although we did not include all children with heterotaxy and single-ventricle physiology, our group of patients is similar to that reported by Gaynor and associates [13]. In a previous report from our institution, neither the presence of TAPVC nor TAPVC repair was found to be a definite risk factor for early death in newborn patients with TAPVC and visceral heterotaxy [11]. We did not find the presence of TAPVC to be a risk factor for mortality in this study, but obstruction of the pulmonary veins was a significant risk factor. Five of 13 patients with obstructed TAPVC died (mortality, 38%), and 4 patients were neonates. One explanation for the finding that the presence of TAPVC was not found to be a risk factor in our cohort may be the timing of surgical repair of TAPVC. Of the 30 children with TAPVC, all but 8 had the pulmonary veins connected to the atrium at a surgical procedure before the Fontan operation, with 1 death in the Fontan group (Fig 2). One may speculate that subtle degrees of obstruction result in elevated or labile pulmonary vascular resistance postoperatively in the children with unrepaired TAPVC at the time of the Fontan procedure. If so, then a more aggressive approach with early repair of anomalous pulmonary venous connection is warranted.

The presence of CAVC defect frequently limits the ability to achieve a two-ventricle repair, particularly when associated with unbalanced ventricular chambers [15]. Atrioventricular regurgitation is known to be a risk factor for Fontan operation [16]. The natural history of common AVVR in patients with functional single ventricle is poor. Moak and Gersony [16] reported that AVVR was a significant and specific complicating factor that developed in patients with single ventricle and common AV valve. In a similar report with 242 patients undergoing a Fontan procedure including 99 patients with AVVR, Imai and associates [17] reported a significantly higher mortality in children who had AVVR compared with those without AVVR, even if valve repair was attempted. In our study, 64 patients had mild or worse valve regurgitation including 22 patients with moderate or severe AVVR. Eleven of the 22 patients with greater than mild regurgitation underwent AV valve surgery, including 2 patients requiring valve replacement. Seven of 11 patients who underwent atrioventricular valve repair or replacement died after the surgery. Two patients died of ventricular failure before Fontan palliation and another patient who also did not undergo Fontan procedure died on postoperative day 26, probably related to the high pulmonary vascular resistance. But other patients died of variable diseases including late cardiac tamponade, thrombosed valve, sepsis, and intracranial hemorrhage. Clearly, 2 deaths were related to ventricular dysfunction and another to high pulmonary vascular resistance. Younger children, particularly neonates who have high pulmonary vascular resistance, may rapidly progress to obstructive pulmonary disease. Imai and associates [17] reported the same findings as ours that AVVR would be risk factor for death even if it was repaired. They also found that ventricular dysfunction was not rare after repair of AV valve. Although we do not have a clear explanation for this, ventricular dysfunction may be related to relative increase of afterload and reduced ventricular volume. We found that moderate to severe AVVR was a significant risk factor of mortality with 7 deaths among the 22 children having valve repair alone or in conjunction with other surgical procedures.

Another independent risk factor for mortality found in this study is symptomatic neonatal presentation with 7 deaths early and 1 late in the group of 17 newborns. The observation that 4 of the 7 children with obstructed pulmonary venous return died early after surgery reflects the difficulty in regulating pulmonary blood flow with shunt-dependent pulmonary circulation and labile pulmonary vascular resistance and pulmonary edema. Conversely, the fact that the other 3 neonates with obstructed TAPVC, 1 of whom also had a Norwood procedure, are long-term survivors suggests that early surgical intervention to palliate all the critical anatomic defects may be warranted.

This study has several limitations. It is retrospective, and the data are limited by the review of the medical records. To ensure inclusion of all patients, we reviewed multiple sources including the echocardiography, surgical, and cardiac catheterization databases.

In conclusion, this study demonstrates that children having complex forms of DORV including heterotaxy and/or CAVC defect can be treated with reasonable risk for surgery. Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are independent risk factors for mortality. Although high mortality was seen in symptomatic neonates even with surgical intervention, the monthly odds of death were estimated to be more than 13 times higher for neonates requiring surgery compared with patients operated on after 30 days of life, and there were survivors of neonates with surgical intervention. Thus, early surgical intervention for symptomatic neonates may reduce mortality and improve outcome after a staged operation.


   The Society of Thoracic Surgeons: Forty-Third Annual Meeting
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Please mark your calendars for the Forty-Third Annual Meeting of The Society of Thoracic Surgeons, to be held in San Diego, California, from January 29–31, 2007. The program will provide in-depth coverage of thoracic surgical topics selected to enhance and broaden the knowledge of cardiothoracic surgeons. Attendees will benefit from traditional Abstract Presentations, as well as Surgical Forums, Breakfast Sessions, Surgical Motion Pictures, and Town Hall Meetings on specific topics.

Advance registration forms, hotel reservation forms, and details regarding transportation arrangements, as well as the complete meeting program, will be mailed to Society members this fall. Also, complete meeting information will be available on the Society's Web site at www.sts.org. Nonmembers who wish to receive information on the Annual Meeting may contact the Society's secretary, Douglas E. Wood.

Abstracts for the meeting must be submitted electronically. The electronic submission form may be accessed at www.sts.org. There is no charge for submitting abstracts. The submission deadline is June 30, 2006 at 5:00 PM CDT. Please direct any questions regarding your submission to the Society's headquarters.

Douglas E. Wood, MD Secretary

The Society of Thoracic Surgeons

633 N. Saint Clair St, Suite 2320

Chicago, IL 60611-3658

Telephone: (312) 202-5800

Fax: (312) 202-5801

e-mail: mailto:sts{at}sts.org

website: www.sts.org


   References
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 Abstract
 Introduction
 Material and Methods
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 Comment
 The Society of Thoracic...
 References
 

  1. Bartmus DA, Driscoll DJ, Offord KP, et al. The modified Fontan operation in children less than 4 years old J Am Coll Cardiol 1990;15:429-435.[Abstract]
  2. Stein SI, Smallhorn JF, Coles JG, Williams WG, Trusler GA, Freedom RM. Common atrioventricular valve guarding double inlet atrioventricular connectionnatural history and surgical results in 76 cases. Int J Cardiol 1990;28:7-17.[Medline]
  3. Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome Ann Thorac Surg 2002;74:1967-1977.[Abstract/Free Full Text]
  4. Van Praagh S, Santini F, Sanders S, Fyler DF. Cardiac malpositionscongenital heart disease in visceral heterotaxy (the asplenia and polysplenia syndromes). In: Fyler DC, editor. Nadas' pediatric cardiology. Philadelphia: Hanley & Belfus; 1992. pp. 589-608.
  5. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations J Am Stat Assoc 1958;53:457-481.
  6. Cox DR. Regression models and life tables J Roy Stat Soc Series B 1972;34:187-220.
  7. Mayer Jr JE, Bridges ND, Lock JE, Hanley FL, Jonas RA, Castanada AR. Factors associated with marked reduction in mortality for Fontan operations in patients with single ventricle J Thorac Cardiovasc Surg 1992;103:444-452.[Abstract]
  8. Gentles TL, Mayer Jr JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patientsfactors influencing early and late outcome. J Thorac Cardiovasc Surg 1997;114:376-391.[Abstract/Free Full Text]
  9. Mayer Jr JE, Helgason H, Jonas RA, et al. Extending the limits for modified Fontan procedures J Thorac Cardiovasc Surg 1986;92:1021-1028.[Abstract]
  10. Di Donato R, di Carlo D, Squitieri C, et al. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy Ann Thorac Surg 1987;44:35-39.[Abstract]
  11. Heinemann MK, Hanley FL, Van Praagh S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy Ann Thorac Surg 1994;57:88-91.[Abstract]
  12. Culberton CB, George BL, Day RW, Laks H, Williams RG. Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure J Am Coll Cardiol 1992;20:678-684.[Abstract]
  13. Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection J Thorac Cardiovasc Surg 1999;117:506-514.[Abstract/Free Full Text]
  14. Lodge AJ, Rychik J, Nicolson S, Ittenbach RF, Spray TL, Gaynor JW. Improving outcome in functional single ventricle and total anomalous pulmonary venous connection Ann Thorac Surg 2004;78:1688-1695.[Abstract/Free Full Text]
  15. Drinkwater DC, Laks H. Unbalanced atrioventricular septal defects Semin Thorac Cardiovasc Surg 1997;9:21-25.[Medline]
  16. Moak JP, Gersony WM. Progressive atrioventricular valvular regurgitation in single ventricle Am J Cardiol 1987;59:656-658.[Medline]
  17. Imai Y, Takanashi Y, Hoshino S, Terada M, Aoki M, Ohta J. Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation J Thorac Cardiovasc Surg 1997;113:262-269.[Abstract/Free Full Text]



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