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J Thorac Cardiovasc Surg 2006;131:1108-1113
© 2006 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies

Tae-Jin Yun, MD a , Osman O. Al-Radi, MD b , Ian Adatia, MD c , Christopher A. Caldarone, MD b , John G. Coles, MD b , William G. Williams, MD b , Jeffrey Smallhorn, MD b , Glen S. Van Arsdell, MD b , *

a Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan, Ulsan, Republic of Korea
b The Cardiac Program, Hospital for Sick Children, Toronto, Canada
c Division of Pediatric Cardiology, University of California, San Francisco, Calif

Presented at 31st Annual Meeting of the Western Thoracic Surgical Association, Victoria, British Columbia, June 22-25, 2005.

Received for publication June 16, 2005; revisions received November 10, 2005; accepted for publication November 16, 2005.

* Address for reprints: Glen S. Van Arsdell, MD, Division of Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada, M5G 1X8 (Email: glen.vanarsdell{at}sickkids.ca).


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Discussion
 Conclusions
 References
 
OBJECTIVES: Infants with right atrial isomerism have poor outcomes because of a complex combination of cardiac anomalies. Aggressive management of total anomalous pulmonary venous drainage might have a positive effect on the prognosis.

METHODS: Outcomes of all children with right atrial isomerism from 1994 to the present were reviewed. Management of total anomalous pulmonary venous drainage evolved from no repair or conventional surgical technique to primary sutureless repair on initial palliation. Cox survival models were used to identify variables associated with reduced survival.

RESULTS: There were 55 children enrolled in the study. The median age at the initial visit was 2 days. Fifty-one patients had total anomalous pulmonary venous drainage (obstructive in 22 patients). Withdrawal of treatment occurred in 11 (20%) of 55 patients during an interval of institutional bias toward no treatment. Thirteen (24%) of 55 patients had palliations without total anomalous pulmonary venous drainage repair, and 3 (23%) of 13 survived. Thirty-one (56%) of 55 patients had operations that included total anomalous pulmonary venous drainage repair, of whom 13 (42%) of 31 underwent primary sutureless repair for total anomalous pulmonary venous drainage. Sixteen (52%) of 31 survived, and their current status 1 to 10 years (median, 5.8 years) after repair is post-Fontan (7/16 [44%]), postbidirectional Glenn (6/16 [38%]), and others (3 [20%]). In patients who underwent total anomalous pulmonary venous drainage repair (n = 31), 2 risk factors of decreased survival were identified: drainage site obstruction and infracardiac or mixed-type total anomalous pulmonary venous drainage. After adjustment, sutureless repair appeared to be associated with improved survival (hazard ratio, 0.43), but this beneficial effect did not reach significance (P = .19).

CONCLUSIONS: Mortality continues to be high; however, aggressive total anomalous pulmonary venous drainage repair for right atrial isomerism has resulted in improved survival. The role of primary sutureless repair for total anomalous pulmonary venous drainage remains to be defined.

Abbreviations and Acronyms AVSD = atrioventricular septal defect; BCPS = bidirectional cavopulmonary shunt; ECMO = extracorporeal membrane oxygenation; PVS = pulmonary vein stenosis; RAI = right atrial isomerism; TAPVD = total anomalous pulmonary venous drainage; VAD = ventricular assist device


   Introduction
Top
 Abstract
 Introduction
Patients and Methods
 Results
 Discussion
 Conclusions
 References
 
GoDespite strategic and technical improvements in the treatment of complex congenital heart anomalies, right atrial isomerism (RAI) associated with abnormal pulmonary venous drainage has remained a clinical challenge with high early and late mortality. 1,2 Go Discouraged by a number of clinical reports and by experiences of their own, some cardiac programs have been tempted to withdraw aggressive treatment from children with RAI, particularly when it is associated with obstructive total anomalous pulmonary venous drainage (TAPVD). Previously, we reported our 26-year experience of RAI between 1970 and 1996, which demonstrated that early mortality of RAI after palliative surgical procedures, including TAPVD repair, was as high as 95%. This disappointing experience led us to have an institutional bias toward no treatment in this disease entity between 1998 and 1999. With the development of the sutureless technique for TAPVD repair and extracorporeal life support systems, however, our institutional strategy has been changed from a conventional treatment or treatment withdrawal to surgical management, including primary sutureless repair of TAPVD, extracorporeal membrane oxygenation (ECMO)–ventricular assist device (VAD) support, and control of supraventricular tachyarrhythmias. In this study we sought to determine whether these changes of therapeutic strategy had a positive effect on early and late outcome of RAI.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Discussion
 Conclusions
 References
 
A review of children who registered at The Hospital for Sick Children, Toronto, Canada, between February 1994 and October 2003 was performed. During this time, the treatment of RAI evolved from conventional management or withdrawal of care to primary sutureless repair of TAPVD with aggressive cardiac and pulmonary support strategies (VAD and ECMO) if needed for management of arrhythmia, ventricular dysfunction, or pulmonary hypertension. The sutureless repair technique for pulmonary vein anomalies, which was originally developed for the surgical management of pulmonary vein stenosis (PVS) after TAPVD repair, 3 Go has been modified and adopted as a primary procedure for TAPVD associated with RAI since 2000. 4,5 Go For the retrospective review and collation of the data, "ethics approval of human subject health record/database research" was obtained from the research ethical board in the hospital.

Data are presented as frequencies, medians with ranges, or means with standard deviations. Survival data are represented by using the Kaplan-Meier method. Stratified survival curves were plotted to explore unadjusted group differences for variables of interest. After excluding the treatment withdrawal group, the Cox proportional hazards model was fitted with time to death as the dependent variable and stratified by indication categories (described below) to allow different baseline hazards for each category. Variables were selected by means of backward elimination with bootstrapping. The assumption of proportional hazards was tested by using an interaction term between time and each variable in the final model. The final model validation (to assess for overfitting) and calibration were done by means of bootstrapping. Analyses were conducted with S-plus 6.1 software (Insightful Corp).


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Discussion
 Conclusions
 References
 
Patients
Fifty-five children with RAI were identified during the study period. Age at the first visit ranged from the day of birth to 6.5 years (median, 2 days). There were 34 male and 21 female patients. Associated anomalies were as follows: atrioventricular septal defect (AVSD; 45/55 [82%]), TAPVD (51/55 [93%]; extracardiac types: 42/55 [76%]), d-transposition of great arteries (16/55 [29%]), double-outlet right ventricle (18/55 [33%]), pulmonary stenosis or atresia (45/55 [82%]), and bilateral superior vena cava (16/55 [29%]). Ventricles were balanced in 20 patients, including balanced AVSD (18 patients) and normal ventricular morphology (2 patients). The ventricles were unbalanced in 35 patients, including unbalanced AVSD (27 patients) and mitral or tricuspid atresia (8 patients). The type of TAPVD is described in Table 1.


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  		TABLE 1. Types of TAPVD (n = 55)
 
Surgical Treatment
Treatment withdrawal occurred in 11 (20%) of 55 children, with 6 withdrawals among 7 registered patients in 1998. The last treatment withdrawal in this reported group took place in 2000. There has been no treatment withdrawal in the 17 patients from January 2001 through October 2003. In 10 of 11 treatment-withdrawal patients, death occurred between 1 day and 3.6 months (median, 20 days) from progressive PVS, hypoxia, or both. One boy has survived 44.5 months without any surgical intervention. Thirteen (14%) of 55 patients underwent various palliative operations that did not include TAPVD repair. Thirty-one (56%) of 55 patients had TAPVD repair, of which 18 were a standard anastomosis between the common pulmonary venous channel and atrium, and 13 were a primary sutureless repair. Palliative or corrective procedures for the treatment group (44/55 patients) are summarized in Table 2. Four patients who had sutureless repair for TAPVD received ECMO-VAD support postoperatively. Three patients were successfully removed from the support without any neurologic complications and were discharged home. Of these, one child with supraventricular arrhythmia associated with double atrioventricular nodes received cryoablation in the critical care unit. Ablation of a left accessory atrioventricular node by using an epicardially applied cryoprobe controlled the medically unmanageable supraventricular arrhythmia. On follow-up, 2 of 3 babies who survived ECMO-VAD support, including one who had bedside cryoablation in the intensive care unit, died unexpectedly at home at 4 and 6 months after initial palliation, respectively. Autopsy was not done for these patients, and the causes of death remain unknown. The third patient survived and underwent the Fontan operation at 20 months after initial repair. The relationship between ventricular morphology and surgical strategies is described in Table 3.


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  		TABLE 2. Operative procedures
 

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  		TABLE 3. Ventricular morphology and surgical strategies
 
Early and Late Outcome
In the 13 patients who had a palliative procedure without TAPVD repair, 10 patients died 0 to 4.6 months after initial palliation or after bidirectional cavopulmonary shunt (BCPS). Repair of pulmonary venous return was not initially performed because there was no abnormality (n = 4) or the obstruction was thought to be clinically manageable without repair (n = 1) or clinically insignificant (n = 8). Causes of death were sudden hemodynamic instability in 5 patients, sepsis in 1 patient, progressive PVS in 1 patient, and unclear in 3 patients. In the 31 patients who underwent TAPVD repair, 15 patients died after initial operations or subsequent to BCPS, heart transplantation, or biventricular repair (Figure 1). The cause of death was sudden hemodynamic instability in 6 patients, obstruction in the BCPS or Fontan pathway in 2 patients, progressive individual PVS in 2 patients, systemic thromboembolism in 1 patient, cerebral hemorrhage in 1 patient, and an unclear cause in the remaining 4 patients. Among the 16 patients who survived (16/31 [51.6%]), 3 patients had PVS (2 after conventional TAPVD repair and 1 after sutureless repair), which necessitated secondary sutureless repair (n = 2) or pulmonary endovenectomy (n = 1). Their current status 1 to 10 years (median, 5.8 years) after repair is post-Fontan (7/16 [44%]), postbidirectional Glenn (6/16 [38%]), and others (3 [20%]). Overall survival and unadjusted survival according to age at initial surgical intervention, TAPVD repair strategy, and treatment-withdrawal strategy are shown in Figures 2 through 5. Go Go Go Patients who needed surgical intervention after 30 days of age (P = .0028), who underwent TAPVD repair (P = .036), and who registered in an era when no treatment withdrawal occurred (P = .0033) had better outcome by unadjusted comparisons. In the cohort of patients who underwent surgical intervention for TAPVD (n = 31), 3 groups were identified. Group 1 had cardiac or supracardiac TAPVD without obstruction of the pulmonary venous drainage, group 2 had cardiac or supracardiac TAPVD with obstruction of pulmonary venous drainage, and group 3 had infracardiac or mixed-type TAPVD. Decreased survival was associated with group 2 versus group 1 and group 3 versus group 1. The hazard ratios of death were 5.53 (95% confidence interval, 1.15-26.63) and 16.51 (95% confidence interval, 2.81-97.21), respectively. The sutureless repair of TAPVD appeared to show a trend for improved survival (hazard ratio, 0.43; 95% confidence interval, 0.12-1.52) but did not reach statistical significance (P = .19).


Figure 1
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  		Figure 1. Outcome after treatment withdrawal, palliations without total anomalous pulmonary venous drainage (TAPVD) repair, and palliations with TAPVD repair.BCPS, Bidirectional cavopulmonary shunt; Tx, transplantation; BVR, biventricular repair.

 

Figure 2
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  		Figure 2. Survival after birth in all patients (n = 55).

 

Figure 3
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  		Figure 3. Survival after birth for patients with first surgical intervention after age 30 days (A), with first surgical intervention during the neonatal period (B), and with no treatment (C). Patients with neonatal surgical intervention had significantly worse prognosis than patients who had surgical intervention after 1 month of age (P = .0028), although the neonatal palliation group had better outcome compared with the treatment-withdrawal group (P = .0406).

 

Figure 4
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  		Figure 4. Survival after birth according to total anomalous pulmonary venous drainage (TAPVD) repair strategy.

 

Figure 5
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  		Figure 5. Survival after birth according to treatment withdrawal strategy. A, Survival in an era when all patients underwent surgical treatment (from 2001); B, survival in an era when withdrawal of treatment occurred in selected cases (before 2001).

 

   Discussion
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
Conclusions
 References
 
The outcome of RAI depends on the patient population enrolled for the analysis. Early and late mortality after bidirectional cavopulmonary anastomosis or the Fontan operation in patients with RAI are reasonably low and comparable with surgical outcomes in patients without RAI. 6-9 Go This might be misleading because only selected patients who survived initial palliation were enrolled in those studies. In contrast to the relatively favorable prognosis of left atrial isomerism, 10-12 Go babies with RAI have a poor outcome. The present data demonstrate that need for neonatal repair and the presence of pulmonary venous obstruction are risk factors for mortality but also show that outcomes for those treated are better than for those having treatment withdrawal. Broad-category risk factors do not always explain the reasons for the poor outcome. Some patients having an anatomically correct repair have sudden death, unexplained end-organ complications, or a protracted illness leading to death. Some have hemodynamically compromising supraventricular or ventricular arrhythmia after a surgical procedure. Given the fact that withdrawal of treatment occurs frequently 1,2 Go and surgical mortality after palliative procedures is still high in patients with RAI, we developed a strategy for operative and postoperative care that dealt with key issues.

Rationale for VAD or ECMO Support
One potential mechanism for sudden unexplained early death after neonatal repair for obstructed TAPVD is a profoundly reactive pulmonary vasculature. In this situation a pulmonary hypertensive crisis would have a self-compounding effect. Achieving a high pulmonary arterial saturation might not be possible where there is a balanced circulation. Hypoxia would induce further pulmonary hypertension, followed by further hypoxia and so forth. VAD and ECMO support the myocardium through a pulmonary hypertensive crisis and can be discontinued after a stabilization of pulmonary reactivity. This rationale and strategy have been useful in our patient cohort. Patients having severely compromising arrhythmia can also be maintained with adequate oxygenation and cardiac output until effective medical management of the arrhythmia is achieved. In one of our patients requiring VAD support for arrhythmia, we successfully performed cryoablation of a secondary atrioventricular node in the critical care. We have not used VAD-ECMO for all recent cases but believe it is a useful adjunct for the above-outlined reasons.

Rationale for Primary Sutureless Pulmonary Vein Repair
Successful early outcome for palliation of RAI might be compromised by late development of PVS. This has also been noted with primary heart transplantation for RAI. 13 Go A child with pulmonary venous obstruction, regardless of cause, in our institution undergoes a sutureless pulmonary vein repair as a final attempt to treat the disease. We rationalized that if infants with RAI were at high risk for development of pulmonary vein obstruction, then we should perform the most aggressive surgical therapy for pulmonary vein obstruction at the initial operation. This is based on the findings of others 14-17 Go and our own findings 1,18 Go that small size of the pulmonary vein is a major risk factor for later development of PVS after conventional TAPVD repair and that high mortality of RAI is related, at least in part, to intrinsically small pulmonary veins. 1,14,16,18,19 Go Furthermore, most of the patients with RAI are not anatomic candidates for biventricular repair. PVS is a risk factor for poor Fontan operation outcome. 7,9 Go The acute anatomic benefit for the sutureless repair is that each vein is its own native size, without any suture material to cause an excessive inflammatory reaction or luminal compromise. Our findings of relatively worse outcome for those not undergoing primary repair of pulmonary venous abnormalities suggest that addressing any potentially obstructive pulmonary venous anomaly at the first operation might be important.

Because primary sutureless repair for RAI is performed without intrapericardial adhesion, longitudinal division of the pulmonary veins into the pulmonary hilum might be associated with violation of the thin pleura at the junction between the parietal and visceral pleura overlying the pulmonary hilum and left atrial bleeding into the pleural space. This can be managed by means of reapproximation of the parietal and visceral pleura with a fine running suture to achieve hemostasis. 4 Go Given that the rationale for primary sutureless repair of the pulmonary vein in RAI is the small size of the confluent pulmonary vein rather than the individual PVS, excessive division of the extrapericardial portion of the pulmonary veins is generally unnecessary. Therefore it should be avoided in the first instance.

Single and Biventricular Repair
Those with balanced ventricles might present a challenge for biventricular repair because of a combined AVSD 20-26 Go and conotruncal anomaly. 1,27 Go Clinical reports of biventricular repair for RAI have been rare and sporadic. 27-30 Go A neonate with obstructed TAPVD, an AVSD, double-outlet right ventricle with the aorta distant from the ventricular septal defect, and pulmonary atresia might not ever be thought to be amenable to a biventricular repair, despite balanced ventricles. However, in view of the present findings, along with current trends toward biventricular repair for various congenital anomalies (eg, marginally hypoplastic left heart syndrome, unbalanced AVSD, and congenitally corrected transposition of the great arteries), it might be rational to shift the competing risks of TAPVD and complex intracardiac repair versus TAPVD and single-ventricle repair more to the biventricular repair side of the risk analysis. Because there were 20 children with balanced ventricles in our series, it is conceivable that biventricular repair might have been achievable in some of them.

Limitations of the Study
The small number of patients with RAI and specifically the small numbers undergoing a sutureless repair for TAPVD and ECMO-VAD support make the statistical strength of the analysis weak. The potential for the sutureless pulmonary vein repair to improve early and late outcome of RAI in terms of the prevention of postrepair PVS cannot be statistically shown. On the basis of favorable findings in an analysis of development of PVS, for all patients after a sutureless repair, 4 Go there is some reason to believe that the sutureless technique might be beneficial for patients with RAI and pulmonary venous abnormalities. Generalized improvements in knowledge, expertise, and delivery of perioperative care are difficult to quantify but might, nevertheless, also contribute to improved outcomes. Improved outcomes in RAI might therefore be multifactorial. In this regard ECMO-VAD and antiarrhythmia surgery were addressed as new therapeutic strategies for critically ill babies with RAI in this article. Any beneficial or detrimental effect was not statistically significant.


   Conclusions
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 Conclusions
References
 
Outcomes for RAI continue to be less than optimal, particularly when there is infracardiac or mixed TAPVD with draining site obstruction, presentation requiring repair in the neonatal period, or both. A strategy for primary sutureless repair of the pulmonary venous anomaly and aggressive use of ECMO-VAD support until the pulmonary vascular reactivity, arrhythmia, or both have subsided appears to be beneficial. Other unmeasured improvements in care might also be contributory. Primary sutureless repair of the pulmonary veins has been associated with a low incidence of development of subsequent pulmonary venous obstruction.


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   References
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 Abstract
 Introduction
 Patients and Methods
 Results
 Discussion
 Conclusions
 References
 

  1. Hashimi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcome of right atrial isomerism. a 26-year experience. J Am Coll Cardiol 1998;31:1120-1126.[Abstract/Free Full Text]
  2. Di Donato R, di Carlo D, Squitieri C, Rossi E, Ammirati A, Marino B, et al. Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorac Surg 1987;44:35-39.[Abstract]
  3. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg 1998;115:468-470.[Free Full Text]
  4. Yun TJ, Coles JG, Constantinov IE, Al-Radi OO, Wald RM, Guerra V, et al. Conventional and sutureless techniques for management of the pulmonary veins. evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies. J Thorac Cardiovasc Surg 2005;129:167-174.[Abstract/Free Full Text]
  5. Caldarone CA. "Sutureless" pulmonary vein stenosis repair. The Cardiothoracic Surgery Network. Clinical resources. congenital cardiac expert technique. 2004Available at: www.ctsnet.org..
  6. Azakie A, Merklinger SL, Williams WG, Van Arsdell GS, Coles JG, Adatia I. Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndrome. Ann Thorac Surg 2001;72:1636-1640.[Abstract/Free Full Text]
  7. Jacobs ML. Complications associated with heterotaxy syndrome in Fontan patients. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:25-35.[Medline]
  8. McElhinney DB, Reddy VM, Moore P, Hanley FL. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997;63:1676-1684.[Abstract/Free Full Text]
  9. Stamm C, Fiehs I, Duebener LF, Zurakowski D, Mayer JE, Jonas RA, 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]
  10. Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Card 2000;36:908-916.[Abstract/Free Full Text]
  11. Uemura H, Yagihara T, Kawahira Y, Yoshikawa Y. Anatomic biventricular repair by intraatrial and intraventricular re-routing in patients with left isomerism. Cardiol Young 2001;11:12-16.[Medline]
  12. Carotti A, Marino B, Oppido G. Biventricular repair in patients with left isomerism. J Thorac Cardiovasc Surg 1995;110:1151-1153.[Free Full Text]
  13. Razzouk AJ, Gundry SR, Chinnock RE, Larsen RL, Ruiz C, Zuppan CW, et al. Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart disease. J Heart Lung Transplant 1995;14:713-717.[Medline]
  14. Cheung YF, Cheng VYW, Chau AKT, Chiu CSW, Yung TC, Leung MP. Outcome of infants with right atrial isomerism. is prognosis better with normal pulmonary venous drainage?. Heart 2002;87:146-152.[Abstract/Free Full Text]
  15. Bando K, Turrentine MW, Ensing GJ, Sun K, Sharp TG, Sekine Y, et al. Surgical management of total anomalous pulmonary venous connection. thirty year trends. Circulation 1996;94(suppl):II12-II16.
  16. Jenkins KJ, Sanders SP, Orav EA, Mayer JE, Colan SD. Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. J Am Coll Cardiol 1993;22:201-206.[Abstract]
  17. Heinmann MK, Hanley FL, Van Praagh S, Fenton KN, Jonas RA, Mayer JE, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg 1994;57:88-91.[Abstract]
  18. Freedom RM, Hashmi A. Total anomalous pulmonary venous connections and consideration of the Fontan or one-ventricle repair. Ann Thorac Surg 1998;66:681-682.[Abstract/Free Full Text]
  19. Rubino M, Van Praagh S, Kadoba K, Pessotto R, Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg 1995;110:641-650.[Abstract/Free Full Text]
  20. Oshima Y, Yamaguchi M, Yoshimura N, Oka S, Ootaki Y. Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies. Ann Thorac Surg 2001;72:424-429.[Abstract/Free Full Text]
  21. Oku H, Iemura J, Hitoshi K, Saga T, Shirotani H. Bivalvation with bridging for common atrioventricular valve regurgitation in right isomerism. Ann Thorac Surg 1994;57:1324-1326.[Abstract]
  22. Okita Y, Miki S, Kusuhara K, Ueda Y, Tahata T, Yamanaka K, et al. Annuloplastic reconstruction for common atrioventricular valvular regurgitation in right isomerism. Ann Thorac Surg 1989;47:302-304.[Abstract]
  23. Uemura H, Anderson RH, Ho SY, Devine WA, Neches WH, Smith A, 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]
  24. Uemura H, Ho SY, Anderson RH, Yagihara T. The structure of the common atrioventricular valve in hearts having isomeric atrial appendages and double inlet ventricle. J Heart Valve Dis 1998;7:580-585.[Medline]
  25. Kawahira Y, Kishimoto H, Kawata H, Ikawa S, Ueda H, Nakajima T, et al. Morphologic analysis of common atrioventricular valves in patients with right atrial isomerism. Pediatr Cardiol 1997;18:107-111.[Medline]
  26. De Tommasi SM, Daliento L, Ho SY, Macartney FJ, Anderson RH. Analysis of atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 specimens with atrial isomerism. Br Heart J 1981;45:236-247.[Abstract/Free Full Text]
  27. Ohasi T, Yagihara T, Kishimoto H, Isobe F, Yamamoto F, Kawashima Y. Biventricular repair for right isomerism associated with transposition of great arteries and common atrioventricular canal. Nippon Kyobu Geka Gakkai Zasshi 1992;40:1025-1028.[Medline]
  28. Sinzobahamvya N, Arenz C, Brecher AM, Urban AE. Atrial isomerism. a surgical experience. Cardiovasc Surg 1999;7:436-442.[Medline]
  29. van Son JA, Black MD, Haas GS, Falk V, Hambsch J, Onnasch JF, et al. Extracardiac repair versus intracardiac baffle repair of complex unroofed coronary sinus. Thorac Cardiovasc Surg 1998;46:371-374.[Medline]
  30. Hirooka K, Yagihara T, Kishimoto H, Isobe F, Yamamoto F, Nishigaki K, et al. Biventricular repair in cardiac isomerism. report of seventeen cases. J Thorac Cardiovasc Surg 1995;109:530-535.[Abstract/Free Full Text]



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