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Ann Thorac Surg 1997;63:1676-1684
© 1997 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Bidirectional Cavopulmonary Shunt in Patients With Anomalies of Systemic and Pulmonary Venous Drainage

Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of California, San Francisco, San Francisco, California

Accepted for publication December 19, 1996.

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Background. Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure.

Methods. Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described.

Results. There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome.

Conclusions. Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
When Fontan and Baudet introduced their technique for repair of tricuspid atresia [1], the operation was thought to be contraindicated in patients with anomalies of systemic and pulmonary venous return [2]. However, the Fontan procedure has since undergone numerous modifications and has been extended to more complex single ventricle anatomies, including those with anomalies of systemic venous drainage (ASVD), pulmonary venous drainage (APVD), or both [3–6]. Although early and late survival after the Fontan operation have tended to be less favorable in patients with ASVD/APVD [7], recent studies have shown results to be improving in this group of patients [6]. Increasingly, pre-Fontan palliation of functional single-ventricle hearts is carried out using a bidirectional cavopulmonary shunt (BCPS), especially in patients with complex lesions who are thought to be at increased risk for early Fontan operation [8–11]. Several reported series of BCPS have included patients with ASVD/APVD, but results observed in such patients are inconsistent, and there is no clear evidence concerning relative risks of morbidity and mortality [9–15].

Several reports have discussed technical considerations for performing the Fontan operation in patients with ASVD/APVD [4–6]. In the present article, we discuss our experience with and technical approaches to BCPS in patients with ASVD, APVD, or both. Because our preferred approach to single-ventricle palliation includes an extracardiac conduit-type Fontan operation, neonatal palliative procedures and BCPS are undertaken with this end in mind. We believe that the extracardiac Fontan operation is well suited to patients with ASVD and APVD, which we will also address in the present report.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Patients
Between January 1990 and December 1995, 117 patients underwent BCPS at our institution. Of these patients, 36 (31%) had ASVD, APVD, or both and constitute the study group for the present report. Data on patient ages, diagnoses, and morphologic characteristics are summarized in Table 1. Combined ASVD and APVD were present in 12 patients (33%), whereas 19 patients (53%) had only ASVD and 5 patients (14%) had isolated APVD. Eighteen patients (50%) had diagnoses of visceral heterotaxy syndrome, on the basis of criteria described by Van Praagh and associates [16]. All 6 of the polysplenia patients also had left atrial isomerism, and 11 of the 12 asplenia patients had right atrial isomerism. Median age at BCPS was 11 months (range, 34 days to 32 years), and median weight was 7.5 kg (range, 3.4 to 100 kg). Bidirectional cavopulmonary shunt was performed electively in 34 patients, 20 of whom were noted to have increasing cyanosis or decreased exercise tolerance, 3 of whom had poor or no flow across systemic-to-pulmonary artery (PA) shunts, and 3 of whom had branch PA stenosis. One patient presented in extremis, with respiratory distress and metabolic acidosis, and 1 patient was receiving a prostaglandin E₁ infusion and required preoperative ventilatory support. Bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 patients (28%), whereas 19 patients (53%) had undergone one previous procedure and 7 patients (19%) had undergone two or more previous procedures. Before BCPS, interventions to repair ASVD/APVD had been performed in only 1 patient, who underwent repair of obstructed total APVD at 2 weeks of age and revision of a left pulmonary vein-to-left atrium anastomosis at 6 months of age, both at another institution.

	Operative Techniques

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Patients were prepared and draped in the supine position. After median sternotomy, the ascending aorta, both cavae, and PAs were exposed. Cardiopulmonary bypass was instituted in 28 patients, either at this point or later in the procedure, with total perfusion time ranging from 29 to 292 minutes. In the remaining patients, superior vena cava (SVC)-to-right atrial bypass was employed.

SYSTEMIC VENOUS DRAINAGE AND PULMONARY BLOOD FLOW.
Systemic venous drainage can be broken down into three categories for the present study: normal, anomalous but not requiring special intervention, and anomalous and necessitating special intervention. The first category includes patients with a single SVC and inferior vena cava draining to the systemic venous atrium. The second category includes, for example, patients with incommensurate SVC and inferior vena cava situs, and patients with unilateral SVCs and azygos/hemiazygos continuation of the inferior vena cava with hepatic venous drainage directly to the right atrium (although techniques of hepatic vein exclusion, which may be a means of preventing the development of arteriovenous fistulas in these patients, would place them in the third category). The third category includes patients with forms of ASVD requiring specific interventions, including bilateral SVC. Because the SVC(s) become the primary or sole source of pulmonary blood flow in BCPS, other issues of pulmonary blood flow, such as PA interventions, are discussed in this section.

In patients with a single right (n = 8) or left (n = 1) SVC, the SVC was clamped and divided above the cavoatrial junction, after which the cardiac end was oversewn and the cranial end was anastomosed end-to-side with the PA. The patient with the left SVC and right-sided inferior vena cava did not require special intervention for this form of ASVD, and simply underwent left BCPS. Of 3 patients with azygos continuation of the inferior vena cava, 1 had a single right SVC, whereas the other 2 had bilateral SVCs. In all 28 patients with bilateral SVCs, bilateral BCPS were performed. The SVCs were clamped and divided, with the cardiac ends oversewn and the craniad ends anastomosed end-to-side to the PAs.

In 13 patients, including 6 who underwent bilateral BCPS, PA augmentation was performed along with the cavopulmonary anastomosis. In 1 patient, extensive intrapulmonary arterioplasty was performed. In 4 patients with pulmonary atresia (n = 2) or stenosis (n = 2) and discontinuous central PAs, PA confluence was achieved by tube reconstruction or patch augmentation along with the extension of a tongue of SVC laterally from a unilateral (n = 3) or bilateral (n = 1) BCPS. A source of pulmonary blood flow in addition to the BCPS was placed (n = 6) or allowed to remain from the native anatomy or a previous procedure (n = 14) in 20 patients (57%), as either antegrade flow through a banded or stenotic main PA (n = 10) or a systemic-to-PA shunt (n = 10). Extra pulmonary blood flow was added in an almost identical percentage (58%) of the 117 patients undergoing BCPS during this period. Extra pulmonary blood flow is left in patients with antegrade flow by banding the pulmonary artery at the time of BCPS, or by tightening the band if the pulmonary artery has already been banded. However, in patients with a prior arterial shunt, the shunt is generally removed, because the cavopulmonary anastomosis is performed at the same site. Another shunt is only added if arterial blood gases show a partial pressure of oxygen of less than approximately 30 mm Hg. In older patients (beyond the toddler age group) extra pulmonary blood flow is added in all patients.

PULMONARY VENOUS DRAINAGE.
Pulmonary venous drainage can be classified into the same three categories: normal, anomalous but not requiring special intervention, and anomalous and necessitating special intervention. Normal drainage includes return of all pulmonary venous blood to the atrium contralateral to the systemic venous atrium. Because we plan eventually to perform an extracardiac conduit Fontan operation in almost all single-ventricle patients, APVD not requiring intervention includes all forms of cardiac APVD, such as drainage to the middle or systemic side of a common atrium. Most forms of high supracardiac and infracardiac APVD and all cases of obstructed APVD require intervention, as do most cases of cardiac APVD when one-and-a-half ventricle repair is planned.

Nineteen patients had normal pulmonary venous drainage and underwent no pulmonary venous interventions. Seven patients had unobstructed APVD to the middle or systemic side of a common atrium. In these patients, none of whom underwent one-and-a-half ventricle repair, nothing was done to modify the APVD. In a patient with anomalous drainage of the left upper lobe pulmonary vein to a persistent left SVC, which drained into the left atrium, the left SVC was divided for BCPS above the entrance of the pulmonary vein, and the SVC stump was oversewn to allow drainage of the pulmonary venous blood into the left atrium (Fig 1). A secundum atrial septal defect was also closed in this patient as part of a one-and-a-half ventricle repair. A similar pattern of APVD of one or more right pulmonary veins into a right SVC just craniad to the cavoatrial junction was present in 3 patients. This pattern was managed surgically by dividing the SVC sufficiently above the entrance of the pulmonary veins to avoid obstruction, performing a BCPS with the craniad portion of the ligated SVC, and oversewing the SVC stump to allow the pulmonary veins to drain into a common atrium (Fig 2). These techniques are not applicable if a subsequent lateral tunnel-type Fontan operation is planned. In 1 of these patients, there was also a tortuous common left pulmonary vein that travelled across the mediastinum, posterior to the main bronchi, and drained into the high right SVC. This ascending vein was ligated, filleted open longitudinally, and anastomosed to a transverse incision in the left atrium (see Fig 2). In another patient with pulmonary venous confluence behind the left atrium and total anomalous drainage into the right superior cavoatrial junction, the pulmonary venous confluence was opened longitudinally and anastomosed to the left atrium, along with an atrial baffle septation procedure, as part of a one-and-a-half ventricle repair. In 2 patients, total APVD entered high up into the right SVC. In both of these patients, the ascending vein was ligated, and the pulmonary venous confluence was filetted open longitudinally and anastomosed to the posterior atrial wall. In 1 of the patients with total APVD high on the right SVC, the cardiac stump of a left SVC ligated for left BCPS was used as part of the atrial anastomosis with the pulmonary venous confluence. In 1 patient, anomalous right pulmonary veins drained into the right innominate vein. This was corrected by ligating the ascending vein and anastomosing it end-to-end to the tip of the right-sided (ie, systemic) atrial appendage (Fig 3). Total APVD below the diaphragm was encountered in only 1 patient. In this patient, the descending vein was ligated, opened longitudinally, and anastomosed to a transverse posterior atriotomy.

View larger version (28K): [in this window] [in a new window]   Fig 1. . (A) Anatomy of patient 17, who had neither left nor right atrial isomerism. (B) Technique of repair, as described in text. (Note: This and subsequent figures may not accurately reflect the anatomy of the atrial appendages in the patients represented; atrial appendage anatomy is mentioned in the figure legends for the sake of clarity.) (Ao = aorta; LA = left atrium; LPA = left pulmonary artery; LPV = left pulmonary vein; LSVC = left superior vena cava; RA = right atrium; RPA = right pulmonary artery; RPV = right pulmonary vein; RSVC = right superior vena cava.)

View larger version (26K): [in this window] [in a new window]   Fig 2. . (A) Anatomy of patient 15 (right atrial isomerism). (B) Technique of repair, as described in text. (C) Alternative technique of repair, with anastomosis of the anomalous LPV to the atrial stump of the LSVC, effectively conserving suture line. (Abbreviations are as in Figure 1.)

View larger version (22K): [in this window] [in a new window]   Fig 3. . (A) Anatomy of patient 4 (right atrial isomerism). (B) Technique of repair, as described in text. (C) Alternative technique of repair, with anastomosis of the anomalous RPV to the cardiac stump of the superior vena cava, effectively conserving suture line. (LIV = left innominate vein; other abbreviations are as in Figure 1.)

	Data Collection and Statistical Analysis

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Preoperative and perioperative data were collected on retrospective review of patient records. Follow-up was conducted from November 1995 to January 1996 by means of physician contact or direct review of patient records, and was complete in all patients. Patients were followed up until their most recent physician contact before the time follow-up was conducted, or until they underwent Fontan completion. Descriptive statistics were performed using Microsoft Excel Version 5.0 (Microsoft Corp, Redmond, WA). SPSS for Windows v.6.01 (SPSS Inc, Chicago, IL) was used to calculate paired two-tailed t test analysis, general factorial analysis of variance, multiple logistic regression, and survival analysis with the Kaplan-Meier product limit method and the Cox proportional hazards regression model. For the purposes of Cox regression and actuarial survival analysis, patients who have undergone Fontan completion were censored at the date of their Fontan procedure.

	Results

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Early Results
There were two early deaths in the study group (5.6%), which was not significantly different than the early mortality rate for all patients undergoing BCPS during this period (4.3%). One early death occurred in a 61-day-old patient (patient 6) with asplenia syndrome and total APVD who had previously undergone placement of a systemic-to-PA shunt and presented for operation in extremis, with severe respiratory distress and metabolic acidosis. Bidirectional cavopulmonary shunt was performed despite an elevated pulmonary vascular resistance of 4.7 Wood units, which would have argued against BCPS in a less emergent situation. Due to continued poor oxygenation postoperatively, reoperation was required 2 days after BCPS, at which time an additional systemic-to-PA shunt was placed. The patient arrested and died on postoperative day 5 after continued low output and poor oxygen saturation. The other early death was in a 16-year-old patient (patient 36) with unbalanced double-outlet right ventricle, pulmonary stenosis, and normal pulmonary venous return who experienced malignant arrhythmias postoperatively, required reoperation 2 days postoperatively for tamponade, and arrested on the fourth postoperative day.

One patient, who underwent primary BCPS at 30 days of age for palliation of tricuspid atresia with pulmonary stenosis and bilateral SVC, experienced respiratory distress that required extracorporeal membrane oxygenation and BCPS take-down to an aortopulmonary shunt on postoperative day 3. Four patients, 2 with asplenia, 1 with double-outlet right ventricle, and 1 with double-inlet single left ventricle, underwent reoperation 2 to 24 days postoperatively for ligation or clipping of systemic-to-PA shunts placed at the time of BCPS (n = 2) or at a previous palliative procedure (n = 2). In the patient who underwent atrial septation and left atrial anastomosis of total APVD as part of a one-and-a-half ventricle repair, persistent cyanosis led to reexploration on the second postoperative day, which revealed a previously unrecognized hepatic vein draining to the left of the atrial baffle. The baffle was revised to admit drainage from this hepatic vein into the systemic venous atrium. Two other patients underwent reoperation for bleeding within 1 day of BCPS. The incidence of early reoperation did not differ significantly between patients with ASVD/APVD and the entire cohort of patients undergoing BCPS during the study period.

Statistical analysis did not reveal any significant factors for poor outcome. There was no significant difference in early mortality or complication rates between study group patients with and without heterotaxy syndrome, and no demonstrable differences between patients with polysplenia and asplenia. In addition, among all 117 patients who underwent BCPS during the study period, patients with ASVD/APVD and those with heterotaxy syndrome were not subject to higher rates of morbidity or mortality.

	Late Results

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Patients were followed up for a mean of 19.9 ± 12.2 months after BCPS (range, 1 to 47 months). During the follow-up period, 11 patients underwent Fontan completion an average of 22 months after BCPS (range, 1 to 47 months). With the exception of a patient who underwent Fontan completion at another institution, all of these patients had extracardiac conduit total cavopulmonary anastomosis-type Fontan procedures performed. One patient with asplenia syndrome and total APVD to a common atrium, who did not require pulmonary vein intervention at BCPS, underwent unroofing of the pulmonary veins at Fontan completion 18 months after BCPS, and died on the second postoperative day after sustaining a cerebrovascular accident. There were no other pulmonary vein interventions performed at Fontan completion and no additional Fontan-related deaths or significant perioperative morbidity. Fontan results in this cohort are comparable with those in the total group of 52 patients who underwent extracardiac conduit Fontan operations during the study period, among whom there was one other early post-Fontan death, which occurred in a patient undergoing revision of a previous atriopulmonary Fontan [17]. There were no other deaths among patients who underwent both BCPS and Fontan operation at our institution.

Late reoperations other than Fontan completion were performed in 2 patients. The patient who had undergone repair of total APVD at 2 weeks of age at another institution, before BCPS, required repair of mild left and severe right pulmonary vein stenoses 5 months after BCPS. These stenoses were not noticed during the work-up for BCPS, but they had probably been present, as they were caused by scarring at the site of anastomosis to the left atrium. Another patient, who subsequently underwent extracardiac Fontan operation 34 months after BCPS, had a systemic-to-PA shunt placed 1 year before Fontan completion. This late reoperation rate was lower than among the overall BCPS cohort of 117 patients, 9 of whom underwent late reoperations other than the Fontan procedure or conversion to a one-and-a-half ventricle repair.

Aside from the patient who died after the Fontan operation, there have been three late deaths after BCPS. One patient with polysplenia syndrome and normal pulmonary venous return who underwent BCPS at 61 days of age (patient 18) died 5 months after BCPS with severe desaturations secondary to multiple pulmonary arteriovenous fistulas [18]. A patient with asplenia syndrome and total APVD who underwent BCPS at 56 days of age (patient 5) died 9 months after BCPS of an unknown pulmonary illness. This patient had been intubated and receiving prostaglandin E₁ before coming to operation, and did poorly after BCPS, requiring several general surgical operations and suffering numerous problems characteristic of severe heterotaxy syndrome. A third patient, with situs inversus double-inlet left ventricle and partial APVD to the right superior vena cava (patient 16), died 21 months after undergoing BCPS at 16 months of age, due to complications of chronic lung disease that developed secondary to respiratory syncytial virus pneumonia. This patient never thrived postoperatively, and required postoperative hospitalization for nearly 5 months due to primary conditions and hospital complications that included thrombocytopenia, primary hypothyroidism, postoperative seizure disorder, respiratory syncytial virus pneumonia, Candida sepsis, chronic lung disease, and nutrition problems resulting from poor gastric emptying.

Actuarial survival among all study patients (including early and late events) was 87% at 1 year and 81% at 3 years after BCPS (Fig 4). Among the study population, the only significant predictor of poorer survival by Cox regression analysis was lower preoperative arterial oxygen saturation (p = 0.018; continuous variable). Survival over time among patients with heterotaxy syndrome was slightly lower than in patients without heterotaxy, but this difference did not approach significance. Among all patients undergoing BCPS during the study period (n = 117), neither heterotaxy syndrome nor ASVD/APVD was significantly associated with poorer postoperative survival by Cox regression. Actuarial survival curves for all patients undergoing BCPS during the study period and those in the study group are shown in Figure 4.

View larger version (17K): [in this window] [in a new window]   Fig 4. . Actuarial survival curves for all patients undergoing bidirectional cavopulmonary shunt during the study period, patients with anomalies of systemic or pulmonary venous drainage (ASVD/APVD), and patients with heterotaxy syndrome. Differences in survival were not significant between groups.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Since the introduction of the Fontan operation for repair of tricuspid atresia [1], the Fontan concept has been increasingly applied to patients with a variety of complex single-ventricle anatomies, including those that occur with ASVD, APVD, or both. Several reports have addressed the issue of managing ASVD/APVD at Fontan operation, but most of the techniques described require inventive manipulations to reroute anomalous venous blood, and often employ extensive synthetic patches and conduits [4–6].

In the development of a staged approach to the palliation of functional single-ventricle hearts with ASVD/APVD, first- and second-stage procedures can facilitate straightforward techniques for managing the anomalous venous return at Fontan operation. For the Fontan procedure, it is our policy to perform extracardiac conduit total cavopulmonary anastomosis whenever possible, preceded by BCPS and neonatal palliation if early intervention is necessary. In almost all cases, primary palliative procedures and BCPS (which was the primary palliative procedure in 28% of patients in the present series) performed in single-ventricle patients at our institution are done with the eventual aim of performing an extracardiac conduit Fontan operation.

The extracardiac Fontan operation lends itself well to complex single-ventricle lesions with ASVD/APVD. Because the systemic venous atrium is bypassed in this technique, an effective common atrium receives all pulmonary venous blood. If a native common atrium or nonrestrictive atrial septal defect is present, no atrial septal intervention is necessary; otherwise, atrial septectomy can be performed at BCPS or a previous palliative operation. For most forms of unobstructed cardiac and low supracardiac APVD, which constitute a significant portion of the patterns encountered in patients with both visceral heterotaxy syndromes [19–21] and nonheterotaxy APVD [22, 23], no pulmonary vein intervention is required if an eventual extracardiac Fontan operation is planned. For example, the patients reported in the present study with pulmonary venous drainage to the middle or systemic side of a common atrium underwent no pulmonary venous intervention at BCPS. Similarly, in patients with anomalous drainage of the right pulmonary veins just above the superior cavoatrial junction, transection of the SVC for the BCPS procedure was performed sufficiently superior to the entrance of the pulmonary veins to avoid possible obstruction, which leaves adequate SVC tissue for the cavopulmonary anastomosis (see Fig 2). This technique would not be applicable in preparation for Fontan repairs other than the extracardiac conduit-type Fontan operation. Although these forms of pulmonary venous drainage are amenable to diversion with intraatrial conduit or atrial baffle septation procedures, as is standard practice for biventricular repairs of APVD [22–24] and has been described for Fontan operation [4, 6], the risk of complications such as secondary pulmonary venous obstruction are important to consider in a single-ventricle situation. Moreover, our technique and the others we have described involve only natural tissue connections, with the eventual extracardiac Fontan conduit as the only nonnative tissue connection. Although long-term patency of the extracardiac conduit has yet to be firmly established, our experience and that of others [25] suggests that is not a significant problem, at least in the intermediate term.

With infradiaphragmatic and high supracardiac APVD, or any form of obstructed APVD, various techniques can be used to achieve pulmonary venoatrial connection. In such circumstances, the effective common atrium allows for greater flexibility in anastomosis placement when the eventual aim is to perform an extracardiac conduit Fontan operation. One of the cases described in the present series is a good example: partial anomalous drainage of the right pulmonary veins into the right innominate vein was corrected by ligating the ascending vein and anastomosing it end-to-end to the tip of the right-sided (ie, systemic) atrial appendage (see Fig 3). As shown in Figure 3C, this technique might be further improved by anastomosing the pulmonary vein to the cardiac stump of the transected right SVC, thus minimizing atrial suture line. This patient subsequently underwent successful Fontan completion with an extracardiac conduit-type Fontan operation.

With techniques of BCPS that accommodate pulmonary venous drainage into the right side of a common atrial chamber, such as those described above, a right-sided extracardiac conduit Fontan operation can be performed with little modification. If necessary, the conduit from the inferior vena cava can be run slightly anterior or posterior, so as to afford clearance between the conduit and the pulmonary venoatrial connection, with an oblique cavopulmonary anastomosis and a pericardial hood used to enlarge the communication.

In conclusion, BCPS can be performed in patients with ASVD, APVD, or both, including those with visceral heterotaxy syndrome, with morbidity, mortality, and reoperation rates that do not differ significantly from those achieved in all patients undergoing BCPS. Increasingly, it is being recognized that BCPS can be carried out effectively in young infants, often as a primary palliative procedure, for various forms of functional single ventricle [26, 27]. Although the management of neonates with heterotaxy syndrome and obstructed APVD is probably best handled with palliation in the first few days of life [28], BCPS appears to be a good option for primary palliation of some single-ventricle patients with unobstructed ASVD/APVD and adequate pulmonary blood flow. A number of risk factors have been identified for Fontan failure in patients with heterotaxy syndrome or venous anomalies, including atrioventricular valve regurgitation, high preoperative PA pressure, PA hypoplasia, and asplenia syndrome [29]. We did not find any of these factors to correlate with BCPS failure or morbidity. Thus, BCPS does not appear to predispose patients with ASVD/APVD to the development of factors that have been found to place such patients at increased risk of Fontan failure. One issue of concern, however, is the development of pulmonary arteriovenous fistulas in 3 patients, all with heterotaxy syndromes, two of the asplenia variety and one with polysplenia and interrupted inferior vena cava. There have been several reports suggesting that patients with heterotaxy are more likely to have development of arteriovenous fistulas than other patients [13, 30, 31, 32], but it is not known what might be responsible for any such predisposition. Two of the patients in whom arteriovenous fistulas developed were very young at BCPS (less than 2 months); thus, age may be an important consideration is planning the timing of BCPS and Fontan operation in patients with heterotaxy. Nevertheless, it is clear that this requires further investigation.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 
Address reprint requests to Dr Reddy, Division of Cardiothoracic Surgery, 505 Parnassus Ave, M593, San Francisco, CA 94143-0118.

	References

Top Footnotes Abstract Introduction Patients and Methods Operative Techniques Data Collection and Statistical... Results Late Results Comment References

 

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