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Ann Thorac Surg 2006;82:1621-1628
© 2006 The Society of Thoracic Surgeons
a Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas
b Department of Physical Medicine and Rehabilitation, Baylor College of Medicine, Houston, Texas
c Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas
Accepted for publication May 11, 2006.
* Address correspondence to Dr Morales, Division of Congenital Heart Surgery, Texas Children's Hospital, 6621 Fannin St, MC-WT 19345H, Houston, TX 77030 (Email: dlmorale{at}texaschildrenshospital.org).
Presented at the Forty-second Annual Meeting of The Society of Thoracic Surgeons, Chicago, IL, Jan 30–Feb 1, 2006.
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Abstract |
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METHODS: Between July 7, 1995, and November 22, 2005, 122 consecutive patients underwent primary repair of total anomalous pulmonary venous return. Characteristics were as follows: mean age, 0.35 ± 1.8 years; mean weight, 4.3 ± 2.8 kg; single ventricle, 40 (33%); heterotaxy, 38 (31%); pulmonary atresia, 13 (11%). Concomitant procedures included pulmonary artery banding in 4 (3%), systemic-to-pulmonary shunt in 14 (12%), bidirectional Glenn in 11 (9%), and other in 16 (14%).
RESULTS: With a mean follow-up of 2.6 ± 2.5 years including all patients, overall 30-day and 5-year survival was 93% and 86%, respectively; for heterotaxy patients it was 84% and 79%; and for nonheterotaxy patients it was 96% and 89%. Heterotaxy and nonheterotaxy survival curves were not significantly different (p> 0.05). Overall, there were 12 (9.8%) patients who required reoperation for pulmonary vein stenosis, at a mean of 5.1 ± 5.5 months postoperatively. Seven (58%) reoperations were in heterotaxy patients. Rate of pulmonary vein reoperation is statistically greater (p < 0.05) for heterotaxy patients than nonheterotaxy patients. At last follow-up, all patients were in New York Heart Association class II or less. Using Cox proportional hazards models, pulmonary atresia and need for systemic-to-pulmonary shunt were risk factors for death (not heterotaxy). Single ventricle, operation after July 2000, and need for systemic-to-pulmonary shunt were risk factors for pulmonary vein reoperation.
CONCLUSIONS: At midterm follow-up, heterotaxy patients undergoing total anomalous pulmonary venous return repair have a higher pulmonary vein reoperative rate but a comparable overall survival to nonheterotaxy patients.
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Introduction |
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TopAbstractIntroductionPatients and MethodsResultsCommentDiscussionReferences |
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Patients and Methods |
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TopAbstractIntroductionPatients and MethodsResultsCommentDiscussionReferences |
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The repair of TAPVR was guided by the principles of (1) limiting dissection to the confluence only and minimizing manipulation of the PVs, (2) small endocardial-to-endothelial bites minimizing the thickness of the anastomosis, (3) exposure of the confluence by lifting the apex of the heart toward the right shoulder and creating an extracardiac anastomosis when possible, and (4) performing the anastomosis under circulatory arrest, especially in neonates, to optimize visualization and accuracy. Sixty-eight percent (n = 83) of the total cohort had circulatory arrest (87% of neonates, 38% of nonneonates). The technique of lifting the apex of the heart toward the right shoulder is especially preferred for infradiaphragmatic TAPVR repair in which it was applied in 92% (34 of 37) of these patients.
Other concomitant procedures included the creation of a systemic-to-pulmonary shunt in 14 (12%), pulmonary artery band in 4 (3%), bidirectional Glenn in 11 (9%), pulmonary artery augmentation in 5 (4%), extracorporeal membrane oxygenation in 2 (2%), repair of hypoplastic arch in 2 (2%), and other in 6 (5%). Mean cardiopulmonary bypass time was 130 ± 43 minutes. Mean aortic cross-clamp time was 69 ± 27 minutes. Mean circulatory arrest time was 24 ± 22 minutes. Inhaled nitric oxide was first used at Texas Children's Hospital in a TAPVR patient in March 1999 and since then has been used postoperatively in 34% (31 of 92) of TAPVR patients and in 47% (20 of 43) of obstructed TAPVR patients. On discharge, patients were followed up at the discretion of their cardiologist.
Statistical Summary
Nonparametric, binomial, and descriptive statistics were computed when appropriate. Fisher's exact test and the 
2 test were used to analyze binary variables. Kaplan–Meier survival analyses plotted survival curves from the follow-up data, and survival probabilities were compared for the two treatment groups. For these analyses, patients were censored at the time of death or were withdrawn alive at the point of last contact. Survival time was defined as age in days with date of operation taken to be time zero. Multivariable analyses were performed using Cox proportional hazard models. Fourteen variables were specified and tested for significance as risk factors in the entire TAPVR cohort for mortality and PV reoperation. The variables analyzed were continuous variables (age, weight, length of deep hypothermic circulatory arrest) and discontinuous variables (obstruction, age < 30 days, heterotaxy, operation date after July 2000, systemic-to-pulmonary shunt, pulmonary artery band, complete atrioventricular canal, pulmonary atresia, single ventricle, use of inhaled nitric oxide postoperatively, asplenia). Thirteen variables were specified and tested for significance as risk factors in the TAPVR with heterotaxy cohort for mortality and PV reoperation. Variables analyzed were continuous variables (age, weight, length of deep hypothermic circulatory arrest) and discontinuous variables (obstruction, age < 30 days, operation date after July 2000, systemic-to-pulmonary shunt, pulmonary artery band, complete atrioventricular canal, pulmonary atresia, single ventricle, use of inhaled nitric oxide postoperatively, asplenia). All analyses were conducted with SPSS 13.0 (SPSS, Inc, Chicago, IL).
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Results |
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Kaplan–Meier survival curves in Figure 1 demonstrate a 30-day, 1-year, and 5-year survival for the entire group of 93%, 86%, and 86%, respectively; for the nonheterotaxy group it was 96%, 89%, and 89%, respectively, and for the heterotaxy group it was 84%, 79%, and 79%, respectively. The survival curves between the heterotaxy and nonheterotaxy groups were not statistically different (p > 0.05). There were 16 deaths (12 hospital and 4 late deaths) during the entire study period: 8 occurred in the nonheterotaxy group (n = 84) at a mean postoperative day (POD) of 70.1 ± 69.3 days, and 8 occurred in the heterotaxy group (n = 38) at a mean POD of 28.3 ± 38.2 days. All mortality occurred in the first 6 months postoperatively.
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Within the heterotaxy cohort, there was no significant difference in regard to mortality or PV reoperation rate comparing the subpopulations of patients with or without the following conditions: obstruction, early or late era, single ventricle, and systemic-to-pulmonary shunts as shown in Table 2.
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Comment |
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The current study does not find that heterotaxy is a risk factor for death or that the survival curve of heterotaxy patients is statistically different from the survival curve of the nonheterotaxy cohort after TAPVR repair. The heterotaxy group does have a tendency toward early death (7 of the 8 heterotaxy deaths were in-hospital); however, the overall survival curves probably do not differ because of the low incidence (n = 1) of late heterotaxy deaths in the series. The latter perhaps can be explained by the heart center's vigilance in recognizing the early signs of restenosis, an aggressive surgical stance on reoperation of PV stenosis with a hospital survival rate of 92%, and the overall improvement in interstage and perioperative management of single ventricle patients. Also recently, in an attempt to avoid Fontan and interstage mortalities, heterotaxy patients with native pulmonary blood flow who at the time of bidirectional Glenn can be predicted to be poor Fontan candidates for anatomic reasons, are undergoing pulsatile Glenn procedures as perhaps their final palliation, as seen in Figure 2. The 1 late death in the heterotaxy group was after PV reoperation during the interstage period between stage I (TAVPR repair and systemic-to-pulmonary shunt) and II (bidirectional Glenn). No heterotaxy patient achieving stage II (bidirectional Glenn) has died. The Texas Children's Hospital heart center makes a concerted effort during the heterotaxy patient's initial admission to involve the other services these patients often require (ie, gastroenterology and pediatric surgery for the diagnosis and repair of malrotation before discharge). This programmatic management of the heterotaxy patient is an attempt to decrease the morbidity and mortality from cardiac and noncardiac complications of heterotaxy syndrome. Despite these efforts, as the length of follow-up increases in the current series, late morbidity and mortality from heterotaxy syndrome would be expected to rise.
The risk factors for death in the entire cohort are the diagnosis of pulmonary atresia and placement of a systemic-to-pulmonary shunt at the time of TAPVR repair. In this series, almost all pulmonary atresia patients (11 of 12) and patients getting a systemic-to-pulmonary shunt (12 of 14) were single ventricle patients. Although pulmonary atresia and systemic-to-pulmonary shunt are independent risk factors, both appear to identify the same subpopulation of single ventricle patients with ductal-dependent pulmonary blood flow. This group has always been identified throughout the literature as a subpopulation at risk for death [2–4]. Volume overload from a systemic-to-pulmonary shunt in a newborn with a single ventricle that is noncompliant because of recent surgery and a period of ischemic arrest is hazardous. With the addition of a particularly reactive pulmonary vasculature owing to preoperative TAPVR obstruction, the clinical scenario is often quite unstable and can result in death. The survival results of heterotaxy patients have improved to a level comparable to nonheterotaxy patients as evidenced by the comparison of their survival curves (Fig 1B) and the fact that heterotaxy was not a risk factor for death in the overall TAPVR cohort. However, caution should be exercised before stating that heterotaxy TAPVR patients have the same survival rates as nonheterotaxy TAPVR patients, without performing a larger scale study. Notable is the lack of influence of age, weight, and obstruction (ie, emergent status) on TAPVR and TAPVR with heterotaxy survival.
The overall reoperation rate on the PVs in this study compares favorably with the recent reports by large single centers [3, 8]. The PV reoperation rate in the heterotaxy patients was significantly higher than in the nonheterotaxy patients. This difference is underlined by the risk factors identified for PV reoperation for the entire cohort as the vast majority of the patients with these risk factors are heterotaxy patients. Heterotaxy was not a risk factor (p = 0.07) but was approaching significance and probably was underpowered. Single ventricle (90% of which are heterotaxy) was a risk factor for reoperation. However, 50% (4 of 8) of single ventricle patients underwent reoperation on the PV confluence during promotion to the next step in the single ventricle pathway (bidirectional Glenn, 3, and completion Fontan, 1). Therefore, it remains unclear whether these 4 patients, all of whom had mild PV stenosis, would have had reoperations because of PV stenosis alone. Need for a systemic-to-pulmonary shunt at the time of TAPVR repair (12 of 14 were heterotaxy) was also a risk factor for reoperation on the PVs. The lack of pulmonary flow evidenced by requiring a shunt is thought to contribute to the underdevelopment of PVs in these patients. This type of intrinsic PV stenosis or hypoplasia has been demonstrated in these patients by several histopathology studies [8–10]. Also, any degree of intrinsic PV stenosis is probably unmasked more often because of the increased flow from a systemic-to-pulmonary shunt [11, 12]. These findings may explain why patients receiving a systemic-to-pulmonary shunt, most of whom are heterotaxy patients, are at an increased risk for PV reoperation. Repair during the latter half of the series was also a risk factor. The percentage of heterotaxy patients operated on during the later era (36%) was higher than the number of heterotaxy patients operated on in the earlier era (24%). This may explain why era is a risk factor and again underlines the effect that heterotaxy syndrome has on PV reoperation rate.
The practice of performing minimal dissection and limiting it to the confluence is based on the theory that dissection and manipulation of the PVs themselves increases the inflammatory and eventual adhesive or fibrotic reaction of and around the PVs. This reaction may contribute to PV or anastomotic stenosis and need for reoperation. The often-unique PV and confluence anatomy in heterotaxy patients requires the PVs to be partially or fully dissected at times to identify all PVs and make an operative plan. This difference in technique may contribute to the increased PV reoperative rate in heterotaxy patients. The advances in operative techniques and perioperative care that have decreased the need for PV reoperation on TAPVR patients as a whole are unlikely to neutralize the effect of intrinsic anatomic and histopathologic differences in the PVs of heterotaxy patients [9,10]. This perhaps explains why in comparison with nonheterotaxy patients, the PV reoperation rate for TAPVR with heterotaxy patients remains uniformly higher in the literature.
Interestingly, there were no risk factors found in the heterotaxy cohort for mortality or for reoperation on the pulmonary venous confluence. This result probably can be attributed to two features: (1) this is a small cohort that is quite homogenous (ie, 95% are single ventricles, which is probably one of their most defining features); and (2) no subpopulation of the heterotaxy patients (ie, obstructed TAPVR) differed significantly from the rest of the heterotaxy cohort in mortality or reoperation rate (Table 2).
Heterotaxy patients continue to require a significantly higher reoperative rate on the PVs than do nonheterotaxy patients. However, recent advancements in the care of TAPVR patients have not only improved their survival as a whole but, in this series, have conferred this effect on the subpopulation with heterotaxy syndrome, making it no longer a risk factor for death. At midterm follow-up, heterotaxy patients undergoing TAPVR repair have comparable overall survival to that of nonheterotaxy patients.
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Discussion |
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DR MORALES: We do not normally perform transesophageal echos or epicardial echos. Our suture technique is usually a running Prolene suture.
DR HENRY L. WALTERS (Detroit, MI): Your results are dramatically different and better than in many reported series. To what do you ascribe these improved results?
DR MORALES: After looking at all the data and looking at the results, we have thought long and hard about why our outcomes are different and it remains slightly unclear. I will say that when we are repairing patients with heterotaxy and TAPVR, we try to avoid placing a Blalock-Taussig shunt in those patients with any prograde pulmonary flow, even if they have severe pulmonary stenosis. Placement of a shunt makes these patients' management quite difficult, as shown in the literature, where patients requiring a shunt have consistently been the subpopulation of heterotaxy TAPVR patients with the highest mortality.
DR CHRISTOPHER KNOTT-CRAIG (Oklahoma City, OK): The heterotaxy patients with TAPVR who requires surgery during the neonatal period behave very differently from the older child. Did you specifically look at neonates with heterotaxy and TAPVR, and were their outcomes less flattering?
DR MORALES: Well, we looked at obstruction to see if it was a risk factor, which it was not. However, the vast majority of patients with obstruction were repaired as neonates. In terms of looking at the neonates specifically, no, but age analyzed as a continuous variable was not a risk factor nor was it when combined with obstruction. You are right in that the older patients did tend to get a bidirectional Glenn at the time of repair. This bidirectional Glenn group, which consists of about 11 patients, was on average around 7 months at the time of repair. Four of these patients had prior operations consisting of BT (Blalock-Taussig) shunts.
DR KIRK R. KANTER (Atlanta, GA): I'm struck by the survival curve for the heterotaxy patients. These patients have other problems other than their heart disease, including asplenia and polysplenia, and tend to die of other things such as noncardiac-related infection. Yet you had no mortality in your group after the first early period. This brings into question your follow-up. Is your follow-up accurate or is it just that in Texas heterotaxy patients don't die of other things?
DR MORALES: Well, having gone through all the records and seeing that most of these patients are being followed at Texas Children's, I would have to say our follow-up is complete—I believe that our curves aren't statistically different between the heterotaxy and nonheterotaxy cohorts because as you've pointed out, once patients reached about 7 months or really, once anyone got to bidirectional Glenn, we have had no decline in our heterotaxy curve. There has been no mortality after bidirectional Glenn or Fontan. However, I am sure that as our follow-up continues we will see mortality.
I think contributing to our low late mortality is that those patients, who at time of their Glenns we identify as having unfavorable anatomy for a Fontan, receive a pulsatile Glenn. These patients are often left with this circulation as their final palliation, with which they do quite well. We feel this is better than trying to create some complex baffle in a patient with unfavorable anatomy. These attempts may represent the still significant mortality and morbidity reported for heterotaxies after Fontan. Therefore our avoiding this situation by performing pulsatile Glenns in selective patients may help explain our decreased late mortality.
DR CHARLES D. FRASER (Houston, TX): I might just comment on that last question by Dr. Kanter. Since 1995, we have employed sort of a multidisciplinary team to look after these heterotaxy syndrome patients, although it's probably not as formalized as our colleagues in Wisconsin. We do employ the assistance of our infectious disease service. All of these patients get introduced to the general surgeons at the time of diagnosis. We prophylactically do Ladd's procedures on these patients. We follow them very closely. So that may explain the lack of late attrition.
And it's also a relatively short follow-up, and I expect that there will be attrition over time in even the patients who have done well after the Glenn.
DR KANTER: That is a very important point, that you have a special clinic, because they don't all die of their heart disease. I applaud you for that and I will go back and try to do that in our institution.
DR PHILIP C. SMITH (Akron, OH): I'm just curious about the subgroup of patients whoi you're performing a concomitant bidirectional Glenn on. Are any of those patients presenting with pulmonary venous obstruction?
DR MORALES: A couple of those patients did present with obstruction, but some of those patients had a Blalock-Taussig shunt as a primary procedure, and then presented for their bidirectional Glenn as well as repair of their TAPVR. The increased flow going through the confluence from the shunt perhaps increased the pressure gradient, so they had obstruction in that sense. However, none of these patients had more than mild obstruction, and none required emergent repair.
DR FRANÇOIS LACOUR-GAYET (Denver, CO): The term TAPVR and heterotaxy is one aspect of this disease that associate, as you know, complete AV (atrioventricular) canal, DORV (double-outlet right ventricle), TAPVR, and heterotaxy. As you have observed, and we have the same experience, the risk of pulmonary vein obstruction is huge. And for a reason that is not clear, this risk is more important in our experience than in yours. I don't know if it is the altitude, but we have very concerning incidence of pulmonary vein stenosis in this group of patients.
In the group of complete AV canal and DORV, we have found that it was better to try to go on and do a biventricular repair. Would you consider doing a biventricular repair in those patients with two viable ventricles provided the risk of pulmonary vein stenosis?
DR MORALES: Out of the 38 heterotaxy patients, there were 2 who had biventricular repair eventually, and both had double-outlet right ventricle and complete AV canal. The other double-outlet right ventricle patients were unbalanced canals and did not undergo biventricular repair. When possible, yes, we are aggressive about trying to do a biventricular repair.
DR CARL L. BACKER (Chicago, IL): I would like to make an editorial comment relating to the fact that you used circulatory arrest for all these patients, correct?
DR MORALES: Yes.
DR BACKER: But it was a relatively short mean period of about 25 minutes, correct?
DR MORALES: Yes.
DR BACKER: So in the debate between continuous cardiopulmonary bypass with low-flow perfusion and deep hypothermic circulatory arrest, this seems to be a group of patients in whom circulatory arrest was very useful. Is that correct?
DR MORALES: Yes, we feel that it is for this one particular operation. As you know, we favor selective cerebral perfusion but we feel that being extremely precise and having good visualization at the initial repair is essential, because, as everyone knows, once restenosis occurs, it is quite difficult to ever achieve a satisfactory result.
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  T. Nakata, Y. Fujimoto, K. Hirose, M. Osaki, Y. Tosaka, Y. Ide, M. Tachi, and K. Sakamoto Functional single ventricle with extracardiac total anomalous pulmonary venous connection Eur. J. Cardiothorac. Surg., July 1, 2009; 36(1): 49 - 56. [Abstract] [Full Text] [PDF]
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P. V. Anagnostopoulos, J. M. Pearl, C. Octave, M. Cohen, A. Gruessner, E. Wintering, and M. F. Teodori Improved current era outcomes in patients with heterotaxy syndromes Eur. J. Cardiothorac. Surg., May 1, 2009; 35(5): 871 - 878. [Abstract] [Full Text] [PDF]
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