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J Thorac Cardiovasc Surg 1999;117:506-514
© 1999 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

LONG-TERM OUTCOME OF INFANTS WITH SINGLE VENTRICLE AND TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION

From The Divisions of Cardiothoracic Surgery,^a Pathology,^b and Cardiology,^c The Children's Hospital of Philadelphia, and the Department of Biostatistics,^d Temple University School of Medicine, Philadelphia, Pa.

Read at the Seventy-eighth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 3-6, 1998.

Received for publication May 8, 1998. Revisions requested June 10, 1998. Revisions received Oct 27, 1998. Accepted for publication Nov 3, 1998. Address for reprints: J. William Gaynor, MD, 34th St & Civic Center Blvd, Philadelphia, PA 19104.

	Abstract

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Background and methods: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival.
Results: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14. Obstructed total anomalous pulmonary venous connection was present in 21 patients. The pulmonary venous connection was supracardiac in 32 patients, cardiac in 21 patients, infracardiac in 13, and mixed in 7. Twelve patients died before the operation. The remaining 61 patients underwent surgery at a median age of 5 days (range 1 day–2.5 years). Overall survival was 45% at 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5 years. By univariate analysis with the Cox proportional hazards model, younger age at the time of the initial operation and repair of total anomalous pulmonary venous connection were predictors of mortality. Lung tissue from 14 patients was available for histologic examination. The pulmonary veins were dilated and wall thickness was increased. Increased muscularization of the arteries was seen in 11 patients.
Conclusions: The long-term prognosis for children undergoing staged reconstructive operations for single ventricle and total anomalous pulmonary venous connection is poor. Early mortality is high and late death is a continuing risk. Development of the pulmonary vasculature, especially the pulmonary veins, is abnormal, even in children without clinical evidence of pulmonary venous obstruction.

	Introduction

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The term functional single ventricle encompasses a heterogeneous group of anomalies including hypoplastic left heart syndrome (HLHS), tricuspid atresia, heterotaxy syndrome, and other complex cardiac defects. Many children with these anomalies are referred for treatment in the neonatal period and undergo a series of staged reconstructive procedures leading to an eventual Fontan operation. The short- and long-term outcomes for these children have improved significantly in recent years. Improvements in surgical techniques and perioperative management have contributed to significant decreases in mortality and morbidity after the Fontan procedure. Certain anatomic factors, however, including HLHS, heterotaxy syndrome, and total anomalous pulmonary venous connection (TAPVC), have been associated with increased early and late mortality after the Fontan procedure. The current study was undertaken to assess the impact of TAPVC on early mortality and long-term outcome in children with functional single ventricle.

	Methods

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All patients with functional single ventricle and TAPVC treated at The Children's Hospital of Philadelphia between July l, 1984, and December l, 1997, were eligible for entry into the study. Patients were retrospectively identified by review of the cardiac surgical and echocardiography databases, as well as autopsy records. The medical records were reviewed and follow-up information was obtained from medical records and contact with referring physicians.

Data are presented as mean ± standard deviation or median as appropriate. Variables were compared by means of the Student unpaired t test. Confidence levels (95%) are presented as appropriate. For survival analysis, date of birth was taken as zero time. The patients were censored at the time of death or were withdrawn alive at the time of last follow-up contact. The Kaplan-Meier survival estimates with 95% confidence limits are provided. Survival distributions between groups were compared by means of the log rank test. The Cox proportional hazards model was used to examine the effects of anatomic and procedural variables on survival time both in the entire population (n = 73) and in the subgroup who underwent surgery (n = 61) (see Table III). A univariable analysis was performed and variables with P < .1 were included in the multivariable analysis. Data analysis was performed by means of SPSS version 7.5.1 (SPSS, Inc, Chicago, Ill) and SAS version 6.12 (SAS Institute, Inc, Cary, NC).

	Results

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Recurrent pulmonary venous obstruction necessitating reoperation developed in 7 of the 13 survivors (54%) of initial TAPVC repair. Only 1 of these patients is a long-term survivor. Infracardiac TAPVC was present in 5 of these patients and supracardiac TAPVC in 2. Pulmonary venous obstruction had been present in 3 of the patients before the initial TAPVC repair.

Follow-up is complete (death or December 1, 1997) in 71 of the 73 patients (97%). In the 2 remaining patients, partial follow-up was available until their families moved abroad (11 months and 15 years after the operation). As of December 1, 1997, 12 of the 73 patients were alive and 9 of these patients had successfully undergone the modified Fontan procedure. The median duration of follow-up for the entire group (n = 73) is 4 months (range 1 day–17 years). The median follow-up for patients who survived the initial hospitalization (n = 34) is 26 months (range 0.1 month–17 years). Fifty-one patients were followed up for at least 6 months, 25 for 1 year, 18 for 2 years, and 11 patients for more than 5 years. For the entire group (n = 73), survival was 45% (95% CL, 34%, 57%) at 6 months, 37% (95% CL 25%, 48%) at 1 year, and 19% (95% CL 10%, 29%) at 5 years. Survival was better for the patients who underwent surgery: 54% (95% CL 42%, 67%) at 6 months, 44% (95% CL 31%, 56%) at 1 year, and 23% (95% CL 12%, 34%) at 5 years, P < .0001 by log rank test (Fig. 1). For the entire group, survival was worse for patients with obstructed TAPVC than for those with unobstructed TAPVC (P = .02). Repair of TAPVC at the initial operation was associated with worse survival (P = .02) (Fig. 2).

View larger version (16K): [in this window] [in a new window]   Fig. 1. Actuarial survival (Kaplan-Meier) comparing outcome for all patients with outcome of those undergoing staged reconstructive operations.

View larger version (18K): [in this window] [in a new window]   Fig. 2. Actuarial survival (Kaplan-Meier) comparing outcome for patients who underwent TAPVC repair with outcome for those in whom TAPVC repair was not performed.

View larger version (154K): [in this window] [in a new window]   Fig. 3. A, A pulmonary vein from a patient with HLHS and normal venous return appears in the center of the photograph as a dilated vessel without markedly thickened media; a dilated lymphatic vessel is at the right of the vein in the interlobular septum. B, A very abnormal pulmonary vein from a study patient is seen in the interlobular septum, surrounded by alveolar spaces. The vein wall is very thick and nuclei are seen in the mural fibers. (A and B, Hematoxylin and eosin stain; original magnifications x400.)

View larger version (150K): [in this window] [in a new window]   Fig. 4. A, An elastic tissue stain of a pulmonary vein of the same patient as in Fig 3, A, shows a thin wall, without distinct inner and outer elastic tissue laminae. B, In contrast, an elastic tissue stain from a vein of the same patient as in Fig 3, B, shows multiple elastic tissue laminae, so-called arterialization of the venous wall. (A and B, Elastic van Gieson stain; original magnifications x400.)

	Discussion

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The current study was undertaken to assess the impact of TAPVC on early mortality and long-term outcome in infants with single ventricle. Heterotaxy syndrome was present in the majority of these children. This study demonstrates that the long-term prognosis for these children is poor. Palliative surgery resulted in improved survival; however, the mortality for the initial palliative procedure is high, and late death is a continuing risk. Mortality rates at the time of superior cavopulmonary connection and the Fontan operation were much greater than for children without TAPVC. ¹ The cause of death often could not be determined from the medical record; however, in the majority of cases significant hypoxemia and inadequate cardiac output were evident. Obstruction to pulmonary venous drainage was a risk factor for mortality for the entire group. Younger age at the time of the initial operation and repair of TAPVC were associated with increased mortality. Early and late mortality were significantly greater in patients who underwent repair of TAPVC. Recurrent pulmonary venous obstruction was prevalent after TAPVC repair (>50%). Only 1 patient with recurrent pulmonary venous obstruction is a long-term survivor. The reasons for the increased incidence of recurrent pulmonary venous obstruction are unclear. Histologic examination of lung tissue from patients in this series demonstrates that development of the pulmonary vasculature, particularly the pulmonary veins, is abnormal in infants with single ventricle and TAPVC. The veins were abnormal even in the absence of clinical evidence of pulmonary venous obstruction, suggesting that current diagnostic techniques may underestimate the severity of obstruction.

DeLeon and colleagues ⁴ reported early results after palliative surgery in 8 infants with obstruction to pulmonary blood flow and TAPVC. Heterotaxy syndrome was present in all patients and 7 patients had functional single ventricle. Four of these 7 patients died. Di Donato and colleagues ⁵ reported outcome after palliation in 14 children with right atrial isomerism (asplenia) and TAPVC. Thirteen patients underwent palliative operations consisting of aortopulmonary shunts in 10 patients and shunts with TAPVC repair in 3 patients. There were 7 hospital deaths (54%) and 2 late deaths. No patients had undergone cavopulmonary connection or a Fontan procedure at the time of these reports.

Heinemann and associates ⁶ reported their experience with initial palliation in 21 patients with heterotaxy syndrome and TAPVC. Pulmonary venous drainage was obstructed in 12 patients and unobstructed in 9. None of these patients had obstruction to systemic blood flow. The overall early mortality was 28.6% (6/21). The mortality for aortopulmonary shunt alone was 25% (2/8) and for shunt in combination with TAPVC repair, 33.3% (2/6). Mortality for TAPVC repair alone was 50% (2/4). Long-term follow-up was not reported.

Sadiq and coworkers ⁷ recently reported outcomes after surgical palliation of 20 children with right atrial isomerism. TAPVC was present in 16 patients. The overall mortality was 55% (11/20) and was 82% (9/11) in patients who underwent surgery during the first month after birth. Seven patients have undergone the Fontan procedure, with 2 deaths. No patient who was referred to us during the first week of life with obstructed TAPVC and pulmonary atresia survived.

Hashmi and associates ⁸ reported outcomes for 91 patients with right atrial isomerism treated at The Hospital for Sick Children in Toronto between 1970 and 1996. Surgery was undertaken in 69 patients, and 64 of these underwent staged reconstructive surgery with the plan of an eventual Fontan procedure. TAPVC repair alone or in association with other procedures was performed in 20 children with an early mortality of 95%. At last follow-up, 40 of the 64 patients had died, 10 had successfully undergone the Fontan procedure, 13 were alive and awaiting the Fontan procedure, and 1 patient had undergone cardiac transplantation. Overall actuarial survival was 49% at 1 year and 35% at 5 years. Risk factors for mortality were absence of pulmonary outflow obstruction, presence of major atrioventricular valve anomaly, and obstructed TAPVC.

Alejos and colleagues ⁹ evaluated risk factors for mortality in 129 patients undergoing bidirectional Glenn shunts. Anomalous pulmonary venous connection, either partial or total, and heterotaxy syndrome were risk factors for death. McElhinney and colleagues ¹⁰ reported outcome after bidirectional Glenn shunts in patients with anomalies of systemic and pulmonary venous drainage. In this series, 11 patients had single ventricle and TAPVC. Heterotaxy syndrome was present in 10 of the 11 patients. Repair of TAPVC was necessary in only 3 patients. One early death and 1 late death occurred among the 11 patients.

Razzouk and associates ¹¹ reported outcomes after cardiac transplantation in 12 children with complex cardiac defects and TAPVC. Heterotaxy syndrome was present in 9 patients and HLHS in 2. TAPVC was supracardiac in 10, infracardiac in 1, and mixed in 1. Six children had undergone a previous operation, but only 1 had undergone TAPVC repair. Transplantation was performed during the first 6 months after birth in 8 of the 12 patients. Overall survival was excellent, with only 1 early and 1 late death. Recurrent pulmonary venous obstruction developed in 2 patients. A more recent report of transplantation in 20 patients with heterotaxy syndrome (13 with TAPVC) demonstrated a 1-year survival of 93% and a 5-year survival of 65%. ¹²

The findings of the current study are consistent with those of previously published results and demonstrate a significant early mortality and poor long-term outcome for children with functional single ventricle and TAPVC. Risk factors for mortality include younger age at the time of the initial operation, obstructed TAPVC, and need for TAPVC repair at the initial operation. Children with more severe obstruction to pulmonary venous drainage are likely to have more severe changes in the pulmonary vasculature and to be referred for treatment at an earlier age. The greatest risk for early death was in patients undergoing simultaneous creation of an aortopulmonary shunt and TAPVC repair. Interestingly, the presence of heterotaxy syndrome or HLHS was not associated with worse outcomes. Examination of the pulmonary vasculature from patients with single ventricle and TAPVC reveals very abnormal development, especially of the pulmonary veins. It is likely that increased pulmonary vascular resistance is a major cause of mortality for these children. The cause of the increased mortality for superior cavopulmonary connection and the Fontan procedure is less clear. The preoperative hemodynamic data do not suggest that the patients are at increased risk. However, the pulmonary vascular abnormalities may contribute to the increased mortality. Interestingly, despite the abnormal pulmonary vasculature, the early results after cardiac transplantation in these patients appear to be satisfactory. ^11,12 Perhaps the pulmonary vascular abnormalities are better tolerated when a ventricle provides pulmonary blood flow rather than an arterial or venous shunt.

This study has several limitations. It is retrospective, and the data are limited by review of the medical records. To ensure inclusion of all patients, even those who did not undergo surgery, we reviewed multiple sources including the echocardiography database, surgical database, and autopsy records. The criteria for diagnosis of pulmonary venous obstruction were not standardized. A patient was classified as having obstructed TAPVC if the echocardiogram was considered to show obstruction of the draining vein or if the physician caring for the patient diagnosed obstructed venous drainage on the basis of the clinical course. There is obviously some bias in the selection of patients for histologic examination of the pulmonary vasculature. Because only autopsy specimens were available, the findings likely represent the more severely affected patients.

In conclusion, this study demonstrates that children with functional single ventricle and TAPVC have a very poor prognosis. The outcome of staged reconstructive surgery is poor. Alternative therapy such as cardiac transplantation may be indicated for these patients.

	Appendix: Discussion

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Dr William G. Williams (Toronto, Ontario, Canada). Dr Gaynor's review of infants with single ventricle and TAPVC demonstrates the dismal outlook for these children despite the authors' best efforts. I congratulate them for their candor, honesty, and courage in presenting this information.

Although better techniques in pediatric cardiac care have produced substantially improved results for newborn infants with either univentricular heart or TAPVC in isolation, the combination of the two lesions appears to be almost universally fatal.

First of all, I applaud their decision to include the babies identified with the diagnosis but not surviving long enough to undergo operation. The 16% who died before surgical intervention are an index of the severity of these lesions. As surgeons, we do have an obligation to these patients who do not survive long enough for our care. Our management protocols must evolve to salvage these children.

The most telling statistic is the failure to manage these babies at the time of cavopulmonary shunt. The 38% mortality at cavopulmonary shunt, an operation which for other lesions is a very low-risk procedure, is an index of the severity of the underlying disease that we do not clearly understand.

The cavopulmonary shunt is used as a staging procedure in patients with single ventricle to lessen the risk of a subsequent Fontan operation. In the authors' series, only 26% of the patients actually had a Fontan operation. This outcome is very similar to our experience in patients with heterotaxy, among whom only 22% eventually had the Fontan operation. Cavopulmonary shunt was not apparently protective, as illustrated by the 42% operative risk at the time of the Fontan operation and the subsequent 20% late mortality.

In an analysis of cavopulmonary shunts in our center, it is clear that there are some patients in whom conversion from a cavopulmonary shunt to a Fontan circulation offers no survival advantage and is probably contraindicated. Patients with heterotaxy, single ventricle, and TAPVC are good examples of this problem. Dr Gaynor's experience is similar to the series from Toronto reported by Hashmi and the series from Boston reported by Jenkins. In our experience, patients treated more recently have an even worse prognosis than those treated in the earlier era.

Dr Gaynor's group identified the following as risk factors: abnormal pulmonary veins, thickening of the muscular walls, increased elastin, and dilated lumen. Jenkins previously reported that small pulmonary artery diameter and a small pulmonary artery confluence are predictors of poor outcome.

Is there hope for these children? Razzouk and associates, from Loma Linda, reported on 30 children with heterotaxy who had a 65% survival 5 years after heart transplantation. Heterotaxy was not a risk factor among their transplant series. I have two questions for Dr Gaynor. First, is the histology of dilated pulmonary veins that you report compatible with the observations of Jenkins that small pulmonary veins are detrimental to survival?

Second, what is the current or future protocol at The Children's Hospital of Philadelphia for managing infants with single ventricle and TAPVC in combination? If transplantation is recommended, what priority do these children warrant to procure a very scarce resource?

Dr Gaynor. Dr Williams, thank you very much for your comments. The pathology that we examined is of children who have undergone repair of TAPVC, and they may have had high atrial pressures. We examined small veins within the lung parenchyma. Because this is a retrospective study, we were unable to assess the size of the pulmonary veins at the site of entrance into the heart to know how small or how large those veins were. If there is obstruction at that level, the upstream veins may be dilated.

In terms of the current management of the children at our institution, certain children can be managed initially with only an aortopulmonary shunt. The major group includes children who have unobstructed pulmonary venous drainage, particularly drainage to the cardiac veins or to the cavoatrial junction. The mortality for shunt alone as the initial palliative procedure was acceptable. We would probably use a shunt in those children and then try to determine the optimal method of treatment at a later date, either cavopulmonary anastomosis leading to a Fontan operation or cardiac transplantation.

For children who are brought for treatment in the neonatal period and have pulmonary veins that must be repaired, particularly those with obstructed veins, cardiac transplantation is the best option. I think the presence of either a systemic shunt or a venous shunt in children who have these abnormal pulmonary vessels and a high degree of reactivity is the reason for the high mortality.

Many of the infant hearts available for transplantation are currently used for children with HLHS who do have another option, the Norwood procedure. In these children with TAPVC and single ventricle, there really is no other option. Under the current organ allocation system, infants are not stratified as possible recipients for cardiac transplantation according to the availability of other possible procedures. As a group, we should amend that system to give priority to infants for whom cardiac transplantation is the only option.

	Acknowledgments

 
We appreciate the contributions of the many physicians and other personnel who cared for these patients, including Larry W. Stephenson, MD, John D. Pigott, MD, William I. Norwood, MD, and Marshall L. Jacobs, MD.

	References

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11. 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;14:713-7. [Medline]
    
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