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Ann Thorac Surg 2005;79:613-617
© 2005 The Society of Thoracic Surgeons

Original article: Cardiovascular

Early Outcome After Glenn Shunt and Fontan Palliation and the Impact of Operation During Viral Respiratory Season: Analysis of a 19-year Multi-Institutional Experience

Ramzi T. Nicolas, MDa,*, Christine Hills, BAb, James H. Moller, MDc, Charles B. Huddleston, MDd, Mark C. Johnson, MDa

a Division of Pediatric Cardiology, Washington University in St. Louis, School of Medicine, St Louis, Missouri
d Division of Cardiothoracic Surgery, Washington University in St. Louis, School of Medicine, St Louis, Missouri
b Pediatric Cardiac Care Consortium, University of Minnesota, School of Medicine, Minneapolis, Minnesota
c Division of Pediatric Cardiology, University of Minnesota, School of Medicine, Minneapolis, Minnesota

Accepted for publication July 6, 2004.

* Address reprint requests to Dr Nicolas, Suite 5S30, One Children's Place, St. Louis, MO 63110 (E-mail: nicolas_r{at}kids.wustl.edu).


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 Acknowledgments
 References
 
BACKGROUND: This study was done to investigate the impact of season of operation, age at operation, and surgical era on short-term outcome after single ventricle palliation. One small study suggests that operation during viral respiratory season is associated with prolonged pleural effusion and hospitalization. Single-institution studies also find improved outcomes after the Glenn shunt and Fontan over time, despite operation at a younger age.

METHODS: The Pediatric Cardiac Care Consortium (PCCC) database for Fontan (n = 2,713) and Glenn shunt (n = 2,239) performed between 1982 and 2000 was utilized. Death during initial hospitalization and length of stay after each procedure were investigated. Viral respiratory season was defined as the interval of November to March.

RESULTS: The mortality rate after Fontan was higher during the viral respiratory season (14 versus 11%, p = 0.03) and in children under age 2 years. Glenn shunt mortality was higher with operation before age of 120 days, but was not affected by operation during viral respiratory season. Age at operation for the Fontan procedure has decreased with declines in hospital mortality (14% to 7%, p < 0.0001) and hospital stay (14 to 11 days, p < 0.0001) comparing 1992 to 1995 with 1996 to 2000.

CONCLUSIONS: Fontan procedure performed during viral respiratory season is associated with an increased mortality. Short-term mortality and morbidity after the Glenn and Fontan procedures have improved in the recent era. Operation in the youngest age groups may adversely impact mortality.


   Introduction
Top
 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 Acknowledgments
 References
 
Children undergoing cardiac surgery with active respiratory tract infections suffer greater postoperative complications [1, 2]. Winter season is associated with a higher incidence of respiratory illnesses, especially infection with respiratory syncytial virus [3, 4].

Some congenital heart centers avoid elective operations during winter months for children with single ventricle physiology because of higher anticipated risk of complications from respiratory infections; however, few data are available to support this policy. One recent study suggested that performance of the Fontan operations during the viral respiratory season was associated with prolonged pleural effusion and longer hospitalization [5].

The investigation of the impact of seasonal timing of operation on outcome is complicated by the many refinements in the surgical palliation of single ventricle congenital heart defects that have been made in the past 2 decades. These advancements include performance of Glenn shunt and Fontan procedures at an earlier age, more frequent use of a staged repair with a Glenn shunt before a Fontan procedure, use of a lateral tunnel or extracardiac conduits, and fenestration of the Fontan baffle. Single-institution studies suggest that these revisions have resulted in lower mortality, shorter duration of pleural effusions, shorter hospital stay, and fewer additional postoperative procedures [6–9].

We utilized a large multi-institutional database to investigate the hypothesis that operation during viral respiratory season adversely affects mortality and morbidity after Glenn and Fontan procedures. Additionally, we investigated the impact of patient age and surgical era on early morbidity and mortality after these operations.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 Acknowledgments
 References
 
Patient Population
The multi-institutional Pediatric Cardiac Care Consortium (PCCC) database was utilized to investigate Fontan operation (n = 2,713) and Glenn shunt (n = 2,239) procedures performed between 1982 and 2000. Patients with take down of previous Fontan (n = 61) were excluded from analysis. Outliers (more than 4 standard deviations from the mean and more than 1 standard deviation from the proximal value) for age and hospital stay were excluded from the study. These included 142 Fontan patients and 25 Glenn patients who were older than 21 years at time of the procedure. Two Fontan patients whose lengths of stay were 381 and 382 days and 3 Glenn patients who were hospitalized for 220, 227, and 370 days were also excluded. Cases of hospital death were excluded from length of stay analysis.

Data Collection
The PCCC collects and analyzes data for all cardiac procedures performed at member institutions on an annual basis for purpose of quality improvement. To ensure high-quality data and consistency among institutions, the program coordinator manually reviews cardiac diagnosis and procedure codes of the PCCC data. The information is entered into the computer system by two different persons, and the two versions of the data are compared electronically; computer programs check for out-of-range values, nonsequential dates, and missing data. The University of Minnesota Institutional Review Board has exempted PCCC data outcome studies from review.

Statistical Analysis
Viral respiratory season was defined as the time period between November 1 and March 31. Death during initial hospitalization and length of stay after each procedure were investigated. Cases with hospital death were excluded from length of stay analysis. Patients were classified based on their cardiac surgical history before the Glenn or Fontan operation: 1, previous open heart operation; 2, previous closed heart procedure (pulmonary artery band, Blalock-Taussig shunt, or patent ductus arteriosus ligation); or 3, no previous cardiac operations. Cardiac anatomy was classified as tricuspid atresia (n = 761) versus other diagnoses. Data were analyzed with Statview (SAS Institute, Cary, NC). The nonparametric method was used to compare stratified data. The SAS Proc Logistic program (SAS Institute) was used for multivariate analysis. Continuous data were compared by unpaired t test, and categorical data by {chi}2 test.


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 Acknowledgments
 References
 
The number of Fontan procedures reported to the PCCC each year has increased and hospital mortality has declined during the period of the study (Fig 1). Some of the increase in number of operations is due to addition of centers reporting to PCCC over time. Median age at operation for the Fontan procedure has decreased despite more frequent use of the Glenn shunt before the Fontan procedure (Table 1). The number of Glenn shunts reported in 1992 to 2000 era compared with the 1982 to 1991 era has increased (1,877 versus 362 cases) with a decline in the median age at operation (0.8 versus 3.2 years, p < 0.0001).



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  		Fig 1. Number of Fontan operations (bars) and mortality (line), 1982 to 2000.

 

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  		Table 1. Fontan Patient Characteristics by Surgical Era
 
The effect of viral respiratory season on the outcome of both procedures was investigated. No seasonal effect was observed for mortality and hospital stay after a Glenn shunt for all patients (p = 0.6 and 0.7) or for the subgroup of patients less than 2 years of age (p = 0.9 and 0.7). Mortality after the Fontan procedure was higher when operation occurs during the November to March viral respiratory season (14% versus 11%, p = 0.03), although fewer operations were performed in these winter months (Fig 2). There was no relationship between length of stay after Fontan procedures and season of operation (p = 0.42). When the analysis was restricted to patients with Fontan performed before age 5 years in a more recent era (1992 to 2000), the effect of season of operation on mortality was more pronounced (14% versus 7%, p = 0.0005).



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  		Fig 2. Fontan monthly number of procedures (bars) and mortality (line), 1992 to 2000, for age less than 5 years.

 
A multivariate analysis was utilized to investigate confounding factors that may impact timing of operation. Diagnosis of tricuspid atresia was included in this analysis because tricuspid atresia has a more favorable prognosis in previous studies [10, 11] and in the current dataset (8% mortality for tricuspid atresia versus 14% for all other anatomic variants, p < 0.0001). Season remained as a predictor of mortality in this multivariate analysis. For Fontan procedures in patients younger than age 5 years from 1992 to 2000, later date of operation (p < 0.0001), older age (p = 0.002), diagnosis of tricuspid atresia (p = 0.001), and operation outside the viral respiratory season (p = 0.01) predicted lower mortality. Prior Glenn shunt did not predict mortality in this model (p = 0.85). In the multivariate analysis of length of stay after Fontan procedure, tricuspid atresia (p = 0.002) and year of operation (p = 0.0002) are associated with shorter length of stay, whereas operation during viral respiratory season (p = 0.9), age at operation (p = 0.1), and prior Glenn shunt (p = 0.4) had no impact on length of stay.

Further analysis was done to investigate mortality and length of stay in regard to age at operation and surgical era. Hospital mortality has decreased (14% versus 9%, p < 0.0001) and hospital stay is shorter (18 versus 12 days, p < 0.0001) with the Fontan procedure in the more recent surgical era (1982 to 1991 versus 1992 to 2000) for all patients. This is also true for patients less than age 5 years at the time of operation (mortality 20% versus 9% and length of stay 18 versus 12 days, p < 0.0001 for both). Further improvements are seen when comparing outcome of Fontan procedures performed between 1992 to 1995 versus 1996 to 2000. Mortality declined (14% versus 7%, p < 0.0001) and median hospital stay shortened (14 versus 11 days, p < 0.0001). The relationship of mortality to patient age in the more recent time period is shown in Fig 3.



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  		Fig 3. Number of Fontan procedures (bars) and mortality (line) by age, 1992 to 2000, for age less than 5 years.

 
Because the Glenn shunt was performed infrequently before 1992, further analysis of the impact of age of operation and surgical era on mortality compared the time periods of 1992 to 1995 and 1996 to 2000. In this analysis, open heart operations before a Glenn shunt have increased from 16% to 28%. The overall mortality has not significantly changed for the entire series (9% versus 7%, p = 0.2) or for patients less than 2 years of age (12% versus 7%, p = 0.06). Median length of hospital stay after a Glenn shunt decreased from 11 to 8 days for all ages (p < 0.0001), and from 9 to 8 days for patients less than 2 years of age (p = 0.05). For patients with Glenn shunt performed at age less than 2 years from 1992 to 2000, older age (p < 0.0001) and later date of operation (p = 0.0003) predicted lower mortality. The mortality rate for a Glenn shunt operation was higher when it was performed at an age younger than 120 days, compared with those performed on patients between the ages of 120 days to 2 years (15% versus 5% for operations done between 1992 and 1995, and 13 versus 4% for those done between 1996 and 2000, p < 0.0001 for both; Fig 4).



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  		Fig 4. Number of Glenn shunts (bars) and mortality (line), 1992 to 2000, for age less than 420 days.

 

   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
Acknowledgments
 References
 
This large multiinstitutional dataset demonstrates increased mortality associated with the Fontan procedure during the months of November to March coincident with the increased prevalence of viral respiratory infections [4]. This seasonal effect is more pronounced among younger patients and persists in a multivariate analysis. We speculate that the increased mortality is related to viral infections. The presence of an upper respiratory tract infection in children undergoing cardiac surgery has been found to be an independent risk factor for postoperative respiratory complications, infections, and intensive care unit length of stay [1]. While respiratory infection did not predict mortality in this earlier prospective study, only 27% of these children had single ventricle physiology. Fedderly and colleagues [5] posit higher transpulmonary gradients from subclinical respiratory abnormalities to explain their observation that performance of the Fontan procedure during viral respiratory season resulted in prolonged pleural effusions and increased length of stay. Our observation that younger patients undergoing Fontan procedures have greater seasonal variation in mortality is consistent with the observation that the incidence of upper respiratory infections before cardiac surgery varies inversely with age [1]. Our inference regarding viral infections and mortality is also supported by the observation that postoperative pulmonary hypertension in children is associated with symptomatic respiratory syncytial virus infection [2]. In patients with single ventricle physiology and elevated pulmonary vascular resistance, severe cyanosis or right heart failure may develop, with resultant increased mortality [6].

We did not find a higher mortality rate among patients with Glenn shunt performed during viral respiratory season. This may be because patients with Glenn shunts can more effectively maintain cardiac output in the face of elevated pulmonary vascular resistance as compared with patients with Fontan physiology. Furthermore, shorter hospital stay after a Glenn operation may limit patients' exposure to hospital-acquired respiratory infection.

These data demonstrate a progressive temporal decline in death and length of stay after palliative procedures for patients with single ventricle physiology, despite a younger age of operation. These findings are consistent with the results from smaller single-institution studies [6–9, 12, 13]. Completion of a Fontan procedure at an age younger than 4 years has been advocated to minimize the adverse long-term consequences of cyanosis and a volume-loaded ventricle. Glenn shunts are now used in most patients before completion of a Fontan operation. Our analysis suggests that this approach has not increased early death, as was originally predicted by Fontan [14]. Age limits may remain, however, as we found an increased mortality rate under age 2 years for the Fontan and under age 120 days for the Glenn shunt. These findings are consistent with the theoretical model of Senzaki and associates [15] showing that small body surface area, or lower age, has minimal effects on the Fontan circulation until it comes close to the infant value. Although a recent single-center report found no increase in deaths with the Glenn shunt performed before age 4 months, these younger patients had a longer duration of mechanical ventilation, pleural drainage, and hospitalization [16].

Study Limitations
A seasonal effect can be alternatively explained by a case selection bias with fewer high-risk operations being undertaken in the winter months as compared with a larger number of low-risk patients in the summer. However, season remains as a predictor of mortality in a multivariate analysis that utilizes tricuspid atresia and age of operation as surrogates for risk related to variable anatomy and physiology. Because Fontan procedures are typically scheduled electively, the higher number of operations performed outside of the winter months may be explained by family preference due to holiday, school, and work schedules as well as travel difficulties during cold weather months.

The PCCC data collection design limits the scope of our findings. Cause of death and hemodynamic data are not available for this analysis. Our conclusions regarding age limited mortality are tempered by the small number of patients in the youngest age groups and the possibility that these young patients represent exceptions to typical clinical scenarios.

Clinical Implications
These results suggest that the early Fontan mortality rate is lower when the operation is performed between April and October. Alternatively, more aggressive screening for viral respiratory infections and the use of the respiratory syncytial virus monoclonal antibody [17, 18] may eliminate some of the seasonal variation in mortality. Additional studies are needed to balance the risk of earlier age of operation and possible medium- and long-term benefits to be gained by early palliation of patients with single ventricle physiology.


   Acknowledgments
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Acknowledgments
References
 
Special thanks to Avril Adelman from the Department of Biostatistics at Washington University in St. Louis, Missouri.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Acknowledgments
 References
 

  1. Malviya S, Voepel-Lewis T, Siewert M, Pandit U, Riegger L, Tait A. Risk factors for adverse postoperative outcomes in children presenting for cardiac surgery with upper respiratory tract infections Anesthesiology 2003;98:628-632.[Medline]
  2. Khongphatthanayothin A, Wong PC, Samara Y, et al. Impact of respiratory syncytial virus infection on surgery for congenital heart disease: postoperative course and outcome Crit Care Med 1999;27:1974-1981.[Medline]
  3. Long SS. Respiratory syncytial virusIn: McMillan JA, DeAngelis CD, Feigin RDM, et al. editors. Oski's pediatrics: Principles and practice. Philadelphia: Lippincott Williams & Wilkins; 1999. pp. 1094-1098.
  4. Law BJ, Carbonell-Estrany X, Simoes E. An update on respiratory syncytial virus epidemiology: a developed country perspective Resp Med 2002;96(Suppl. B):1-7.[Medline]
  5. Fedderly RT, Whitstone BN, Frisbee SJ, Tweddell JS, Litwin SB. Factors related to pleural effusions after Fontan procedure in the era of fenestration. Circulation 2001;104(Suppl):I148–51..
  6. Gentles T, Mayer J, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome J Thorac Cardiovasc Surg 1997;114(Suppl):376-391.[Abstract/Free Full Text]
  7. Lemler MS, Scott WA, Leonard SR, Stromberg D, Ramaciotti C. Fenestration improves clinical outcome of the Fontan procedure: a prospective, randomized study Circulation 2002;105:207-212.[Abstract/Free Full Text]
  8. Yamauchi H, Imura H, Maruyama Y, et al. Evolution of staged approach for Fontan operation J Nippon Med Sch 2002;69:154-159.[Medline]
  9. Tokunaga S, Kado H, Imoto Y, et al. Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients Ann Thorac Surg 2002;73:76-80.[Abstract/Free Full Text]
  10. Gale AW, Danielson GK, McGoon DC, Wallace RB, Mair DD. Fontan procedure for tricuspid atresia Circulation 1980;62:91-96.[Abstract/Free Full Text]
  11. Mair DD, Puga FJ, Danielson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients J Am Coll Cardiol 2001;37:933-939.[Abstract/Free Full Text]
  12. Cetta F, Feldt RH, O'Leary PW, et al. Improved early morbidity and mortality after Fontan operation: the Mayo Clinic experience, 1987 to 1992 J Am Coll Cardiol 1996;28:480-486.[Abstract]
  13. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bi-directional Glenn procedure as staged reconstruction for the functional single ventricle Am J Cardiol 1993;71:959-962.[Medline]
  14. Choussat A, Fontan F, Besse P. Selection criteria for Fontan's procedure. In: Anderson RH, Shinebourne EA, eds. Pediatric cardiology 1997. Edinburgh: Churchill Livingstone, 1978:559–566..
  15. Senzaki H, Naito C, Kobayashi T, et al. Influence of age (body size) on the Fontan circulation—analysis by a theoretical model Jpn Circ J 2000;64:943-948.[Medline]
  16. Jaquiss R, Ghanayem N, Hoffman G, et al. Early cavopulmonary anastomosis in very young infants after the Norwood procedure: impact on oxygenation, resource utilization, and mortality J Thorac Cardiovasc Surg 2004;127:982-988.[Abstract/Free Full Text]
  17. Meissner HC, Long SS. American Academy of Pediatrics committee on infectious diseases and committee on fetus and newbornRevised indications for the use of palivizumab and respiratory syncytial virus immune globulin intravenous for the prevention of respiratory syncytial virus infections. Pediatrics 2003;112:1447-1452.[Abstract/Free Full Text]
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