Abstract
Background
The impact of age and weight on outcomes following the Fontan operation is unclear. Previous analyses have suggested that lower weight-for-age z-score is an important predictor of poor outcome in patients undergoing bidirectional Glenn. We evaluated variation in age, weight, and weight-for-age z-score at Fontan across institutions, and the impact of these variables on post-operative morbidity and mortality.
Methods
Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database undergoing the Fontan operation (2000–2009) were included. Center variation in age, weight, and weight-for-age z-score were described. Multivariable analysis was performed to evaluate the impact of age, weight, and weight-for-age z-score on in-hospital mortality, Fontan failure (combined in-hospital mortality and Fontan takedown/revision), post-operative length of stay, and complications, adjusting for other patient and center factors.
Results
A total of 2747 patients (68 centers) were included: 61% male; 45% right dominant lesions (38% left dominant, 17% undifferentiated). An extracardiac conduit Fontan (vs. lateral tunnel) was performed in 63%; 65% were fenestrated. Median age and weight at Fontan operation and proportion with weight-for-age z-score <−2 varied across centers ranging from 1.7–4.8 yrs, 10.5–16.1 kg, and 0%–30%, respectively. In multivariable analysis, age and weight were not significantly associated with outcome. Weight-for-age z-score <−2 was associated with increased in-hospital mortality (OR 2.73, 95%CI 1.09–6.86), Fontan failure (OR 2.59, 95%CI 1.24–5.40), and longer length of stay (+1.2 days, 95%CI 0.1–2.4).
Conclusions
Weight-for-age z-score <−2 is associated with significant morbidity and mortality following the Fontan operation independent of other patient and center characteristics.
Keywords: Congenital Heart Disease, Outcomes
Introduction
In 1978, Choussat published ten criteria describing optimal conditions under which to perform the Fontan operation, including age >4 years (1). In more recent years, the Fontan operation has been performed in younger and smaller patients at many centers. However, the impact of patient age and weight on outcome remains unclear. Some have reported satisfactory outcome following Fontan operation performed in children as young as 7 months (2). Others have reported that younger age at Fontan is associated with increased morbidity and mortality (3–5). There are also conflicting reports regarding weight with some studies suggesting that lower weight at Fontan operation (in particular weight <10 kg) is a risk factor for post-operative morbidity, while others have not shown this to be the case (6–8).
The majority of studies to date on this topic are single center reports limited by relatively small sample size (2–12). In addition, most studies have evaluated weight without taking into account weight relative to age as a predictor of outcome. A previous study has suggested that low weight-for-age z-score is an important predictor of poor outcome following the bidirectional Glenn operation (13). Further, recent data suggests that growth in single ventricle patients is modifiable, and may be optimized through a standardized feeding and monitoring program (14).
The purpose of this study was to evaluate variation across institutions in age, weight, and weight-for-age z-score at the time of Fontan operation, and to assess the impact of these factors on post-operative morbidity and mortality utilizing the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database.
Patients and Methods
Data Source
The STS Congenital Heart Surgery Database contains de-identified operative, peri-operative, and outcomes data on more than 160,000 patients, and represents nearly three quarters of all US centers performing congenital heart surgery (15). Data quality and reliability are assured through intrinsic verification of data as well as a formal process of site visits and data audits (16). The Duke Clinical Research Institute serves as the data warehouse for the STS Databases. This study was approved by the Duke University Medical Center institutional review board with waiver of informed consent, and by the Access and Publications Committee of the STS Workforce for National Databases.
Patient Population
To maximize data integrity, analysis was restricted to 68 STS centers with >85% complete data for all study variables. From these centers, patients ≤18 years who underwent primary Fontan operation from 2000–2009 were eligible for inclusion. Patients undergoing Fontan conversion or repeat Fontan operation were excluded. In cases where it was unclear if the patient had undergone previous Fontan operation and was >6 years of age, the patient was excluded from analysis as it was felt to be less likely the patient was undergoing primary Fontan operation. Patients with missing or invalid data for key variables of age and weight were excluded, along with those undergoing types of Fontan operation other than lateral tunnel or extracardiac conduit.
Data collection
Patient characteristics included age, weight, weight-for-age z-score, height, height-for-age z-score, and weight-for-height z-score (all calculated using standard growth curves) cardiac diagnosis (categorized as right dominant lesions, left dominant lesions, and undifferentiated), non-cardiac/genetic abnormality, number of prior cardiothoracic surgeries, preoperative length of stay, and other pre-operative factors including pre-operative neurologic deficit/seizures, arrhythmia, and complete heart block requiring pacemaker (these pre-operative factors represent those captured by the STS Database which occurred in >0.5% of patients undergoing the Fontan operation) (17). Center characteristics included annual Fontan operation volume and region. Operative characteristics included Fontan type (extracardiac conduit vs. lateral tunnel, and fenestration status as coded by the surgeon at the time of the operation) and secondary procedures performed at the time of Fontan operation.
Outcomes
The primary outcome was in-hospital mortality. Deaths occurring outside the hospital were not included in this analyses as these are not consistently captured in the STS Congenital Heart Surgery Database currently. Secondary outcomes included Fontan failure (combined in-hospital mortality, transplant, or Fontan revision/takedown), post-operative length of stay, and complications. Post-operative complications included any of those defined in the STS Congenital Heart Surgery Database (18).
Statistical Analysis
Patient, center, and operative characteristics were described. Age and weight-for-age z-scores were categorized for descriptive purposes based on the distribution of the data: age 0–2 years, 2–4 years, and >4 years; and weight-for-age z-score <−2, −2 to 0, and >0. Weight was categorized as <10 kg vs. ≥10 kg based on previous studies suggesting weight <10 kg was a significant risk factor for poor outcome (6).
Outcomes were compared across age, weight, and weight-for-age z-score groups in univariate analysis using the Chi-square and Kruskal-Wallis tests. The association of these variables with outcome was then evaluated in multivariable analysis utilizing hierarchical logistic and linear regression, with hospital specific intercepts to account for within center clustering. Age, weight, and weight-for-age z-score were evaluated both as continuous and as categorical variables. Multivariable analyses adjusted for other patient, operative, and center factors [sex, cardiac diagnosis, non-cardiac/genetic abnormality, other pre-operative factors as described above, preoperative length of stay, number of previous cardiac surgeries, Fontan type (extracardiac conduit vs. lateral tunnel, and fenestrated vs. non-fenestrated), other secondary surgical procedures performed at the time of Fontan operation, and center Fontan volume]. Length of stay was not normally distributed and was therefore log transformed for analysis. Results from logistic regression models are displayed as odds ratios (OR) and 95% confidence intervals and results from linear regression models as parameter estimates and 95% confidence intervals. Missing data were rare (< 0.5% for all variables). Patients with missing data for an endpoint were excluded from analysis involving that endpoint. All analyses were performed using STATA version 11.0 (StataCorp, College Station, TX). A p-value < 0.05 was considered statistically significant.
Results
Patient and operative characteristics
A total of 2747 patients from 68 centers (42.6% South, 22.1% Midwest, 19.1% West, 16.2% Northeast) were included (Table 1). Most patients were between 2–4 years, weighed 10–15 kg, and had a weight for age z-score between −2 and 1 at the time of Fontan operation (Figures 1–3). However, 13.1% were <2 years, 5.1% were <10 kg and 14.3% had a weight for age z-score <−2. Overall, both weight-for-age z-score and height-for-age z-score were low (Table 1). Evaluation of weight-for-height z-score suggested disproportionately lower weight in relation to height in the smallest patients (Table 1).
Table 1.
Patient, Operative, and Center Characteristics
| Variable | Overall (n=2747) |
<−2 (n=393) |
Weight-for-age z-score −2 to 0 (n=1580) |
>0 (n=774) |
|---|---|---|---|---|
| Patient Characteristics | ||||
| Age, years | 3.0 (2.3, 3.6) | 3.0 (2.3, 4.0) | 3.0 (2.3, 3.7) | 2.8 (2.3, 3.4) |
| Male | 1664 (60.6%) | 213 (54.2%) | 992 (62.8%) | 459 (59.3%) |
| Diagnosis | ||||
| Right dominant lesions | 1239 (45.1%) | 195 (49.6%) | 730 (46.2%) | 314 (40.6%) |
| Left dominant lesions | 1031 (37.5%) | 128 (32.6%) | 574 (36.3%) | 329 (42.5%) |
| Undifferentiated | 477 (17.4%) | 70 (17.8%) | 276 (17.5%) | 131 (16.9%) |
| Anthropometric Data | ||||
| Weight, kg | 13.2 (11.7, 14.8) | 10.8 (9.8, 12.0) | 12.9 (11.7, 14.2) | 15.0 (13.8, 16.5) |
| Weight-for-age z-score | −0.7 (−1.5, 0.1) | −2.5 (−3.1, −2.3) | −0.9 (−1.4, −0.4) | 0.6 (0.2, 1.0) |
| Height, cm | 91 (86, 97) | 87 (81, 93) | 91 (86, 97) | 93 (88, 99) |
| Height-for-age z-score | −0.8 (−1.5, −0.6) | −2.1 (−2.8,−1.4) | −0.9 (−1.5,−0.3) | 0 (−0.6, 0.7) |
| Weight-for-height z-score | −0.2 (−1.1, 0.7) | −1.8 (−2.5, −1.2) | −0.4 (−1.1, 0.2) | 1.0 (0.4, 1.6) |
| Pre-operative factors | ||||
| Neurologic deficit/seizures | 82 (3.0%) | 14 (3.6%) | 50 (3.2%) | 18 (2.3%) |
| Arrhythmia | 50 (1.8%) | 7 (1.8%) | 29 (1.8%) | 14 (1.8%) |
| Complete heart block/pacemaker | 16 (0.6%) | 5 (1.3%) | 9 (0.6%) | 2 (0.3%) |
| Any non-cardiac/genetic abnormality | 536 (19.5%) | 100 (25.5%) | 298 (18.9%) | 138 (17.8%) |
| Pre-operative LOS >2 days | 58 (2.1%) | 7 (1.8%) | 37 (2.3%) | 14 (1.8%) |
| Number of prior CT operations | 2 (2, 3) | 2 (2, 3) | 2 (2, 3) | 2 (2, 3) |
| Operative Characteristics | ||||
| Fontan type | ||||
| Lateral tunnel fenestrated | 881 (32.0%) | 154 (39.2%) | 532 (33.6%) | 195 (25.2%) |
| Lateral tunnel non-fenestrated | 136 (5.0%) | 16 (4.1%) | 74 (4.7%) | 46 (5.9%) |
| Extracardiac conduit fenestrated | 907 (33.0%) | 121(30.8%) | 513 (32.5%) | 273 (35.3%) |
| Extracardiac conduit non-fenestrated | 823 (30.0%) | 102 (26.0%) | 461(29.2%) | 260 (33.6%) |
| Secondary procedure at Fontan, n (%) | 1058 (38.5%) | 162 (41.2%) | 611 (38.7%) | 285 (36.8%) |
| Center annual Fontan volume, median operations/year | 10.8 (7.7, 31.4) | 13.0 (8.8, 34.9) | 10.8 (7.6, 31.4) | 10.6 (7.7, 31.4) |
Data for continuous variables are displayed as median (interquartile range)
LOS=length of stay, CT = cardiothoracic
Figure 1.
Age distribution
Figure 3.
Weight-for-age z-score distribution
An extracardiac Fontan (vs. lateral tunnel) was performed in 63%, and 65% of all Fontans were fenestrated (Table 1). Thirty-nine percent (n=1058) of patients underwent a total of 1466 secondary procedures at the time of the Fontan operation. The most common secondary procedure was pulmonary arterioplasty in 10.8%; 4.8% underwent atrioventricular valve repair/replacement and 0.8% underwent arch surgery.
Center Variation
Median age and weight at Fontan operation and proportion with weight-for-age z-score <−2 varied from center-to-center with a range of 1.7 – 4.8 years, 10.5 – 16.1 kg, and 0% – 30%, respectively.
Outcomes
Unadjusted outcomes are displayed in Table 2. Of note, there were no in-hospital transplants performed following the Fontan operation, thus the data on in-hospital Fontan failure includes only mortality or Fontan revision/takedown. In multivariable analysis, age and weight were not significantly associated with outcome when analyzed as both continuous (data not shown) and categorical variables (Table 3). Lower weight-for-age z-score was associated with significantly increased in-hospital mortality, Fontan failure, and longer length of stay.
Table 2.
Unadjusted In-hospital Outcomes in Age, Weight, and Weight-for-age z-score Groups
| A. Age | ||||
|---|---|---|---|---|
| Outcome | <2 years (n=361) |
2–4 years (n=1918) |
>4 years (n=468) |
p-value |
| In hospital mortality | 5 (1.4%) | 30 (1.6%) | 10 (2.1%) | 0.63 |
| Fontan failure | 9 (2.5%) | 49 (2.6%) | 15 (3.2%) | 0.72 |
| Postoperative LOS, days* | 9.7 (7.0, 13.0) | 10.8 (7.0, 15.0) | 11.5 (7.0, 15.0) | 0.01 |
| Postoperative complications | 148 (41.0%) | 772 (40.3%) | 191 (40.8%) | 0.95 |
| B. Weight | ||||
| Outcome |
<10kg (n=139) |
≥ 10 kg (n=2608) |
p-value | |
| In hospital mortality | 4 (2.9%) | 41 (1.6%) | 0.24 | |
| Fontan failure | 6 (4.3%) | 67 (2.6%) | 0.21 | |
| Postoperative LOS, days* | 10.9 (7.0, 15.0) | 10.6 (7.0, 14.0) | 0.80 | |
| Postoperative complications | 63(45.3%) | 1048 (40.2%) | 0.23 | |
| C. Weight-for-age z-score | ||||
| Outcome |
z-score <−2 (n=393) |
z-score −2 to 0 (n=1580) |
z-score >0 (n=774) |
p-value |
| In hospital mortality | 15 (3.8%) | 20 (1.3%) | 10 (1.3%) | 0.001 |
| Fontan failure | 22 (5.6%) | 34 (2.2%) | 17 (2.2%) | <0.001 |
| Postoperative LOS, days* | 11.8 (7.0, 17.0) | 10.6 (7.0, 14.0) | 10.5 (7.0, 14.0) | 0.33 |
| Postoperative complications | 175 (44.5%) | 613 (38.8%) | 323 (41.7%) | 0.08 |
LOS=length of stay
data are presented as 10% trimmed mean (interquartile range)
Table 3.
Adjusted In-hospital Outcomes
| In-hospital mortality | Adjusted OR (95% CI) | p-value |
|---|---|---|
| Age | ||
| <2 years | 0.97 (0.26, 3.67) | 0.96 |
| 2–4 years | 0.94 (0.42, 2.07) | 0.87 |
| >4 years | (reference) | |
| Weight | ||
| <10 kg | (reference) | |
| ≥10 kg | 0.94 (0.24, 3.65) | 0.93 |
| Weight-for-age z-score | ||
| <−2 | 2.73 (1.09, 6.86) | 0.03 |
| −2 to 0 | 0.98 (0.45, 2.16) | 0.96 |
| >0 | (reference) | |
| Fontan failure | Adjusted OR (95% CI) | |
| Age | ||
| <2 years | 1.37 (0.48, 3.51) | 0.60 |
| 2–4 years | 1.03 (0.54, 1.96) | 0.93 |
| >4 years | (reference) | |
| Weight | ||
| <10 kg | (reference) | |
| ≥10 kg | 0.99 (0.33, 2.98) | 0.98 |
| Weight-for-age z-score | ||
| <−2 | 2.59 (1.24, 5.40) | 0.01 |
| −2 to 0 | 1.02 (0.56, 1.87) | 0.95 |
| >0 | (reference) | |
| Complications | Adjusted OR (95% CI) | |
| Age | ||
| <2 years | 1.01 (0.69, 1.47) | 0.96 |
| 2–4 years | 0.97 (0.76, 1.25) | 0.83 |
| >4 years | (reference) | |
| Weight | ||
| <10 kg | (reference) | |
| ≥10 kg | 0.76 (0.48, 1.21) | 0.25 |
| Weight-for-age z-score | ||
| <−2 | 1.06 (0.78, 1.44) | 0.73 |
| −2 to 0 | 0.88 (0.72, 1.07) | 0.20 |
| >0 | (reference) | |
| Length of stay | Estimate* (95% CI) | |
| Age | ||
| <2 years | −0.01 (−0.11, 0.09) | 0.84 |
| 2–4 years | 0.04 (−0.02, 0.11) | 0.22 |
| >4 years | (reference) | |
| Weight | ||
| <10 kg | (reference) | |
| ≥10 kg | −0.007 (−0.13, 0.12) | 0.91 |
| Weight-for-age z-score | ||
| <−2 | 0.09 (0.01, 0.18)** | 0.03 |
| −2 to 0 | 0.02, (−0.03, 0.07) | 0.41 |
| >0 | (reference) |
CI=confidence interval, OR=odds ratio,
log days,
estimate of difference in predicted length of stay in days from multivariable model in weight-for-age z-score <−2 group vs. >0 group = +1.2 days, 95%CI 0.1–2.4
Comment
In this large multi-institutional cohort, we found substantial variation from center-to-center in age, weight, and weight-for-age z-score at the time of Fontan operation. In multivariable analysis, age and weight were not significantly associated with outcome, while lower weight-for-age z-score was associated with significantly increased in-hospital morbidity and mortality.
Previous studies have suggested that age <4 years was a risk factor for early Fontan failure (4,5,19). However, these reports included data from the time period when atriopulmonary and atrioventriuclar Fontan operations were favored techniques. More recent studies in the era of lateral tunnel and extracardiac Fontan operation have suggested that age is not significantly associated with post-operative outcome (8,11,12,20). Pizarro et al evaluated children <18 months and found that early mortality, Fontan takedown, and length of stay did not differ compared with older patients (20). Our study supports these findings in a large multi-intsitutional population. While our analysis focused on peri-operative outcomes, other studies have suggested that age at surgery may impact longer-term outcomes, such as aerobic capacity and hemodynamic status (21,22).
In our analysis of anthropometric data, we found overall impaired growth in regard to both height and weight in the population undergoing Fontan, similar to others (23). There was substantial variation from center-to-center in patient weight at Fontan operation, but similar to age, we did not find weight to be significantly associated with post-operative outcomes. Ikai et al. evaluated 72 patients from a single institution and found that weight <10kg was a significant risk factor for prolonged length of stay (6). In contrast, our findings are consistent with several more recent studies suggesting lower weight at Fontan operation is not a risk factor for poor outcome (8,11,24–26).
We also found significant variation from center-to-center in weight-for-age z-score at the time of Fontan operation. In contrast to age and raw weight, we did find that lower weight-for-age z-score was significantly associated with post-operative morbidity and mortality independent of other patient, operative, and center factors. To our knowledge, the impact of weight-for-age z-score on outcome following the Fontan operation has not been evaluated previously. However, similar findings have been noted in an evaluation of 100 children undergoing bidirectional Glenn, where lower weight-for-age z-score was significantly associated with longer length of stay and a trend toward increased mortality and other morbidities (13).
We found that those in the lowest weight-for-age z-score group had disproportionately lower weight in relation to height. There are several factors which may impact weight gain, and in turn, impact outcome. Adequate nutrition can be impaired by feeding difficulties which are common in the single ventricle population (27). Poor nutritional status and low protein reserves could predispose to pleural effusions, infection, and poor wound healing (13,28,29). Impaired growth may also be present in patients with genetic syndromes (30). However, we found that the association of low weight-for-age z-score with outcome was independent of the presence of genetic syndrome/non-cardiac abnormality. Factors increasing metabolic demand or impairing the ability to meet metabolic demand can also impair growth. Vogt et al demonstrated a significant association between pre-operative cardiac medications and the presence of venous collaterals and lower weight-for-age z-scores at Fontan operation (23). Cardiac medication use may be a surrogate for congestive heart failure which may increase metabolic demand, and venous collaterals may be associated with cyanosis which can impair ability to meet metabolic demand. We did adjust for atrioventricular valve regurgitation and coarctation/arch hypoplasia requiring intervention at the time of the Fontan operation (among other secondary procedures at the time of Fontan) in our analyses, both of which may be associated with pre-operative heart failure and poor cardiac function. However there are likely other variables related to heart failure and cyanosis which we were unable to account for. Finally, lower weight-for-age z-score at the time of Fontan operation has also been associated with requirement for additional surgical procedures prior to the Fontan and older age at Fontan operation (23). However, we adjusted for age and number of previous cardiothoracic operations in our analysis, such that the impact of lower weight for age z-score on outcome in our study is likely independent of these factors.
Recent data from Hehir and colleagues suggest that growth is a modifiable factor in the single ventricle population (14). Through a comprehensive feeding and monitoring protocol, investigators demonstrated growth velocity in single ventricle patients similar to that of a normal child despite the presence of other comorbidities, and excellent outcomes through stage 2 palliation with an interstage survival of 98% (14).
Limitations
The limitations of this study are primarily related to the limitations of the STS Database and the observational nature of the analysis. Although this is largest study to date evaluating age and weight at Fontan operation, the STS Database does not include the entire US population and thus, may underestimate center variation.
In addition, pre-operative hemodynamic and echocardiographic variables are not available in the STS Database. Information regarding cardiologist and surgeon decision making concerning the timing of surgery is also not available. Therefore we were not able to account for these factors in our analysis. We were able to account for several factors captured by the database which may impact both growth and outcome, including the presence of genetic/non-cardiac abnormalities, diagnosis, previous interventions, other pre-operative comorbidities, as well as hemodynamic/anatomic abnormalities (atrioventricular valve regurgitation, coarctation, etc) requiring intervention at the time of the Fontan. The current state of the database also does not enable us to know with certainty whether all patients have undergone Fontan following a superior cavopulmonary anastomosis, as opposed to being done in a palliated or unoperated state. Thus, it is unknown whether a volume unloading procedure preceded the Fontan, or by how long. Somatic growth parameters may be reflective of a variety of types of hemodynamic burden, of which ventricular volume load certainly is one. Due to these limitations, we are not able to draw inferences regarding the exact mechanism by which weight-for-age z-score is related to outcome as discussed above. Further study is needed to evaluate the relationship with other variables including nutritional status, heart failure, and cyanosis. Recent data from the Children’s Hospital of Wisconsin support the notion that growth velocity in single ventricle patients similar to that of a normal child can be achieved with a comprehensive feeding and monitoring plan despite the presence of these comorbidities (14).
We were also unable to analyze outcomes associated with varying practice patterns within an individual institution as this was precluded by the small number of patients and events. Finally, the STS Database currently only allows for the evaluation of in-hospital outcomes. Plans to expand data collection to include long-term morbidity and mortality will allow analyses of longer-term outcomes to be performed in the future.
Conclusions
In this large multi-institutional analysis, age and weight at Fontan operation did not significantly impact post-operative outcomes. However, lower weight-for-age z-score was associated with significantly increased post-operative mortality, Fontan failure, and length of stay, independent of other patient and center characteristics. Previous analyses have suggested that growth is modifiable with a comprehensive feeding and monitoring plan in single ventricle patients, and that poor growth is also a risk factor for poor outcome in patients undergoing bidirectional Glenn (13,14). These data, along with the results of the present analysis, support the implementation of strategies to optimize growth in this population and further investigation into mediating factors.
Figure 2.
Weight distribution
Acknowledgments
Disclosures
Dr. Wallace: Grant support, Duke Children’s Miracle Network.
Dr. J. Jacobs: Chair, Society of Thoracic Surgeons Congenital Heart Surgery Database Task Force and medical advisor and shareholder, CardioAccess.
Dr. Pasquali: Grant support (1K08HL103631-01), National Heart, Lung, and Blood Institute, and American Heart Association Mid-Atlantic Affiliate Clinical Research Program.
Dr. Smith: Grant support (1K23HD060040-01), National Institute of Child Health and Human Development.
Dr. Peterson: Principal Investigator, STS National Databases Analytic Center
References
- 1.Choussat UK, Fontan F, Besse P. Selection criteria for Fontan’s procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edingburgh: Churchill Livingstone; 1978. p. 55966. [Google Scholar]
- 2.Kaulitz R, Ziemer G, Luhmer I, Paul T, Kallfelz HC. Total cavopulmonary anastamosis in patients less than three years of age. Ann Thorac Surg. 1995;60(6 Suppl):S563–S567. doi: 10.1016/0003-4975(95)00856-x. [DOI] [PubMed] [Google Scholar]
- 3.Fedderly TR, Whitstone BN, Frisbee SJ, Tweddell JS, Litwin SB. Factors related to pleural effusions after Fontan procedure in the era of fenestration. Circulation. 2001;104 supplement I:I148–I151. doi: 10.1161/hc37t1.094817. [DOI] [PubMed] [Google Scholar]
- 4.Gentles TL, Mayer JE, Gauvreau K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg. 1997;114:37691. doi: 10.1016/s0022-5223(97)70183-1. [DOI] [PubMed] [Google Scholar]
- 5.Knott-Craig CJ, Danielson GK, Schaff HV, Puga FJ, Weaver AL, Driscoll DD. The modified Fontan operation. An analysis of risk factors for early postoperative death or takedown in 702 consecutive patients from one institution. J Thorac Cardiovasc Surg. 1995;109:1237–1243. doi: 10.1016/S0022-5223(95)70208-3. [DOI] [PubMed] [Google Scholar]
- 6.Ikai A, Fujimoto Y, Hirose K, et al. Feasibility of the extracardiac conduit Fontan procedure in patients weighing less than 10 kilograms. J Thorac Cardiovasc Surg. 2008;135:1145–1152. doi: 10.1016/j.jtcvs.2007.12.013. [DOI] [PubMed] [Google Scholar]
- 7.Mosca RS, Kulik TJ, Goldberg CS, et al. Early results of the Fontan procedure in one hundred consecutive patients with hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2000;119:1110–1118. doi: 10.1067/mtc.2000.106656. [DOI] [PubMed] [Google Scholar]
- 8.Salvin JW, Scheurer MA, Laussen PC, et al. Factors associated with prolonged recovery after the Fontan operation. Circulation. 2008;118(14 Suppl):S171–S176. doi: 10.1161/CIRCULATIONAHA.107.750596. [DOI] [PubMed] [Google Scholar]
- 9.Chowdhury UK, Airan B, Kothari SS, et al. Specific issues after extracardiac Fontan operation: Ventricular function, growth potential, arrhythmia, and thromboembolism. Ann Thorac Surg. 2005;80:665–672. doi: 10.1016/j.athoracsur.2005.02.024. [DOI] [PubMed] [Google Scholar]
- 10.Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: Is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg. 2002;123:23745. doi: 10.1067/mtc.2002.119337. [DOI] [PubMed] [Google Scholar]
- 11.Hosein RB, Clark AJ, McGuirk SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The “Two Comandments”? Eur J Cardiothorac Surg. 2007;31:344–352. doi: 10.1016/j.ejcts.2006.11.043. [DOI] [PubMed] [Google Scholar]
- 12.Napoleone CP, Oppido G, Angeli E, Giardini A, Resciniti E, Gargiulo G. Results of the modified Fontan procedure are not related to age at operation. Eur J Cardiothorac Surg. 2010;37:645–650. doi: 10.1016/j.ejcts.2009.09.003. [DOI] [PubMed] [Google Scholar]
- 13.Anderson JB, Beekman RH, Border WL, et al. Lower weight for age z-score adversely affects hospital length of stay after bidirectional Glenn procedure in 100 infants with a single ventricle. J Thorac and Cardiovasc Surg. 2009;138:397–404. doi: 10.1016/j.jtcvs.2009.02.033. [DOI] [PubMed] [Google Scholar]
- 14.Hehir DA, Rudd N, Slicker J, et al. Interstage growth velocity after Norwood palliation can parallel normal infant growth through home monitoring and standardized feeding plans. Congenit Heart Dis. 2010;5:512. [Google Scholar]
- 15.Jacobs ML, Mavroudis C, Jacobs JP, Tchervenkov CI, Pelletier GJ. Report of the 2005 STS Congenital Heart Surgery Practice and Manpower Survey. Ann Thorac Surg. 2006;82:1152–1158. doi: 10.1016/j.athoracsur.2006.04.022. [DOI] [PubMed] [Google Scholar]
- 16.Clarke DR, Breen LS, Jacobs ML, et al. Verification of data in congenital cardiac surgery. Cardiol Young. 2008;18 Suppl2:177–187. doi: 10.1017/S1047951108002862. [DOI] [PubMed] [Google Scholar]
- 17. [Accessed 5/3/2010];Centers for Disease Control and Prevention Growth Charts 2000. Available at: http://www.cdc.gov/growthcharts/
- 18. [Accessed October 26, 2010];STS Congenital Database Full Specifications. http://www.sts.org/documents/pdf/Congenital_DataSpecs_250.pdf.
- 19.Bartmus DA, Driscoll DJ, Offord KP, et al. The modified Fontan operation for children less than 4 years old. J Am Coll Cardiol. 1990;15:429–435. doi: 10.1016/s0735-1097(10)80073-7. [DOI] [PubMed] [Google Scholar]
- 20.Pizarro C, Mroczek T, Gidding SS, Murphy JD, Norwood WI. Fontan completion in infants. Ann Thorac Surg. 2006;81:2243–2248. doi: 10.1016/j.athoracsur.2006.01.016. [DOI] [PubMed] [Google Scholar]
- 21.Mahle WT, Wernovsky G, Bridges ND, Linton AB, Paridon SM. Impact of early ventricular unloading on exercise performance in preadolescents with single ventricle Fontan physiology. J. Am Coll Cardiol. 1999;34:1637–1643. doi: 10.1016/s0735-1097(99)00392-7. [DOI] [PubMed] [Google Scholar]
- 22.Shiraishi S, Yagihara T, Kagisaki K, et al. Impact of age at Fontan completion on postoperative hemodynamics and long term aerobic exercise capacity in patients with dominant left ventricle. Ann Thorac Surg. 2009;87:555–561. doi: 10.1016/j.athoracsur.2008.11.015. [DOI] [PubMed] [Google Scholar]
- 23.Vogt KN, Manlhiot C, Van Arsdell G, Russell JL, Mital S, McCrindle BW. Somatic growth in children with single ventricle physiology: Impact of physiologic state. J Am Coll Cardiol. 2007;50:1876–1883. doi: 10.1016/j.jacc.2007.07.050. [DOI] [PubMed] [Google Scholar]
- 24.Gupta A, Daggett C, Behera S, Ferraro M, Wells W, Starnes V. Risk factors for persistent pleural effusions after the extracardiac Fontan procedure. J Thorac Cardiovasc Surg. 2004;127:1664–1669. doi: 10.1016/j.jtcvs.2003.09.011. [DOI] [PubMed] [Google Scholar]
- 25.Hirsch JC, Goldberg C, Bove EL, et al. Fontan operation in the current era:a 15-year single institution experience. Ann Surg. 2008;248:402–410. doi: 10.1097/SLA.0b013e3181858286. [DOI] [PubMed] [Google Scholar]
- 26.Tweddell JS, Nersesian M, Mussatto KA, et al. Fontan palliation in the modern era: Factors impacting mortality and morbidity. Ann Thorac Surg. 2009;88:1291–1299. doi: 10.1016/j.athoracsur.2009.05.076. [DOI] [PubMed] [Google Scholar]
- 27.Davis D, Davis S, Cotman K, et al. Feeding Difficulties and Growth Delay in Children with Hypoplastic Left Heart Syndrome versus d-Transposition of the Great Arteries. Pediatr Cardiol. 2008;29:328–333. doi: 10.1007/s00246-007-9027-9. [DOI] [PubMed] [Google Scholar]
- 28.Leite HP, Fisberg M, de Carvalho WB, de Camargo Carvalho AC. Serum albumin and clinical outcome in pediatric cardiac surgery. Nutrition. 2005;21:553–558. doi: 10.1016/j.nut.2004.08.026. [DOI] [PubMed] [Google Scholar]
- 29.Pichard C, Kyle UG, Morabia A, et al. Nutritional assessment: lean body mass depletion at hospital admission is associated with an increased length of stay. Am J Clin Nutr. 2004;79:613–618. doi: 10.1093/ajcn/79.4.613. [DOI] [PubMed] [Google Scholar]
- 30.Knirsch W, Zingg W, Bernet V, et al. Determinants of body weight gain and association with neurodevelopmental outcome in infants operated for congenital heart disease. Interact Cardiovasc Thorac Surg. 2010;10:377–382. doi: 10.1510/icvts.2009.216135. [DOI] [PubMed] [Google Scholar]