Society Of Thoracic Surgeons-European Association Research Articles

Associations Between Unplanned Cardiac Reinterventions and Outcomes After Pediatric Cardiac Operations

After pediatric heart operations, we sought to determine the incidence of unplanned cardiac reinterventions during the same hospitalization, assess risk factors for these reinterventions, and explore associations between reinterventions and outcomes. We hypothesized that younger patients undergoing more complex operations would be at greater risk for unplanned cardiac reinterventions and that operative mortality and postoperative length of stay (PLOS) would be greater in patients who undergo reintervention than in those who do not. Patients aged 18 years or younger in The Society of Thoracic Surgeons Congenital Heart Surgery Database (January 2010 to June 2015) were included. We used multivariable regression to evaluate risk factors for unplanned cardiac reintervention (operation or therapeutic catheterization) and associations of reintervention with operative mortality and PLOS. Of 84,404 patients (117 centers), 21% were neonates and 36% infants. An unplanned cardiac reintervention was performed in 5.4% of patients, including 11.8% of neonates, 5.2% of infants, and 2.8% of children. Independent risk factors for unplanned reintervention included presence of noncardiac anomalies/genetic syndromes, nonwhite race, younger age, lower weight among neonates and infants, prior cardiothoracic operations, preoperative mechanical ventilation, other Society of Thoracic Surgeons preoperative risk factors, and higher Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Mortality Category (adjusted p < 0.001 for all). Unplanned reintervention was a risk factor for operative mortality (adjusted odds ratio, 5.3; 95% confidence interval, 4.8 to 5.8; p < 0.001) and longer PLOS (adjusted relative risk, 2.3; 95% confidence interval, 2.2 to 2.4; p < 0.001). Unplanned cardiac reinterventions are not rare, particularly in neonates, and are independently associated with operative mortality and increased PLOS. Patients at greater risk may be identified preoperatively, presenting opportunities for quality improvement.

Higher Programmatic Volume in Neonatal Heart Surgery Is Associated With Lower Early Mortality

The early results of congenital heart surgery in neonates remain a challenge. We sought to determine the nature of the association between annual center volume of neonatal cardiac surgery and operative mortality using a multicenter cohort. The dataset consists of 27,556 neonatal procedures performed between 1999 and 2015 in 90 centers participating in the European Congenital Heart Surgeons Association database. Centers with mean annual volume load of six or more that submitted data for at least 3 consecutive years were included. World Bank annual gross national index per capita was utilized as an indicator of temporal national affluence. Multilevel logistic regression was used to create a model including the significant risk factors and to calculate odds ratios for operative mortality. Iterative modeling of the dataset incrementally excluding centers with lower annual caseload was used to identify the relationship between annual volume and mortality. In the model thus calculated including The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score, operative weight and age, noncardiac genetic anomalies, and annual volume of operations were independent risk factors for operative mortality in the analysis of the entire cohort. In the model containing these variables, annual gross national index and year of surgery were not significantly associated with mortality. In the iterative process, annual volume ceased to be a risk factor when units operating on fewer than 60 neonates annually were excluded. In neonatal congenital heart surgery, the risk of operative death decreased with the increase of volume load. The cutoff point in this cohort was a mean annual volume of 60 neonatal operations per year.

Epidemiology and Outcomes of Cardiac Arrest in Pediatric Cardiac ICUs.

In-hospital cardiac arrest occurs in 2.6-6% of children with cardiac disease and is associated with significant morbidity and mortality. Much remains unknown about cardiac arrest in pediatric cardiac ICUs; therefore, we aimed to describe cardiac arrest epidemiology in a contemporary multicenter cardiac ICU cohort. Retrospective analysis within the Pediatric Cardiac Critical Care Consortium clinical registry. Cardiac ICUs within 23 North American hospitals. All cardiac medical and surgical patients admitted from August 2014 to July 2016. None. There were 15,908 cardiac ICU encounters (6,498 medical, 9,410 surgical). 3.1% had cardiac arrest; rate was 4.8 cardiac arrest per 1,000 cardiac ICU days. Medical encounters had 50% higher rate of cardiac arrest compared with surgical encounters. Observed (unadjusted) cardiac ICU cardiac arrest prevalence varied from 1% to 5.5% among the 23 centers; cardiac arrest per 1,000 cardiac ICU days varied from 1.1 to 10.4. Over half cardiac arrest occur within 48 hours of admission. On multivariable analysis, prematurity, neonatal age, any Society of Thoracic Surgeons preoperative risk factor, and Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 4, 5 had strongest association with surgical encounter cardiac arrest. In medical encounters, independent cardiac arrest risk factors were acute heart failure, prematurity, lactic acidosis greater than 3 mmol/dL, and invasive ventilation 1 hour after admission. Median cardiopulmonary resuscitation duration was 10 minutes, return of spontaneous circulation occurred in 64.5%, extracorporeal cardiopulmonary resuscitation in 27.2%. Unadjusted survival was 53.2% in encounters with cardiac arrest versus 98.2% without. Medical encounters had lower survival after cardiac arrest (37.7%) versus surgical encounters (62.5%); Norwood patients had less than half the survival after cardiac arrest (35.6%) compared with all others. Unadjusted survival after cardiac arrest varied greatly among 23 centers. We provide contemporary epidemiologic and outcome data for cardiac arrest occurring in the cardiac ICU from a multicenter clinical registry. As detailed above, we highlight high-risk patient cohorts and periods of time that may serve as targets for research and quality improvement initiatives aimed at cardiac arrest prevention.

Performance of Pediatric Risk of Mortality Score Among Critically Ill Children With Heart Disease.

To evaluate the performance of the Pediatric Risk of Mortality 3 (PRISM-3) score in critically ill children with heart disease. Patients <18 years of age admitted with cardiac diagnoses (cardiac medical and cardiac surgical) to one of the participating pediatric intensive care units in the Virtual Pediatric Systems, LLC, database were included. Performance of PRISM-3 was evaluated with discrimination and calibration measures among both cardiac surgical and cardiac medical patients. The study population consisted of 87,993 patients, of which 49% were cardiac medical patients (n = 43,545) and 51% were cardiac surgical patients (n = 44,448). The ability of PRISM-3 to distinguish survivors from nonsurvivors was acceptable for the entire cohort (c-statistic 0.86). However, PRISM-3 did not perform as well when stratified by varied severity of illness categories. Pediatric Risk of Mortality 3 underpredicted mortality among patients with lower severity of illness categories (quintiles 1-4) whereas it overpredicted mortality among patients with greatest severity of illness category (fifth quintile). When stratified by Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) categories, PRISM-3 overpredicted mortality among the STS-EACTS mortality categories 1, 2, and 3 and underpredicted mortality among the STS-EACTS mortality categories 4 and 5. Pediatric Risk of Mortality 3 overpredicted mortality among centers with high cardiac surgery volume whereas it underpredicted mortality among centers with low cardiac surgery volume. Data from this large multicenter study do not support the use of PRISM-3 in cardiac surgical or cardiac medical patients. In this study, the ability of PRISM-3 to distinguish survivors from nonsurvivors was fair at best, and the accuracy with which it predicted death was poor.

Risk Factors for Mortality in Reoperations for Pediatric and Congenital Heart Surgery in a Developing Country.

The survival of patients with congenital heart disease has increased in the recent years, because of enhanced diagnostic capabilities, better surgical techniques, and improved perioperative care. Many patients will require reoperation as part of staged procedures or to treat grafts deterioration and residual or recurrent lesions. Reoperations favor the formation of cardiac adhesions and consequently increase surgery time; however, the impact on morbidity and operative mortality is certain. The objective of the study was to describe the risk factors for mortality in pediatric patients undergoing a reoperation for congenital heart disease. Historic cohort of patients who underwent reoperation after pediatric cardiac surgery from January 2009 to December 2015. Operations with previous surgical approach different to sternotomy were excluded from the analysis. In seven years, 3,086 surgeries were performed, 481 were reoperations, and 238 patients fulfilled the inclusion criteria. Mean number of prior surgeries was 1.4 ± 0.6. Median age at the time of reoperation was 6.4 years. The most common surgical procedures were staged palliation for functionally univentricular heart (17.6%). Median cross-clamp time was 66 minutes. Younger age at the moment of resternotomy, longer cross-clamp time, and Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) Mortality Categories risk category greater than three were risk factors for mortality. The number of resternotomies was not associated with mortality. Mortality prior to hospital discharge was 4.6%, and mortality after discharge but prior to 30 days after surgery was 0.54%. Operative mortality was 5.1%. Resternotomy in pediatric cardiac surgery is a safe procedure in our center.

Recurrent Laryngeal Nerve Injury and Swallowing Dysfunction in Neonatal Aortic Arch Repair

We evaluated the incidence, clinical effect, and recovery rate of vocal cord dysfunction (VCD) and swallowing dysfunction in neonates undergoing aortic arch repair. We retrospectively evaluated 101 neonates who underwent aortic arch reconstruction from 2008 to 2015. Direct flexible laryngoscopy was performed in 89 patients before initiation of postoperative oral feeding after Norwood (n= 63) and non-Norwood (n= 26) arch reconstruction. We defined VCD as immobility of vocal cords or their lack of coaptation and poor mobility. The incidence of VCD after aortic arch repair was 48% (n= 43). There was no significant difference between the VCD and non-VCD groups in postoperative length of stay, extubation failure, cardiopulmonary bypass, cross-clamp, selective cerebral perfusion time, operative death, and The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories. Placement of gastrostomy (p= 0.03) and documented aspiration (p= 0.01) were significantly more common in VCD patients. The incidence of VCD was 41% (n= 26) after Norwood and 65% (n= 17) after non-Norwood repairs (p= 0.06). Gastrostomy was required in 44 Norwood patients vs 9 non-Norwood patients (p= 0.004). Median length of stay was similar in Norwood patients with or without VCD (p= .28) but was significantly longer in non-Norwood patients with VCD vs those without (p= 0.002). At follow-up direct flexible laryngoscopy, VCD recovery was 74% (14 of 19) in the Norwood group and 86% (12 of 14) in the non-Norwood group. The incidence of VCD and swallowing dysfunction in neonates undergoing aortic arch reconstruction is high. Patients with VCD have a significantly higher incidence of gastrostomy placement and aspiration. In the Norwood population, length of stay is not associated with presence or absence of VCD. More than 70% of patients in each group who had direct flexible laryngoscopy follow-up recovered vocal cord function.

Invited Commentary

Trisomy 13 and 18 are genetic diagnoses associated with multiple congenital anomalies, significant neurologic impairment, and a markedly reduced lifespan. Consequently, they have often been described as “lethal anomalies” or “incompatible with life,” and invasive medical or surgical interventions were historically not offered. Recently there have been several studies on the effects of active treatment of children with trisomy 13 and 18 [1Nelson K.E. Rosella L.C. Mahant S. Guttmann A. Survival and surgical interventions for children with trisomy 13 and 18.JAMA. 2016; 316: 420-428Crossref PubMed Scopus (114) Google Scholar]. Several of these studies have included cardiac interventions and report reasonable hospital survival [2Graham E.M. Bradley S.M. Shirali G.S. Hills C.B. Atz A.M. Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium).Am J Cardiol. 2004; 93: 801-803Abstract Full Text Full Text PDF PubMed Scopus (95) Google Scholar]. Despite this, the role of invasive cardiac interventions remains anything but clear, in part because of the lack of long-term follow-up. In this issue of The Annals, Peterson and colleagues [3Peterson J.K. Kochilas L.K. Catton K.G. Moller J.H. Setty S.P. Long-term outcomes of children with trisomy 13 and 18 after congenital heart disease interventions.Ann Thorac Surg. 2017; 103: 1941-1949Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar] report their retrospective review of the Pediatric Cardiac Care Consortium (PCCC) to determine short-term and long-term mortality after congenital cardiac interventions in children with trisomy 13 and 18. This study represents one of the largest cohorts of patients with trisomy 13 or 18 undergoing cardiac interventions in a national registry. The authors identified 171 children with trisomy 13 or 18 of whom 98 underwent either surgical cardiac intervention, catheter-based cardiac intervention, or both. Hospital mortality was high—27.6% and 13% for trisomy 13 and 18, respectively—and was higher than in the general congenital cardiac surgical population for some surgical risk categories by as much as tenfold. What makes this study unique is its report of the long-term survival in this population by use of the National Death Index (NDI). In individuals who survived hospital discharge, the authors report median survival of 14.8 and 16.2 years for trisomy 13 and 18, respectively. The authors appropriately describe the study limitations, but it is worth highlighting that the reported survival is conditioned on survival to hospital discharge. In addition, the process of linking the PCCC to the NDI reduced the number of individuals eligible for NDI evaluation from the original 81 hospital survivors to 44. Finally, those not linked by the NDI are assumed to be alive. Despite these minor limitations, this is longer survival than previously reported. These findings are important considerations when we counsel families and make program-level decisions regarding the care of children with trisomy 13 or 18. Even for those selected for cardiac interventions, mortality remains high: ten times the “expected” hospital mortality for “simple lesions” (Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT] Mortality Categories 1 and 2). However, a subset of children can survive until adolescence or even adulthood, challenging us as providers to stop using descriptions like “lethal anomalies” or “incompatible with life” when describing these syndromes. Whereas an improvement in quality of life after cardiac interventions has not been addressed, some improvement might be expected and should be explored in future studies. Predicting the child most likely to benefit from cardiac interventions remains challenging, although this work and others suggest that mosaicism may be beneficial. Families, providers, and health care institutions will continue to be faced with the complex and multifaceted issues surrounding the best course of treatment for these children. It is imperative that we provide realistic, accurate, and balanced information so the potential benefits and burdens of any proposed intervention can be weighted in a collaborative decision-making process. Dr Graham and his institution receive support from the National Heart, Lung, and Blood Institute of the National Institutes of Health (NIH) under Award number R01 HL112968 for an ongoing randomized control trial “Corticosteroid Therapy in Neonates Undergoing Cardiopulmonary Bypass.” The content of this work is solely the responsibility of the author and does not necessarily represent the official views of the NIH. Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease InterventionsThe Annals of Thoracic SurgeryVol. 103Issue 6PreviewThe purpose of this study is to report short- and long-term outcomes after congenital heart defect (CHD) interventions in patients with trisomy 13 or 18. Full-Text PDF

Duration of Mechanical Ventilation and Perioperative Care Quality After Neonatal Cardiac Operations

This study was conducted to determine whether the duration of mechanical ventilation (DOMV) could be used to benchmark the overall quality of care after neonatal congenital heart operations. Children aged younger than 30 days undergoing cardiac operations were reviewed. Technical adequacy was assessed using the Technical Performance Score (TPS), a previously validated tool for determining the adequacy of a palliative or corrective surgical procedure that uses echocardiography criteria and need for unplanned reintervention to determine technical adequacy. Preoperative risk factors and postoperative complications were determined using The Society of Thoracic Surgeons Congenital Heart Surgery Database definitions. Surgical complexity was assessed using The Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery (STAT) Mortality Categories. We explored associations between preoperative risk factors, STAT category, TPS, and postoperative complications with DOMV. Of 601 patients studied, 49 were not included in the multivariable analysis due to a STAT nonclassifiable operation or unmeasured TPS, or both. Multiple risk factors were associated with longer DOMV, including weight (p= 0.005), The Society of Thoracic Surgeons Congenital Heart Surgery Database preoperative factors (p= 0.005), STAT mortality category (p < 0.001), TPS (p < 0.001), and The Society of Thoracic Surgeons Congenital Heart Surgery Database-defined complications (p < 0.001). Multivariable regression showed that preoperative factors increased DOMV by 1.23 days (p= 0.01), class 3 TPS by 2.16 days (p < 0.001), and postoperative complications by 2.03 days (p < 0.001), with adjusted R2= 0.42. Neonates with major residual lesions and postoperative complications have prolonged DOMV. DOMV may reflect the quality of care after congenital heart operations.

Early extubation in pediatric heart surgery across a spectrum of case complexity: Impact on hospital length of stay and chest tube days

Early extubation is increasingly common in congenital heart surgery, but there are limited outcomes data across the spectrum of case complexity. We performed a retrospective review of 201 pediatric operations using cardiopulmonary bypass between 2012 and 2014. Patients extubated in the operating room or immediately on arrival to the ICU were compared to those extubated by traditional protocols. In-hospital mortality, major complications, need for re-intubation, hospital length of stay, and chest-tube days were compared between groups and by Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Congenital Heart Surgery (STAT) mortality category. Outcome measures of hospital length of stay and chest tube days were analyzed using multivariable regression modeling. Early extubation subjects were older, weighed more, had shorter bypass and aortic cross-clamp time, more often received caudal anesthesia, and had shorter hospital length of stay and fewer chest tube days. Subjects not extubated early had more chromosomal abnormalities, more preoperative co-morbidities, and had more major complications. Inhospital death, major complications, and re-intubation were rare outcomes for both groups across all STAT categories. Multivariable regression analysis showed that cardiopulmonary bypass time was a significant predictor of hospital length of stay and chest tube days. Hospital LOS and chest tube days were significantly lower for the early extubation group in both the unadjusted and adjusted analyses. Early extubation can be performed safely in congenital heart surgery across a spectrum of case complexity. No increased early mortality or re-intubation was observed with early extubation although there were important differences between the groups that merits further study. The potential benefits of early extubation include decreased hospital length of stay and fewer chest tube days, particularly in young children and patients with long bypass times.

Mortality Trends in Pediatric and Congenital Heart Surgery: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

Previous analyses of The Society of Thoracic Surgeons (STS) Adult Cardiac Surgery Database have demonstrated a reduction over time of risk-adjusted operative mortality after coronary artery bypass grafting. The STS Congenital Heart Surgery Database (STS CHSD) was queried to assess multiinstitutional trends over time in discharge mortality and postoperative length of stay (PLOS). Since 2009, operations in the STS CHSD have been classified according to STAT (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) Congenital Heart Surgery Mortality Categories. The five STAT Mortality Categories were chosen to be optimal with respect to minimizing variation within categories and maximizing variation between categories. For this study, all index cardiac operations from 1998 to 2014, inclusive, were grouped by STAT Mortality Category (exclusions: patent ductus arteriosus ligation in patients weighing less than or equal to 2.5 kg and operations that could not be assigned to a STAT Mortality Category). End points were discharge mortality and PLOS in survivors for the entire period and for 4-year epochs. The Cochran-Armitage trend test was used to test the null hypothesis that the mortality was the same across epochs, by STAT Mortality Category. The analysis encompassed 202,895 index operations at 118 centers. The number of centers participating in STS CHSD increased in each epoch. Overall discharge mortality was 3.4% (6,959 of 202,895) for 1998 to 2014 and 3.1% (2,308 of 75,337) for 2011 to 2014. Statistically significant improvement in discharge mortality was seen in STAT Mortality Categories 2, 3, 4, and 5 (p values for STAT Mortality Categories 1 through 5 are 0.060, <0.001, 0.015, <0.001, and <0.001, respectively). PLOS in survivors wasrelatively unchanged over the same time intervals. Sensitivity analyses reveal that the finding of declining risk-stratified rates of discharge mortality over time is not simply attributable to the addition of more centers to the cohort over time. This 16-year analysis of STS CHSD reveals declining discharge mortality over time, especially for more complex operations.

Pediatric Index of Cardiac Surgical Intensive Care Mortality Risk Score for Pediatric Cardiac Critical Care.

Comparison of clinical outcomes is imperative in the evaluation of healthcare quality. Risk adjustment for children undergoing cardiac surgery poses unique challenges, due to its distinct nature. We developed a risk-adjustment tool specifically focused on critical care mortality for the pediatric cardiac surgical population: the Pediatric Index of Cardiac Surgical Intensive care Mortality score. Retrospective analysis of prospectively collected pediatric critical care data. Pediatric critical care units in the United States. Pediatric cardiac intensive care surgical patients. Prospectively collected data from consecutive patients admitted to ICUs were obtained from The Virtual PICU System (VPS, LLC, Los Angeles, CA), a national pediatric critical care database. Thirty-two candidate physiologic, demographic, and diagnostic variables were analyzed for inclusion in the development of the Pediatric Index of Cardiac Surgical Intensive care Mortality model. Multivariate logistic regression with stepwise selection was used to develop the model. A total of 16,574 cardiac surgical patients from the 55 PICUs across the United States were included in the analysis. Thirteen variables remained in the final model, including the validated Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery Congenital Heart Surgery Mortality (STAT) score and admission time with respect to cardiac surgery, which identifies whether the patient underwent the index surgical procedure before or after admission to the ICU. Pediatric Index of Cardiac Surgical Intensive Care Mortality (PICSIM) performance was compared with the performance of Pediatric Risk of Mortality-3 and Pediatric Index of Mortality-2 risk of mortality scores, as well as the STAT score and STAT categories by calculating the area under the curve of the receiver operating characteristic from a validation dataset: PICSIM (area under the curve = 0.87) performed better than Pediatric Index of Mortality-2 (area under the curve = 0.81), Pediatric Risk of Mortality-3 (area under the curve = 0.82), STAT score (area under the curve = 0.77), STAT category (area under the curve = 0.75), and Risk Adjustment for Congenital Heart Surgery-1 (area under the curve = 0.74). This newly developed mortality score, PICSIM, consisting of 13 risk variables encompassing physiology, cardiovascular condition, and time of admission to the ICU showed better discrimination than Pediatric Index of Mortality-2, Pediatric Risk of Mortality-3, and STAT score and category for mortality in a multisite cohort of pediatric cardiac surgical patients. The introduction of the variable "admission time with respect to cardiac surgery" allowed prediction of mortality when patients are admitted to the ICU either before or after the index surgical procedure.

Estimating Mortality Risk for Adult Congenital Heart Surgery: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

Adjustment for case mix is critical to accurate outcomes analysis in congenital heart surgery. Established tools encompass all age groups and are not specific to the growing population of adults undergoing congenital heart operations. We derived an empirically based adult congenital heart surgery (ACHS) mortality score. In-hospital mortality was analyzed for the 152 most common procedures/procedural groups in adults 18 years of age and older in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) (2000-2013). Procedure-specific adult mortality rate estimates were calculated using Bayesian methods adjusting for small denominators for procedures with 30 cases or more (N = 52). Each procedural group was assigned an ACHS mortality score ranging from 0.1 to 3.0 based on the estimated mortality rate. Discrimination was assessed using the c-index in a separate validation sample. A total of 12,513 procedures (116 centers) were analyzed. Overall unadjusted mortality was 1.8%. Significant differences in mortality rates in adults compared with all ages were seen for several procedures, including Ebstein's repair (0.7% versus 4.9%; p = 0.003) and Fontan operations (6.8% versus 1.4%; p < 0.01). The procedure with the lowest model-based estimate of mortality and accompanying ACHS mortality score was atrial septal defect repair (0.2%, 0.1), and the highest was Fontan revision (9.7%, 3.0). The c-index for the ACHS mortality score was 0.809 versus 0.777 for the "non-age-specific" Society of Thoracic Surgeons-European Association for Cardio-thoracic Surgery (STAT) mortality score applied to adults. Risk estimation based on the aggregate of all age groups is suboptimal when analyzing outcomes specifically among adults. An empirically based ACHS mortality score can facilitate case-mix adjustment by providing accurate estimation of mortality risk for adults.

Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience.

To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively. Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO. In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

Association of Center Volume With Outcomes: Analysis of Verified Data of European Association for Cardio-Thoracic Surgery Congenital Database

The relation between surgical volumes and outcome in congenital heart surgery (CHS) was investigated with no clear conclusions. We sought to quantify the relationship between surgical volume andsurgical performance defined as the relation between outcome and Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) Mortality Score and The Society of Thoracic Surgeons (STS) Morbidity Score. We have used only the verified data of theEuropean Association for Cardio-Thoracic Surgery Congenital Database. The verified dataset consists of 17,861 procedures performed in 23 congenital heart surgery centers between 2003 and 2011. The centers were divided into 4 volume-related groups with annual caseload of below 150, 150 to 250, 250 to 350, and over 350. Stepwise logistic regression was used to calculate the ratio between volume and mortality, as well as between volume and onset of complications. The relations between volume and STAT Mortality Score, and STS Morbidity Score were evaluated using the analysis of variance test.The performance was calculated as the following: 100 - observed mortality/STAT Mortality Score; and 100 - observed complications/STS Morbidity Score. The study showed no relation between volume and raw mortality (p= 0.94) and between volume and complications (p= 0.6). The STAT Mortality Score and STS Morbidity Score were higher in larger volume centers (p < 0.001). Surgical performances measured as related to mortality and morbidity were higher at high-volume centers (R(2)= 0.95 and R(2)= 0.92). Our analysis suggests that after adjustment for case mix higher programmatic volume is associated with lower rates of mortality and morbidity. The small- and medium-volume centers have higher rates of major complications. When complications occurred the chance of rescue is higher in large-volume centers.

Twenty-Four Hour In-Hospital Congenital Cardiac Surgical Coverage Improves Perioperative ECMO Support Outcomes

Extracorporeal membrane oxygenation (ECMO) support is often required in the management of perioperative congenital heart surgery (CHS) patients. However, 24-hour in-hospital congenital cardiac surgical coverage (24-CCSC) is not available at all institutions. The purpose of this study is to evaluate the effect of 24-CCSC on perioperative ECMO outcomes in CHS patients. An institutional review board approved, retrospective review of 128 perioperative CHS ECMO patients at a single, quaternary care children's hospital between January 2003 and December 2012 was performed. Primary endpoints evaluated were mortality in children supported with ECMO after undergoing cardiac surgery and ECMO-related morbidity after initiation of 24-CCSC with advanced congenital cardiac surgical fellows. Patients were divided into 2 groups based on whether 24-CCSC was absent (cohort 1: January 2003 to July 2007) or present (cohort 2: August 2007 to December 2012) at the time of ECMO management. The surgical procedures performed were similar in both cohorts based on STAT Mortality Categories (5 Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories). The overall mortality rate in children supported with ECMO after undergoing cardiac surgery was 53%. This mortality was significantly reduced from 68% to 43% (p= 0.007) with24-CCSC. Multivariate logistic regression analysis revealed that 24-CCSC (p= 0.009) and lower STAT Mortality Category (p= 0.042) were independent predictors of operative survival. Cardiac arrhythmias (36% to 16%; p= 0.012) and pulmonary complications (32% to 8%; p < 0.001) were significantly reduced with 24-CCSC. The presence of 24-CCSC significantly decreased the rate of mortality in children supported with ECMO after undergoing cardiac surgery, as well as cardiac arrhythmias and pulmonary complications for perioperative CHS patients receiving ECMO support. This study demonstrates that CHS programs would benefit from 24-CCSC in the care of this critically ill patient population.

Outcomes of cardiac surgery in patients weighing

A recent Society of Thoracic Surgeons database study showed that low weight (<2.5 kg) at surgery was associated with high operative mortality (16%). We sought to assess the outcomes after cardiac repair in patients weighing <2.5 kg versus 2.5 to 4.5 kg in an institution with a dedicated neonatal cardiac program and to determine the potential role played by prematurity, the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) risk categories, uni/biventricular pathway, and surgical timing. We analyzed the outcomes (hospital mortality, early reintervention, postoperative length of stay, mortality [at the last follow-up point]) in patients weighing <2.5 kg at surgery (n = 146; group 1) and 2.5 to 4.5 kg (n = 622; group 2), who had undergone open or closed cardiac repairs from January 2006 to December 2012 at our institution. The statistical analysis was stratified by prematurity, STAT risk category, uni/biventricular pathway, and usual versus delayed surgical timing. Univariate versus multivariate risk analysis was performed. The mean follow-up was 21.6 ± 25.6 months. Hospital mortality in group 1 was 10.9% (n = 16) versus 4.8% (n = 30) in group 2 (P = .007). The postoperative length of stay and early unplanned reintervention rate were similar between the 2 groups. Late mortality in group 1 was 0.7% (n = 1). In group 1, early outcomes were independent of the STAT risk category, uni/biventricular pathway, or surgical timing compared with group 2. A lower gestational age at birth was an independent risk factor for early mortality in group 1. A dedicated multidisciplinary neonatal cardiac program can yield good outcomes for neonates and infants weighing <2.5 kg independently of the STAT risk category and uni/biventricular pathway. A lower gestational age at birth was an independent risk factor for hospital mortality.

Low-weight infants are at increased mortality risk after palliative or corrective cardiac surgery

Low weight is an established risk factor for mortality after congenital cardiac surgery. Given the advances in the care of neonates and infants after surgery, we sought to examine the effect of low weight on outcomes in the current era. From 2002 to 2012, 2051 infants aged 90 days or less underwent cardiac surgery including 534 (26.0%) with single-ventricle pathology. Regression models examined the effect of low weight (≤ 2.5 kg; n = 274, 13.4%) on early and late outcomes. Overall, the incidence of prematurity, associated chromosomal/extracardiac abnormalities was higher in infants who weighed 2.5 kg or less than in those who weighed more than 2.5 kg; the incidence of single-ventricle pathology was comparable between the 2 groups. In addition, infants who weighed 2.5 kg or less underwent more palliation and had a higher proportion of STAT (Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) risk category 4 and 5 procedures. Adjusted regression models showed that low weight (≤ 2.5 kg) did not increase unplanned reoperation (odds ratio [OR], 0.90; 95% confidence interval [CI], 0.48-1.67; P = .73) or extracorporeal membrane oxygenation requirement (OR, 1.23; 95% CI, 0.68-2.22; P = .49), however it was associated with significant increase in hospital mortality (OR, 2.15; 95% CI, 1.33-3.50; P = .002). In addition, there was a significant association between low weight and increased duration of postoperative mechanical ventilation and intensive care unit and hospital stays. Adjusted hazard analysis showed that weight equal to or less than 2.5 kg was associated with diminished late survival (hazard ratio, 1.89; 95% CI, 1.39-2.55; P < .001) and that was evident in all patients subgroups (P < .001 for all). In a large single-center series, low weight continues to be associated with increased early mortality risk and resource utilization after palliative and corrective cardiac surgery. The hazard of death in low-weight patients continues beyond the perioperative period for at least 1 year before normalizing. Strategies to improve outcomes for this high-risk population must address perioperative care, outpatient surveillance, and management.

Predictors of 90-day mortality after congenital heart surgery: The first report of risk models from a Japanese database

The purpose of this study was to develop risk models for congenital heart surgery short-term and midterm outcomes from a nationwide integrated database drawn from hospitals in Japan. The Japan Congenital Cardiovascular Surgery Database collects clinical information from institutions throughout Japan specializing in congenital heart surgery. Variables and definitions used in the Japan Congenital Cardiovascular Surgery Database are almost identical to those of the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery database for congenital heart surgery. We used logistic regression to develop risk models, which were then validated through spilt-sample validation. In addition to procedural complexity categories by Risk Adjustment in Congenital Heart Surgery (RACHS-1) score, we incorporated patient characteristics to predict surgical outcome. Among 8923 congenital heart operations performed at 69 sites with cardiac surgical programs, 30-day mortalities by RACHS-1 category were as follows: I, 0.1% (n=1319); II, 0.5% (n=3211); III, 2.2% (n=3285); IV, 4.3% (n=818); and V and VI, 8.6% (n=290). From the test data set (n=7223), we developed 3 risk models (30-day mortality, 90-day mortality, and 90-day and in-hospital mortality) with 11 variables, including age category, RACHS-1 category, preoperative risk factors, number of surgical procedures, unplanned reoperations, status of surgery, surgery type, asplenia, and prematurity (<35 weeks). For the performance metrics of the risk models, C statistic values of 30-day, 90-day, and 90-day and in-hospital mortalities for the test data set were 0.85, 0.85, and 0.84, respectively. When only the RACHS-1 score was used for discrimination, the C statistic values of 30-day, 90-day, and 90-day and in-hospital mortalities for the validation data set were 0.73, 0.73, and 0.77, respectively. The proposed risk scores and categories have high discrimination power for predicting mortality, demonstrating improvement relative to existing consensus-based methods. Risk models incorporating these measures may be useful for comparing mortality outcomes cross institutions or countries with mixed cases.

Does ICU Structure Impact Patient Outcomes After Congenital Heart Surgery? A Critical Appraisal of “Care Models and Associated Outcomes in Congenital Heart Surgery” by Burstein et al (Pediatrics 2011; 127

To review the findings and discuss the implications of different ICU care models on morbidity and mortality in pediatric patients after congenital heart surgery. The electronic PubMed database was used to perform the clinical query, as well as to search for additional pertinent literature. The article by Burstein DS et al "Care Models and Associated Outcomes in Congenital Heart Surgery. Pediatrics 2011; 15:77-81" was selected for critical appraisal and literature review. The authors evaluated in-hospital mortality, postoperative length of stay, and postoperative complications in pediatric patients after congenital heart surgery and compared the odds of these outcomes by model of care received (cardiac ICU or mixed ICU). The data for the study was extracted from the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) database. Overall, the cardiac ICU group represented hospitals with higher surgical volumes and included more patients with high-risk defects. After multivariate analysis, the adjusted in-hospital mortality was not associated with the care model (cardiac ICU vs. ICU). The only significant finding was a lower morality in the STS-EACTS risk category 3 (odds ratio, 0.47 [95% CI, 0.25-0.86]). There were no significant differences between groups for adjusted postoperative length of stay or postoperative complications. This paper suggests that the composition of the ICU is not a critical factor in determining outcomes after congenital heart surgery. Other factors, such as expertise of the nurses, physicians, and surgeons, as well as technical performance, should be considered.

Contemporary Outcomes in Infants With Congenital Heart Disease and Bochdalek Diaphragmatic Hernia

Fifteen percent of infants with congenital diaphragmatic hernia (CDH) are born with a coexisting cardiac anomaly. The purpose of this study was to evaluate contemporary outcomes in this patient population and to identify potential risk factors for in-hospital mortality. Data from all CDH neonates with congenital heart disease managed at a single pediatric tertiary care referral center between 1997 and 2011 were retrospectively analyzed. Forty (18%) of 216 CDH patients had a cardiac anomaly. This group was associated with a significant decrease in overall survival when compared with patients without cardiac anomaly (55% versus 81%; p = 0.001). There was no association between type of cardiac anomaly and mortality based on risk stratification according to the Risk Adjustment for Congenital Heart Surgery and The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery scoring systems (p = 0.86 and p = 0.87, respectively). Birth weight was similarly no different between survivors and nonsurvivors (2.8 ± 0.6 kg versus 2.8 ± 0.9 kg, respectively; p = 0.98). There was a trend toward increased extracorporeal membrane oxygenation use among nonsurvivors (p = 0.13). Infants with hemodynamic stability enabling subsequent cardiac repair were associated with lower mortality (p = 0.04). Survivors had a wide spectrum of long-term morbidity, but most had some evidence of neurodevelopmental impairment. This large single-institution series suggests that the overall prognosis of infants with concomitant CDH and congenital heart disease can be quite variable, regardless of the type of heart anomaly. Hemodynamic instability and need for extracorporeal membrane oxygenation correlate with higher mortality. Although some long-term survivors have excellent outcomes, most suffer from chronic, long-term morbidities.