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Introduction: Congenital heart disease (CHD) is the most prevalent congenital disability globally. This study aimed to describe parents' perspectives on financial stressors related to having a child with CHD using a descriptive qualitative approach., Method: Qualitative data were obtained from parents of children with CHD in a cross-sectional web-based survey study. Iterative data analysis was used to develop essential themes that enabled a rich description of 147 parents' perspectives., Results: Parents identified five financial stressors: perpetual worries about health insurance, facing the dilemma of "making too much money," struggling to balance work, worrying over having an emerging adult with CHD, and constant constraints because of financial needs., Discussion: As experts in pediatric care, pediatric advanced practice providers need to work with policymakers to provide further financial assistance and sufficient insurance coverage for families that struggle to balance finances for the whole family and children with CHD., Competing Interests: CONFLICTS OF INTEREST None to report., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Data regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized., Methods: This was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link., Results: Among 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P < .001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values < .05)., Conclusions: The presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: There is an increased risk of mortality in patients in whom acute kidney injury and fluid accumulation develop after cardiothoracic surgery, and the risk is especially high when renal replacement therapy is needed. However, renal replacement therapy remains an essential intervention in managing these patients. The objective of this study was to identify risk factors for mortality in surgical patients requiring renal replacement therapy in a pediatric cardiac intensive care unit., Methods: We performed a retrospective review of patients requiring renal replacement therapy for acute kidney injury or fluid accumulation after cardiothoracic surgery between January 2009 and December 2017. Survivors and nonsurvivors were compared with respect to multiple variables, and a multivariable logistic regression analysis was performed to identify independent risk factors associated with mortality., Results: The mortality rate for the cohort was 75%. Nonsurvivors were younger (nonsurvivors: 0.8 years; interquartile range, 0.1-8.2; survivors: 14.6 years; interquartile range, 4.2-19.7; P = .002) and had a lower weight-for-age z-score (nonsurvivors: -1.5; interquartile range, -3.1 to -0.4; survivors: -0.5; interquartile range, -0.9 to 0.3; P = .02) compared with survivors. There was no difference with respect to fluid accumulation. In multivariable analysis, a longer duration of stage 3 acute kidney injury before initiation of renal replacement therapy was independently associated with mortality (adjusted odds ratio, 1.39; 95% confidence interval, 1.05-1.83; P = .021)., Conclusions: Mortality in patients requiring renal replacement therapy after congenital heart disease surgery is high. A longer duration of acute kidney injury before renal replacement therapy initiation is associated with increased mortality., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Previous studies demonstrate racial and ethnic disparities among children undergoing congenital heart surgery. Extracorporeal membrane oxygenation (ECMO) is used to support critically ill children after congenital heart surgery and improve survival. Thus, racial or ethnic variations in postoperative ECMO use following congenital heart surgery may be associated with racial/ethnic disparities in hospital survival., Methods: All children in the Pediatric Health Information Systems dataset undergoing congenital heart surgery from 2004 to 2015 were examined. Multivariable, multinomial regression models examining hospital survival without ECMO use, survival after ECMO, death after ECMO, and death without ECMO support were constructed., Results: Of 130,860 congenital cardiac surgery patients, 95.4% survived to hospital discharge without requiring ECMO support, whereas 1.3% survived after ECMO support, 1.3% died after ECMO support, and 1.9% died without receiving ECMO support. After adjustment for other covariates, black patients (odds ratio, 1.22; 95% confidence interval [CI], 1.05-1.42) and patients of other race (odds ratio, 1.36; 95% CI, 1.17-1.58) were at increased odds of mortality compared with white patients. In multivariable multinomial models, black patients had increased risk of death without ECMO support (relative risk, 1.31; 95% CI, 1.11-1.56). Patients of other race (relative risk, 1.37; 95% CI, 1.10-1.69) and governmental insurance (relative risk, 1.24; 95% CI, 1.12-1.37) were also at increased risk of death without ECMO., Conclusions: Black children and children of other race are at increased odds of mortality after congenital heart surgery. These disparities can be traced to variations in ECMO utilization across racial/ethnic groups., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: To update previously described trends for neonates with congenital diaphragmatic hernia (CDH) receiving ECMO with changes in recommendations for care, and to determine how recent advancements in respiratory care have affected this patient population., Study Design: This study is a retrospective review of more than 2500 neonates with CDH who received ECMO listed in the Extracorporeal Life Support Organization (ELSO) registry. Cochran-Armitage and multivariate regression analyses were used to analyze changes in the patient population over time and in mortality-related risk factors., Results: Almost one-half (48.1%) of the term neonates survived to discharge, representing a 13.8% decline in survival over the past 25 years (P < .0001). Over the past 10 years, the prevalence of respiratory acidosis more than doubled (P < .0001) and the prevalence of major complications increased (P < .001). During the same period, the number of ECMO courses longer than 1 week increased (P < .001), whereas the prevalence of multiple complications (>4) decreased (P < .0001). Surgeries performed on ECMO were associated with worse outcomes than those performed off ECMO. ECMO duration no longer represents a mortality-related risk factor., Conclusions: Survival rates for neonates with CDH receiving ECMO have continued to drop in the modern era. Although the safety of ECMO has improved over the last decade, the number of patients experiencing significant respiratory acidosis has more than doubled-increasing the risk of intracranial hemorrhage and overall mortality. The evidence for permissive hypercapnia remains mixed; nonetheless, we believe that the risks outweigh the rewards in this patient population., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: Publicly available data from the Pediatric Heart Network's Single Ventricle Reconstruction Trial was analyzed to determine the prevalence, timing, risk factors for, and impact of second- and third-degree heart block (HB) on outcomes in patients who underwent stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS)., Methods: The presence and date of onset of post-S1P HB occurring within the first year of life, potential risk factors for HB, and factors known to predict poor outcomes after S1P were extracted. Multivariable logistic and Cox regression analyses were performed to identify risk factors for HB and to determine the effect of HB on 3-year transplantation-free survival., Results: Among the 549 patients in the cohort, 33 (6%) developed HB after S1P. The median interval between S1P and HB was 8 days (interquartile range, 0-133 days). Regression analysis showed that tricuspid valve repair during S1P and obstruction of pulmonary venous drainage requiring pre-S1P intervention were independently associated with HB (adjusted odds ratio [aOR], 11.6, 95% confidence interval [CI] 3.3-40; P < .001 and aOR, 5.1; 95% CI, 1.3-20.6; P = .02, respectively). Transplantation-free survival at 3 years was lower for those with HB (39% vs 65%; P = .004). HB remained associated with transplantation-free survival after controlling for known risk factors (adjusted hazard ratio, 3.1; 95% CI, 1.9-5.0; P < .001). Nine children (27%) had a pacemaker implanted, and 7 of these children (78%) died or underwent heart transplantation., Conclusions: HB after S1P is rare but heralds a poor outcome. Careful monitoring of these patients is recommended given their significantly increased risks of death and heart transplantation., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Support with extracorporeal membrane oxygenation for cardiopulmonary failure is done so with venoarterial cannulation in the majority of children with single-ventricle anatomy. However, there is a growing experience for patients with pure oxygenation/ventilation impairment supported with venovenous extracorporeal membrane oxygenation. We describe that experience., Methods: Data were collected from the Extracorporeal Life Support Organization registry for patients with single-ventricle anatomy supported with venovenous extracorporeal membrane oxygenation from 1990 to 2012. Descriptive statistics and multivariate analyses for associations with mortality were conducted., Results: A total of 89 patients with single-ventricle anatomy had venovenous extracorporeal membrane oxygenation performed at a median age of 66 days (8-221). Survival to discharge was 48%. Fifty-four patients (61%) had shunt physiology, 22 patients (25%) had cavopulmonary connections, and 13 patients (14%) had single-ventricle anatomy but with no previous cardiac surgery. Indication for extracorporeal membrane oxygenation was respiratory failure in 59 patients (63%) and cardiac failure in 30 patients (32%). Double-lumen cannulas were used in 62 patients (70%). Bivariate analysis demonstrated that the duration of intubation before extracorporeal membrane oxygenation, mean airway pressure before cannulation, partial pressure carbon dioxide before cannulation, peak inspiratory pressure before cannulation, pump flow at 24 hours, extracorporeal membrane oxygenation run duration, and presence of renal injury were associated with mortality. Multivariate logistic analysis demonstrated that the duration of intubation (adjusted odds ratio, 1.01; 95% confidence interval, 1.003-1.016; P = .003), partial pressure carbon dioxide (adjusted odds ratio, 1.04; 95% confidence interval, 1.01-1.068; P = .007), mean airway pressure (adjusted odds ratio, 1.16; 95% confidence interval, 1.0-1.342; P = .05), and renal injury (adjusted odds ratio, 6.6; 95% confidence interval, 1.879-23.2; P = .003) were associated with mortality., Conclusions: Patients with single-ventricle anatomy in respiratory failure may be treated successfully with venovenous extracorporeal membrane oxygenation, with survival comparable to those treated with venoarterial extracorporeal membrane oxygenation for cardiac failure. Future research on indications for venovenous extracorporeal membrane oxygenation may aid clinicians in deciding the optimal approach for this challenging cohort., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To assess the characteristics associated with high hospital cost for patients receiving extracorporeal membrane oxygenation (ECMO) to identify a cohort of high-resource users., Study Design: Cost for hospitalization, during which ECMO support was used, was calculated from hospital charges reported in the 2012 Health Care Cost and Use Project Kid's Inpatient Database. Patients were categorized into 6 diagnostic groups: (1) cardiac surgery; (2) nonsurgical heart disease; (3) congenital diaphragmatic hernia; (4) neonatal respiratory failure; (5) pediatric respiratory failure; and (6) sepsis. We categorized cost into 4 groups based on quartiles. We compared ECMO cost with hospital cost for bone marrow, liver, and kidney transplants performed during the same year., Results: Median hospital cost for children supported with ECMO (n = 1465) was $230,425 (IQR: $126,599-$420,960). In a multivariable model, lower cost was associated with neonatal respiratory failure (OR: 0.19) and sepsis (OR 0.53) compared with cardiac surgery (OR: 1.88), whereas greater cost was associated with smaller hospital bed-size <99 (OR: 3.49) and 100-399 beds (OR: 3.03) compared with hospitals >400 beds, hospital location (Midwest [OR: 1.74] and West [OR 2.18] compared with North-East), and complications such as renal failure (OR: 3.77) and thromboembolic complications (OR 1.60). Hospital cost per survivor was greater for ECMO ($519,450) than bone marrow transplantation ($207,212), liver ($231,755), or kidney transplantation ($82,008) groups., Conclusions: Hospitalization cost for children supported with ECMO is high. Diagnosis, hospital characteristics, and presence of complications are associated with increased cost., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: To describe the use of extracorporeal membrane oxygenation (ECMO) in patients with trisomy 21 (T21), to identify risk factors for hospital mortality, and to compare outcomes with those of patients without T21., Study Design: Children under age 18 years registered in the Extracorporeal Life Support Organization Registry were included. Comparisons between patients with T21 and patients without T21 were performed using the χ(2) or Wilcoxon rank-sum test and multivariable logistic regression., Results: The study cohort included 623 patients with T21 and 46 239 patients without T21. The prevalence of T21 was 13.5/1000 patients receiving ECMO. ECMO utilization in patients with T21 increased over time, with 60% of cases occurring in the last decade. There was no significant difference in survival between patients without T21 and those with T21 (63% vs 57%; P = .23). In patients with T21, independent risk factors for mortality before cannulation were a cardiac indication for ECMO support and milrinone use (P ≤ .001 for both). Multivariable risk factors for mortality on ECMO included hemorrhagic, neurologic, renal, and pulmonary complications (P < .04 for all)., Conclusion: The use of ECMO in patients with T21 has increased over time. Patients with a cardiac indication for ECMO have higher mortality compared with those supported for respiratory indications. Despite differences in indications for ECMO, patients with T21 have similar hospital survival as those without T21; thus, by itself, a diagnosis of T21 should not be considered a risk factor for in-hospital mortality when contemplating ECMO cannulation., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: Acute kidney injury (AKI) is a predictor of outcomes in heterogeneous populations of children undergoing cardiac surgery. We investigated its causes and consequences in a cohort undergoing Fontan completion, hypothesizing that central venous pressure is independently associated with development of AKI., Methods: In this retrospective cohort study of patients undergoing Fontan (n = 211), univariable and multivariable analyses identified factors associated with AKI within 3 days of surgery. Secondary analyses identified factors associated with hospital length of stay, and examined effects of perioperative kidney injury on follow-up renal function., Results: Acute kidney injury occurred in 42% of cases (n = 89), with the following independent risk factors: mean renal perfusion (mean arterial minus central venous) pressure on postoperative day zero (per mm Hg; adjusted odds ratio [AOR] 0.83; P < .001); preoperative atrioventricular valve regurgitation > mild (AOR 6.78; P = .02); bypass time (per 10 minutes, AOR 1.08; P = .04); peak inotrope score on postoperative day zero (per point, AOR 1.17; P < .001); and preoperative pulmonary vascular resistance (per Wood unit, AOR 1.69; P = .04). Central venous pressure was not independently associated with AKI. Moderate and severe (but not mild) AKI were independently associated with prolonged hospital length of stay (adjusted hazard ratios, 0.56; P = .004, and .41; P = .006, respectively). Perioperative injury was not associated with longer-term renal dysfunction., Conclusions: Acute kidney injury is common after Fontan completion and has several potentially modifiable risk factors. Moderate-to-severe injury is associated with longer hospital length of stay but not with renal dysfunction at follow-up., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Patients who have undergone the superior cavopulmonary anastomosis (Glenn procedure) have unique cardiopulmonary-cerebral physiology that may limit the success of cardiopulmonary resuscitation and extracorporeal membrane oxygenation (ECMO). Limited data published to date suggest grim morbidity and mortality when ECMO is used. We utilized the Extracorporeal Life Support Organization registry database to more thoroughly assess outcomes in these patients., Methods: Data from the Extracorporeal Life Support Organization registry from 1999 to 2012 for children with Glenn physiology aged 3 months to 1 year were retrospectively analyzed. Demographics and ECMO characteristics were compared between survivors and nonsurvivors. Factors associated with mortality were evaluated using multivariate logistic regression., Results: Of 103 infants, 42 (41%) survived to hospital discharge. Neurologic complications (eg, seizure, hemorrhage, or embolic stroke) were documented in 23% of patients (24 of 103) and 14% of survivors (6 of 42). In univariate analysis, inotropic requirement before ECMO, duration of ECMO, mechanical complications with the ECMO circuit, renal failure, and pulmonary hemorrhage or pneumothorax were predictors of mortality. In multivariate logistic regression, inotrope requirement (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.3-9.8), longer duration of ECMO support (OR, 7.2; 95% CI, 1.8-28), combined cardiopulmonary indication for ECMO (OR, 3.7; 95% CI, 1.4-9.7), and renal failure (OR, 4.2; 95% CI, 1.5-12) were associated with mortality., Conclusions: Mortality in infants with Glenn physiology supported with ECMO is lower than that previously reported, but the incidence of neurologic injury is high. These data support use of ECMO in patients with Glenn physiology with refractory cardiopulmonary failure., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Children who undergo cardiac surgery are at high risk for renal insufficiency and abdominal compartment syndrome. Peritoneal dialysis catheter (PDC) implantation is used in this population for abdominal decompression and access for dialysis. However, there is no consensus regarding PDC use, and the practice varies widely. This study was undertaken to assess associated factors, outcomes, and variability in the use of PDC in patients who have undergone cardiac surgery., Methods: The cohort was obtained from the Kids' Inpatient Database, years 2006 and 2009. Patients who underwent cardiac surgery were included and the subset that underwent PDC implantation during the same hospitalization was identified. Univariable and multivariable analyses assessed factors associated with PDC and survival., Results: A cohort of 28,259 patients underwent cardiac surgery, of whom 558 (2%) had PDCs placed. In the PDC group, 39.1% (n = 218) had acute renal failure whereas 3.5% or patients (n = 974) in the non-PDC group had acute renal failure. Among patients receiving PDC, mortality was 20.3% (n = 113; vs 3.4% overall mortality, n = 955). Excluding patients with acute renal failure, mortality remained 12% (n = 41) for the PDC group. Factors associated significantly with PDC placement in the overall cohort were younger age, greater surgical complexity, nonelective admission, hospital region, use of cardiopulmonary bypass, and acute renal failure., Conclusions: Patients receiving PDC after cardiac surgery had 20% mortality, which remained 12% after excluding patients with acute renal failure. Given the variability in PDC use and poor outcomes, further research is needed to assess the possible benefit of earlier intervention for peritoneal access in this high-risk cohort., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: The effect of mild acute kidney injury (AKI) on outcomes after heart surgery in children is unclear. We sought to characterize the epidemiology of mild AKI associated with surgery for congenital heart disease (CHS-AKI) in children., Methods: We conducted a single-center, retrospective cohort study of 693 patients (aged 6 days-18 years) who underwent heart surgery in 2009. The prevalence of AKI within 72 hours of surgery was determined using the 3-stage Acute Kidney Injury Network criteria. Factors associated with both hospital length of stay and AKI were used in a proportional hazards model to test the association of stage 1 AKI with hospital length of stay., Results: The median age of the patients was 11.5 months (interquartile range, 3-54 months). Eighteen percent of the cohort had single ventricle heart disease and 54% underwent RACHS-1 category 3 or higher surgery. The prevalence of stages 1, 2, and 3 AKI in this cohort was 11% (n = 77), 3% (n = 19), and 1% (n = 8), respectively. Factors independently associated with AKI were prematurity, single ventricle physiology, peak postoperative lactic acid concentration, cardiopulmonary bypass time, and a history of heart surgery. Stage 2 or greater CHS-AKI was associated with hospital length of stay (adjusted hazard ratio [AHR], 0.53; 95% confidence interval [CI], 0.33-0.87; P = .01), but stage 1 was not (AHR, 0.85; 95% CI, 0.66-1.10; P = .22)., Conclusions: AKI occurs after surgery for congenital heart disease but may be less common than previously reported. Although moderate to severe CHS-AKI is independently associated with prolonged recovery after heart surgery, mild disease does not appear to be., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: To report the outcomes from a large multicenter cohort of neonates requiring extracorporeal membrane oxygenation (ECMO) after stage 1 palliation for hypoplastic left heart syndrome., Methods: Using data from the Extracorporeal Life Support Organization (2000-2009), we computed the survival to hospital discharge for neonates (age ≤30 days) supported with ECMO after stage 1 palliation for hypoplastic left heart syndrome. The factors associated with mortality were evaluated using multivariate logistic regression analysis., Results: Among 738 neonates, the survival rate was 31%. The median age at cannulation was 7 days (interquartile range, 4-11). Black race (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.6), mechanical ventilation before ECMO (>15-131 hours: OR, 1.6; 95% CI, 1.1-2.4; >131 hours: OR, 1.9; 95% CI, 1.3-2.9), use of positive end expiratory pressure (>6-8 cm H(2)O: OR, 1.7; 95% CI, 1.1-2.7; >8 cm H(2)O: OR, 1.9; 95% CI, 1.2-3.1), and longer ECMO duration (per day, OR, 1.2; 95% CI, 1.1-1.3) increased mortality. ECMO support for failure to wean from cardiopulmonary bypass (OR, 1.6; 95% CI, 1.02-2.4) also decreased survival. ECMO complications, including renal failure (OR, 1.9; 95% CI, 1.2-3.1), inotrope requirement (OR, 1.5; 95% CI, 1.1-2.1), myocardial stun (OR, 3.2; 95% CI, 1.3-7.7), metabolic acidosis (OR, 2.9; 95% CI, 1.3-6.7), and neurologic injury (OR, 1.7; 95% CI, 1.1-2.6), during support also increased mortality., Conclusions: Mortality for neonates with hypoplastic left heart syndrome supported with ECMO after stage 1 palliation is high. Longer ventilation before cannulation, longer support duration, and ECMO complications increased mortality., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: We have previously shown that surgical Technical Performance Scores (TPS) are important predictors of early postoperative morbidity across a wide spectrum of procedures and that intraoperative recognition and intervention of residual defects resulted in improved outcomes. We hypothesized that these scores would also be important predictors of midterm outcomes., Methods: Neonates and infants aged younger 6 months were prospectively followed from the index surgery for a minimum of 1 year. The TPS were calculated using previously published criteria, including intraoperative course, predischarge echocardiograms or catheterizations, and clinical data, and graded as optimal, adequate, or inadequate. Case complexity was determined by the Risk Adjustment for Congenital Heart Surgery-1 category. The primary outcome was mortality, and the secondary outcome was the need for unplanned reinterventions. Outcomes were analyzed using nonparametric methods and a logistic regression model., Results: A total of 166 patients were included in our study, with 7 early deaths. The remaining 159 patients (Risk Adjustment for Congenital Heart Surgery-1 category 4-6, 76 [48%]; neonates, 78 [49%]) were followed for a minimum of 1 year after surgery. There were 14 late deaths or late transplantations and 55 late reinterventions. On univariate analysis, the TPS were associated with mortality (P < .001) and reintervention (P = .04). On logistic regression analysis, inadequate TPS was associated with late mortality (P < .001; odds ratio, 7.2; 95% confidence interval, 2.2-23.6), and Risk Adjustment for Congenital Heart Surgery-1 category (P = .004; odds ratio, 3.7; 1.5-8.8) at index surgery was associated with need for late unplanned reintervention., Conclusions: Technical performance affects midterm survival after infant heart surgery. Inadequate TPS can be used to prospectively identify patients at ongoing risk of demise and the need for reintervention. An aggressive approach to diagnosing and treating residual lesions at the initial operation is warranted., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Our objective was to define the relationship between surgical technical performance score, intraoperative adverse events, and major postoperative adverse events in complex pediatric cardiac repairs., Method: Infants younger than 6 months were prospectively followed up until discharge from the hospital. Technical performance scores were graded as optimal, adequate, or inadequate based on discharge echocardiograms and need for reintervention after initial surgery. Case complexity was determined by Risk Adjustment in Congenital Heart Surgery (RACHS-1) category, and preoperative illness severity was assessed by Pediatric Risk of Mortality (PRISM) III score. Intraoperative adverse events were prospectively monitored. Outcomes were analyzed using nonparametric methods and a logistic regression model., Results: A total of 166 patients (RACHS 4-6 [49%]), neonates [50%]) were observed. Sixty-one (37%) had at least 1 intraoperative adverse event, and 47 (28.3%) had at least 1 major postoperative adverse event. There was no correlation between intraoperative adverse events and RACHS, preoperative PRISM III, technical performance score, or postoperative adverse events on multivariate analysis. For the entire cohort, better technical performance score resulted in lower postoperative adverse events, lower postoperative PRISM, and lower length of stay and ventilation time (P < .001). Patients requiring intraoperative revisions fared as well as patients without, provided the technical score was at least adequate., Conclusions: In neonatal and infant open heart repairs, technical performance score is one of the main predictors of postoperative morbidity. Outcomes are not affected by intraoperative adverse events, including surgical revisions, provided technical performance score is at least adequate., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Currently, there are no Food and Drug Administration-approved devices available that can provide long-term mechanical circulatory support to smaller children with severe heart failure as a bridge to heart transplant (HT). In recent years, the Berlin Heart EXCOR Pediatric ventricular assist device (VAD) has emerged as a potential treatment option. Systematic data on the safety and efficacy of the EXCOR are limited., Methods: The Investigational Device Exemption (IDE) clinical study is designed to evaluate the safety and probable benefit of the EXCOR to support regulatory review of the device under the Humanitarian Device Exemption regulation. The study design and rationale are reviewed in light of the well-described challenges inherent in small population studies., Results: The Berlin Heart EXCOR IDE clinical study is a prospective, multicenter, single-arm, clinical cohort study. Children aged 0 to 16 years with severe heart failure (Interagency Registry for Mechanically Assisted Circulatory Support profile 1 or 2) due to 2-ventricle heart disease and actively listed for HT comprise the primary study cohort. The control population is a propensity-matched retrospective cohort of children supported with extracorporeal membrane oxygenation, the only bridge device available to smaller children before the EXCOR. The primary efficacy end point is survival to heart transplantation or recovery. The primary safety end point is the incidence of serious adverse events as defined by pediatric Interagency Registry for Mechanically Assisted Circulatory Support criteria. The study will enroll a total of 48 subjects in 2 cohorts based on body surface area (cohort 1 <0.7 m(2), cohort 2 0.7-1.5 m(2)) and is powered to show safety superiority to a prespecified performance goal of 0.25 serious adverse events per day of support. Children ineligible for the primary cohort will still have access to the device in a third compassionate-use cohort where adverse event data will be collected for additional safety characterization of the device., Conclusion: The Berlin Heart IDE clinical study will be the first bridge-to-HT VAD study designed exclusively for children. It is anticipated that the study will provide important information on the safety and efficacy of the Berlin Heart EXCOR Pediatric in children while providing valuable lessons into the design and conduct of future VAD studies in children., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Objective: Extracorporeal membrane oxygenation has been used to support children with cardiac failure after the Fontan operation. Mortality is high, and causes of mortality remain unclear. We evaluated the in-hospital mortality and factors associated with mortality in these patients., Methods: Extracorporeal Life Support Organization registry data on patients requiring extracorporeal membrane oxygenation after the Fontan operation from 1987 to 2009 were retrospectively analyzed. Demographics and extracorporeal membrane oxygenation data were compared for survivors and nonsurvivors. A multivariable logistic regression model was used to identify factors associated with mortality., Results: Of 230 patients, 81 (35%) survived to hospital discharge. Cardiopulmonary resuscitation was more frequent (34% vs 17%, P = .04), and median fraction of inspired oxygen concentration was higher (1 [confidence interval, 0.9-1.0] vs 0.9 [confidence interval, 0.8-1.0], P = .03) before extracorporeal membrane oxygenation in nonsurvivors compared with survivors. Extracorporeal membrane oxygenation duration and incidence of complications, including surgical bleeding, neurologic injury, renal failure, inotrope use on extracorporeal membrane oxygenation, and bloodstream infection, were higher in nonsurvivors compared with survivors (P < .05 for all). In a multivariable model, neurologic injury (odds ratio, 5.18; 95% confidence interval, 1.97-13.61), surgical bleeding (odds ratio, 2.36; 95% confidence interval, 1.22-4.56), and renal failure (odds ratio, 2.81; 95% confidence interval, 1.41-5.59) increased mortality. Extracorporeal membrane oxygenation duration of more than 65 hours to 119 hours (odds ratio, 0.33; 95% confidence interval, 0.14-0.76) was associated with decreased mortality., Conclusions: Cardiac failure requiring extracorporeal membrane oxygenation after the Fontan operation is associated with high mortality. Complications during extracorporeal membrane oxygenation support increase mortality odds. Prompt correction of surgical bleeding when possible may improve survival., (Published by Mosby, Inc.)

Objective: To investigate factors associated with mechanical circulatory support and survival in patients with acute fulminant myocarditis (AFM)., Study Design: Retrospective cohort of AFM patients admitted to the cardiac intensive care unit during 1996-2008. AFM was defined as distinct onset of symptoms ≤14 days before admission, rapid-onset cardiogenic shock, and normal left ventricular size. Demographic and physiological variables were compared between patients treated with extracorporeal membrane oxygenation (ECMO) and those who were not and between survivors and nonsurvivors., Results: Twenty patients (median age 12.7 years) met inclusion criteria. Seventeen patients (85%) survived to hospital discharge. One underwent heart transplantation. Ten (50%) patients required ECMO, and 7 (70%) of these survived. On admission, patients requiring ECMO had elevated lactate (9 vs 1 mmol/L), creatinine (0.8 vs 0.6 mg/dL), and aspartate aminotransferase (256 vs 35 IU/L) (all P < .05) and a trend towards increased incidence of dysrhythmias (80% vs 30%, P = .07). During hospitalization, non-survivors had higher peak lactate (10 vs 3 mmol/L), creatinine (1.5 vs 0.8 mg/dL), and aspartate aminotransferase (3007 vs 156 IU/L) (all P < .05) compared with survivors., Conclusions: Patients with AFM with end organ dysfunction or arrhythmias on admission may require mechanical circulatory support. The transplant-free survival rate in this critically ill cohort was excellent (80%)., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Objective: Infants undergoing heart transplantation have the highest early posttransplant mortality of any age group. We sought to determine the pretransplantation factors associated with in-hospital mortality in transplanted infants in the current era., Methods: All infants under 12 months of age who underwent primary heart transplantation during a recent 10-year period (1999-2009) in the United States were identified using the Organ Procurement and Transplant Network database. Multivariable logistic regression was used to identify independent pretransplantation factors associated with in-hospital mortality., Results: Of 730 infants in the study (median age 3.8 months), 462 (63%) had congenital heart disease, 282 (39%) were supported by a ventilator, 94 (13%) with extracorporeal membrane oxygenation, and 22 (3%) with a ventricular assist device at the time of transplantation. Overall, 82 (11.2%) infants died before their initial hospital discharge. In adjusted analysis, in-hospital mortality was associated with repaired congenital heart disease (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.8, 7.2), unrepaired congenital heart disease not on prostaglandin E (OR, 2.8; CI, 1.3, 6.1), extracorporeal membrane oxygenator support (OR, 6.1; CI, 2.8, 13.4), ventilator support (OR, 4.4; CI, 2.3, 8.3), creatinine clearance less than 40 mL·min(-1)·1.73 m(-2) (OR, 3.1; CI, 1.7, 5.3), and dialysis (OR, 6.2; CI, 2.1, 18.3) at transplantation., Conclusions: One in 9 infants undergoing heart transplantation dies before hospital discharge. Pretranplantation factors associated with early mortality include congenital heart disease, extracorporeal membrane oxygenator support, mechanical ventilation, and renal failure. Risk stratification for early posttransplant mortality among infants listed for heart transplantation may improve decision-making for transplant eligibility, organ allocation, and posttransplant interventions to reduce mortality., (Copyright © 2011 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: Resuscitation of pediatric cardiac patients involves unique and complex physiology, requiring multidisciplinary collaboration and teamwork. To optimize team performance, we created a multidisciplinary Crisis Resource Management training course that addressed both teamwork and technical skill needs for the pediatric cardiac intensive care unit. We sought to determine whether participation improved caregiver comfort and confidence levels regarding future resuscitation events., Methods: We developed a simulation-based, in situ Crisis Resource Management curriculum using pediatric cardiac intensive care unit scenarios and unit-specific resuscitation equipment, including an extracorporeal membrane oxygenation circuit. Participants replicated the composition of a clinical team. Extensive video-based debriefing followed each scenario, focusing on teamwork principles and technical resuscitation skills. Pre- and postparticipation questionnaires were used to determine the effects on participants' comfort and confidence regarding participation in future resuscitations., Results: A total of 182 providers (127 nurses, 50 physicians, 2 respiratory therapists, 3 nurse practitioners) participated in the course. All participants scored the usefulness of the program and scenarios as 4 of 5 or higher (5 = most useful). There was significant improvement in participants' perceived ability to function as a code team member and confidence in a code (P < .001). Participants reported they were significantly more likely to raise concerns about inappropriate management to the code leader (P < .001)., Conclusions: We developed a Crisis Resource Management training program in a pediatric cardiac intensive care unit to teach technical resuscitation skills and improve team function. Participants found the experience useful and reported improved ability to function in a code. Further work is needed to determine whether participation in the Crisis Resource Management program objectively improves team function during real resuscitations., (2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Interplay of baseline physiologic status, case complexity, technical performance, and outcomes in high-acuity operations has been poorly defined. This study explored these interactions to determine whether a technically optimal operation can mitigate effects of baseline physiology and high case-complexity on outcomes for the stage I Norwood procedure., Methods: Technical performance was categorized as optimal, adequate, or inadequate from adequacy of the anatomic repair of the stage I subprocedures according to anatomic areas where intervention is performed. Physiological illness severity statuses in preoperative and postoperative periods were determined with Pediatric Risk of Mortality III system, which uses 17 physiologic variables. Case complexity was calculated with Aristotle comprehensive system. All patients undergoing stage I procedure from January 2004 to December 2007 were retrospectively studied., Results: One hundred thirty-five procedures were included. Five were excluded from the technical performance assessment because of inadequate postoperative data. Eighty-one (62.3%), 26 (20%), and 23 (17.7%), respectively, were scored as optimal, adequate, and inadequate. Overall hospital mortality was 14.1%. Inadequate technical performance, high-complexity Aristotle comprehensive scores, and high preoperative illness severity scores correlated with significantly higher hospital mortality, longer stay, and greater frequency of major postoperative complications. In subgroup analysis of patients with optimal technical performance, outcomes were favorable irrespective of high or low preoperative physiologic illness severity or case complexity., Conclusions: In stage I Norwood procedures, optimal technical performance attenuated effects of poor preoperative physiologic status and high case complexity, with reduced hospital mortality. Inadequate technical performance resulted in poor outcomes regardless of preoperative status., (Copyright 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: We investigated survival and predictors of mortality for infants and children with heart disease treated with extracorporeal membrane oxygenation as an aid to cardiopulmonary resuscitation., Methods: Children (<18 years) with heart disease who received extracorporeal cardiopulmonary resuscitation and were reported to the Extracorporeal Life Support Organization database were evaluated. Patients were classified into one of 3 groups based on underlying cardiac physiology: single ventricle, 2 ventricles, and cardiac muscle disease. Patients with eligible procedure codes were assigned a Risk Adjustment for Congenital Heart Surgery-1 classification., Results: Four hundred ninety-two patients were eligible for analysis, and 279 (57%) were assigned a Risk Adjustment for Congenital Heart Surgery-1 category. Overall survival was 42%. In a multivariable logistic regression analysis, significant pre-extracorporeal predictors for mortality included single-ventricle physiology (odds ratio, 1.6; 95% confidence interval, 1.05-2.4), a history of a stage 1-type procedure (odds ratio, 2.7; 95% confidence interval, 1.2-6.2), and extreme acidosis (arterial blood gas pH < 7.01; odds ratio, 2.2; 95% confidence interval, 1.3-3.7). Right carotid artery cannulation was associated with decreased mortality risk (odds ratio, 0.6; 95% confidence interval, 0.4-0.9). During extracorporeal support, complications, including renal injury, evidence of neurologic injury, and persistent acidosis, were associated with an increased risk of hospital mortality., Conclusion: Use of extracorporeal membrane oxygenation as an adjunct to cardiopulmonary resuscitation resulted in hospital survival in 42% of infants and children with heart disease. Underlying cardiac physiology and associated cardiac surgical procedures influenced mortality, as did pre-extracorporeal resuscitation status and extracorporeal membrane oxygenation-associated complications.

Objectives: The use of extracorporeal membrane oxygenation to support patients with shunted single-ventricle physiology has been controversial. Variable survivals are reported in a number of small case series. We sought to evaluate outcomes and identify predictors of survival for patients with shunted single-ventricle physiology who require extracorporeal membrane oxygenation support., Methods: We retrospectively reviewed the medical records of all patients aged less than 1 year with shunted single-ventricle physiology who were supported with extracorporeal membrane oxygenation at Children's Hospital Boston between 1996 and 2005. Survivors and nonsurvivors were compared with respect to demographics, diagnosis, operative variables, indication for extracorporeal membrane oxygenation, and extracorporeal membrane oxygenation variables., Results: Forty-four infants with shunted single-ventricle physiology were supported with extracorporeal membrane oxygenation. Diagnoses included hypoplastic left heart syndrome (24), other single-ventricle lesions (12), and pulmonary atresia/intact ventricular septum or a variant (8). Overall survival to discharge was 48%. Indication for extracorporeal membrane oxygenation was the strongest predictor of survival to discharge, with 81% of patients cannulated for hypoxemia but only 29% of those cannulated for hypotension surviving to discharge. Specifically, patients cannulated for shunt obstruction had the highest survival (83%)., Conclusions: Overall survival to discharge for patients with shunted single-ventricle physiology is similar to survival reported in the Extracorporeal Life Support Organization registry for all infants supported with cardiac extracorporeal membrane oxygenation. Thus, shunted single-ventricle physiology should not be considered a contraindication to extracorporeal membrane oxygenation. Patients cannulated for hypoxemia, particularly shunt thrombosis, had markedly improved survival compared with those supported primarily for hypotension/cardiovascular collapse. Survival did not differ depending on anatomic diagnosis.