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Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th-85th percentile 11.2-41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0-5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Given pulmonary artery interventions following the Norwood procedure can recur, the average number of occurrences per patient over time is likely more informative than the crude percentage of patients who required an intervention. Pulmonary artery intervention was defined as any surgical or catheter-based procedure after the Norwood procedure. The number of pulmonary artery interventions for patients with hypoplastic left heart syndrome were compared between patients with modified Blalock-Taussig Shunts (MBTS) and right ventricle-to-pulmonary artery conduits (RVPA) at a single institution from 2011 to 2018. The comparison was replicated using data from the Single Ventricle Reconstruction Trial (SVR), a nonoverlapping dataset. The mean number of pulmonary artery interventions per patient over time (mean cumulative function, MCF) is described using Nelson-Aalen estimates and compared using the pseudo-score test. The number of patients requiring intervention was compared using the chi-square test. Using our institutional dataset, the Norwood operation was performed on 117 patients (59 MBTS, 58 RVPA). In total, 73 patients had a pulmonary artery intervention, including 32 of 58 (55%) after MBTS and 41 of 59 (69%) after RVPA (P= 0.11). The MCF did not vary between cohorts (P = 0.55). Using the SVR trial dataset, 140 of 549 patients required pulmonary artery intervention, including 55 (21%) after MBTS and 85 (30%) after RVPA (P = 0.0090). The MCF did not vary between cohorts (P = 0.067). Although more patients with RVPA than MBTS require pulmonary artery interventions after the Norwood procedure, the MCFs are not different, which may be of greater importance to patients and families., (Copyright © 2020 Elsevier Inc. All rights reserved.)

In patients with Pierre Robin sequence (PRS), understanding the mechanism of airway obstruction and the potential for concurrent airway lesions is important for determining appropriate intervention. When concomitant airway anomalies are present with PRS, mandibular distraction osteogenesis alone is often contraindicated and unsuccessful at alleviating the obstruction, mandating the need for tracheostomy. Herein, the authors present the second-reported case of PRS with concomitant complete tracheal rings and highlight the importance collaborative surgical efforts to avoid tracheostomy. Our patient was born with signs and symptoms of PRS and concomitant complete tracheal rings. At birth, he developed respiratory compromise that was resistant to invasive and noninvasive intervention and was transferred to our care. The severity of his airway compromise necessitated operative intervention. Due to the patient's complex medical condition, avoiding the long-term sequelae of a tracheostomy and performing mandibular distraction was favorable. However, due to his multi-level airway disease, a 2-staged approach was planned: sliding tracheoplasty to release the tracheal rings, followed by mandibular distraction to correct the micrognathia. The patient tolerated both procedures and was successfully distracted and eventually weaned into room air. When managing patients with PRS in the setting of severe respiratory distress, securing the airway and carefully evaluating for concomitant airway anomalies is crucial. Even in the setting of multi-level airway disease where mandibular distraction is typically contraindicated, by directly addressing each anatomic level of airway obstruction, the airway can be safely secured and the resultant consequences of tracheostomy can be avoided. Careful planning with a multidisciplinary team is essential.

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: The use of ventricular assist devices for pediatric patients with heart failure is increasing, but is associated with significant morbidity and mortality. Our objectives were to describe the admission outcomes and resource utilization of pediatric patients supported with ventricular assist devices, utilizing a multicenter database., Data Sources: Pediatric Health Information System database (comprising 49 nonprofit children's hospitals)., Study Selection: Retrospective cohort analysis of the database from January 2006 to September 2015 for all admissions less than or equal to 21 years old with ventricular assist device implantation., Data Extraction: The primary outcome was hospital mortality. The secondary outcomes were hospital length of stay and adjusted cost., Data Synthesis: We analyzed 744 ventricular assist device implantations (740 patients), 422 (57%) males, and 363 (49%) non-Hispanic white. Median age at admission was 5.9 years (interquartile range, 0.9-13.5 yr), and median length of stay was 69 days (interquartile range, 36-122 d). The overall hospital mortality was 188 (25%), whereas 395 (53%) were transplanted and 141 (19%) were discharged on ventricular assist device. Extracorporeal membrane oxygenation was used, in addition to ventricular assist device, in 340 (46%). The majority of ventricular assist device implantations (453, 61%) were from 2011 to 2015 (compared to 2006-2010). More patients discharged on ventricular assist device from 2011 to 2015 (23% vs 13% in 2006-2010; p = 0.001). There was no difference in median age, mortality, length of stay, or adjusted costs between these time periods. On multivariable analysis, underlying congenital heart disease, renal failure, liver congestion, sepsis, cerebrovascular accident, and extracorporeal membrane oxygenation were associated with hospital mortality. Sepsis and ventricular assist device replacement/repair were associated with higher adjusted cost and longer length of stay., Conclusions: The pediatric ventricular assist device experience continues to grow, with a significant increase in the number of patients undergoing ventricular assist device implantation and a higher proportion being discharged from hospital on ventricular assist device support in recent years. Underlying congenital heart disease, renal failure, sepsis, cerebrovascular accident, and extracorporeal membrane oxygenation are significantly associated with hospital mortality.

Objective: The exact incidence and risk factors for reoperation in partial and transitional atrioventricular septal defects are unclear. The goal of this study was to assess risk factors for left atrioventricular valve and left ventricular outflow tract reoperation in partial and transitional atrioventricular septal defects., Methods: All patients undergoing partial and transitional atrioventricular septal defects repair between 1995 and 2017 were reviewed. Patients were classified as infants (<1 year), toddlers (1-3 years), children (3-17 years), and adults (≥18 years). Survival and reoperation were assessed using log-rank test and Cox models for univariate and multivariable analyses, respectively., Results: Overall, 265 patients underwent partial and transitional atrioventricular septal defects repair (partial: 177 [67%]). Median age was 2 years. The cohort included 73 infants (28%), 85 toddlers (32%), 94 children (35%), and 13 adults (5%). Trisomy 21 was present in 76 patients (29%), and in 216 patients (83%), the zone of apposition was completely closed. Perioperative mortality was 0.8%. Complete heart block did not develop in any patients. Ten-year survival and freedom from reoperation were 98% and 81%, respectively. On multivariable analysis, trisomy 21 (hazard ratio [HR], 0.16) and older age compared with infants (toddlers: HR, 0.35; children: HR, 0.25) were protective for any reoperation, whereas heterotaxy (HR, 3.43) was a risk factor. For left atrioventricular valve reoperation, toddlers (HR, 0.35), children (HR, 0.25), and trisomy 21 (HR, 0.16) remained protective, whereas left atrioventricular valve anomaly was a risk factor (HR, 2.61). Likewise, for left ventricular outflow tract reoperation, toddlers (HR, 0.24) and children (HR, 0.06) were protective., Conclusions: Mortality after partial and transitional atrioventricular septal defects repair is minimal, yet reoperation for left atrioventricular valve disease and left ventricular outflow tract obstruction remains significant. Patients requiring repair during infancy are at higher risk of reoperation., (Copyright © 2018. Published by Elsevier Inc.)

Background: Extracardiac birth defects are associated with worse outcomes in congenital heart disease (CHD). The impact of esophageal atresia/trachea-esophageal fistula (EA/TEF) on outcomes after surgery for ductal-dependent CHD is unknown., Methods: Retrospective matched cohort study using the Pediatric Health Information System database from 07/2004 to 06/2015. Hospitalizations with ductal-dependent CHD and EA/TEF, undergoing CHD surgery were included as cases. Admissions with ductal-dependent CHD without EA/TEF were matched 3:1 for age at admission and Risk Adjustment for Congenital Heart Surgery-1 classification. Comparisons were performed using generalized estimating equations., Results: There were 124 cases and 372 controls. Cases included 32 (25.8%) low-risk, 86 (69.3%) intermediate-risk, and 6 (4.8%) high-risk patients. Cases had more females compared to controls (53.2% vs 41.1%, P = .022). Cases were more likely to be premature (28.2% vs 13.7%, P = .001) and low birth weight (29.8% vs 11.8%, P < .001). Cases had a similar frequency of Down syndrome, and DiGeorge/Velocardiofacial syndrome, but a higher frequency of anorectal malformations (4.3% vs 2.4%, P < .001) and renal anomalies (27.4% vs 9.9%, P < .001) than controls. Cases had a higher mortality on univariate (22.0% vs 8.4%, P < .001) and multivariable analysis (odds ratio 2.45, 95%, confidence interval 1.34 - 4.49). Prematurity also was significantly associated with mortality on multivariable analysis. Cases had a longer duration of mechanical ventilation, longer hospital duration of stay, and higher total cost than controls (all P < .001)., Conclusion: In children with ductal-dependent CHD, EA/TEF is associated with increased morbidity, mortality and resource utilization. A majority of patients undergo EA/TEF repair prior to congenital heart disease surgery. (Surgery 2017;160:XXX-XXX.)., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Patients with right heart obstructive lesions develop residual or recurrent right ventricle outflow tract pathology as a result of native or implanted pulmonary valve (PV) dysfunction. Until recently, the standard of care has been surgical placement of a PV or valved right ventricle to pulmonary artery conduit. Catheter-based options are being increasingly applied in patients with PV dysfunction. The purpose of our study was to evaluate outcomes of surgical pulmonary valve/conduit replacement (PVR) at a large pediatric hospital to provide contemporary benchmark data for comparison with developing technologies., Methods: Retrospective review of patients undergoing PVR not associated with complex concomitant procedures from July 1995 to December 2010 was completed. Inclusion criteria were designed to generally match those applied to patients promoted for catheter-based valve replacement based on age and weight (age≥5 years and weight≥30 kg)., Results: There were 148 PVRs with all patients having undergone ≥1 previous interventions (tetralogy of Fallot [53%] and pulmonary atresia [17%]). Surgical indications were PV insufficiency (60%), PV stenosis (26%), and both (13%). Valves used included bioprosthetic (n=108; 73%) and homografts (n=40; 27%). Time-to-extubation, intensive care unit stay, and hospital length of stay were <1 day (interquartile range, 0-1 day), 2 days (interquartile range, 1-2 days), and 5 days (interquartile range, 4-6 days), respectively, with no hospital deaths. Freedom from PV reintervention at 1, 3, and 5 years was 99%, 99%, and 94%, respectively. Multivariable analysis showed age<13 years (P=.003), and smaller valve size (P=.025) were associated with increased risk of valve reintervention. Patient survival at follow-up (mean, 5.0±3.9 years) was 99%., Conclusions: Surgical PVR is safe with low in-hospital and midterm follow-up mortality and reoperation rates. These outcomes provide a useful benchmark for treatment strategy comparisons., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: The present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL., Methods: The present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated., Results: At follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the survey's Core Module, nor were any differences found in the outcomes assessed using the survey's Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores., Conclusions: Over the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy., Methods: Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months., Results: Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 (P = .19), Language Scores were 92.4 ± 12.4 (P = .33), and Motor Scale scores were 92.6 ± 14.1 (P = .51)., Conclusions: Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Pediatric diffuse lung diseases comprise a heterogeneous group of rare lung disorders which may lead to end stage lung disease and referral for lung transplantation. Previous studies are limited by small numbers of patients with specific forms of diffuse lung disease. Children with all forms of diffuse lung disease who underwent lung transplantation at two pediatric centers were evaluated in terms of several pre- and post-transplant factors and compared to children with other end stage lung disorders., Methods: A retrospective chart review was performed on all patients transplanted between October 1, 2002 and June 15, 2007 at Texas Children's Hospital and St. Louis Children's Hospital. Multiple pre-transplant characteristics and post-transplant morbidities and mortality were compared between diffuse lung disease, cystic fibrosis, and pulmonary vascular disease groups., Results: There were 31 diffuse lung disease (DLD), 57 cystic fibrosis (CF), and 16 pulmonary vascular disease (PVD) patients included in our analysis. Patients with DLD had significantly higher pre-transplant morbidity including lower percent predicted of forced expiratory volume in first second (P = 0.013) and more patients with pulmonary hypertension (P = 0.001) and hypercapnia (P = 0.031). Compared to CF patients, more DLD and PVD patients required invasive ventilation (P = 0.001) and care in the pediatric intensive care unit (P = 0.001). After transplant, there was a difference among the three groups with regards to number of acute allograft rejections but statistical limitations preclude knowing between which group the difference lies. A difference in time to bronchiolitis obliterans was found between the PVD and CF groups but not when compared to the DLD patients. The three groups had similar time to post-transplant lymphoproliferative disease, rate of infections, and survival., Conclusion: Lung transplantation is as successful for patients with end stage diffuse lung disease as compared to other lung transplant candidates., (Copyright © 2012 Wiley Periodicals, Inc.)

Objective: Few data are available on the frequency or importance of perioperative atrial tachycardia in infants. We hypothesized that atrial tachycardia in infants undergoing cardiac surgery is not rare and is associated with increased morbidity and mortality., Methods: From 2007 through 2010, 777 infants (median age, 1.8 months; interquartile range, 0.33-5.73) underwent cardiac surgery. Their medical records were reviewed for atrial tachycardia during the perioperative period., Results: Of the 777 patients, 64 (8.2%) developed atrial tachycardia. The independent risk factors for developing atrial tachycardia included surgical age 6 months or younger (odds ratio, 4.4; 95% confidence interval, 1.1-19.15), use of 3 or more inotropes (odds ratio, 2.9; 95% confidence interval, 1.4-6.2), and heterotaxy syndrome (odds ratio, 2.9; 95% confidence interval, 1.1-7.4). All-cause mortality in the atrial tachycardia group was increased (21.9% vs 7.2%, P<.001) during a median follow-up period of 14.6 months (interquartile range, 6.8-24.6), and atrial tachycardia was independently associated with decreased survival (hazard ratio, 1.9; 95% confidence interval, 1.1-3.8). Infants with perioperative atrial tachycardia had a longer hospital length of stay (32 vs 17 days, P<.001) and duration of inotrope use (10.5 vs 3.0 days, P<.001). A total of 57 patients received antiarrhythmic therapy, with propranolol the most common (n=31). Among the survivors, 48 patients received outpatient antiarrhythmic therapy, which was successfully discontinued in 23 patients at a median duration of 14 months (interquartile range, 5.7-18.6) without recurrence., Conclusions: Atrial tachycardia is common in infants undergoing cardiac surgery and is independently associated with decreased survival. Among survivors, antiarrhythmic agents successfully controlled atrial tachycardia in most patients with a low recurrence risk after discontinuation., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Few data exist on prevalence, morbidity, and mortality of pediatric heart failure hospitalizations. We tested the hypotheses that pediatric heart failure-related hospitalizations increased over time but that mortality decreased. Factors associated with mortality and length of stay were also assessed., Methods and Results: A retrospective analysis of the Healthcare Cost and Utilization Project Kids' Inpatient Database was performed for pediatric (age ≤18 years) heart failure-related hospitalizations for the years 1997, 2000, 2003, and 2006. Hospitalizations did not significantly increase over time, ranging from 11,153 (95% confidence interval [CI] 8,898-13,409) in 2003 to 13,892 (95% CI 11,528-16,256) in 2006. Hospital length of stay increased from 1997 (mean 13.8 days, 95% CI 12.5-15.2) to 2006 (mean 19.4 days, 95% CI 18.2 to 20.6). Hospital mortality was 7.3% (95% CI 6.9-8.0) and did not vary significantly between years; however, risk-adjusted mortality was less in 2006 (odds ratio 0.70, 95% CI 0.61 to 0.80). The greatest risk of mortality occurred with extracorporeal membrane oxygenation, acute renal failure, and sepsis., Conclusions: Heart failure-related hospitalizations occur in 11,000-14,000 children annually in the United States, with an overall mortality of 7%. Many comorbid conditions influenced hospital mortality., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Coarctation of the aorta (CoA) is often associated with clinically significant hypoplasia of the aortic arch. Historically, patch aortoplasty or bypass procedures have been the preferred techniques when arch augmentation is required in children beyond infancy. While safe and effective, these approaches require prosthetic or biologic material without the potential for growth, or normal endothelial and physiologic elastic function. This retrospective study reviews the use of a novel technique, ascending sliding arch aortoplasty, that utilizes viable autologous tissue for repair of arch obstruction in children beyond infancy., Methods: Between April 2002 and January 2007, 8 patients ranging in age from 18 months to 15 years underwent repair of CoA with arch hypoplasia using ascending sliding arch aortoplasty. All patients were approached through median sternotomy, utilizing cardiopulmonary bypass and selective antegrade cerebral perfusion., Results: There was no mortality or major morbidity. One toddler had pneumonia, resulting in an increased length of stay. Median duration of hospitalization was 5.8 days, ranging from 3 to 10 days. No patient had evidence of residual obstruction or recurrent CoA at a mean follow-up interval of 36 months., Conclusions: Ascending sliding arch aortoplasty for CoA with arch obstruction in children beyond infancy is a safe technique that can be accomplished without deep hypothermic circulatory arrest. There is no evidence of recurrence at midterm follow-up. Because the augmentation is accomplished with viable autologous aortic tissue, the potential for growth, preserved elasticity and endothelial function, and resistance to infection make this method attractive for use in the young., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Lung retransplantation (re-LTx) in children has been associated with lower survival rates compared with primary lung transplantation. However, improving survival for primary LTx has led to more patients presenting for re-LTx. Therefore, an analysis of the UNOS (United Network of Organ Sharing) database to evaluate the effectiveness of pediatric lung retransplantation in the United States was completed., Methods: The UNOS registry was queried for pediatric re-LTx patients from May 1988 to May 2008. There were 81 (10%) re-LTx out of a total 802 pediatric lung transplants., Results: Median age and weight at re-LTx were 14 (range, 0 to 18) years and 32 (4 to 58) kg. Indications for re-LTx were obliterative bronchiolitis in 50 patients (62%), primary graft failure in 8 (10%), and other in 23 (28%). The Kaplan-Meier graft survival for re-LTx patients was worse than for primary transplant patients (p < 0.001, graft half-life 0.9 vs 4.0 years), especially if re-LTx was done less than 1 year after primary transplant (graft half-life 0.25 years). Graft survival in patients who underwent re-LTx greater than 1 year after primary transplant was not statistically different than for primary LTx patients (p = 0.21; graft half-life 2.8 vs 4.0 years), and if re-LTx greater than 1 year posttransplant occurred in patients who were not ventilator dependent, survival was further improved (p = 0.68; graft half-life 4.7 vs 4.0 years)., Conclusions: Pediatric lung retransplantation within the first year after primary transplant does not appear advisable. Pediatric re-LTx greater than 1 year after primary transplantation may be a reasonable strategy for end-stage graft failure. Patients greater than 1 year posttransplant and not ventilator dependent appear an even more compelling group in which to consider lung retransplantation., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Recently, there has been a surge of interest by clinicians, industry, and the government in the development and use of ventricular assist devices (VAD) in children. Despite this rapidly expanding interest, the incidence of VAD use in children across the United States is unknown. The Healthcare Cost and Utilization Project (H-CUP) Kids' Inpatients Database (KID) was analyzed to characterize the current utilization of VADs in children nationwide., Methods: The most recent HCUP-KID (2006) was analyzed (n = 7.5 million). This database is a nationwide sampling of hospital discharges of patients less than 20 years old weighted to provide national estimates., Results: In 2006, 187 children had a VAD implanted in the United States. Mean age was 13 ± 7 years. Forty patients (21%) were bridged to VAD by extracorporeal membrane oxygenation. Forty-nine patients (26%) were bridged to heart transplant. Fifty-six patients (30%) died in hospital. Eighty-six patients (46%) were discharged or transferred. Length of stay was 29 days (range, 0 to 285). Total cost was $174,743 (range, $4,230 to $1,911,588). Sixty-seven hospitals placed VADs; 66% of VADs (124) were implanted at large teaching hospitals (more than 500 beds), and 46% (85) were at high-volume hospitals (more than 5 VADs per year). High-volume, large teaching hospitals (10) had better survival (89% versus 61%; p < 0.001) and lower hospital cost ($236,000 ± $184,000 versus $300,000 ± $355,000; p = 0.002) compared with all other hospitals. On multivariate analysis, acute renal failure and extracorporeal membrane oxygenation were risk factors for mortality, whereas transplant and being at a high-volume large teaching hospital were protective factors from death., Conclusions: Preliminary data suggest that the growing use of VADs in children may be better served in regard to resource utilization and outcomes if centralized to high-volume large teaching hospitals., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Ventricular septal defect (VSD) is the most commonly recognized congenital heart defect. With the development of device closure for intracardiac defects, we sought to evaluate current expectations for surgical closure of isolated VSD., Methods: Between January 1, 2000, and December 31, 2006, 215 patients underwent isolated VSD repair at a median age of 10 months (range, 20 days to 18 years) and a median weight of 7 kg (range, 2 to 66 kg). The following VSD types were found: 172 perimembranous (80%), 28 supracristal (13%), 6 inlet (3%), and 9 muscular (4%). One hundred eight patients (50%) had evidence of congestive heart failure or failure to thrive preoperatively. Thirty-one patients (14%) had aortic valve cusp prolapse, and 63 (29%) had genetic abnormalities., Results: Incidence of significant postoperative complications was extremely low. No patient underwent reoperation for a residual VSD. None had complete heart block. One operative mortality (0.5%) and 2 late deaths (0.9%) occurred. Median postoperative hospital length of stay was 5 days (range, 2 to 187 days). In the immediate postoperative period, 6 patients (2.8%) required reoperation. No patients were discharged on antiarrhythmic agents, had complete heart block, or required permanent pacing. At mean follow-up of 2.1 +/- 2.0 years, 99.5% (211 of 212) of patients were asymptomatic from a cardiac standpoint. None exhibited greater than mild new-onset tricuspid valve regurgitation. No aortic valve injuries occurred., Conclusions: Surgical closure of isolated VSD is a safe, effective therapy. Risk of death, complete heart block, and reoperation is minimal. As new technologies for VSD closure evolve, results such as these should be considered when evaluating patients, choosing therapeutic options, and counseling families., (2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The risk of repeat sternotomy (RS) is often taken into account when making clinical management decisions. Current literature on RS suggests a risk of approximately 5% to 10% for major morbidity. We sought to establish the true risk of RS in a contemporary pediatric series., Methods: All RS between October 2002 and August 2006 were analyzed (602 RS in 558 patients). Median age was 3.6 years (range, 0.1 to 45.1); weight, 14.2 kg (2.0 to 112.2). Operations performed at RS were Glenn 22% (131), Fontan 21% (129), aortic valve repair/replacement 12% (72), right ventricle-pulmonary artery conduit 11% (67), Rastelli 7% (39), heart transplant 5% (31), and other 22% (133). Forty-seven percent of patients (280) had single-ventricle physiology. Incidence of second sternotomy was 67% (406), third 28% (166), fourth 4% (24), fifth 0.8% (5), and sixth 0.2% (1). A major injury upon RS was defined as one causing hemodynamic instability requiring vasopressor support or emergent transfusion; femoral cannulation or emergent cardiopulmonary bypass; and any morbidity. A minor injury is any other injury during RS., Results: The incidence of a major injury was not different between RS (0.3%; 2 of 602) and first-time sternotomy (0%; 0 of 1,274; p > 0.1). Incidence of a minor injury was 0.66% (4 of 602). No injury resulted in hemodynamic instability, neurologic injury, or death. Two patients (0.3%) required a nonemergent blood transfusion secondary to injury. (Nonemergent was defined as adminstration rate of less than 0.2 cc/kg/min and less than 10 cc/kg in total.) Femoral cannulation was performed in 4 of 602 RS cases (< 0.6%). Sternal wound infection was 0.5% (3 of 602); reoperation for postoperative bleeding was 1% (8 of 602). Median intensive care unit stay was 3 days (1 to 174); median hospital stay was 7 days (1 to 202). Hospital survival was 98%., Conclusions: Repeat sternotomy can represent a negligible risk of injury and of subsequent morbidity or mortality. Therefore, the choice of management strategies for patients should not be affected by the need for RS.

We describe a female infant with complex single ventricle physiology who had undergone median sternotomy for placement of a right-sided systemic-to-pulmonary artery shunt, division of a patent ductus arteriosus, and left pulmonary artery augmentation. Her early postoperative course was complicated by cardiac arrest requiring institution of extracorporeal membrane oxygenation (ECMO) support. The brain natriuretic peptide (BNP) levels acutely improved after left ventricular decompression by insertion of a left atrial cannula. In this setting BNP levels may be an indicator of left ventricular stretch and are potentially a useful index to monitor left ventricular distension.