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Background Despite excellent operative survival, correction of tetralogy of Fallot frequently is accompanied by residual lesions that may affect health beyond the incident hospitalization. Measuring resource utilization, specifically cost and length of stay, provides an integrated measure of morbidity not appreciable in traditional outcomes. Methods and Results We conducted a retrospective cohort study, using de‐identified commercial insurance claims data, of 269 children who underwent operative correction of tetralogy of Fallot from January 2004 to September 2015 with ≥2 years of continuous follow‐up (1) to describe resource utilization for the incident hospitalization and subsequent 2 years, (2) to determine whether prolonged length of stay (>7 days) in the incident hospitalization was associated with increased subsequent resource utilization, and (3) to explore whether there was regional variation in resource utilization with both direct comparisons and multivariable models adjusting for known covariates. Subjects with prolonged incident hospitalization length of stay demonstrated greater resource utilization (total cost as well as counts of outpatient visits, hospitalizations, and catheterizations) after hospital discharge ( P Conclusions This study is the first to report short‐ and medium‐term resource utilization following tetralogy of Fallot operative correction. It also demonstrates that prolonged length of stay in the initial hospitalization is associated with increased subsequent resource utilization. This should motivate research to determine whether these differences are because of modifiable factors.

Background The surgical strategy for neonates with tetralogy of Fallot (TOF) consists of complete or staged repair. Assessing the comparative effectiveness of these approaches is facilitated by a large multicenter cohort. We propose a novel process for cohort assembly using the Pediatric Health Information System (PHIS), an administrative database that contains clinical and billing data for inpatient and emergency department stays from tertiary children's hospitals. Methods A 4-step process was used to identify neonates with TOF: (1) screen neonates in PHIS with International Classification of Diseases-9 (ICD-9) diagnosis or procedure codes for TOF; (2) include patients with TOF procedures before 30 days of age; (3) exclude patients with missing 2-year follow-up data; (4) analyze patients' 2-year surgery sequence patterns, exclude patients inconsistent with a treatment strategy for TOF, and designate patients as complete or staged repair. Manual chart review at 1 PHIS center was performed to validate this process. Results Between January 2004 and March 2015, 5862 patients were identified in step 1. Step 2 of cohort assembly excluded 3425 patients (58%); step 3 excluded 148 patients (3%); and step 4 excluded 54 patients (1%). The final cohort consisted of 2235 neonates with TOF from 45 hospitals. Manual chart review of 336 patients showed a positive predictive value for accurate PHIS identification of 44% after step 1 and 97% after step 4. Conclusions This systematic cohort identification algorithm resulted in a high positive predictive value to appropriately categorize patients. This carefully assembled cohort offers a unique opportunity for future studies in neonatal TOF outcomes.

Background There is ongoing debate about the best strategy to treat patients with tetralogy of Fallot who are symptomatic in the neonatal period. Objectives The aim of this study was to compare the outcomes of complete versus staged surgery (i.e., initial palliative procedure for possible later complete repair). Methods A retrospective cohort study was performed using the Pediatric Health Information System database, including patients who underwent complete or staged tetralogy of Fallot repair prior to 30 days of age. The primary outcome was death during 2-year follow-up after the initial procedure. Inverse probability–weighted Cox and logistic regression models were used to examine the association between surgical approach group and mortality while accounting for patient- and hospital-level factors. Causal mediation analyses examined the role of intermediate variables. Results A total of 2,363 patients were included (1,032 complete and 1,331 staged). There were 239 deaths. Complete neonatal repair was associated with a significantly higher risk for mortality during the 2-year follow-up period (hazard ratio: 1.51; 95% confidence interval: 1.05 to 2.06), between 7 and 30 days after the initial procedure (hazard ratio: 2.29; 95% confidence interval: 1.18 to 4.41), and during the initial hospital admission (odds ratio: 1.72; 95% confidence interval: 1.15 to 2.62). Post-operative cardiac complications were more common in the complete repair group and mediated the differences in 30-day and 2-year mortality. Conclusions Complete surgical repair for neonates with tetralogy of Fallot is associated with a significantly higher risk for early and 2-year mortality compared with the staged approach, after accounting for patient and hospital characteristics. Post-operative cardiac complications mediated these findings.

Vascular rings occur due to some perturbation in the normal sequence of development and selective involution of paired aortic arches to form a definitive left or right aortic arch. The two most common types of vascular rings are double aortic arch (DAA) and right aortic arch with an aberrant left subclavian artery and a left patent ductus arteriosus or ligamentum arteriosum (RAA-ALS) (1,2). The prevalence of vascular rings is unknown because many patients remain asymptomatic and are undiagnosed.

Background Adolescents often fare poorly after heart transplantation. However, whether the effect of age varies according to the etiology of heart failure is unknown. We tested the hypothesis that age-related heart transplantation outcomes are different in patients with myocarditis and congenital heart disease (CHD). Methods A retrospective analysis of the United Network of Organ Sharing database was performed for patients with myocarditis ( n = 709) and CHD ( n = 1,631) undergoing heart transplantation from 1987 to 2011. The effect of age on graft survival was assessed. Age was categorized as children (6–12 years), adolescents (13–18 years), younger adults (19–30 years), and older adults (31–50 years). Results For myocarditis, the median graft survival for adolescents was 6.9 years (95% confidence interval [CI], 5.6–9.6), which was significantly lower than other age groups (children: 14.1 [95% CI, 9.8–10.9] years, p = 0.004; younger adults: 11.8 [95% CI, 8.3–15.2] years, p = 0.172; older adults: 12.0 years [95% CI, 10.0–14.3 years], p = 0.033). For CHD, the median graft survival for adolescents was 7.4 years (95% CI, 6.8–8.6), which was not significantly different from other age groups (children: 9.0 [95% CI, 7.9–11.0] years, p = 0.737; younger adults: 11.2 [95% CI, 8.6–13.3] years, p = 0.744; older adults: 11.6 [95% CI, 9.2–15.3] years, p = 0.608). Multivariable analysis showed adolescent age was independently associated with worse graft survival for patients with myocarditis but not for CHD. Conclusions Adolescents with myocarditis have significantly worse graft survival after heart transplantation, but adolescents with CHD have similar outcomes to other patients with CHD. Further study is needed to improve outcomes in this vulnerable population.

Background Post-operative chylothorax in patients with congenital heart disease is a challenging problem with substantial morbidity and mortality. Currently, the etiology of chylothorax is poorly understood and treatment options are limited. Objectives This study aimed to report lymphatic imaging findings, determine the mechanism of chylothorax after cardiac surgery, and analyze the outcomes of lymphatic embolization. Methods We conducted a retrospective review of 25 patients with congenital heart disease and post-operative chylothorax who presented for lymphatic imaging and intervention between July 2012 and August 2016. Results Based on dynamic contrast-enhanced magnetic resonance lymphangiography and intranodal lymphangiography, we identified 3 distinct etiologies of chylothorax: 2 patients (8%) with traumatic leak from a thoracic duct (TD) branch, 14 patients (56%) with pulmonary lymphatic perfusion syndrome (PLPS), and 9 patients (36%) with central lymphatic flow disorder (CLFD), the latter defined as abnormal central lymphatic flow, effusions in more than 1 compartment, and dermal backflow. Patients with traumatic leak and PLPS were combined into 1 group of 16 patients without CLFD, of whom 14 (88%) had an intact TD. Sixteen patients underwent lymphatic intervention, including complete TD embolization. All 16 patients had resolution of chylothorax, with a median of 7.5 days from intervention to chest tube removal and 15 days from intervention to discharge. The 9 patients with CLFD were considered a separate group, of whom 3 (33%) had an intact TD. Seven patients underwent lymphatic intervention but none survived. Conclusions Most patients in this study had nontraumatic chylothorax and dynamic contrast-enhanced magnetic resonance lymphangiography was essential to determine etiology. Lymphatic embolization was successful in patients with traumatic leak and PLPS and, thus, should be considered first-line treatment. Interventions in patients with CLFD were not successful to resolve chylothorax and alternate approaches need to be developed.

Background Heart retransplant ( HRT ) recipients represent a growing number of transplant patients. The impact of concurrent kidney transplants ( KTs ) in this population has not been well studied. We tested the hypothesis that recipients of HRT with concurrent KT ( HRT ‐ KT ) would have worse survival than recipients of HRT alone. Methods and Results A retrospective analysis of the United Network of Organ Sharing database was performed for all patients undergoing HRT from 1987 to 2011. There were 1660 HRT patients, of which 116 (7%) received concurrent KT . Those who received HRT ‐ KT had older age, longer wait‐list time, worse kidney function, and more known diabetes. Survival among recipients of HRT ‐ KT was significantly better than that of recipients of HRT alone ( P =0.005). A subgroup of 323 HRT patients with severe kidney dysfunction (estimated glomerular filtration rate 2 or on dialysis) was studied in more detail, and 76 (24%) received concurrent KT . Those on dialysis at the time of HRT had better survival with versus without concurrent KT ( P KT was independently associated with better outcomes for all patients with HRT and for the subgroup of patients with severe kidney dysfunction. Conclusions Recipients of HRT ‐ KT have better survival than recipients of HRT alone. Further research is needed to determine which HRT patients may benefit the most from concurrent KT .

s S225 (34-98%) derived from catheterizations. 31% (8-89%) of the total exposure occurred during the transplant admission, 59% (11-88%) during planned and 3% (0-56%) during unplanned follow-up testing. Age at transplant was a significant risk factor for increased radiation exposure, with older patients receiving higher dose (p= 0.006). When adjusted for age, congenital heart disease as indication for transplant (p= 0.08), pre-sensitization (p= 0.12), and a positive crossmatch (p= 0.09) showed a trend toward increased exposure. Conclusion: At our center, pediatric heart transplant patients are exposed to significant amounts of ionizing radiation over the first post-transplant year, the majority of which occurs during scheduled catheterization. As survival improves, it is important to consider the potential long term risks associated with these levels of exposure.

Purpose Adolescents often fare poorly after heart transplantation (HT) compared to other age groups. However, it is unknown if the impact of age varies across diseases leading to HT. We tested the hypothesis that age-related HT outcomes are different in patients with congenital heart disease (CHD) vs myocarditis. Methods and Materials A retrospective analysis of the United Network of Organ Sharing database was performed for patients with myocarditis (n=709) and CHD (n=1,631) undergoing HT from 1987-2011, to assess the effect of age on graft survival. Age was categorized as children (6-12 years), adolescents (13-18 years), younger adults (19-30 years), and older adults (31-50 years). Results Adolescents comprised 28% of CHD patients and 14% of myocarditis patients (p Conclusions Adolescents with myocarditis have significantly worse graft survival after HT, but adolescents with CHD have similar outcomes to other patients with CHD. Further study is needed to improve outcomes in this vulnerable population.