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Background The Fontan circulation challenges the lymphatic system. Increasing production of lymphatic fluid and impeding lymphatic return, increased venous pressure may cause lymphatic dilatation and decrease lymphatic contractility. In-vitro studies have reported a lymphatic diameter-tension curve, with increasing passive stretch affecting the intrinsic contractile properties of each thoracic duct segment. We aimed to describe thoracic duct occlusion pressure and asses if thoracic duct dilation impairs contractility in individuals with a Fontan circulation and lymphatic failure. Methods Central venous pressure and thoracic duct measurements were retrospectively collected from 31 individuals with a Fontan circulation. Thoracic duct occlusion pressure was assessed during a period of external manual compression and used as an indicator of lymphatic vessel contractility. Measurements of pressure were correlated with measurements of the thoracic duct diameter in images obtained by dynamic contrast-enhanced MR lymphangiography. Results The average central venous pressure and average pressure of the thoracic duct were 17 mm Hg. During manual occlusion, the thoracic duct pressure significantly increased to 32 mm Hg. The average thoracic duct diameter was 3.3 mm. Thoracic duct diameter correlated closely with the central venous pressure. The rise in pressure following manual occlusion showed an inverse correlation with the diameter of the thoracic duct. Conclusion Higher central venous pressures are associated with increasing diameters of the thoracic duct. When challenged by manual occlusion, dilated thoracic ducts display a decreased ability to increase pressure. Dilatation and a resulting decreased contractility may partly explain the challenged lymphatic system in individuals with a Fontan circulation.

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Introduction: Fetuses with heterotaxy syndrome (HS) and congenital heart disease continue to have worse outcomes compared to other lesions. We sought to explore differences between asplenia and polysplenia subtypes and to identify risk factors for poor outcome in the current era. Methods: All cases of fetal HS seen at our center between 1/2005 and 3/2018 were included in this study. Prenatal echocardiographic parameters and clinical outcomes were recorded. Findings were compared between the asplenia and polysplenia subtypes via Chi-square or Fisher’s exact analysis. Univariate logistic regression was utilized to identify prenatal variables predictive of fetal demise. Postnatal survival was examined with Kaplan-Meier curves and univariate Cox proportional hazards models. Results: The cohort consisted of 155 subjects: 86 with asplenia and 69 with polysplenia. There were 36 with biventricular (2V) and 119 with univentricular (1V) physiology. Asplenia subjects were more likely to have 1V physiology, common atrioventricular canal (CAVC), conotruncal anomaly, and pulmonary venous obstruction (all p Conclusions: Fetuses with HS continue to have high rates of postnatal mortality, particularly if CHB, ventricular dysfunction, or pulmonary venous obstruction are identified. CHB and ventricular dysfunction are also significant risk factors for fetal demise. Further research is needed to improve management of CHB and pulmonary venous obstruction in this high-risk group.

Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies.Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value.The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant atlt;32 weeks with a BW z score of -2 to -1.This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

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 Percutaneous lymphatic intervention (PCL) is a promising new therapy for plastic bronchitis (PB). We characterized bronchoalveolar lavage (BAL) and cast morphology in surgically repaired congenital heart disease (CHD) patients with PB during PCL. We quantified respiratory and bronchoscopic characteristics and correlated them with post-intervention respiratory outcomes. Methods We retrospectively reviewed patients with PB and surgically repaired CHD undergoing PCL and bronchoscopy at our institution. Pre-intervention characteristics, bronchoscopy notes, BAL cell counts, virology, and cultures were collected. A pathologist blinded to clinical data reviewed cast specimens. Respiratory outcomes were evaluated through standardized telephone questionnaire. Results Sixty-two patients were included with a median follow-up of 20 months. No patients experienced airway bleeding, obstruction, or prolonged intubation related to bronchoscopy. Of BAL infectious studies, the positive results were 4 (8%) fungal, 6 (11%) bacterial, and 6 (14%) viral. Median BAL count per 100 cells for neutrophils, lymphocytes, and eosinophils were 13, 10, and 0, respectively. Of 23 bronchial casts analyzed, all contained lymphocytes, and 19 (83%) were proteinaceous, with 14 containing neutrophils and/or eosinophils. Median BAL neutrophil count was greater in patients with proteinaceous neutrophilic or eosinophilic casts compared to casts without neutrophils or lymphocytes (P = 0.030). Post-intervention, there was a significant reduction in respiratory medications and support and casting frequency. Conclusions The predominance of neutrophilic proteinaceous casts and high percentage of positive BAL infectious studies support short-term fibrinolytic and anti-infective therapies in PB in select patients. Flexible bronchoscopy enables safe assessment of cast burden. PCL effectively treats PB and reduces respiratory therapies.

In 2004, the United Network for Organ Sharing (UNOS) added the label "high-risk donor" (HRD) for any organ donor who met the Centers for Disease Control (CDC) criteria for high-risk behavior for infection. The aim of this study was to calculate the rate of HRD graft use in heart transplantation in children and determine the differences in outcome from those who received standard-risk donor (SRD) grafts.We reviewed information from the UNOS database regarding transplants performed between June 30, 2004 and July 31, 2012. Heart transplant recipients18 years old were divided into two groups based on the donor's risk status. Demographic data on donors and recipients were collected. Survival analysis was performed to compare survival based on donor status. We also compared episodes of rejection before hospital discharge and the length of stay after transplantation by donor status.During the study period, 2,782 pediatric heart transplantations were performed and 116 (4.1%) patients received a CDC HRD graft. Recipients of HRD grafts were significantly older and heavier than those who received an SRD graft (8.5 vs 6.5 years, p0.001 and 35.7 vs 26.9 kg, p0.001). There was no difference in patient survival (log rank, p = 0.88) between groups. There was no difference in rejection prior to discharge (17.2 vs 16.4%, p = 0.81) or length of stay after transplantation (26.1 vs 27.6 days, p = 0.58).CDC HRD graft status does not appear to significantly affect recipient outcome after heart transplantation in children.

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.