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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.

BACKGROUND Children with single ventricle heart disease have significant morbidity and mortality. The maternal–fetal environment (MFE) may adversely impact outcomes after neonatal cardiac surgery. We hypothesized that impaired MFE would be associated with an increased risk of death after stage 1 Norwood reconstruction. METHODS AND RESULTS We performed a retrospective cohort study of children with hypoplastic left heart syndrome (and anatomic variants) who underwent stage 1 Norwood reconstruction between 2008 and 2018. Impaired MFE was defined as maternal gestational hypertension, preeclampsia, gestational diabetes, and/or smoking during pregnancy. Cox proportional hazards regression models were used to investigate the association between impaired MFE and death while adjusting for confounders. Hospital length of stay was assessed with the competing risk of in‐hospital death. In 273 children, the median age at stage 1 Norwood reconstruction was 4 days (interquartile range [IQR], 3–6 days). A total of 72 children (26%) were exposed to an impaired MFE; they had more preterm births (18% versus 7%) and a greater percentage with low birth weights P P CONCLUSIONS Children exposed to an impaired MFE had a higher risk of death following stage 1 Norwood reconstruction. Prenatal exposures are potentially modifiable factors that can be targeted to improve outcomes after pediatric cardiac surgery.

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.

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 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 .