Single versus multiple arterial coronary artery bypass grafting in men and women: results from Polish National Registry of Cardiac Surgery Procedures.

BACKGROUND: The objective of this multicenter study aimed to investigate the impact of sex on long-term survival among patients with multivessel coronary artery disease undergoing coronary artery bypass grafting (CABG) using multiple arterial grafting (MAG) or a single artery with saphenous vein grafts. MATERIALS AND METHODS: Data were obtained from the Polish National Registry of Cardiac Surgery Procedures database. This study included 81 136 patients who underwent CABG for multivessel disease between January 2012 and December 2020 (22.9 were women and 77.1% were men). MAG was performed in 8.3 and 11.7% of female and male patients, respectively. A 1:1 propensity score (PS)-matching was performed. Long-term mortality was compared between matched groups of men and women. Subgroup analyses of patients aged <70 and ≥70 years, with an ejection fraction (EF) >40% and ≤40%, and with and without diabetes, obesity, peripheral artery disease (PAD), or chronic lung disease (CLD) were performed separately in women and men. RESULTS: MAG was associated with lower long-term mortality than saphenous vein grafts in 1528 PS-matched female pairs [hazard ratio (HR): 0.74; 95% CI: 0.59-0.92; P =0.007) and 7283 PS-matched male pairs (HR: 0.80; 95% CI: 0.72-0.88; P <0.001). Subgroup analyses confirmed the results among female patients aged <70 years, with diabetes and EF >40%, and without PAD or CLD, and of male patients aged <70 and ≥70 years; with EF >40%; with or without diabetes, obesity, or PAD; and without CLD. CONCLUSIONS: In patients undergoing CABG, MAG was associated with significantly improved survival in both sexes. The long-term benefits of MAG observed across subgroups of men and women support the consideration of a multiarterial revascularization strategy for a broader spectrum of patients.

Surgical ablation, left atrial appendage occlusion or both? Nationwide registry analysis of cardiac surgery patients with underlying atrial fibrillation.

OBJECTIVES: The aim of this study was to evaluate in-hospital outcomes and long-term survival of patients undergoing cardiac surgery with preoperative atrial fibrillation (AF). We compared different strategies, including no-AF treatment, left atrial appendage occlusion (LAAO) alone, concomitant surgical ablation (SA) alone or both. METHODS: A retrospective analysis using the KROK registry included all patients with preoperative diagnosis of AF who underwent cardiac surgery in Poland between between January 2012 and December 2022. Risk adjustment was performed using regression analysis with inverse probability weighting of propensity scores. We assessed 6-year survival with Cox proportional hazards models. Sensitivity analysis was performed based on index cardiac procedure. RESULTS: Initially, 42 510 patients with preoperative AF were identified, and, after exclusion, 33 949 included in the final analysis. A total of 1107 (3.26%) received both SA and LAAO, 1484 (4.37%) received LAAO alone, 3921 (11.55%) SA alone and the remaining 27 437 (80.82%) had no AF-directed treatment. As compared to no treatment, all strategies were associated with survival benefit over 6-year follow-up. A gradient of treatment was observed with the highest benefit associated with SA + LAAO followed by SA alone and LAAO alone (log-rank P < 0.001). Mortality benefits were reflected when stratified by surgery type with the exception of aortic valve surgery where LAAO alone fare worse than no treatment. CONCLUSIONS: Among patients with preoperative AF undergoing cardiac surgery, surgical management of AF, particularly SA + LAAO, was associated with lower 6-year mortality. These findings support the benefits of incorporating SA and LAAO in the management of AF during cardiac surgery.

Validation of EuroSCORE II in atrial fibrillation heart surgery patients from the KROK Registry.

The study aimed to validate the European System for Cardiac Operative Risk Evaluation score (EuroSCORE II) in patients with atrial fibrillation (AF). All data were retrieved from the National Registry of Cardiac Surgery Procedures (KROK). EuroSCORE II calibration and discrimination performance was evaluated. The final cohort consisted of 44,172 patients (median age 67, 30.8% female, 13.4% with AF). The in-hospital mortality rate was 4.14% (N = 1830), and 5.21% (N = 2303) for 30-day mortality. EuroSCORE II significantly underestimated mortality in mild- and moderate-risk populations [Observed (O):Expected (E)-1.1, 1.16). In the AF subgroup, it performed well [O:E-0.99), whereas in the very high-risk population overestimated mortality (O:E-0.9). EuroSCORE II showed better discrimination in AF (-) [area under curve (AUC) 0.805, 95% CI 0.793-0.817)] than in AF (+) population (AUC 0.791, 95%CI 0.767-0.816), P < 0.001. The worst discriminative performance for the AF (+) group was for coronary artery bypass grafting (CABG) (AUC 0.746, 95% CI 0.676-0.817) as compared with AF (-) population (AUC 0.798, 95% CI 0.774-0.822), P < 0.001. EuroSCORE II is more accurate for patients with AF. However, it underestimated mortality rates for low-to-moderate-risk patients and had a lower ability to distinguish between high- and low-risk patients with AF, particularly in those undergoing coronary artery bypass grafting.

Comparison of Repair vs Replacement in Calcific and Rheumatic Mitral Disease.

BACKGROUND: The benefit of repair over replacement of rheumatic or calcified mitral valve (MV) is debatable. METHODS: Patients who underwent MV repair or replacement for rheumatic or calcified MV disease between 2006 and 2020 were identified in the Polish National Registry of Cardiac Surgery Procedures. Patients who underwent additional procedures other than coronary artery bypass grafting or tricuspid valve surgery, as well as redo or emergency cases, were excluded. The long-term survival was verified based on National Health Fund registry data. The survival was compared between MV repair and replacement in the whole cohort and after propensity score matching. RESULTS: We included 4338 patients: 1859 (43%) with pure mitral regurgitation and 2479 (57%) with mitral stenosis. MV was repaired in 543 patients (29%) with pure regurgitation and 126 (5.1%) with stenosis (P < .001). In total, 984 (23%) patients underwent concomitant coronary artery bypass grafting and 1358 (32%) tricuspid valve surgery. MV repair improved survival (hazard ratio 0.81; 95% CI 0.68-0.97; P = .022) in patients with no mitral stenosis, and had no effect in mitral stenosis (hazard ratio 1.17; 95% CI 0.85-1.59; P = .332). The results were confirmed in propensity-matched cohorts. The freedom from MV reoperation at 10 years was 95.5% ± 1.2% after repair and 96.0% ± 0.7% after MV replacement (P = .416) in the absence of stenosis and 91.8% ± 3.4% after repair vs 95.9% ± 0.5% after replacement in patients with mitral stenosis (P = .065). CONCLUSIONS: Repair of rheumatic/calcified mitral valve should be a preferred option in patients with no mitral stenosis, but confers no benefit if mitral stenosis is present.

Repair or Replacement for Secondary Mitral Regurgitation: Results From Polish National Registry.

BACKGROUND: The optimal surgical strategy (repair vs replacement) for patients with secondary mitral (MV) regurgitation is questionable. METHODS: Patients who underwent MV repair or replacement for functional or ischemic mitral regurgitation between 2006 and 2017 were identified in Polish National Registry of Cardiac Surgery Procedures. Patients, who underwent additional procedures other than coronary artery bypass grafting or tricuspid valve surgery, as well as redo or emergency cases, were excluded. The long-term survival was verified based on National Health Fund registry. The survival was compared between MV repair and replacement both in the whole cohort and after propensity score matching. The Cox regression was used to seek for independent predictors of survival. RESULTS: Of 7633 identified patients, 1793 (23%) underwent MV replacement and 5840 (77%) underwent MV repair. Coronary artery bypass surgery was performed together with MV repair in 3992 (69%) patients and together with MV replacement in 915 (52%) patients (P < .001). Tricuspid valve surgery was added to 1393 (24%) MV repairs and to 561 (32%) MV replacements (P < .001). The crude actuarial 5-year survival was 71% (95% confidence interval [CI], 70%-72%) in the repair group and 66% (95% CI, 63%-68%) in the replacement group (P < .001). MV replacement was an independent predictor of mortality (hazard ratio, 1.32; 95% CI, 1.17-1.49) (P < .001) in Cox regression modeling. In the propensity-matched cohort (1105 pairs), the long-term mortality was also significantly higher in the replacement group (hazard ratio, 1.24; 95% CI, 1.06-1.45; P = .008). CONCLUSIONS: Repair of secondary mitral regurgitation has an associated survival benefit compared with MV replacement.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

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.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

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.

Early Impact of the COVID-19 Pandemic on Congenital Heart Surgery Programs Across the World: Assessment by a Global Multi-Societal Consortium.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

Long-Term Survival Following Surgical Ablation for Atrial Fibrillation Concomitant to Isolated and Combined Coronary Artery Bypass Surgery-Analysis from the Polish National Registry of Cardiac Surgery Procedures (KROK).

The current investigation aimed to evaluate long-term survival in patients undergoing isolated and combined coronary artery bypass grafting (CABG) with concomitant surgical ablation for atrial fibrillation (AF). Procedural data from KROK (Polish National Registry of Cardiac Surgery Procedures) were retrospectively collected. Eleven thousand three hundred sixteen patients with baseline AF (72.4% men, mean age 69.6 ± 7.9) undergoing isolated and combined CABG surgery between 2006-2019 in 37 reference centers across Poland and included in the registry were analyzed. The median follow-up was four years (3.7 IQR 1.3-6.8). Over a 12-year study period, there was a significant survival benefit (Hazard Ratio (HR) 0.83; (95% Confidence Interval (CI): 0.73-0.95); p = 0.005) with concomitant ablation as compared to no concomitant ablation. After rigorous propensity matching (LOGIT model, 432 pairs), concomitant surgical ablation was associated with over 25% improved survival in the overall analysis: HR 0.74; (95% CIs: 0.56-0.98); p = 0.036. The benefit of concomitant ablation was maintained in the subgroups, yet the most benefit was appraised in low-risk patients (EuroSCORE < 2, p = 0.003) with the three-vessel disease (p < 0.001) and without other comorbidities. Ablation was further associated with significantly improved survival in patients undergoing CABG with mitral valve surgery (HR 0.62; (95% CIs: 0.52-0.74); p < 0.001) and in patients in whom complete revascularization was not achieved: HR 0.43; (95% CIs: 0.24-0.79); p = 0.006.

Surgical ablation for atrial fibrillation during isolated coronary artery bypass surgery.

OBJECTIVES: Our goal was to evaluate early sequelae and long-term survival in patients undergoing isolated coronary artery bypass grafting (CABG) with concomitant surgical ablation for atrial fibrillation (AF). METHODS: Procedural data from KROK (Polish National Registry of Cardiac Surgery Procedures) were collected. A total of 7879 patients with underlying AF underwent isolated CABG between 2006 and 2018 in 37 reference centres across Poland. The mean follow-up was 4.7 ± 3.5 years [median (interquartile range) 4.3 (1.7-7.4)]. Propensity score matching and Cox proportional hazards models were used to compare isolated CABG + ablation with isolated CABG. RESULTS: Of the included patients, 346 (4.39%) underwent surgical ablation. Patients in this group were significantly younger (66.4 ± 7.5 vs 69.2 ± 8.2; P < 0.001) but had a non-significant, different baseline surgical risk (EuroSCORE: 2.11 vs 2.50; P = 0.088). After a rigorous 1:3 propensity matching (LOGIT model: 306 cases of isolated CABG + ablation vs 918 of isolated CABG alone), surgical ablation was associated with a lower 30-day risk of death [risk ratio 0.37, 95% confidence interval (CI) 0.15-0.91; P = 0.032] and multiorgan failure (risk ratio 0.29, 95% CI 0.10-0.94; P = 0.029). In the long term, surgical ablation was associated with a significant 33% improved overall survival rate: hazard ratio 0.67, 95% CI 0.49-0.90; P = 0.008. The benefit of ablation was sustained in the subgroups but was most pronounced in lower risk older patients (age >70 years, P = 0.020; elective status, P = 0.011) with 3-vessel disease (P = 0.036), history of a cerebrovascular accident (P = 0.018) and preserved left ventricular function [left ventricular ejection fraction >50%; P = 0.017; no signs of heart failure (per New York Heart Association functional class); P = 0.001] and those undergoing on-pump CABG (P < 0.001). CONCLUSION: Surgical ablation for AF in patients undergoing isolated CABG is safe and associated with significantly improved long-term survival.

Outcome Prediction After Coronary Surgery and Redo Surgery for Bleeding (From the KROK Registry).

OBJECTIVES: To assess the reliability of EuroSCORE II in an entire population after isolated coronary artery surgery and separately among patients who underwent redo surgery due to bleeding, and to create a model predicting hospital death among patients who underwent redo surgery owing to bleeding. DESIGN: Retrospective study based on data from the Polish National Registry of Cardiac Surgical Procedures. SETTING: Multi-institutional study. PARTICIPANTS: The study comprised 41,353 patients who underwent isolated coronary artery surgery in Poland between January 2012 and December 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: EuroSCORE II reliability was estimated using the area under the receiver operating characteristics curve (AUC), the observed-to-expected surgical mortality ratio (O/E), and the Hosmer-Lemeshow test. Parameters of the function correcting the original EuroSCORE II were determined using the least squares method. The original score was adjusted using a created formula. Among the 41,353 patients, 1,406 (3.4%) underwent reexploration. Even though EuroSCORE II was reliable in predicting hospital mortality in the entire population (AUC 0.76, O/E ratio 1.08), it greatly underestimated mortality for patients who required reexploration (AUC 0.74, O/E ratio 4.33). In this subpopulation, the worst performance of the EuroSCORE II was noted among patients with the lowest predicted mortality (0.50%-0.82%) Accurate calibration was obtained by adding a coefficient and creating a nomogram. CONCLUSIONS: EuroSCORE II was reliable in a Polish population undergoing isolated coronary surgery. After redo surgery for bleeding, the observed mortality was much higher than in the overall coronary population, but the rate was made more accurate by adding a coefficient to the initially calculated EuroSCORE II.

In-hospital and mid-term outcomes in patients reoperated on due to bleeding following coronary artery surgery (from the KROK Registry).

OBJECTIVES: Surgical re-exploration due to postoperative bleeding that follows coronary artery surgery is associated with significant morbidity and mortality. The aim of this study was to assess a relationship between re-exploration, major postoperative complications, in-hospital mortality and mid-term outcomes in patients following coronary surgery, on the basis of nationwide registry data. METHODS: We identified all consecutive patients enrolled in Polish National Registry of Cardiac Surgical Procedures (KROK Registry) who underwent isolated coronary surgery between January 2012 and December 2014. Preoperative data, major postoperative complications, hospital mortality and mid-term all-cause mortality were, respectively, analysed. Comparisons were performed in all patients, low-risk patients (EuroSCORE II < 2%, males, aged 60-70 years) and propensity-matched patients. The starting point for follow-up was the date of hospital discharge. RESULTS: Among 41 353 analysed patients, 1406 (3.4%) underwent re-exploration. Reoperated patients had more comorbidities, more frequent major postoperative complications, higher in-hospital mortality (13.2% vs 1.8%, P < 0.001) and higher mid-term mortality in survivors (P < 0.001). In the low-risk population, 3.0% of patients underwent re-exploration. Reoperated low-risk patients and propensity-matched patients also had more frequent major postoperative complications and higher in-hospital mortality, but mid-term mortality in survivors was similar. In a multivariable analysis, re-exploration was an independent predictor of death and all major postoperative complications. CONCLUSIONS: Surgical re-exploration due to postoperative bleeding following coronary artery surgery carries a high risk of perioperative mortality and is linked to major postoperative complications. Among patients who survive to hospital discharge, mid-term mortality is associated primarily with preoperative comorbidities.

Survival after surgical ablation for atrial fibrillation in mitral valve surgery: Analysis from the Polish National Registry of Cardiac Surgery Procedures (KROK).

OBJECTIVES: Surgical ablation for atrial fibrillation (AF) performed at the time of other valvular- or nonvalvular cardiac procedure is a mainstay of therapy; yet the data regarding its influence on remote survival are sparse. We aimed to evaluate late survival in patients undergoing mitral valve (MV) surgery with concomitant surgical ablation for AF. METHODS: Procedure-related data from the Polish National Registry of Cardiac Surgery Procedures (Krajowy Rejestr Operacji Kardiochirurgicznych) were retrospectively collected. A total of 11,381 patients with baseline AF (46.6% men; mean age 65.6 ± 9.0 years) undergoing MV surgery between 2006 and 2017 in 37 reference centers across Poland and included in the registry were analyzed. Median follow-up was 5 years (mean, 4.6 years; interquartile range, 1.9-7.9 years). Cox proportional hazards models were used for computations. Propensity score matching for the comparison of MV + ablation versus MV alone was performed. RESULTS: Of included patients, 2449 (21.5%) underwent surgical ablation for AF. Patients in this group were significantly younger (63.8 ± 8.7 years vs 66.1 ± 9.0 years; P < .001) and were at lower baseline surgical risk (EuroSCORE, 2.86 vs 3.69; P < .001). During the 12-year study period, there was a significant survival benefit (hazard ratio, 0.71; 95% confidence interval, 0.63-0.79; P < .001) for MV + ablation compared with MV alone. After rigorous propensity matching (logit model, 1784 pairs) surgical ablation was associated with nearly 20% improved survival (hazard ratio, 0.82; 95% confidence interval, 0.70-0.96; P = .011). Benefit of surgical ablation was maintained in subgroup analyses, yet most benefit was appraised in low-risk patients such as those with EuroSCORE of 2 to 5 or age < 50 years. CONCLUSIONS: Concomitant surgical ablation for AF in patients undergoing mitral valve procedures is safe, feasible, and significantly improves late survival.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11).

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many "short list" versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various "short lists". In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the "short list" for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.