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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.

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.

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.

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.

Surgical intervention in the setting of cardiogenic shock (CS) is burdened with high mortality. Due to acute condition, detailed diagnoses and risk assessment is often precluded. Atrial fibrillation (AF) is a risk factor for perioperative complications and worse survival but little is known about AF patients operated in CS. Current analysis aimed to determine prognostic impact of preoperative AF in patients undergoing heart surgery in CS. We analyzed data from the Polish National Registry of Cardiac Surgery (KROK) Procedures. Between 2012 and 2021, 332,109 patients underwent cardiac surgery in 37 centers; 4852 (1.5%) patients presented with CS. Of those 624 (13%) patients had AF history. Cox proportional hazards models were used for computations. Propensity score (nearest neighbor) matching for the comparison of patients with and without AF was performed. Median follow-up was 4.6 years (max.10.0), mean age was 62 (± 15) years and 68% patients were men. Thirty-day mortality was 36% (1728 patients). The origin of CS included acute myocardial infarction (1751 patients, 36%), acute aortic dissection (1075 patients, 22%) and valvular dysfunction (610 patients, 13%). In an unadjusted analysis, patients with underlying AF had almost 20% higher mortality risk (HR 1.19, 95% CIs 1.06–1.34; P  = 0.004). Propensity score matching returned 597 pairs with similar baseline characteristics; AF remained a significant prognostic factor for worse survival (HR 1.19, 95% CI 1.00–1.40; P  = 0.045). Among patients with CS referred for cardiac surgery, history of AF was a significant risk factor for mortality. Role of concomitant AF ablation and/or left atrial appendage occlusion or more aggressive perioperative circulatory support should be addressed in the future.

No single randomized study has ever before addressed the safety of On-Pump coronary artery bypass grafting (CABG) vs Off-Pump CABG in the setting of atrial fibrillation (AF) and data from small observational samples remain inconclusive. Procedural data from KROK (Polish National Registry of Cardiac Surgery Procedures) were retrospectively collected. Of initial 188,972 patients undergoing CABG, 7,913 presented with baseline AF (76.0% men, mean age 69.1±8.2) and underwent CABG without concomitant valve surgery between 2006-2019 in 37 reference centers across Poland. Mean follow-up was 4.7±3.5 years (median 4.3 IQR 1.7-7.4). Cox proportional hazards models were used for computations. Of included patients, 3,681 underwent On-Pump- (46.52%) as compared to 4,232 (53.48%) who underwent Off-Pump CABG. Patients in the latter group less frequently were candidates for complete revascularization (P<0.001). In an unadjusted comparison, On-Pump surgery was associated with significantly worse survival at 30 days: HR: 1.28; 95%CIs: (1.07-1.53); P = 0.007. Along the 13-year study period, the trend shifted in favor of On-Pump CABG: HR: 0.92; 95%CIs: (0.83-0.99); P = 0.005. After rigorous propensity matching, 636 pairs were identified. The direction and magnitude of treatment effects was sustained with HRs of 3.58; (95%CIs: 1.34-9.61); p = 0.001 and 0.74; [95%CIs: 0.56-0.98]; p = 0.036) for 30-day and late mortality respectively. Off-Pump CABG offered 30-day survival benefit to patients undergoing CABG surgery and presenting with underlying AF. On-Pump CABG was associated with significantly improved survival at long term.

In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term “heterotaxy”: “Heterotaxy is synonymous with ‘visceral heterotaxy’ and ‘heterotaxy syndrome’. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as ‘situs solitus’, nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as ‘situs inversus’.” “Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements.” The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connections, since these are so often abnormal. The malformations within the heart are then analysed and described separately as for any patient with suspected congenital cardiac disease. The relationship and arrangement of the remaining thoraco-abdominal organs, including the spleen, the lungs, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. One of the clinically important implications of heterotaxy syndrome is that splenic abnormalities are common. Investigation of any patient with the cardiac findings associated with heterotaxy, therefore, should include analysis of splenic morphology. The less than perfect association between the state of the spleen and the form of heart disease implies that splenic morphology should be investigated in all forms of heterotaxy, regardless of the type of cardiac disease. The splenic morphology should not be used to stratify the form of disease within the heart, and the form of cardiac disease should not be used to stratify the state of the spleen. Intestinal malrotation is another frequently associated lesion that must be considered. Some advocate that all patients with heterotaxy, especially those with isomerism of the right atrial appendages or asplenia syndrome, should have a barium study to evaluate for intestinal malrotation, given the associated potential morbidity. The cardiac anatomy and associated cardiac malformations, as well as the relationship and arrangement of the remaining thoraco-abdominal organs, must be described separately. It is only by utilizing this stepwise and logical progression of analysis that it becomes possible to describe correctly, and to classify properly, patients with heterotaxy.

This study aimed to compare perioperative outcomes and long-term mortality between off-pump coronary artery bypass grafting and on-pump coronary artery bypass grafting in patients with ischaemic cardiomyopathy who had a left ventricle ejection fraction of ≤35%. A retrospective cohort analysis was conducted using data from the Polish National Registry of Cardiac Surgery Procedures database, encompassing patients who underwent isolated coronary artery bypass grafting in Poland between 2012 and 2022. Patients were divided into two groups: on-pump and off-pump. Propensity score matching was used to balance the groups. The primary outcome was long-term all-cause mortality following surgical revascularization. A total of 9920 patients were included, with 3116 patients in each group after propensity score matching. The median follow-up period was 4 years. The off-pump group was associated with a lower 30-day mortality rate (6.4% vs 9.1%, P = 0.002) and fewer perioperative complications. However, long-term survival analysis revealed a modest but statistically significant advantage for on-pump group at the 10-year follow-up (P = 0.047). Off-pump provides short-term benefits, including reduced early mortality and fewer complications compared to on-pump technique. However, these advantages do not translate into improved long-term survival, where on-pump demonstrates a slight benefit. The choice between off-pump and on-pump technique should be individualized based on patient-specific factors and surgical expertise.

This position paper, jointly authored by the Polish Society of Cardiothoracic Surgeons and the Heart Rhythm Association of the Polish Society of Cardiology, defines the role of hybrid approaches in the treatment of cardiac arrhythmias, particularly atrial fibrillation. We present a rationale for integrating epicardial and endocardial ablation strategies, summarize current evidence, and advocate for multidisciplinary planning, including arrhythmia treatment during cardiac surgery for other indications and atrial fibrillation treatment as a stand-alone procedure. The paper highlights the importance of minimally invasive approaches, the need for heart team-based planning, structured curricula, and postoperative referral pathways for comprehensive management of atrial fibrillation and non-atrial fibrillation arrhythmias.

Clinicians working in the field of congenital and paediatric cardiology have long felt the need for a common diagnostic and therapeutic nomenclature and coding system with which to classify patients of all ages with congenital and acquired cardiac disease. A cohesive and comprehensive system of nomenclature, suitable for setting a global standard for multicentric analysis of outcomes and stratification of risk, has only recently emerged, namely, The International Paediatric and Congenital Cardiac Code. This review, will give an historical perspective on the development of systems of nomenclature in general, and specifically with respect to the diagnosis and treatment of patients with paediatric and congenital cardiac disease. Finally, current and future efforts to merge such systems into the paperless environment of the electronic health or patient record on a global scale are briefly explored. On October 6, 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. In January, 2005, the International Nomenclature Committee was constituted in Canada as The International Society for Nomenclature of Paediatric and Congenital Heart Disease. This International Society now has three working groups. The Nomenclature Working Group developed The International Paediatric and Congenital Cardiac Code and will continue to maintain, expand, update, and preserve this International Code. It will also provide ready access to the International Code for the global paediatric and congenital cardiology and cardiac surgery communities, related disciplines, the healthcare industry, and governmental agencies, both electronically and in published form. The Definitions Working Group will write definitions for the terms in the International Paediatric and Congenital Cardiac Code, building on the previously published definitions from the Nomenclature Working Group. The Archiving Working Group, also known as The Congenital Heart Archiving Research Team, will link images and videos to the International Paediatric and Congenital Cardiac Code. The images and videos will be acquired from cardiac morphologic specimens and imaging modalities such as echocardiography, angiography, computerized axial tomography and magnetic resonance imaging, as well as intraoperative images and videos. Efforts are ongoing to expand the usage of The International Paediatric and Congenital Cardiac Code to other areas of global healthcare. Collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the representatives of the steering group responsible for the creation of the 11th revision of the International Classification of Diseases, administered by the World Health Organisation. Similar collaborative efforts are underway involving the leadership of The International Nomenclature Committee for Pediatric and Congenital Heart Disease and the International Health Terminology Standards Development Organisation, who are the owners of the Systematized Nomenclature of Medicine or “SNOMED”. The International Paediatric and Congenital Cardiac Code was created by specialists in the field to name and classify paediatric and congenital cardiac disease and its treatment. It is a comprehensive code that can be freely downloaded from the internet (http://www.IPCCC.net) and is already in use worldwide, particularly for international comparisons of outcomes. The goal of this effort is to create strategies for stratification of risk and to improve healthcare for the individual patient. The collaboration with the World Heath Organization, the International Health Terminology Standards Development Organisation, and the healthcare industry, will lead to further enhancement of the International Code, and to its more universal use.