Haematological and infectious complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the haematological system and to infectious complications. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a near-exhaustive list of haematological and infectious complications. Within each subgroup, complications are presented in alphabetical order. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatmen

Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Europe and China:An Analysis of the European Congenital Heart Surgeons Association Congenital Heart Surgery Database

Background: The European Congenital Heart Surgeons Association (ECHSA) Congenital Heart Surgery Database (CHSD) was founded in 1999 and is open for worldwide participation. The current dataset includes a large amount of surgical data from both Europe and China. Thepurposeofthisanalysisistocomparepatternsof practice and outcomes among pediatric congenital heart defect surgeries in Europe and China using the ECHSA-CHSD. Methods: We examined all European (125 centers, 58,261 operations) and Chinese (13 centers, 23,920 operations) data in the ECHSA-CHSD from 2006-2018. Operative mortality, postoperative length of stay, median patient age and weight were calculated for the ten benchmark operations for China and Europe, respectively. Results: Benchmark procedure distribution frequencies differed between Europe and China. In China, ventricular septal defect repair comprised approximately 70% of procedures, while Norwood operations comprised less than one percent of all procedures. Neonatal cardiac procedures were rare in China overall. For procedures in STAT mortality category 1, Chinese centers had lower operative mortality rates, while procedures in categories 3 and 5 mortality is lower in European centers. Operative mortality over the time period decreased from 3.89% to 1.64% for the whole cohort, with a sharper decline in China. This drop coincides with an increase of submitted procedures over this 13-year-period. Conclusion: Chinese centers had higher program-matic volume of congenital heart surgeries, while European centers have a more complex case mix. Palliation for patients with functionally univentricular heart was performed less commonly in China. These comparison of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement

Pediatric Cardiac Surgical Patterns of Practice and Outcomes in Japan and Europe

Objectives: The Japan Cardiovascular Surgery Database-Congenital section (JCVSD-Congenital) and the European Congenital Heart Surgeons Association (ECHSA) Congenital Heart Surgery Database (CHSD) share the same nomenclature. We aimed at comparing congenital cardiac surgical patterns of practice and outcomes in Japan and Europe using the JCVSD-Congenital and ECHSA-CHSD. Methods and Results: We examined Japanese (120 units, 63,365 operations) and European (96 units, 90,098 operations) data in JCVSD-Congenital and ECHSA-CHSD from 2011 to 2017. Patients' age and weight, periprocedural times, mortality at hospital discharge, and postoperative length of stay were calculated for ten benchmark operations. There was a significantly higher proportion of ventricular septal defect closures and Glenn operations and a significantly lower proportion of coarctation repairs, tetralogy of Fallot repairs, atrioventricular septal defect repairs, arterial switch operations, truncus repairs, Norwood operations, and Fontan operations in JCVSD-Congenital compared to ECHSA-CHSD. Postoperative length of stay was significantly longer following all benchmark operations in JCVSD-Congenital compared to ECHSA-CHSD. Mean STAT mortality score (Society of Thoracic Surgeons European Association for Cardio-Thoracic Surgery mortality score) was significantly higher in JCVSD-Congenital (0.78) compared to ECHSA-CHSD (0.71). Mortality at hospital discharge was significantly lower in JCVSD-Congenital (4.2%) compared to ECHSA-CHSD (6.0%, P < .001). Conclusions: The distribution of the benchmark procedures and age at the time of surgery differ between Japan and Europe. Postoperative length of stay is longer, and the mean complexity is higher in Japan compared to European data. These comparisons of patterns of practice and outcomes demonstrate opportunities for continuing bidirectional transcontinental collaboration and quality improvement

On-Pump vs Off-Pump coronary artery bypass surgery in atrial fibrillation : analysis from the polish national registry of cardiac surgery procedures (KROK)

BackgroundNo 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.Methods and findingsProcedural 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 (PConclusionsOff-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

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. View Full-Text

Napadowy częstoskurcz nadkomorowy u dzieci z zespołem Ebsteina

Wstęp: Zespół Ebsteina jest stosunkowo rzadką wadą wrodzoną serca (< 1%). Celem pracy była analiza częstoskurczów u dzieci z zespołem Ebsteina. Materiał i metody: Badaniami objęto 30 dzieci (20 dziewczynek, 10 chłopców) w wieku od 1 doby życia do 12 lat (śr. 16 miesięcy) w chwili postawienia diagnozy. U 23 dzieci stwierdzono inne wady serca: ubytek w przegrodzie międzyprzedsionkowej typu drugiego (12 pacjentów), atrezja, hipoplazja, bądź zwężenia pnia i tętnic płucnych (7 osób), ubytki w przegrodzie międzykomorowej (2 chorych), skorygowane przełożenie wielkich pni tętniczych (1 pacjent), niedomykalność tętnicy płucnej (1 osoba). U 11 chorych stwierdzono zespół preekscytacji Wolffa-Parkinsona- White&#8217;a (WPW), u 10 - blok przedsionkowo-komorowy I°. Wyniki: Czas obserwacji wynosił od 5 dób do 18 lat (śr. 8 lat). U 2 osób wykonano plastykę balonową tętnicy płucnej lub tętnic płucnych. Jednemu pacjentowi założono stent do tętnic płucnych. U pozostałych chorych wykonano następujące typy operacji kardiochirugicznych: zespolenie systemowo płucne typu Blalock-Taussig, plastykę tętnic płucnych, zespolenie Glena, plastykę zastawki trójdzielnej z wszczepieniem homograftu aortalnego, dwukierunkowe zespolenie Glena, zamknięcie ubytków w przegrodzie międzykomorowej i międzyprzedsionkowej. Częstoskurcz nadkomorowy występował u 10 pacjentów: u 8 chorych częstoskurcz ortodromowy, u 2 osób częstoskurcze orto- i antydromowe. U wszystkich dzieci napadom częstoskurczu towarzyszyły zaburzenia hemodynamiczne z obniżeniem ciśnienia parcjalnego tlenu we krwi. W leczeniu profilaktycznym częstoskurczów stosowano propafenon, sotalol i amiodaron. Dwóch pacjentów poddano zabiegowi ablacji dodatkowych dróg przewodzenia z bardzo dobrym wynikiem końcowym. Wnioski: Z zespołem Ebsteina często współistnieje zespół WPW, a napady częstoskurczu leczy się trudno. Zespołowi WPW w zespole Ebsteina zazwyczaj towarzyszy blok przedsionkowo-komorowy I°. (Folia Cardiol. 2003; 10: 683-688

The nomenclature, definition and classification of discordant atrioventricular connections

Congenitally corrected transposition is a complex cardiac lesion that is often associated with ventricular septal defect, obstruction of the outflow tract of the morphologically left ventricle, and abnormalities of the morphologically tricuspid valve.1,2Nomenclature for this lesion has been variable and confusing.1In this review, we define, and hopefully clarify this terminology. The lesion is a combination of discordant union of the atrial chambers with the ventricles, and the ventricles with the arterial trunks.1,2In rare circumstances, discordant atrioventricular connections can be associated with concordant ventriculo-arterial connections. This malformation has been called "isolated ventricular inversion". The term is less than precise, and the descriptive approach using the phrase "discordant atrioventricular connections with concordant ventriculo-arterial connections" is preferred, as discussed below

Tricuspid intervention for less-than-severe regurgitation at time of minimally invasive mitral valve surgery in patients with AF

Background: While to address moderate tricuspid regurgitation (TR) at time of left-side heart surgery is recommended by the guidelines, the procedure is still seldom performed and especially in the minimally invasive setting. Atrial fibrillation (AF) is known marker of both mortality and TR progression after mitral valve surgery. Aims: The current study aimed to address the safety of adding a tricuspid intervention to minimally invasive mitral valve surgery (MIMVS) in patients with preoperative AF. Methods: We retrospectively analyzed data from Polish National Registry of Cardiac Surgery Procedures between 2006 and 2021. We included all patients who underwent MIMVS (mini-thoracotomy-, totally thoracoscopic- or robotic surgery) and presented with moderate tricuspid regurgitation and AF preoperatively. The primary endpoint was death from any cause at 30 days and at longest available follow between MIMVS with tricuspid intervention vs MIMVS alone. We used propensity score matching to account for baseline differences between groups. Results: We identified 1,545 patients with AF undergoing MIMVS, 54.7% were men aged 66.7±9.2years. Of those, 733 (47.4%) underwent concomitant tricuspid valve intervention. At 13 years, addition of tricuspid intervention was associated with 33% higher mortality as compared to MIMVS alone. HR 1.33; 95 CIs (1.05-1.69); P=0.02. PS matching resulted in 565 well-balanced pairs. Concomitant tricuspid intervention did not influence long-term follow-up: HR, 1.01; 95 CIs (0.74-1.38); P=0.94. Conclusions: After adjusting for baseline cofounders the addition of tricuspid intervention for moderate tricuspid regurgitation to MIMVS did not increase perioperative mortality nor influence long-term survival

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS)

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. CONCLUSIONS: The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P = 0.01) and lower weight (<20 kg) (P = 0.015). Transplant rates at 6 months contin

The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy

AbstractIn 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