Using cryopreserved allogeneic pericardium to repair congenital heart defects in children.

Patches prepared from autologous, allogeneic, or xenogeneic tissues are widely used in the repair of congenital heart defects in children. Since 2002, cryopreserved allogeneic pericardial patches have been prepared in our institution as an alternative to commercially available patches. This study retrospectively reviewed donor and patient data concerning cryopreservation time and the clinical use of the pericardium in 382 children who were operated on at a single center between 2004 and 2021. There were 177 donors: 98 males and 79 females. The median donor age was 13 years (range: 1 month to 53 years) and the median cryopreservation time was 72 days (range: 3-685). There were 382 pediatric patients: 224 males and 158 females. The median patient age was 1 month (range: 3 days to 17.8 years). The patches were used for primary surgeries in 228 patients and for reoperations in 154. The patches were implanted into the right heart or venous circulation in 209 patients, the left heart or arterial circulation in 246 patients, and both sides of the circulatory system in 73. Extracardiac patch implantation was performed in 339 patients, intracardiac in 79 patients, and both intracardiac and extracardiac in 36 patients. Our study presents a single-center experience in the use of cryopreserved allogeneic pericardium. The pericardium can be used on the systemic and pulmonary sides of the circulatory system, in either extracardiac or intracardiac positions. However, there is no uniform strategy for selecting the "patch of choice" for correcting congenital heart defects in children, especially since there are few studies comparing several types of patches.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Functional aortic annulus 3-dimensional remodelling during bicuspid aortic valve repair: complete external and internal annuloplasty.

The internal geometric ring provides full three-dimensional annular stability while minimizing the dissection of the aortic root, pulmonary artery and right ventricle and avoids reimplanting the coronaries. The external annuloplasty provides a secondary stabilization of the easily accessed fibrous portion of the annulus using the sutures for the internal annuloplasty device and minimizes the sutures above the leaflets. Together they form a complete remodelling of the ventriculo-aortic junction following its exact course. The junction together with stabilization of the subcommissural triangles defines functional aortic annulus remodelling. External annuloplasty supports the virtual basal ring.

Outcomes of Transannular Repair of Tetralogy of Fallot With a Contegra® Monocuspid Patch.

BACKGROUND: Surgical repair of tetralogy of Fallot (ToF) depends on the anatomical variations of the heart defect. A group of patients with a hypoplastic pulmonary valve annulus required a transannular patch. This study aimed to evaluate the early and late outcomes of ToF repair with a transannular Contegra® monocuspid patch in a single center. METHODS: A retrospective review of medical records was conducted. This study included 224 children with a median age of 13 months who underwent ToF repair with a Contegra® transannular patch in over 20 years of observation. The primary outcomes were hospital mortality and need for early reoperations. The secondary outcomes were late death and event-free survival. RESULTS: The hospital mortality in our group was 3.1%, whereas two patients required early reoperation. Three patients were excluded from the study because follow-up data were not available. In the remaining group of patients (212 patients), the median follow-up was 116 (range, 1-206) months. One patient died because of sudden cardiac arrest at home six months after surgery. Event-free survival was observed in 181 patients (85.4%), whereas the remaining 30 patients (14.1%) required graft replacement. The median time to reoperation was 99 (range, 4-183) months. CONCLUSIONS: Although surgical treatment of ToF has been performed for more than 60 years worldwide, the optimal approach in children with a hypoplastic pulmonary valve annulus remains debatable. Among options, the Contegra® monocuspid patch can be effectively used in transannular repair of ToF with good long-term results.

TGA + IVS + LVOTO: patterns of practice and outcomes.

PURPOSE: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.

Postoperative kinetics of pentraxin 3 (PTX3) after congenital heart surgery with cardiopulmonary bypass in pediatric patients.

BACKGROUND: Pentraxins are inflammatory proteins and markers of acute-phase responses. They are divided into short and long subgroups based on the length of the N-terminal region. The most studied short pentraxin is the C-reactive protein (CRP), which is known to be expressed in various inflammatory conditions, including surgical procedures. On the other hand, much less is known about the kinetics of long pentraxin 3 (PTX3) in surgical patients, especially in the pediatric population. The aim of this prospective study was to determine the early postoperative kinetics of PTX3 in relation to procalcitonin (PCT) and CRP levels in children undergoing congenital heart surgery with cardiopulmonary bypass (CPB). METHODS: A total of 21 children (9 boys and 12 girls, mean age 12 months) were included in the study. Blood samples for determination of CRP, PCT, and PTX3 levels were collected before the surgery and then immediately after its completion (postoperative day 0, POD 0) and subsequently at POD 1, 2, and 3. RESULTS: Serum PTX3 concentrations increased significantly between POD 0 and POD 1 (mean values were 12.2 and 72.4 ng/ml, respectively, p<0.001), decreased between POD 1 and POD 2 (mean values were 72.4 and 23.6 ng/ml, respectively, p<0.001), and normalized on POD 3 (the mean value was 1.2 ng/ml). CONCLUSIONS: PTX3 concentrations are markedly elevated during the first postoperative day. Under normal circumstances, PTX3 rises and falls quickly, and its second rise in the early postoperative period may be abnormal, however, further studies are necessary.

Ten Years of Data Verification: The European Congenital Heart Surgeons Association Congenital Database Audits.

BACKGROUND: Congenital heart surgery databases are tools for internal programmatic evaluation, benchmarking institutional results to multi-institutional aggregate data, and research. Therefore, it is essential to ensure the completeness and accuracy of data. This study analyzes the results of ten years of on-site source data verification of the European Congenital Heart Surgeons Association Congenital Heart Surgery Database (ECHSA CHSD). METHODS: All data forms verified between 2009 and 2018 were analyzed. The data form consists of 12 data elements: dates of birth, admission, surgery, discharge, and death; weight; case category; cardiopulmonary bypass time; aortic cross-clamp time; validation rules; diagnoses; and procedures. Descriptive data calculation and rates of completeness and accuracy were determined. The trend of error rate of seven centers with ≥5 visits was analyzed. RESULTS: Sixty-nine on-site verification visits took place at 17 centers. A total of 26,245 cases were verified; 2,841 of these 26,245 cases (10.8%) showed an error. The total mean error rate of centers for all years was 12.3 ± 2.1%. Rates of completeness and accuracy were 99% and 89.2%, respectively. Coded diagnoses and procedure analysis revealed that 716 (2.7%) and 456 (1.7%) datasets were incorrect, respectively. Rates of completeness and accuracy of dates were 100%, and 97.1%, respectively. Validation fields showed no errors. CONCLUSION: Source data verification is an appropriate tool to determine completeness and accuracy of data. The ECHSA CHSD verification analysis of a ten-year period showed a high level of completeness and accuracy. The verified data of the ECHSA CHSD are well-suited for benchmarking and research.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Background and Objectives: Over the years, surgical repair of total anomalous pulmonary venous connection (TAPVC) outcomes have improved, however, morbidity and mortality still remain significant. This study aims to assess the early and long-term outcomes of surgical treatment of TAPVC children, operated on between 2006 and 2016, in one pediatric center in Poland. Materials and Methods: Diagnostics, surgical treatment, and follow-up data from 83 patients were collected. In addition, survival and risk factor analyses, control echocardiographic, and electrocardiographic examinations were performed. Results: In the analyzed group (n = 83), there were seven hospital deaths (within 30 days after the operation) (8.4%) and nine late deaths (10.8%). The mean follow-up time was 5.5 years, and, for patients who survived, it was 7.1 years. The mean survival time in patients with completed follow-up (n = 70) was 10.3 years; the overall five-year survival rate was 78.4%. Independent mortality risk factors were type I TAPVC, single ventricle physiology, time from admission to operation, intensive care unit stay, postoperative hospital stay, and temporary kidney insufficiency requiring dialysis. Conclusions: The presence of single ventricle physiology and the supracardiac subtype of TAPVC might be negative prognostic factors, while normal heart physiology presents with good post-repair results. This study indicates that cardiac arrhythmias may occur. Morbidity and mortality, related to surgical TAPVC correction, still remain significant.

Bicuspid aortic valve repair with external or subcommissural annuloplasty-echocardiographic prospective trial.

BACKGROUND: The incompetent bicuspid aortic valve (BAV) can be repaired using various techniques. This study presents a prospective comparison of external and subcommissural aortic annuloplasty. METHODS: Fifty consecutive patients (38 males, age: 43.9 ± 15.8 years) with BAV insufficiency with or without aortic dilatation underwent valve repair in a single institution. They were prospectively allocated to one of two groups based on the aortic annulus stabilization technique: 25 patients were operated on using the subcommissural annuloplasty (SCA) and 25 using the external complete annuloplasty (EA). Transthoracic echocardiography was performed in all patients before the operation and 1 and 3 years after the operation. Moreover, mortality and morbidity at 7 years were evaluated. RESULTS: In prospective echocardiographic comparison, EA was associated with smaller diameter of the aortic annulus (24.1 ± 2.6 mm vs. 25.8±2.1 mm, p < .05) and lower mean and peak transvalvular gradients (7 ± 4 mmHg vs. 13 ± 4 mmHg, p = .02 and 15.3 ± 9.7 mmHg vs. 20.7 ± 5.6 mmHg, p = .03, respectively). No patients died or required reoperation due to recurrent insufficiency at 6,81 (interquartile range-0,17) years after the operation. The Kaplan-Meier actuarial freedom from aortic regurgitation (AR) grade =2 or gradient > 20 mmHg at 35.1 ± 3.6 months years was 96% (24 out of 25) for patients who had external annuloplasty and amounted to 76% (19 out of 25) for those who had SCA, p = .05). CONCLUSIONS: External annuloplasty performed during repair of the BAV is associated with better hemodynamics at medium-term follow-up compared to SCA.

Perioperative Antibiotic Prophylaxis in Pediatric Cardiac Surgery-Simple Is Better.

Pediatric cardiac surgery requires perioperative antibiotic prophylaxis (PAP) to reduce the risk of surgical site infections. However, the complexity of these procedures and the metabolic immaturity of children impede the establishment of PAP regimens that are both efficacious and in line with antimicrobial stewardship (AMS). In this study, we compared two PAP regimens: cefazolin with gentamicin (in a retrospective group) and cefazolin only (prospectively) in children undergoing elective cardiac surgery. In the prospective group, additional elements of AMS were introduced, i.e., restricted access to cefazolin and more diligent use of empirical antibiotics proceeded by consultation with an AMS team. The rate of surgical site infections (SSI), the scope of PAP deviations, and the postoperative use of antibiotics other than PAP within 30 days after surgery were analyzed. There were no significant differences in the rate of SSIs between the groups (3.9% vs. 1.2% in the prospective and retrospective groups, respectively (p = 0.35)). However, in the prospective group, the PAP violation was significantly reduced compared with the retrospective group (full compliance with the PAP regimen was 45.5% vs. 4.8%, p < 0.001, respectively). In addition, a reduction of postoperative antibiotic use was observed in the prospective group (0.991 vs. 1.932 defined daily doses, respectively).

Cefazolin prophylaxis in children undergoing cardiac surgery with the use of cardiopulmonary bypass-is the dosing correct?

OBJECTIVES: To determine the recommended concentrations of cefazolin to be used for antibiotic prophylaxis during paediatric cardiac surgery with extracorporeal circulation (ECC). METHODS: Twenty paediatric patients undergoing cardiac surgery with ECC and cefazolin antibiotic prophylaxis were included in the study. Blood samples for measurement of total cefazolin plasma concentration were collected at the following measurement time points: directly after skin incision, 15 min after ECC start, 5 min after ECC cessation and at skin closure. The target concentration was set for ≥40 mg/l, which corresponded to ≥8 mg/l of unbound cefazolin concentration. RESULTS: The median total cefazolin plasma concentrations at the measurement time points were 62.8, 67.7, 45.8 and 34.2 mg/l, respectively, and target concentrations were achieved in 90%, 85%, 65% and 40% of children, respectively. Among patients who received ≥30 mg of cefazolin per 100 ml of ECC priming, target concentrations after ECC cessation were reached in 80% of patients, while in those with <30 mg cefazolin per 100 ml in 20% of patients (P = 0.031). CONCLUSIONS: Direct extrapolation of antibiotic prophylaxis recommendations from adults to children may result in suboptimal antibiotic concentrations. An additional cefazolin dose to ECC priming appears necessary and the dosing should be based on ECC priming volume rather than on the weight of the patient.

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.

Bicuspid aortic valve repair: outcomes after 17 years of experience.

OBJECTIVES: This study presents the results of 17 years of experience with bicuspid aortic valve (BAV) repair and the analysis of factors associated with repair failure and early echocardiographic outcome. METHODS: Between 2003 and 2020, a total of 206 patients [mean age: 44.5 ± 15.2 years; 152 males (74%)] with BAV insufficiency with or without aortic dilatation underwent elective aortic valve repair performed by a single surgeon with a mean follow-up of 5 ± 3.5 years. The transthoracic echocardiography examinations were reported. RESULTS: There were no deaths during the hospital stay, and all but 1 patient survived the follow-up period (99.5%). Overall, 10 patients (5%) developed severe insufficiency and 2 (1%) developed aortic dilatation requiring reoperation. Freedom from reoperation at 7 years reached 91.8%. Type 2 BAV configuration [hazard ratio (HR) 3.9; 95% confidence interval (CI): 1.01-60; P = 0.049], no sinotubular junction remodelling (HR 7; 95% CI: 1.7-23; P = 0.005), no circumferential annuloplasty (HR 3.9; 95% CI: 1.01-64; P = 0.047) and leaflet resection (HR 5.7; 95% CI 1.2-13. P = 0.017) have been identified as a risk factor of redo operation. Parameters of the postoperative left ventricle reverse remodelling improved significantly early after the operation and later at 2 years evaluation. CONCLUSIONS: The repair of BAV offers good short- and mid-term results providing a significant reverse left ventricular remodelling. Type 0 BAV preoperative configuration, circumferential annuloplasty and sinotubular junction remodelling are associated with better repair durability.

The evaluation of annuloplasty in bicuspid aortic valve repair using cardiac magnetic resonance.

BACKGROUND: The incompetent bicuspid aortic valve (BAV) can be replaced or repaired using various surgical techniques. This study sought to assess the efficacy of external annuloplasty and postoperative reverse remodelling using cardiac magnetic resonance (CMR) and compare the results of external and subcommissural annuloplasty. METHODS: Out of a total of 200 BAV repair performed between 2004 and 2018, 21 consecutive patients (median age 54 years) with regurgitation requiring valve repair with annuloplasty without concomitant aortic root surgery were prospectively referred for CMR and transthoracic echocardiography (TTE) one year after the operation. Two aortic annulus stabilization techniques were used: external, circumferential annuloplasty (EA), and subcommissural annuloplasty (SCA). RESULTS: 11 patients received EA and 10 patients were treated using SCA. There was no in-hospital mortality and all patients survived the follow-up period (median: 12.6 months (first quartile: 6.6; third quartile: 14.1). CMR showed strong correlation between postoperative aortic recurrent regurgitant fraction and left ventricular end-diastolic volume (r = 0.62; p = 0.003) as well as left ventricular ejection fraction (r = -0.53; p = 0.01). Patients treated with EA as compared with SCA had larger anatomic aortic valve area measured by CMR (3.5 (2.5; 4.0) vs. 2.5 cm2 (2.0; 3.4); p = 0.04). In both EA and SCA group, aortic valve area below 3.5 cm2 correlated with no regurgitation recurrency. EA (vs. SCA) was associated with lower peak transvalvular aortic gradients (10 (6; 17) vs. 21 mmHg (15; 27); p = 0.04). CONCLUSIONS: The repair of the bicuspid aortic valve provides significant postoperative reverse remodelling, provided no recurrent regurgitation and durable reduction annuloplasty can be achieved. EA is associated with lower transvalvular gradients and higher aortic valve area assessed by CMR, compared to SCA.