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BACKGROUND: To evaluate indications and results of surgery for primary cardiac tumors in children. METHODS AND RESULTS: Eighty-nine patients aged <=18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). CONCLUSIONS: Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas. [ABSTRACT FROM AUTHOR]

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database. Primary outcome was cohort's mortality, also considering different decades, while secondary outcomes were postoperative complications and reintervention. In the last 3 decades, there were 897 patients undergoing Fontan operation, M/F 512/384, median age: 4.5 years (IQR 3.3-6.4), median weight 16 kg (IQR 14-22). A first palliation was deemed necessary in 710 patients (80%), and most patients underwent a staged Fontan (93%); an extracardiac conduit was used in 790 patients (88%). Postoperative complications (mild to severe) occurred in 410 patients (46%), and early reinterventions were required in 66 patients (7.5%). Overall operative mortality was 1.7% (15 patients). Age at Fontan greater than 4 years was associated with an early need for transcatheter reintervention (adj p value = 0.037) and a higher incidence of postoperative complications (adj p value = 0.017). The Fontan operation has seen significant improvements in immediate outcomes, notably a remarkable reduction in overall mortality to just 1.35% in the last decade. While minor complications have remained steady, there has been a substantial decrease in major early complications, deaths, and the need for reinterventions. Notably, patients aged over 4 years seem to face a higher risk of postoperative morbidity, underscoring the critical role of age in preoperative assessment and management strategies for Fontan patients., (© 2024. The Author(s).)

To date, evidence supporting the efficacy of tricuspid valve (TV) repair in interrupting the progression of systemic right ventricular (RV) adverse remodeling in hypoplastic left heart syndrome (HLHS) is conflicting. We conducted a systematic review and meta-analysis of scientific literature to assess the impact of TV repair in effectively modifying the prognosis of patients with HLHS. We conducted a systematic review of PubMed, Web of Science, and Scopus databases. A random-effect meta-analysis was performed and transplant-free survival, freedom from TV regurgitation, and TV reoperation data were reconstructed using the published Kaplan-Meier curves. Nine studies were included, comprising 203 HLHS patients undergoing TV repair and 323 HLHS controls. The estimated transplant-free survival at 1, 5, and 10 years of follow-up was 75.5% [95% confidence interval (CI) = 67.6-84.3%], 63.6% [95% CI = 54.6-73.9%], and 61.9% [95% CI = 52.7-72.6%], respectively. Transplant-free survival was comparable to HLHS peers without TV regurgitation (p = 0.59). Five-year freedom from recurrence of TV regurgitation and freedom from TV reoperation was 57% [95% CI = 46.7-69.7%] and 63.6% [95% CI = 54.5-74.3%], respectively. Younger age and TV repair at the time of Norwood operation increased the risk of TV regurgitation recurrence and the need for TV reoperation. Our meta-analysis supports the efficacy of TV repair in favorably modifying the prognosis of patients with HLHS and TV regurgitation, reestablishing a medium-term transplant-free survival which is comparable to HLHS peers. However, durability of surgery and long-term fate of TV and RV performance are still unclear., (© 2023. The Author(s).)

Background: We evaluated outcomes of neonatal cardiac surgery at hospitals affiliated with the European Congenital Heart Surgeons Association (ECHSA)., Methods: All patients ≤30 days of life undergoing a cardiac surgical procedure during a 10 year period between January 2013 and December 2022 were selected from the ECHSA Congenital Database. Reoperations during the same hospitalization, percutaneous procedures, and noncardiac surgical procedures were excluded. A total of 12 benchmark operations were identified. Primary outcomes were 30-day mortality and in-hospital mortality. Multivariable logistic regression analysis was performed to determine independent factors associated with higher mortality. A comparison of mortality between the first 5 years and second 5 years was also performed., Results: The overall number of neonatal operations from 2013-2022 was 30,931, and 22,763 patients met the inclusion criteria of the study. The four most common procedures were: arterial switch operation (3,520/22,763=15.5%), aortic coarctation repair (3,204/22,763=14.1%), shunt procedure (2,351/22,763=10.3%), and Norwood Operation (2,115/22,763=9.23%). 30-day mortality rate was: overall population (1,342/22,763=5.9%), arterial switch (110/3,520=3.13%), Norwood Operation (339/2,115=16.0%), and hybrid operation (94/609=15.4%). In-hospital mortality rate was: overall population (2,074/22,763=9.1%), arterial switch (145/3,520=4.12%), Norwood Operation (523/2,115=24.7%), and hybrid operation (186/609=30.5%). Multivariable analysis revealed that major factors impacting mortality were high risk procedures (adjusted odds ratio=2.74 [95% confidence interval=2.33-3.23]; p<0.001), and the need for extracorporeal membrane oxygenation (11.8 [9.9-14]; p <0.001)., Conclusions: Neonatal cardiac surgery continues to pose a significant challenge, with notable mortality, particularly for neonates with functionally univentricular physiology. These data can serve as important benchmarks across Europe and offer insights regarding opportunities for improvement., (Copyright © 2024. Published by Elsevier Inc.)

Down syndrome is one of the most common genetic diseases, generally associated with an increased probability of congenital heart diseases. This increased risk contributes to escalated levels of morbidity and mortality. In this study, we sought to analyze nationwide data of pediatric and adult patients with Down syndrome and congenital heart disease over a 15-year period. Data obtained from the hospital discharge form between 2001 and 2016 of patients diagnosed with Down syndrome in Italy and at least one congenital heart disease were included. Information on 12362 admissions of 6527 patients were included. Age at first admission was 6.2 ± 12.8 years and was a predictor of mortality (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). 3923 (60.1%) patients underwent only one admission, while 2604 (39.9%) underwent multiple (> 1) admissions. There were 5846 (47.3%) admissions for cardiac related symptoms. Multiple admissions (SHR: 3.13; 95% CI: 2.99, 3.27; P < 0.01) and cardiac admissions (SHR: 2.00; 95% CI: 1.92, 2.09; P < 0.01) were associated with an increased risk of additional potential readmissions. There was an increased risk of mortality for patients who had cardiac admissions (HR = 1.45, 95% CI: 1.08-1.94, p = 0.012), and for those who underwent at least 1 cardiac surgical procedure (HR = 1.51, 95% CI 1.13-2.03, p = 0.006)., Conclusions: A younger age at first admission is a predictor for mortality in patients with Down syndrome and congenital heart disease. If patients undergo more than one admission, the risk of further readmissions increases. There is a pivotal role for heart disease in influencing the hospitalization rate and subsequent mortality., What Is Known: • Down syndrome individuals often face an increased risk of congenital heart diseases. • Congenital heart diseases contribute significantly to morbidity and mortality in Down syndrome patients., What Is New: • This study analyzes nationwide data covering a 15-year period of pediatric and adult patients in Italy with Down syndrome and congenital heart disease. • It identifies a younger age at first admission as a predictor for mortality in these patients, emphasizing the criticality of early intervention. • Demonstrates a correlation between multiple admissions, particularly those related to cardiac issues, and an increased risk of further readmissions, providing insights into the ongoing healthcare needs of these individuals., (© 2024. The Author(s).)

Objectives: In addition to a correct prenatal diagnosis of congenital heart disease (CHD), comprehensive parental counseling is crucial to ensure that parents are well-informed about the condition of the fetus. This study aims to investigate whether there is a significant difference in the information acquired by parents through traditional counseling, utilizing 2-dimensional (2D) illustrations and images, compared to an advanced approach utilizing personalized three-dimensional (3D) printed models of the fetal heart developed from 3D ultrasound imaging., Methods: This study, designed as a pilot randomized control trial, enrolled pregnant women with gestational ages greater than 18 weeks, whose fetuses were diagnosed with CHD and referred to our center between November, 2020 and June, 2021. Two groups of patients were included in the study. The first group received standard medical counseling with 2D images and illustrations, while the second group underwent advanced counseling with 3D-printed patient-specific heart models. Both groups were then required to complete the same survey in which the knowledge of the CHD was investigated. The 3D models were created from 3D ultrasound imaging and printed using resin materials in both 1:1 and 5:1 scale., Results: A comparison of the scores obtained from the two groups revealed that 3D visualization of the fetus's heart has the potential to increase parental knowledge about CHD and the required surgical procedures. Furthermore, all couples expressed interest in receiving a 1:1 scale model of their baby's heart., Conclusion: Personalized prenatal counseling with 3D-ultrasound-based heart models positively impacts parents' understanding of CHD. The use of 3D models provides a more comprehensive and accessible representation of the condition, contributing to an increased knowledge gain, and potentially helping to support informed decisions regarding their child's care., (© 2024 John Wiley & Sons Ltd.)

Pericardial patches are currently used as reconstructive material in cardiac surgery for surgical treatment of cardiac septal defects. Autologous pericardial patches, either treated with glutaraldehyde or not, can be used as an alternative to synthetic materials or xenograft in congenital septal defects repair. The availability of an allogenic decellularized pericardium could reduce complication during and after surgery and could be a valid alternative. Decellularization of allogenic tissues aims at reducing the immunogenic reaction that might trigger inflammation and tissue calcification over time. The ideal graft for congenital heart disease repair should be biocompatible, mechanically resistant, non-immunogenic, and should have the ability to growth with the patients. The aim of the present study is the evaluation of the efficacy of a new decellularization protocol of homologous pericardium, even after cryopreservation. The technique has proven to be suitable as a tissue bank procedure and highly successful in the removal of cells and nucleic acids content, but also in the preservation of collagen and biomechanical properties of the human pericardium., (© 2023. The Author(s).)

Background: Understanding the macroscopic biventricular changes induced by pulmonary artery banding (PAB) in children with dilated cardiomyopathy (DCM) represents the first step to unraveling the regenerative potential of the myocardium. We herein investigated the phases of left ventricular (LV) rehabilitation in PAB responders, using a systematic echocardiographic and cardiac magnetic imaging (CMRI) surveillance protocol., Methods: We prospectively enrolled all patients with DCM treated with PAB from September-2015 at our institution. Among 9 patients, 7 positively responded to PAB and were selected. Transthoracic 2D echocardiography was performed before PAB; and 30, 60, 90, and 120 days after PAB; and at the last available follow-up. CMRI was performed before PAB (whenever possible) and one year after PAB., Results: In PAB responders, LV ejection fraction showed a modest 10% increase 30-60 days after PAB, followed by its almost complete normalization after 120  days (median of 20[10-26]% vs 56[44.5-63.5]%, at baseline and 120 days after PAB, respectively). Parallelly, the LV end-diastolic volume decreased from a median of 146(87-204)ml/m2 to 48(40-50)ml/m2. At the last available follow-up (median of 1.5 years from PAB), both echocardiography and CMRI showed a sustained positive LV response, although myocardial fibrosis was detected in all patients., Conclusions: Echocardiography and CMRI show that PAB can promote a LV remodeling process, which starts slowly and can culminate in the normalization of LV contractility and dimensions 4 months later. These results are maintained up to 1.5 years. However, CMRI showed residual fibrosis as evidence of a past inflammatory injury whose prognostic significance is still uncertain., Competing Interests: Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

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.

Objectives: Two limitations of the clinical use of 3-dimensional (3D) reconstruction and virtual reality systems are the relatively high cost and the amount of experience required to use hardware and software to effectively explore medical images. We have tried to simplify the process and validate a new tool developed for this purpose with a novel software package., Methods: Five patients with right partial anomalous pulmonary venous return with adequate preoperative images acquired with magnetic resonance imaging were enrolled. Five volunteers with no previous experience in the field of 3D reconstruction were instructed to use the software after viewing a short video tutorial. Users were then asked to create a 3D model of each patient's heart using DIVA software. Their results were compared quantitatively and qualitatively with a benchmark reconstruction performed by an experienced user., Results: All our participants recreated 3D models in a relatively short time, maintaining a good overall quality (average quality score ≥ 3 on a scale of 1-5). The overall trend of all the parameters analysed showed a statistical improvement between case 1 and case 5, as users became more and more experienced., Conclusions: DIVA is a simple software program that allows accurate 3D reconstruction in a relatively short time ("fast-track" virtual reality). In this study, we demonstrated the potential use of DIVA by inexperienced users, with a significant improvement in quality and time after a few cases were performed. Further studies are needed to confirm the potential application of this technology on a larger scale., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Management of end-stage heart failure (ESHF) in children with congenital heart disease is challenging. We report a step-by-step hybrid procedure (transcatheter pulmonary valve and left mechanical assist device implantations) in a child with ESHF after repair of tetralogy of Fallot, as an effective bridge to transplant strategy.

Objectives: Fontan patients are known to suffer from clinical attrition over the years, which has been characterized as Fontan failure. We sought to evaluate the clinical outcomes of such Fontan patients undergoing surgical management in a 25-year, single-centre experience., Methods: A retrospective single-centre analysis of patients undergoing surgical treatment for failing Fontan between 1995 and 2020, including any reoperations when ventricular function was preserved, or a heart transplant (HTx), when ventricular contractility was impaired. We analysed survival, indications for surgery and early and late complication rates., Results: We collected 30 patients (mean age 24.7 years) who required surgery after a mean time of 19.3 years from the original Fontan procedure: Fontan conversion in 21 (70%, extracardiac conduit in 19, lateral tunnel in 2), a HTx in 4 (13.3%) and other reoperations in 5 (16.7%). The most common indications for surgery were tachyarrhythmias (63.3%) and severe right atrial dilatation (63.3%). Overall survival at the 1-, 5-, 10- and 20-year follow-up examinations were 75.9% [95% confidence interval (CI): 91.4-60.4%], 75.9% (95% CI: 91.4-60.4%), 70% (95% CI: 78-52%) and 70% (95% CI: 78-52%), respectively. The most frequent complications were postoperative tachyarrhythmias (50%) and late Fontan-associated liver disease (56.5%). HTx and Fontan conversion provided comparably good outcomes compared to other reoperations (P = 0.022)., Conclusions: Surgery for failing Fontan can be performed effectively with overall good long-term survival. However, early and late morbidities are still a significant burden. Because other reoperations performed when patients presented with contraindications for a HTx have carried high mortality, close clinical follow-up is mandatory, and an earlier indication for Fontan conversion or a HTx is advisable to optimize outcomes., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: We sought to evaluate the predictive power of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database., Methods: The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database. Each operation was assigned a STAT mortality score and category and an ACHS mortality score. Operative mortality was based on the 30-day status and on the status at hospital discharge. The discriminatory power of the STAT and ACHS scores was assessed using the area under the receiver operating characteristic curve (c-index)., Results: A total of 17 214 (97.46%) operations were assigned ACHS scores. The 3 most frequent primary procedures were closure of the atrial septal defect (19.0%), aortic valve replacement (8.8%) and non-valve-sparing aortic root replacement (6.1%). Operative mortality for ACHS-coded operations was 2.07%. The procedures with the highest mortality were atrial septal defect creation/enlargement (19.0%), lung transplantation (18.8%) and heart transplantation (18.2%). A total of 17 638 (99.86%) operations were assigned a STAT score and category. The operative mortality for STAT-coded operations was 2.27%. The c-index for mortality was 0.720 for the STAT mortality score and 0.701 for the ACHS score., Conclusions: The ACHS mortality score and the STAT mortality score reached similar, moderate predictive power in adult patients undergoing congenital heart surgery in ECHSA database., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

End-stage heart failure (ESHF) in pediatric age is an ongoing challenge. Heart transplantation is the final option, but its long-term outcomes are still suboptimal in children. An alternative patient-tailored surgical protocol to manage ESHF in children is described. Retrospective, single-center analysis of pediatric patients admitted to our institution between April 2004 and February 2021 for ESHF. Our current protocol is as follows: (a) Patients <1 year with isolated left ventricular dysfunction due to dilated cardiomyopathy underwent pulmonary artery banding (PAB). (b) Patients <10 years and <20 kg, who did not meet previous criteria were managed with Berlin Heart EXCOR. (c) Patients >10 years or >20 kg, underwent placement of intracorporeal Heartware. Primary outcomes were survival, transplant incidence, and postoperative adverse events. A total of 24 patients (mean age 5.3 ± 5.9 years) underwent 26 procedures: PAB in 6 patients, Berlin Heart in 11, and Heartware in 7. Two patients shifted from PAB to Berlin Heart. Overall survival at 1-year follow-up and 5-year follow-up was 78.7% (95%CI = 62%-95.4%) and 74.1% (95%CI = 56.1%-92.1%), respectively. Berlin Heart was adopted in higher-risk settings showing inferior outcomes, whereas a PAB enabled 67% of patients to avoid transplantation, with no mortality. An integrated, patient-tailored surgical strategy, comprehensive of PAB and different types of ventricular assist devices, can provide satisfactory medium-term results for bridging to transplant or recovery. The early postoperative period is critical and requires strict clinical vigilance. Selected infants can benefit from PAB that has demonstrated to be a safe bridge to recovery., (© 2021 The Authors. Artificial Organs published by International Center for Artificial Organ and Transplantation (ICAOT) and Wiley Periodicals LLC.)

Objectives: The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes., Methods: We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation., Results: A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4-20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P = 0.018). Statistical analysis showed an equal distribution of BVF across time (P = 0.620), and there were no significant differences in terms of early and late survival (P = 0.53 and P = 0.72, respectively) or morbidity (P = 0.45 and P = 0.80, respectively)., Conclusions: A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Introduction: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database., Materials and Methods: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS)., Results: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001)., Conclusions: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients., (© 2021 Wiley Periodicals LLC.)

We reconstructed and printed a 3D model of the fetal heart affected by d-transposition of the great arteries from prenatal ultrasound images. Our 3D model revealed to be very helpful in showing the basic anatomical features of fetal complex Congenital Heart Disease (CHD) and represents an interesting additional diagnostic tool to the current standard imaging armamentarium, improving the quality of prenatal parental counseling.

Background: Extracorporeal membrane oxygenation (ECMO) implantation for neonates with severe cardiorespiratory life-threatening conditions is highly effective. However, since ECMO is a high-risk and complex therapy, this treatment is usually performed in centers with proven expertise., Methods: A retrospective review of neonates, from January 2014 to January 2020, presenting with life-threatening conditions and treated by means of Hub and Spoke (HandS) ECMO in peripheral (spoke) hospitals. Data were retrieved from our internal ECMO registry. Protocols and checklists were revised and shared with all spoke hospitals located in North-Eastern Italy., Results: Eleven neonates receiving maximal respiratory and cardiovascular support at a spoke hospital underwent HandS ECMO management. All but three patients were affected by life-threatening meconium aspiration syndrome (MAS). The median ECMO support duration and hospitalization were four (range 2-32) and 30 days (range 8-50), respectively. All but two patients (with congenital diaphragmatic hernia), were weaned off ECMO and discharged home. At a mean follow up of 33.7 ± 29.2 months, all survivors were alive and well, without medications, and normal somatic growth. All but one had normal neuropsychological development., Conclusion: HandS ECMO model for neonates with life-threatening conditions is effective and successful. A specialized multidisciplinary team and close cooperation between Hub and Spoke centers are essential for success.

Objectives: The one-and-a-half ventricle repair (1.5VR) is a surgical alternative to Fontan circulation or high-risk biventricular repair in patients with complex congenital heart disease (CHD) with a hypoplastic right ventricle (RV). We report our 25 years of experience to evaluate whether the degree of anatomical complexity of the CHD can affect long-term outcomes., Methods: This is a retrospective study including patients undergoing 1.5VR between 1994 and 2018. Clinical records and operative reports were reviewed. Follow-up data were collected from all survivors at the last evaluation or by phone contact., Results: Twenty-nine patients underwent 1.5VR [median age: 3.5 years, interquartile range (IQR) 0.8-7.8]. Fifteen patients had 'simple' (i.e. confined to right heart lesions) anatomical characteristics (48.3%); the median tricuspid valve annulus z-score was -3.5 (IQR -6.2 to +3.6). There were no operative deaths. The median hospital stay was 21 days (IQR 10-33), with postoperative complications in 21 patients (75.8%). At a median follow-up of 13.2 years (IQR 3.2-25.6 years; completeness 96.6%), there were 3 late deaths. There was no significant difference in survival and in freedom from adverse events between simple and complex anatomy groups. However, stress test findings showed a tendency towards a higher maximum oxygen uptake in the simple group (P = 0.055)., Conclusions: The 1.5VR strategy is an effective low-risk surgical option for patients with CHD with hypoplastic RV. When it is planned from birth, late outcomes can be satisfactory. In our experience, the presence of associated major cardiac anomalies was not associated with either worse early or long-term outcomes and functional status., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Several techniques designed to improve long-term results after repair of tetralogy of Fallot are described. We have recently embarked on a program focused on preserving the native pulmonary valve. Here, combined techniques are described in detail, including intraoperative pulmonary valve balloon dilatation, pulmonary valve reconstruction by delamination and resuspension of the leaflets, and pulmonary valve annulus augmentation. As with any other complex congenital heart disease, senior surgeons should select teaching cases, starting from the less severe side of the spectrum.

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.

Patent ductus arteriosus (PDA) is a clinical condition mostly found in premature newborns. Among several medical, surgical and interventional treatment options, extrapleural ligation through a left minithoracotomy is recognized as a safe, efficient and less expensive technique. In fact, it requires short surgical times, grants good exposure of the duct and nearby structures (e.g., thoracic duct, left recurrent laryngeal nerve), and avoids pleural space opening and subsequent pulmonary complications in preterm patients. This approach seems ideal due to its lower costs, especially in developing countries with a high birth rate and limited resources.

Eisenmenger syndrome (ES) has been considered a cause of inoperability in patients with congenital heart disease (CHD). Even if long-standing vasodilators are efficient to reduce pulmonary vascular resistance (PVR), the best approach to grant stable repair of these patients is still unknown. We describe the case of a 28-year-old man with a diagnosis of untreated large ventricular septal defect and established ES. After few years of vasodilator therapy, the patient underwent pulmonary banding with significant reduction of his PVR. His CHD was then repaired, with clinical and functional improvement at 2-year follow-up.

Adults with congenital heart disease (ACHD) represent a growing population. To evaluate frequency, type and outcomes of cardiac surgery in ACHD, we gathered data from the European Congenital Heart Surgeons Association Database of 20,602 adult patients (≥18 years) with a diagnosis of congenital heart disease who underwent cardiac surgery, between January 1997 and December 2017. We demonstrated that overall surgical workload (as absolute frequencies of surgical procedures per year) for this specific subset of patients increased steadily during the study period. The most common procedural groups included septal defects repair ( n = 5740, 28%), right-heart lesions repair ( n = 5542, 27%) and left-heart lesions repair ( n = 4566, 22%); almost one-third of the procedures were re-operations ( n = 5509, 27%). When considering the year-by-year relative frequencies of the main procedural groups, we observed a variation of the surgical scenario during the last two decades, characterized by a significant increase over time for right and left-heart lesions repair ( p < 0.0001, both); while a significant decrease was seen for septal defects repair ( p < 0.0001) and transplant ( p = 0.03). Overall hospital mortality was 3% ( n = 622/20,602 patients) and was stable over time. An inverse relationship between mortality and the number of patients operated in each center ( p < 0.0001) was observed.