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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]

Aim: The Fontan procedure is a palliative surgical treatment for different congenital heart diseases with a univentricular heart, but it has been associated with decreased exercise capacity, cardiovascular morbidity, and premature mortality. The one-and-half ventricle repair (1.5VR) was introduced as an alternative to the Fontan procedure, specifically for selected patients with borderline hypoplastic right ventricle (HRV), aiming for a more physiological circulation. Despite these efforts, the benefit of 1.5VR over Fontan circulation comparison on clinical and functional outcomes remains unclear. The aim of this study was to investigate and compare young patients with HRV after 1.5VR with those with functional single right or left ventricles (FSRV or FSLV) after Fontan palliation over a 10-year follow-up period., Methods and Results: In this retrospective observational study, serial cardiopulmonary exercise tests (CPETs) performed in patients with 1.5VR and Fontan circulation between September 2002 and March 2024 have been analysed. Only patients with at least 10 years of follow-up were considered. A total of 43 patients were included (age at baseline 8.6 ± 2.6 years): 21 with FSLV, 12 with FSRV, and 10 with 1.5VR. No differences in cardiorespiratory fitness and efficiency were shown at the first CPET assessment among the three groups. At 10-year follow-up, 1.5VR had higher cardiorespiratory fitness and efficiency compared to FSLV and FSRV patients., Conclusion: These findings suggest that the 1.5VR may provide superior long-term functional outcomes than the Fontan procedure in patients with borderline HRV. Further studies are needed to evaluate the impact on hard clinical endpoints., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objectives: The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes., Methods: Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed., Results: Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16-26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4-42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m 2 ) in patients with a right dominant ventricle ( p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome ( p = 0.043). Left ventricular dominance was associated with a better ejection fraction ( p = 0.04), less extent of late gadolinium enhancement ( p = 0.022), higher metabolic equivalents ( p = 0.01), and higher peak oxygen consumption ( p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support ( p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test., Conclusions: In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

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).)

The current use of intracorporeal left ventricular assist devices in children is still limited by small body dimensions. Many children weighing of less than 30 kg requiring durable mechanical circulatory support are implanted with the Berlin Heart EXCOR, a paracorporeal device. We present the case of a girl aged 10 years with a body surface area of 1.01 m 2 undergoing a safe and effective HeartMate3 implantation despite extremely small thoracic dimensions. Using computed tomography-derived three-dimensional (3D) reconstruction, it was possible to simulate several device positions finding the best HeartMate3 lodging. Simulation-guided pump placement was then obtained in the operating room. Normal HeartMate3 functioning was registered until heart transplant. Our experience shows that preoperative planning and virtual fitting simulation can be effective to assess safety of HeartMate3 implantation even in small children. The 3D reconstruction and simulation may help to increase the pool of children candidates for this device, even though a larger experience is needed to assess the risk profile of the HeartMate3 in such small patients., Competing Interests: Disclosure: The authors have no conflicts of interest to report., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASAIO.)

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).)

Cracking the selectivity-generality paradox is among the most pressing challenges in asymmetric catalysis. This obstacle prevents the immediate and successful translation of new methods to diverse small molecules. This is particularly rate-limiting for therapeutic development, where availability and structural diversity are often critical components of successful campaigns. Here we describe the union of generality-driven enantioselective catalysis and the preparation of diverse peptidomimetics. A single new organocatalyst provides high selectivity and substrate generality that is matched only by a combination of metal and organocatalysts. Within organocatalysis, this discovery breaks a 16-year monolithic paradigm, uncovering a powerful new scaffold for enantioselective reduction with behavior that suggests the recognition of a nitroethylene minimal catalaphile.

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.)

Introduction: Preformed antibodies against αGal in the human and the presence of αGal antigens on the tissue constituting the commercial bioprosthetic heart valves (BHVs, mainly bovine or porcine pericardium), lead to opsonization of the implanted BHV, leading to deterioration and calcification. Murine subcutaneous implantation of BHVs leaflets has been widely used for testing the efficacy of anti-calcification treatments. Unfortunately, commercial BHVs leaflets implanted into a murine model will not be able to elicit an αGal immune response because such antigen is expressed in the recipient and therefore immunologically tolerated., Methods: This study evaluates the calcium deposition on commercial BHV using a new humanized murine αGal knockout (KO) animal model. Furtherly, the anti-calcification efficacy of a polyphenol-based treatment was deeply investigated. By using CRISPR/Cas9 approach an αGal KO mouse was created and adopted for the evaluation of the calcific propensity of original and polyphenols treated BHV by subcutaneous implantation. The calcium quantification was carried out by plasma analysis; the immune response evaluation was performed by histology and immunological assays. Anti-αGal antibodies level in KO mice increases at least double after 2 months of implantation of original commercial BHV compared to WT mice, conversely, the polyphenols-based treatment seems to effectively mask the antigen to the KO mice's immune system., Results: Commercial leaflets explanted after 1 month from KO mice showed a four-time increased calcium deposition than what was observed on that explanted from WT. Polyphenol treatment prevents calcium deposition by over 99% in both KO and WT animals. The implantation of commercial BHV leaflets significantly stimulates the KO mouse immune system resulting in massive production of anti-Gal antibodies and the exacerbation of the αGal-related calcific effect if compared with the WT mouse., Discussion: The polyphenol-based treatment applied in this investigation showed an unexpected ability to inhibit the recognition of BHV xenoantigens by circulating antibodies almost completely preventing calcific depositions compared to the untreated counterpart., Competing Interests: FN, AG, and GS were employed by Biocompatibility Innovation Srl. AMC and MS were advisors for Biocompatibility Innovation. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The authors declare that this study received funding from Biocompatibility Innovation. The funder/advisors have the following involvement in the study: conception and design, writing of the original draft, project administration, and funding acquisition., (Copyright © 2023 Naso, Colli, Zilla, Calafiore, Lotan, Padalino, Sturaro, Gandaglia and Spina.)

Introduction: The overshoot of the respiratory exchange ratio (RER) after exercise is reduced in patients with heart failure., Aim: The present study aimed to investigate the presence of this phenomenon in young patients with congenital heart disease (CHD), who generally present reduced cardiorespiratory fitness., Methods: In this retrospective study, patients with CHD underwent a maximal cardiopulmonary exercise testing (CPET) assessing the RER recovery parameters: the RER at peak exercise, the maximum RER value reached during recovery, the magnitude of the RER overshoot and the linear slope of the RER increase after the end of the exercise., Results: In total, 117 patients were included in this study. Of these, there were 24 healthy age-matched control subjects and 93 young patients with CHD (transposition of great arteries, Fontan procedure, aortic coarctation and tetralogy of Fallot). All patients presented a RER overshoot during recovery. Patients with CHD showed reduced aerobic capacity and cardiorespiratory efficiency during exercise, as well as a lower RER overshoot when compared to controls. RER magnitude was higher in the controls and patients with aortic coarctation when compared to those with transposition of great arteries, previous Fontan procedure, and tetralogy of Fallot. The RER magnitude was found to be correlated with the most relevant cardiorespiratory fitness and efficiency indices., Conclusions: The present study proposes new recovery indices for functional evaluation in patients with CHD. Thus, the RER recovery overshoots analysis should be part of routine CPET evaluation to further improve prognostic risk stratifications in patients with CHD.

Objectives: A conclusive interpretation of the role of ventricular dominance in outcomes after Fontan palliation has not been formulated yet. We conducted a systematic review and meta-analysis of scientific literature to give an insight into the impact of ventricular morphology in single-ventricle palliation, focusing on its influence on survival, morbidities, ventricular performance and functional capacity., Methods: A systematic review of PubMed, Web of Science and Scopus databases was performed. A random-effect meta-analysis was conducted, and survival data were reconstructed using the published Kaplan-Meier survival curves., Results: Twenty-seven studies were selected, for a total of 4529 left-dominant versus 4844 right-dominant patients. Estimated survival at 1, 5, 10, 20 and 30 years of follow-up was 0.99 [95% confidence interval (CI) = 0.98-0.99], 0.95 [95% CI = 0.94-0.96], 0.92 [95% CI = 0.91-0.93], 0.86 [95% CI = 0.84-0.88] and 0.68 [95% CI = 0.65-0.83] for left-dominant patients and 0.94 [95% CI = 0.93-0.95], 0.89 [95% CI = 0.88-0.9], 0.85 [95% CI = 0.83-0.87], 0.69 [95% CI = 0.63-0.75] and 0.59 [95% CI = 0.5-0.69] for right-dominant patients, respectively. Survival was statistically lower for right-dominant patients (P < 0.001), with an hazard ratio for the mortality of 2.38 (2.03-2.80); also, they displayed significantly longer hospital stay, worse ventricular function, larger ventricular volumes and a higher incidence of moderate or severe atrioventricular valve regurgitation when compared to left-dominant patients., Conclusions: According to our meta-analysis, the morphology of the dominant ventricle has a significant impact on outcomes after Fontan palliation. Right-dominant patients experience an inferior long-term survival when the anatomical right ventricle is included in the systemic circulation., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are a challenge because of their various anatomic and clinical presentation. Although the prevalence is low, the absolute numbers of detected ACAOS are increasing because of the growing use of noninvasive anatomical imaging for ruling out coronary artery disease. As evidence-based guidelines are lacking, treating physicians are left in uncertainty for the optimal management of such patients. The sole presence of ACAOS does not justify surgical correction, and therefore a thorough anatomic and hemodynamic assessment is warranted. Invasive and noninvasive multimodality imaging provides information to the clinical question whether the presence of ACAOS is an innocent coincidental finding, is responsible for the patient's symptoms, or even might be a risk for sudden cardiac death. Based on recent clinical data, focusing on the pathophysiology of patients with ACAOS, myocardial ischemia is dependent on both the extent of fixed and dynamic components, represented by anatomic high-risk features. These varying combinations should be considered individually in the decision making for the different therapeutic options. This state-of-the-art review focuses on the advantages and limitations of the common contemporary surgical, interventional, and medical therapy with regard to the anatomy and pathophysiology of ACAOS. Further, we propose a therapeutic management algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge.

Current treatment paradigms for end-stage dilated cardiomyopathy (DCM) in children include heart transplantation and mechanical support devices. However, waitlist mortality, shortage of smaller donors, time-limited durability of grafts, and thrombo-hemorrhagic events affect long-term outcomes. Moreover, both these options are noncurative and cannot preserve the native heart function. Pulmonary artery banding (PAB) has been reinvented as a possible "regenerative surgery" to retrain the decompensated left ventricle in children with DCM. The rationale is to promote positive ventricular-ventricular interactions that result in recovery of left ventricular function in one out of two children, allowing transplantation delisting. Although promising, global experience with this technique is still limited, and several surgical centers are reluctant to adopt PAB since its exact biological bases remain unknown. In the present review, we summarize the clinical, functional, and molecular known and supposed working mechanisms of PAB in children with DCM. From its proven efficacy in the clinical setting, we described the macroscopic geometrical and functional changes in biventricular performance promoted by PAB. We finally speculated on the possible underlying molecular pathways recruited by PAB. An evidence-based explanation of the working mechanisms of PAB is still awaited to support wider adoption of this surgical option for pediatric heart failure., Competing Interests: The authors declare no conflicts of interest.

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: Many centres have recently adopted pulmonary valve (PV) preservation (PVP) during tetralogy of Fallot (ToF) repair. We sought to identify the midterm functional outcomes and risk factors for pulmonary regurgitation after this procedure., Methods: All patients undergoing PVP during transatrial-transpulmonary repair for ToF with PV stenosis at our institution between January 2007 and December 2020 were reviewed., Results: Overall, 73 patients were included. At the index surgery, the body surface area was 0.31 ± 0.04 m2, the age was 4.9 ± 2.9 months and the preoperative PV z-score was -3.02 ± 1.11. At a mean follow-up of 5.3 ± 2.7 years, the fractional area change of the right ventricle (RV) was 47.1 ± 5.2%, and the tricuspid annular plane systolic excursion z-score was -3.31 ± 1.89%. The 5-year freedom from moderate/severe PV regurgitation was 61.3% [95% confidence interval (CI): 48, 73%]. There was a significant correlation between RV function and moderate/severe PR at follow-up (R2: 0.08; P = 0.03). A comparison with a group of patients undergoing a transannular patch procedure (N = 33) showed superior outcomes for patients with PVP. The preoperative PV z-score and the degree of PR at discharge were risk factors for the early development of moderate/severe PR at follow-up [hazard ratio (HR): 0.64; 95% CI: 0.48, 0.86, P = 0.01 and HR: 2.31; 95% CI: 1.00, 5.36, P = 0.04, respectively]. A preoperative PV annulus z-score ≤ -2.85 was found to be predictive for moderate/severe PR at 5 years after PVP (HR: 2.56; 95% CI: 1.31, 5.01, P = 0.002)., Conclusions: A pulmonary valve preservation strategy during tetralogy of Fallot repair should always be attempted. However, a preoperative PV annulus z-score < -2.85 and moderate/severe regurgitation upon discharge are risk factors for midterm pulmonary regurgitation., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The aim of this study was to evaluate if a 'protective' (low-tidal/low-frequency) ventilation strategy can shorten the postoperative ventilation time and minimize acute lung injury in children with congenital heart disease (CHD) undergoing repair with cardiopulmonary bypass (CPB)., Methods: This is a single-centre prospective, interventional study, including children with CHD under the age of 5 years, undergoing open-heart surgery with a CPB >60 min, in hypothermia, haemodynamically stable, and without evident genetic abnormalities. Assist-control ventilation (tidal volume of 4 ml/kg, 10 breaths/min, positive end-expiratory pressure 5 cmH2O and FiO2 0.21) was applied in a cohort of patients during CPB. We compared clinical outcomes and in fully ventilated versus non-ventilated (control) patients. Propensity score was used to weigh ventilated and control groups to correct for the effect of other confounding clinical variables. Clinical and ventilation parameters and lung inflammatory biomarkers in tracheal aspirates were measured. The primary outcome was the postoperative intubation time of more or less than 48 h., Results: We included 140 children (53 ventilated, 87 non-ventilated) with different CHD. There were no deaths or adverse events in ventilated patients. Using a weighted generalized linear model, we found no sufficient evidence for an effect of intraoperative ventilation on postoperative intubation time [estimate 0.13 (95% confidence interval, -0.08; 0.35), P = 0.22]., Conclusions: Continuous low-tidal/low-frequency mechanical ventilation during CPB is safe and harmless. However, no significant advantages were found when compared to non-ventilated patients in terms of postoperative ventilation time., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

We conducted a systematic review and meta-analysis of the literature to assess the outcomes of surgery for the anomalous aortic origin of a coronary artery in children and young adults (<30 years). Thirteen publications were selected, including a total of 384 patients. Unroofing of the intramural segment was adopted in 92% (95% CI: 81%-98%) of cases, with pooled early and late mortality of 0% (95% CI: 0%-0.3%) and 0.1% (95% CI: 0%-1%), respectively. Reoperation for aortic regurgitation was anecdotal. Surgical management of anomalous aortic origin of coronary arteries can be achieved with excellent results in pediatric patients, but concerns remain about the durability of surgery.

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.)

To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in neonates. This is a retrospective clinical review of neonates with CoAo, who underwent surgery between 1995 and 2019. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by means of relative risk ratio and Cox and logistic multivariate analysis. 218 consecutive neonates (M/F: 129/89, median age 11 days, IQR 7-17 days) were included; 202 (92.7%) had a left thoracotomy; 178 underwent extended end-to-end anastomosis (EEEA, 81.6%). Hypoplastic aortic arch (HAA) was present in 102 patients (46.8%); complex cardiac anomalies in 85 (39%). Significant postoperative complications occurred in 20 (9.2%). Thirty-day mortality was 2.3% (most in complex types). At a median follow-up of 10.4 years (IQR 5.6-15.0 years; FU completeness 95.9%), there were 8 late deaths (3.7%), all associated to complex CoAo. Among 196 survivors, 177 (93.2%) were in NYHA class I; re-interventions on aortic arch occurred in 9.2% (2.0% were surgical). Freedom from mortality and re-intervention on aorta at 10 years were 94.3% and 96.7%, respectively. Surgical repair of CoAo in newborns without CPB in our series was safe and low-risk, with excellent early and late outcomes., (© 2021. The Author(s).)

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.)

Background: Brain injury, impaired brain maturation, and long-term neurodevelopmental disorders are common in infants with congenital heart diseases (CHD). We aimed to assess whether plasma glial fibrillary acidic protein (GFAP) can predict neurodevelopmental anomalies in CHD infants operated on cardiopulmonary bypass (CPB)., Methods: We measured plasma GFAP in 38 infants at multiple CPB phases. Cognitive, neuropsychological, and psychopathological functioning were assessed 5.7 ± 2.2 years after surgery. We identified an impaired global neurodevelopmental index (NDI) when at least two domains were abnormal. The relationships between NDI, GFAP, and clinical variables were explored with non-supervised feature selection methods and modeled with a nested non-linear logistic regression., Results: Intelligence quotient scores were within the normal range in 84% of children, whereas 58% showed an abnormal NDI, with the greatest impairments in the psychopathological area. The plasma GFAP peak was 0.95 (0.44-1.57) ng/mL, and it was correlated with age, weight, duration of surgery phases, and CPB minimum temperature. In the regression model, the GFAP peak was associated with an impaired NDI with a possible flexible point toward NDI impairment at 0.49 ng/mL, keeping constant ICU stay, CPB duration, CHD anatomy, weight, and CPB minimum temperature., Conclusion: GFAP is a promising early marker of abnormal long-term neuropsychological development.

Anomalous aortic origin of a coronary artery (AAOCA) is reported as the second leading cause of sudden cardiac death in otherwise healthy young individuals. Several surgical studies have reported a shallow operative risk, describing repair as safe and effective with short or medium-term follow-up. However, surgical repair can also be associated with a high risk of complications. Numerous repair techniques have been described in the literature, but each technique's indications and limitations are often not well-understood or understated. Since explicit technical knowledge of the most appropriate surgical technique is highly desirable, we sought to thoroughly and clearly outline the safeguards and pitfalls of the most common surgical techniques used to repair AAOCA., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Padalino, Jegatheeswaran, Blitzer, Ricciardi and Guariento.)

Aim: In complex congenital heart diseases (CHD), patients may remain affected by significant morbidity and mortality after surgery. We analysed the end-of-life (EoL) care in children with severe CHD who died in our institution and investigated perspectives of parents and health-care professionals (HCPs)., Methods: Medical records of all children (age < 18 years old) affected by a severe CHD who died in a tertiary cardiac care centre were reviewed. Subsequently, a cross-sectional questionnaire-based study of parents and HCPs of children involved in the study was designed., Results: In total, 30 children died (median age: 45 days; range: 15 days to 3.4 years). Of them, 97% (31/32) died in an intensive care unit setting and were intubated and sedated at EoL. A total of 77% (23/30) died without parents being present at bedside. Eighteen families and 10 HCPs were interviewed. For 61% of the parents (11/18) and 70% of the clinicians (7/10), the goal of therapy at the EoL was 'to lessen your child's suffering as much as possible'. Overall, 44% of parents (8/18) and 50% of HCPs recognised that their child had no chance of survival 'a few days before the child died'., Conclusions: We believe that these data suggest an unconscious reluctance to change goals of care in EoL, shifting from intensive care to comfort and quality of life., (© 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

The role played by the right ventricular (RV) dysfunction has long been underestimated in clinical practice. Recent findings are progressively confirming that when the RV efficiency deteriorates both the right and the left circulation is (significantly) affected, but studies dedicated to a detailed description of RV hemodynamic role still lack. In response to such a gap in knowledge, this work proposes a numerical model that for the first time evaluates the effect of isolated RV dysfunction on the whole circulation. Lumped parameter modelling was applied to represent the physio-pathological hemodynamics. Different grades of impairment were simulated for three dysfunctions i.e., systolic, diastolic, and combined systolic and diastolic. Hemodynamic alterations (i.e., of blood pressure, flow, global hemodynamic parameters), arising from the dysfunctions, are calculated and analysed. Results well accord with clinical observations, showing that RV dysfunction significantly affects both the pulmonary and systemic hemodynamics. Successful verification against in vivo data proved the clinical potentiality of the model i.e., the capability of identifying the degree of RV impairment for given hemodynamic conditions. This study aims at contributing to the improvement of RV dysfunction recognition and treatment, and to the development of tools for the clinical management of pathologies involving the right heart.

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.)