Nationwide hospitalizations of patients with down syndrome and congenital heart disease over a 15-year period.

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.

Enhancing parental understanding of congenital heart disease through personalized prenatal counseling with 3D printed hearts.

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.

Intracorporeal LVAD implantation in pediatric patients: A single-center 10 years' experience.

BACKGROUND: Mechanical cardiac support is currently an effective strategy to reduce morbidity and mortality in pediatric patients. However, solid evidence regarding the feasibility of intracorporeal devices in children still needs to be provided. We report our 10-year experience with intracorporeal left ventricular assist devices (LVAD) in children. MATERIALS AND METHODS: We included all patients undergoing intracorporeal, continuous-flow LVAD implantation between 2012 and 2022. Baseline and postoperative data were collected from the institutional database. RESULTS: Seven HeartWare and 4 HeartMate3 were implanted in 11 patients (median age 13.9 years, median body surface area - BSA - 1.42 m2, IQR 1.06-1.68). The most frequent indication to LVAD implant was dilated cardiomyopathy (72.7%). All candidates underwent a thorough preoperative advanced imaging. Three-dimensional reconstructions and implant fit simulation were performed when BSA was <1.2 m2, weight <30 kg, or internal transverse thoracic diameter <20 cm. There was no operative death. The most common postoperative complication was surgical re-exploration due to bleeding (27.3%). One patient died of severe neurological complications after about 3 months of hospitalization. No late deaths or unplanned re-hospitalizations occurred in the remaining 10, 6 of whom were discharged home. There were no major complications at the follow-up. All survivors underwent successful heart transplantation. CONCLUSIONS: Intracorporeal LVAD implantation proved to be a potentially feasible and safe option in young teenagers and children whose BSA was >1.0 m2. In borderline cases, the 3D reconstruction with implant fit simulation can effectively help to identify those patients who can safely undergo intrathoracic LVAD implantation.

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.

Residual stenosis after right ventricle outflow tract surgery represents a major issue to manage in the children and adult patient with conotruncal defects. Despite a detailed multimodality imaging, the anatomy of distal pulmonary trunk and pulmonary artery bifurcation may be challenging in these patients.The aim of this study was to analyse retrospectively the outcome of the percutaneous transcatheter treatment in children with post-surgical stenosis of pulmonary artery bifurcation.We enrolled 39 patients with a median age of 6.0 years. Standard high-pressure balloon dilation was attempted in 33 patients, effective in 5 of them. Pulmonary branch stenting was performed in 10 patients, effective in 6. A kissing balloon approach was chosen in 17 patients (6 after angioplasty or stenting failure), and this technique was effective in 16 cases. Finally, a bifurcation stenting was performed in 10 patients (second step in 9 cases), effective in all the cases. None of the patients approached by kissing balloon needed a bifurcation stenting.In conclusion, standard balloon angioplasty and standard stenting might be ineffective in post-surgical stenosis involving pulmonary artery bifurcation. In this population, kissing balloon or bifurcation stenting, followed by side branch de-jailing, may be more effective in relieving the gradient.

Matched comparison of decellularized homografts and bovine jugular vein conduits for pulmonary valve replacement in congenital heart disease.

For decades, bovine jugular vein conduits (BJV) and classic cryopreserved homografts have been the two most widely used options for pulmonary valve replacement (PVR) in congenital heart disease. More recently, decellularized pulmonary homografts (DPH) have provided an alternative avenue for PVR. Matched comparison of patients who received DPH for PVR with patients who received bovine jugular vein conduits (BJV) considering patient age group, type of heart defect, and previous procedures. 319 DPH patients were matched to 319 BJV patients; the mean age of BJV patients was 15.3 (SD 9.5) years versus 19.1 (12.4) years in DPH patients (p = 0.001). The mean conduit diameter was 24.5 (3.5) mm for DPH and 20.3 (2.5) mm for BJV (p < 0.001). There was no difference in survival rates between the two groups after 10 years (97.0 vs. 98.1%, p = 0.45). The rate of freedom from endocarditis was significantly lower for BJV patients (87.1 vs. 96.5%, p = 0.006). Freedom from explantation was significantly lower for BJV at 10 years (81.7 vs. 95.5%, p = 0.001) as well as freedom from any significant degeneration at 10 years (39.6 vs. 65.4%, p < 0.001). 140 Patients, matched for age, heart defect type, prior procedures, and conduit sizes of 20-22 mm (± 2 mm), were compared separately; mean age BJV 8.7 (4.9) and DPH 9.5 (7.3) years (p = n.s.). DPH showed 20% higher freedom from explantation and degeneration in this subgroup (p = 0.232). Decellularized pulmonary homografts exhibit superior 10-year results to bovine jugular vein conduits in PVR.

Effect of preoperative pulmonary hemodynamic and cardiopulmonary bypass on lung function in children with congenital heart disease.

In children with congenital heart disease (CHD), pulmonary blood flow (Qp) contributes to alterations of pulmonary mechanics and gas exchange, while cardiopulmonary bypass (CPB) induces lung edema. We aimed to determine the effect of hemodynamics on lung function and lung epithelial lining fluid (ELF) biomarkers in biventricular CHD children undergoing CPB. CHD children were classified as high Qp (n = 43) and low Qp (n = 17), according to preoperative cardiac morphology and arterial oxygen saturation. We measured ELF surfactant protein B (SP-B) and myeloperoxidase activity (MPO) as indexes of lung inflammation and ELF albumin as index of alveolar capillary leak in tracheal aspirate (TA) samples collected before surgery and in 6 hourly intervals within 24 h after surgery. At the same time points, we recorded dynamic compliance and oxygenation index (OI). The same biomarkers were measured in TA samples collected from 16 infants with no cardiorespiratory diseases at the time of endotracheal intubation for elective surgery. Preoperative ELF biomarkers in CHD children were significantly increased than those found in controls. In the high Qp, ELF MPO and SP-B peaked 6 h after surgery and tended to decrease afterward, while they tended to increase within the first 24 h in the low Qp. ELF albumin peaked 6 h after surgery and decreased afterwards in both CHD groups. Dynamic compliance/kg and OI significantly improved after surgery only in the High Qp.  Conclusion: In CHD children, lung mechanics, OI, and ELF biomarkers were significantly affected by CPB, according to the preoperative pulmonary hemodynamics. What is Known: • Congenital heart disease children, before cardiopulmonary run, exhibit changes in respiratory mechanics, gas exchange, and lung inflammatory biomarkers that are related to the preoperative pulmonary hemodynamics. • Cardiopulmonary bypass induces alteration of lung function and epithelial lining fluid biomarkers according to preoperative hemodynamics. What is New: • Our findings can help to identify children with congenital heart disease at high risk of postoperative lung injury who may benefit of tailored intensive care strategies, such as non-invasive ventilation techniques, fluid management, and anti-inflammatory drugs that can improve cardiopulmonary interaction in the perioperative period.

Tricuspid Valve Repair Can Restore the Prognosis of Patients with Hypoplastic Left Heart Syndrome and Tricuspid Valve Regurgitation: A Meta-analysis.

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.

Hybrid approach for end-stage heart failure treatment in a 6-month-old baby.

A 6-month-old baby girl, with a history of 2 months of intensive care management and two episodes of cardiac arrest, was transferred from another European country to initiate the "Giessen approach" for end-stage heart failure in children. At the admission, left ventricular ejection fraction was 20%. Severe mitral valve regurgitation and severe left atrial dilatation were present. Right ventricular function was preserved, and tricuspid valve regurgitation was mild. As a result, the patient underwent surgical pulmonary artery banding. Additionally, unloading of the left atrium was achieved by implanting an 8 mm atrial flow regulator device through a hybrid per-atrial approach. Two months after the procedure, the patient was progressively weaned from the inotropes and transferred to the ward.

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.

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.

Pediatric COVID-19 extracorporeal membrane oxygenation transport during the pandemic.

INTRODUCTION: ExtraCorporeal Membrane Oxygenation (ECMO) in pediatric patients with COVID-19 has a survival rate similar to adults. Occasionally, patients may need to be cannulated by an ECMO team in a referring hospital and transported to an ECMO center. The ECMO transport of a COVID-19 patient has additional risks than normal pediatric ECMO transport for the possible COVID-19 transmissibility to the ECMO team and the reduction of the ECMO team performance due to the need of wearing full personal protective equipment. Since pediatric data on ECMO transport of COVID-19 patients are lacking, we explored the outcomes of the pediatric COVID-19 ECMO transports collected in the EuroECMO COVID_Neo/Ped Survey. METHODS: We reported five European consecutive ECMO transports of COVID-19 pediatric patients collected in the EuroECMO COVID_Neo/Ped Survey including 52 European neonatal and/or pediatric ECMO centers and endorsed by the EuroELSO from March 2020 till September 2021. RESULTS: The ECMO transports were performed for two indications, pediatric ARDS and myocarditis associated to the multisystem inflammatory syndrome related to COVID-19. Cannulation strategies differed among patients according to the age of the patients, transport distance varied between 8 and 390 km with a total transport duration between 5 to 15 h. In all five cases, the ECMO transports were successfully performed without major adverse events. One patient reported a harlequin syndrome and another patient a cannula displacement both without major clinical consequences. Hospital survival was 60% with one patient reporting neurological sequelae. No ECMO team member developed COVID-19 symptoms after the transport. CONCLUSION: Five transports of pediatric patients with COVID-19 supported with ECMO were reported in the EuroECMO COVID_Neo/Ped Survey. All transports were performed by an experienced multidisciplinary ECMO team and were feasible and safe for both the patient and the ECMO team. Further experiences are needed to better characterize these transports and draw insightful conclusions.

3D Reconstruction for a Complex Pediatric Redo Aortic Arch Surgery in a 9-Year-Old Ukrainian Refugee.

Redo aortic surgery poses significant challenges, particularly in complex scenarios involving congenital heart conditions that have been previously operated on several years prior. The integration of three-dimensional (3D) reconstruction and printing holds immense potential to greatly improve surgical precision, particularly in critical situations.

Decellularized cryopreserved human pericardium: a validation study towards tissue bank practice.

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.

Unveiling the gothic aortic arch and cardiac mechanics: insights from young patients after arterial switch operation for d-transposition of the great arteries.

The arterial switch operation (ASO) has become the standard surgical treatment for patients with d-transposition of the great arteries. While ASO has significantly improved survival rates, a subset of patients develop a unique anatomical anomaly known as the gothic aortic arch (GAA). Understanding cardiac mechanics in this population is crucial, as altered mechanics can have profound consequences for cardiac function and exercise capacity. The GAA has been associated with changes in ventricular function, hemodynamics, and exercise capacity. Studies have shown a correlation between the GAA and decreased ascending aorta distensibility, loss of systolic wave amplitude across the aortic arch, and adverse cardiovascular outcomes. Various imaging techniques, including echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography, play a crucial role in assessing cardiac mechanics and evaluating the GAA anomaly. Despite significant advancements, gaps in knowledge regarding the prognostic implications and underlying mechanisms of the GAA anomaly remain. This review aims to explore the implications of the GAA anomaly on cardiac mechanics and its impact on clinical outcomes in young patients after ASO. Advancements in imaging techniques, such as computational modeling, offer promising avenues to enhance our understanding of cardiac mechanics and improve clinical management.

The valuable interaction among cardiac surgeon and electrophysiologist for transvenous rotational mechanical lead extraction.

BACKGROUND: Recent studies have shown that Evolution RL bidirectional rotational mechanical sheath (Cook Medical, USA) is an effective and safe technique for transvenous lead extraction (TLE). We reported our experience with the bidirectional rotational mechanical tools using a multidisciplinary approach highlighting the value of a joint cardiac surgeon and electrophysiologist collaboration. METHODS: The study population comprised 84 patients (77% male; mean age 65 ± 18 years) undergoing TLE. After a multidisciplinary evaluation, a combined procedure was considered. RESULTS: The main indication for TLE was infection in 54 cases (64%). Overall, 152 leads were extracted with a mean implant duration of 94 ± 63 months (range 12-421). Complete procedural success rate, clinical success rate, and lead removal with clinical success rate were 91.6% (77/84), 97.6% (82/84), and 98.6% (150/152), respectively. Eighteen combined procedures were performed in 12 patients (14%), such as "hybrid approach" (n = 2) or TLE concomitant to: 1) transcatheter aspiration procedure for large vegetation (n = 8); 2) left ventricular assistance device implantation as bridge to cardiac transplantation (n = 1); 3) permanent pacing with epicardial leads (n = 6); 4) tricuspid valve replacement (n = 1). One major complication (1.2%) and 11 (13%) minor complications were encountered. No injury to the superior vena cava occurred and no procedure-related deaths were reported. During a mean time follow-up of 21 ± 18 months, 17 patients (20%) died. They were more often diabetics (p = .02), and they underwent TLE more often for infection (p = .004). CONCLUSIONS: Our results support the finding that excellent outcomes can be achieved in performing TLE of chronically implanted leads by using the Evolution RL bidirectional rotational mechanical sheath and a multidisciplinary team approach involving both electrophysiologist and cardiac surgeon as first line operators.

Rescue ross procedure and mitral valve repair on a low birth weight preterm neonate.

Although mid- and long-term outcomes after the Ross procedure for aortic valve disease have been increasingly improving over the years, this is still a rather challenging operation in neonates and small children. This is particularly true for patients with associated congenital heart defects and critical clinical conditions. Herein we describe the application of this procedure as a rescue operation in emergency circumstances in a low-birth-weight neonate with severe aortic stenosis, aortic regurgitation and mitral regurgitation after a previous aortic coartectomy.

Effectiveness of Repair of Aortic Coarctation in Neonates: A Long-Term experience.

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.

Cardiopulmonary bypass-induced coagulopathy in pediatric patients: The role of platelets in postoperative bleeding. A preliminary study.

Pediatric patients are particularly prone to cardiopulmonary bypass (CPB)-induced coagulopathy mainly due to hemodilution, consumption of coagulation factors and hypothermia. The aim of the present study was to examine the possible role of platelet count and function as it relates to the bleeding risk after CPB in the pediatric population. All consecutive patients (age <13 years) scheduled for elective cardiac surgery between January 2019 and November 2019 were retrospectively considered for the study. We gathered demographic characteristics, perioperative laboratory data (mainly platelet count and function), transfusion requirements, and blood loss for each patient. Patients with a chest tube output during the first 24 hours after surgery >75th percentile were bleeders (cases). Controls were nonbleeders. A total of 31 patients were enrolled [median age 17 (4-57) months]. A significant postoperative reduction in platelet count (P < .001) and function either in ADP-test (P < .001), TRAP-test (P < .001) and ASPI-test (P < .001) was found, with positive correlations between chest tube output within the first 24 hours after surgery and postoperative impairment of platelet count (R = 0.553, P = .001), ADP-test (R = 0.543, P = .001), TRAP-test (R = 0.627, P < .001) and ASPI-test (R = 0.436, P = .014). Eight children (26%) experienced major postoperative bleeding. Bleeders were significantly younger (P = .015) and underwent longer CPB duration (P = .015). Despite no significant differences in postoperative platelet count and function between cases and controls, the postoperative reduction (Δ) in platelet count (P = .002) and function in ADP-test (P = .007), TRAP-test (P = .020) and ASPI-test (P = .042) was significantly greater in bleeders vs. nonbleeders. A ΔPLT >262 500 ×109 /L, a ΔADP-test >29 U, a ΔTRAP-test >44 U and a ΔASPI-test >26 U showed to be predictive of major postoperative bleeding. Postoperative bleeding in children undergoing cardiac surgery with CPB was linked to younger age, longer CPB duration, and significant postoperative reduction in platelet count and function. Larger studies are needed to confirm our results and define strategies to reduce postoperative bleeding in these patients.

Surgical strategies for the management of end-stage heart failure in infants and children: A 15-year experience with a patient-tailored approach.

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.

Fast-track virtual reality for cardiac imaging in congenital heart disease.

BACKGROUND AND AIM OF THE STUDY: We sought to evaluate the appropriateness of cardiac anatomy renderings by a new virtual reality (VR) technology, entitled DIVA, directly applicable to raw magnetic resonance imaging (MRI) data without intermediate segmentation steps in comparison to standard three-dimensional (3D) rendering techniques (3D PDF and 3D printing). Differences in post-processing times were also evaluated. METHODS: We reconstructed 3D (STL, 3D-PDF, and 3D printed ones) and VR models of three patients with different types of complex congenital heart disease (CHD). We then asked a senior pediatric heart surgeon to compare and grade the results obtained. RESULTS: All anatomical structures were well visualized in both VR and 3D PDF/printed models. Ventricular-arterial connections and their relationship with the great vessels were better visualized with the VR model (Case 2); aortic arch anatomy and details were also better visualized by the VR model (Case 3). The median post-processing time to get VR models using DIVA was 5 min in comparison to 8 h (range 8-12 h including printing time) for 3D models (PDF/printed). CONCLUSIONS: VR directly applied to non-segmented 3D-MRI data set is a promising technique for 3D advanced modeling in CHD. It is systematically more consistent and faster when compared to standard 3D-modeling techniques.