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.

Impact of age over 70 years in the new allocation system on the outcomes of heart transplantation in the US.

BACKGROUND: United Network for Organ Sharing (UNOS) allocation criteria changed in 2018 to accommodate the increased prevalence of patients on a ventricular assist device as a bridge to heart transplant and prioritize sicker people in anticipation of a heart graft. We aimed to assess the impact of patient age in the new allocation policy on mortality following heart transplantation. Secondary outcomes included the effect of age ≥70 on post-transplant events, including stroke, dialysis, pacemaker, and rejection requiring treatment. METHODS: The UNOS Registry was queried to identify patients who underwent heart transplants alone in the US between 2000 and 2021. Patients were divided into groups according to their age (over 70 and under 70 years old). RESULTS: Patients aged over 70 were more likely to require dialysis during follow-up, but less likely to experience rejection requiring treatment, compared with patients aged <70. Age ≥70 in the new allocation system was a significant predictor of 1-year mortality (adjusted HR: 1.41; 95% CI: 1.05-1.91; p = .024), but its effect on 5-year mortality was not significant after adjusting for potential confounders (adjusted HR: 1.27; 95% CI:.97-1.66; p = .077). Undergoing transplantation under the new allocation policy vs the old allocation policy was not a significant predictor of mortality in patients over 70 years old. CONCLUSIONS: Age ≥70 is a significant predictor of 1-year mortality following heart transplantation, but not at 5 and 10 years; however, the new allocation does not seem to have changed the outcomes for this group of patients.

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.

The introduction of surgical simulation on three-dimensional-printed models in the cardiac surgery curriculum: an experimental project.

AIMS: Training in congenital cardiac surgery has become more and more difficult because of the reduced opportunities for trainees in the operating room and the high patient anatomical variability. The aim of this study was to perform a pilot evaluation of surgical simulation on a simple 3D-printed heart model in training of young surgeons and its potential inclusion in the curriculum of residency programs. METHODS: A group of 11 residents performed a surgical correction of aortic coarctation using a 3D-printed surgical model. After teaching the surgical procedure, a simulation was performed twice, at different times, and was evaluated quantitatively and qualitatively by a senior surgeon. A 3D model-based training program was then developed and incorporated into our cardiac surgery training program. RESULTS: A significant improvement in surgical technique was observed between the first and second surgical simulations: median of 65% [interquartile range (IQR) = 61-70%] vs. 83% (IQR = 82-91%, P < 0.001). The median time required to run the simulation was significantly shorter during the second simulation: 39 min (IQR = 33-40) vs. 45 min (IQR = 37-48; P = 0.02). Regarding the simulation program, a basic and an advanced program were developed, including a total of 40 different simulated procedures divided into 12 sessions. CONCLUSION: Surgical simulation using 3D-printing technology can be an extremely valuable tool to improve surgical training in congenital heart disease. Our pilot study can represent the first step towards the creation of an integrated training system on 3D-printed models of congenital and acquired heart diseases in other Italian residency programs.

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.

Impact of new allocation system on length of stay following heart transplantation in the United States.

BACKGROUND: United Network for Organ Sharing (UNOS) allocation criteria changed in 2018 to accommodate the increased prevalence of ventricular assist device use as a bridge to heart transplant, which consequently prioritized sicker patients. We aimed to assess the impact of this new allocation policy on the length of stay following heart transplantation. Secondary outcomes include other risk factors for prolonged hospitalization and its effect on mortality and postoperative complications. METHODS: The UNOS Registry was queried to identify patients who underwent isolated heart transplants in the United States between 2001 and 2023. Patients were divided into quartiles according to their respective length of stay. RESULTS: A total of 57 020 patients were included, 15 357 of which were allocated with the new system. The median hospital length of stay was 15 days (mean 22.7 days). Length of stay was longer in the new allocation era (25 ± 30 vs. 22 ± 27 days, p < .001). The longer length of stay was associated with increased 5-year mortality in the new allocation system (aHR: 1.18; 95% CI: 1.15, 1.20; p-value: < .001). CONCLUSION: Longer hospital stays and associated observed increased risk for mortality in the era after the allocation criteria change reflect the rationale of this shift which was to prioritize heart transplants for sicker patients. Further studies are needed to track the progress of surgical and perioperative management of these studies over time.

The Impact of Congenital Cardiac Surgery Fellowship on Training and Practice.

BACKGROUND: In 2007, congenital cardiac surgery became a recognized fellowship by the American Council of Graduate Medical Education (ACGME). Beginning in 2023, the fellowship transitioned from a 1-year to a 2-year program. Our objective is to provide current benchmarks by surveying current training programs and assessing characteristics contributing to career success. METHODS: This was a survey-based study in which tailored questionnaires were distributed to program directors (PDs) and graduates of the ACGME accredited training programs. Data collection included responses to multiple-choice and open-ended questions relevant to didactics, operative training, training center characteristics, mentorship, and employment characteristics. Results were analyzed using summary statistics and subgroup and multivariable analyses. RESULTS: The survey yielded responses from 13 of 15 PDs (86%) and 41 of 101 graduates (41%) from ACGME accredited programs. Perceptions among PDs and graduates were somewhat discordant, with PDs more optimistic than graduates. Of PDs, 77% (n = 10) believed current training adequately prepares fellows and is successful in securing employment for graduates. The responses from graduates demonstrated 30% (n = 12) were dissatisfied with operative experience and 24% (n = 10) with overall training. Being supported during the first 5 years of practice was significantly associated with retention in congenital cardiac surgery and greater practicing case volumes. CONCLUSIONS: Dichotomous views exist between graduates and PDs regarding success in training. Mentorship during the early career was associated with increased case volumes, career satisfaction, and retention in the congenital cardiac surgery field. Educational bodies should incorporate these elements during training and after graduation.

Fast-track virtual reality software to facilitate 3-dimensional reconstruction in congenital heart disease.

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.

Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects.

Objective: Restoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP. Methods: This is a single-institution, retrospective cohort study of uAVCD patients who underwent BIVC after SVP, with staged recruitment (staged) or primary BIVC (direct) between 2003 to 2018. Mortality, unplanned reinterventions, imaging, and catheterization data were analyzed. Results: Sixty-five patients underwent BIVC from SVP (17 stage 1, 42 bidirectional Glenn, and 6 Fontan). Decision for conversion was based on poor SVP candidacy (n = 43) or 2 adequately sized ventricles (n = 22). Of the 65 patients, 20 patients underwent recruitment before conversion. The staged group had more severe ventricular hypoplasia than the direct group, reflected in prestaging end-diastolic volume z scores (-4.0 vs -2.6; P < .01), which significantly improved after recruitment (-4.0 to -1.8; P < .01). Median follow-up time was 1.0 years. Survival and recatheterizations were similar between both groups (hazard ratio, 0.9; 95% CI, 0.2-3.7; P = .95 and hazard ratio, 1.9; 95% CI, 0.9-4.1; P = .09), but more reoperations occurred with staged approach (hazard ratio, 3.1; 95% CI, 1.3-7.1; P = .01). Conclusions: Biventricular conversion from SVP is an alternative strategy to manage uAVCD, particularly when risk factors for SVP failure are present. Severe forms of uAVCDs can be converted with staged BIVC with acceptable mortality, albeit increased reinterventions, when primary BIVC is not possible.

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.

Prenatal Diagnosis and Fetopsy Validation of Complete Atrioventricular Septal Defects Using the Fetal Intelligent Navigation Echocardiography Method.

(1) Background: Artificial Intelligence (AI) is a modern tool with numerous applications in the medical field. The case series reported here aimed to investigate the diagnostic performance of the fetal intelligent navigation echocardiography (FINE) method applied for the first time in the prenatal identification of atrioventricular septal defects (AVSD). This congenital heart disease (CHD) is associated with extracardiac anomalies and chromosomal abnormalities. Therefore, an early diagnosis is essential to advise parents and make adequate treatment decisions. (2) Methods: Four fetuses diagnosed with AVSD via two-dimensional (2D) ultrasound examination in the second trimester were enrolled. In all cases, the parents chose to terminate the pregnancy. Since the diagnosis of AVSD with 2D ultrasound may be missed, one or more four-dimensional (4D) spatiotemporal image correlation (STIC) volume datasets were obtained from a four-chamber view. The manual navigation enabled by the software is time-consuming and highly operator-dependent. (3) Results: FINE was applied to these volumes and nine standard fetal echocardiographic views were generated and optimized automatically, using the assistance of the virtual intelligent sonographer (VIS). Here, 100% of the four-chamber views, and after the VISA System application the five-chamber views, of the diagnostic plane showed the atrioventricular septal defect and a common AV valve. The autopsies of the fetuses confirmed the ultrasound results. (4) Conclusions: By applying intelligent navigation technology to the STIC volume datasets, 100% of the AVSD diagnoses were detected.

Three-Dimensional-Enabled Surgical Planning for the Correction of Right Partial Anomalous Pulmonary Venous Return.

Objectives: The surgical technique for right partial anomalous pulmonary venous return (PAPVR) depends on the location of the anomalous pulmonary veins (PVs). With this in mind, we sought to evaluate the impact of 3D heart segmentation and reconstruction on preoperative surgical planning. Methods: A retrospective study was conducted on all patients who underwent PAPVR repair at our institution between January 2018 and October 2021; three-dimensional segmentations and reconstructions of all the heart anatomies were performed. A score (the PAPVR score) was established and calculated using two anatomical parameters (the distance between the most cranial anomalous PV and the superior rim of the sinus venosus defect/the sum of the latter and the distance between the PV and the azygos vein) to predict the type of correction. Results: A total of 30 patients were included in the study. The PAPVR score was found to be a good predictor of the type of surgery performed. A value > 0.68 was significantly associated with a Warden procedure (p < 0.001) versus single/double patch repair. Conclusions: Three-dimensional heart segmentations and reconstructions improve the quality of surgical planning in the case of PAPVR and allow for the introduction of a score that may facilitate surgical decisions on the type of repair required.

Rejection Requiring Treatment within the First Year following Heart Transplantation: The UNOS Insight.

(1) Background: Heart failure is an extremely impactful health issue from both a social and quality-of-life point of view and the rate of patients with this condition is destined to rise in the next few years. Transplantation remains the mainstay of treatment for end-stage heart failure, but a shortage of organs represents a significant problem that prolongs time spent on the waiting list. In view of this, the selection of donor and recipient must be extremely meticulous, considering all factors that could predispose to organ failure. One of the main considerations regarding heart transplants is the risk of graft rejection and the need for immunosuppression therapy to mitigate that risk. In this study, we aimed to assess the characteristics of patients who need immunosuppression treatment for rejection within one year of heart transplantation and its impact on mid-term and long-term mortality. (2) Methods: The United Network for Organ Sharing (UNOS) Registry was queried to identify patients who solely underwent a heart transplant in the US between 2000 and 2021. Patients were divided into two groups according to the need for anti-rejection treatment within one year of heart transplantation. Patients' characteristics in the two groups were assessed, and 1 year and 10 year mortality rates were compared. (3) Results: A total of 43,763 patients underwent isolated heart transplantation in the study period, and 9946 (22.7%) needed anti-rejection treatment in the first year. Patients who required treatment for rejection within one year after transplant were more frequently younger (49 ± 14 vs. 52 ± 14 years, p < 0.001), women (31% vs. 23%, p < 0.001), and had a higher CPRA value (14 ± 26 vs. 11 ± 23, p < 0.001). Also, the rate of prior cardiac surgery was more than double in this group (27% vs. 12%, p < 0.001), while prior LVAD (12% vs. 11%, p < 0.001) and IABP (10% vs. 9%, p < 0.01) were more frequent in patients who did not receive anti-rejection treatment in the first year. Finally, pre-transplantation creatinine was significantly higher in patients who did not need treatment for rejection in the first year (1.4 vs. 1.3, p < 0.01). Most patients who did not require anti-rejection treatment underwent heart transplantation during the new allocation era, while less than half of the patients who required treatment underwent transplantation after the new allocation policy implementation (65% vs. 49%, p < 0.001). Patients who needed rejection treatment in the first year had a higher risk of unadjusted 1 year (HR: 2.25; 95% CI: 1.88-2.70; p < 0.001), 5 year (HR: 1.69; 95% CI: 1.60-1.79; p < 0.001), and 10 year (HR: 1.47; 95% CI: 1.41-1.54, p < 0.001) mortality, and this was confirmed at the adjusted analysis at all three time-points. (4) Conclusions: Medical treatment of acute rejection was associated with significantly increased 1 year mortality compared to patients who did not require anti-rejection therapy. The higher risk of mortality was confirmed at a 10 year follow-up. Further studies and newer follow-up data are required to investigate the role of anti-rejection therapy in the heart transplant population.

Standard versus rapid-deployment aortic valve replacement and concomitant myocardial revascularization: 5-year bi-centre clinical outcomes.

OBJECTIVES: Clinical outcomes of 2 generations of pericardial bioprostheses in concomitant aortic valve and coronary artery bypass graft surgery were analysed. METHODS: Patients were recruited from 2 European centres and divided into 2 groups based on the type of aortic bioprosthesis used: Edwards Intuity Elite™ rapid-deployment (RD) bioprostheses or standard Edwards Magna Ease (ME). A propensity score weighting approach was used for data analysis. RESULTS: A total of 285 patients were included: 144 (50.5%) in the RD group and 141 (49.5%) in the ME group. Thirty-day mortality was 2.8% (RD) and 5% (ME) (P = 0.09). Significantly shorter times of aortic cross-clamp and cardiopulmonary bypass were observed in the RD cohort [94 vs 120 min (P < 0.001); 128 vs 160 min (P < 0.001)]. The RD group was associated with a lower median transvalvular gradient at discharge and follow-up (both P < 0.001). However, 5-year survival was not different, being 93% in RD patients and 91% in the ME group [hazard ratio 0.89 (95% confidence interval: 0.38-2.09), P = 0.784]. The 5-year cumulative incidence of combined events (including percutaneous coronary interventions, endocarditis, thromboembolic events, rehospitalizations and bleeding) favoured the ME group [16.1% (RD) vs 7.3% (ME)] [hazard ratio 2.38 (95% confidence interval:1.03-5.52), P = 0.043]. However, this turned similar when the Cox model analysis was adjusted for revascularization variables (P = 0.067). CONCLUSIONS: RD and ME pericardial bioprostheses used in concomitant aortic valve replacement and coronary artery revascularization provide equivalent clinical and haemodynamic 5-year outcomes, despite constant lower transvalvular gradients and shorter surgical operating times observed with RD technology.

Three-Dimensional Printing Application in a Challenging Case of Type II Endoleak.

PURPOSE: To highlight the importance of 3-dimensional (3D) arterial printing in a case of type II endoleak (EL) embolization. CASE REPORT: An 81-year-old patient, previously treated with endovascular aortic repair (EVAR), developed a type II EL requiring treatment. The EL's main origin was the median sacral artery (MSA). Initial attempts in embolization via a transsealing and transarterial approach were unsuccessful owing to extremely tortuous arterial communications between the left hypogastric artery and the MSA. The construction of a clear resin 3D model of the aorta and iliolumbar arteries improved anatomy understanding and moreover allowed a preoperative simulation. The subsequent transarterial attempt in embolization was resolutive, significantly reducing total procedural time and radiation dose. CONCLUSION: Printing of clear resin 3D arterial models facilitates type II EL transarterial embolization, improving anatomy understanding and allowing simple fluoroscopy-free simulations. CLINICAL IMPACT: The aim of our work is to highlight the additional value of three-dimensional (3D) printing during preoperative planning of challenging endovascular cases. To our best knowledge, this is the first report about 3D printing use in a case of type II endoleak (EL). We believe that realizing life-size aortic models in selected cases where a complex type II EL embolization procedure is indicated, could lead to a better understanding of arterial anatomy, thus allowing to increase procedural success and reduce operative and most importantly fluoroscopy time.