Right disc thrombosis of the new Gore Cardioform ASD Occluder.

BACKGROUND: The GCA is a well performing device in terms of efficacy despite complex anatomies (aortic rim <5 mm and ASD diameter >17 mm) with a good safety profile. AIMS: To evaluate atrial septal defect (ASD) features impacting on right disc device thrombosis in patients who underwent Gore Cardioform ASD Occluder (GCA) implantation. METHODS: A total of 44 consecutive patients undergoing percutaneous ASD with GCA device from January 2020 to September 2022 at our tertiary care Center were evaluated. The minimum follow-up was 6 months. RESULTS: The patients were stratified in two groups according to a cut-off value of ASD diameter equal to 20 mm at sizing balloon, derived from ROC analysis (AUC = 0.894; p = 0.024). Baseline characteristics were comparable between groups in terms of age, sex, weight, height, and interatrial septum dimensions. Patients with ASD > 20 mm (n = 9) had a higher ASD/device dimension ratio, both at echocardiography (p = 0.009) and at sizing balloon (p = 0.001), longer fluoroscopic time (p = 0.022), and higher incidence of device thrombosis (0.006). Right disc thrombosis was observed in three patients of the ASD > 20 mm group, always in the inferior portion of the right disc. On univariate analysis, ASD diameter at sizing balloon (OR 1.360; p = 0.036) was the only positive predictor of device thrombosis. CONCLUSIONS: Right disc thrombosis of the GCA device may be under-recognized at follow-up, hence deserving clinical attention, especially in those patients with larger ASD diameters.

The Appraisal of Adults with Congenital Heart Disease: Lesson from Comparison of Surgical Outcomes.

The population of adults with congenital heart disease (ACHD) is constantly growing. There seems to be a consensus that these patients are difficult to manage especially if compared to patients with acquired heart disease. The aim of this study is to compare outcomes and results of cardiac surgery in ACHD patients with a reference population of adults with acquired cardiac disease. Retrospective study of 5053 consecutive patients older than 18 years hospitalized for cardiac surgery during a 5-years period in our Institution. Two groups of patients were identified. Group I: 419 patients operated for congenital heart disease; Group II: 4634 patients operated for acquired heart disease. In each Group were identified low, medium, and high-risk patients, according to validated scores. Right ventricular outflow tract surgery was the most frequent procedure in Group I, while coronary artery by-pass grafting was the most common in Group II. Patients with ACHD were younger (37.8 vs. 67.7 years), with higher number of previous operations (32.1% vs. 6.9%), had longer post-ICU hospital stay (11 vs. 8 days) but had lower ICU stay (1 vs. 2 days), shorter assisted mechanical ventilation (12 vs. 14 h) and lower surgical mortality (1 vs. 3.7%) (all p < 0.001). No differences were found in term of post-operative complications (12.4 vs. 15%). The surgical treatment of ACHD patients can be done with excellent results and if compared with acquired cardiac disease patients they have better results with shorter ICU stay and lower mortality.

Long-term effects of physical training on cardiopulmonary exercise parameters in young patients with congenital heart diseases.

INTRODUCTION: Physical activity is recognised as an important intervention in patients with CHD. However, more data on the actual magnitude of physical training impact on functional capacity in this group of patients are still warranted. We aim to assess effort tolerance in a contemporary cohort of patients with congenital heart disease, regularly following a training programme, in comparison with a matched control group. METHODS: Patients with CHD followed at the sports medicine department, who had undergone cardiopulmonary exercise test between 2011 and 2019, were included. Variables recorded were maximum workload, absolute and indexed maximum oxygen consumption, maximum heart rate, absolute and indexed maximum O2 pulse, ventilatory equivalent of CO2 and oxygen consumption/Work. Trend of cardiopulmonary parameters was analysed over time. Maximal workload, maximum oxygen consumption and ventilatory equivalent of CO2 were compared with a control group of patients with a more sedentary lifestyle, matched for diagnosis, gender, age, and body mass index. RESULTS: Among one hundred and eleven patients, 73 males (66%) were analysed. Median age was 14 (12-17) years. Twenty-nine patients (27%) were practising sports at competitive level. Maximum oxygen consumption and oxygen consumption % of maximum predicted were not significantly different at follow-up as compared with baseline. Follow-up of maximum oxygen consumption was 38.2 ± 9 ml/kg/min versus 38.6 ± 9.2 ml/kg/min (p = NS) and follow-up of %oxygen consumption was 88 ± 20 versus 87 ± 15 (p = NS). Ventilatory equivalent of CO2 significantly improved in the last test as compared with the baseline: 30 ± 4 versus 33 ± 5 (p = 0.002). As compared with the control group, trained patients displayed a significantly higher maximum workload and oxygen consumption, while ventilatory equivalent of CO2 was not significantly different. CONCLUSIONS: In our cohort, patients following a regular training programme displayed a significantly higher functional capacity as compared with not trained control group, irrespective of NYHA class. Objective functional capacity was stable over a median follow-up of 3 years.

Atrial septal defect in adulthood: a new paradigm for congenital heart disease.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.

Three-dimensional printing, holograms, computational modelling, and artificial intelligence for adult congenital heart disease care: an exciting future.

Congenital heart disease (CHD) is often comprised of complex three-dimensional (3D) anatomy that must be well understood to assess the pathophysiological consequences and guide therapy. Thus, detailed cardiac imaging for early detection and planning of interventional and/or surgical treatment is paramount. Advanced technologies have revolutionized diagnostic and therapeutic practice in CHD, thus playing an increasing role in its management. Traditional reliance on standard imaging modalities including echocardiography, cardiac computed tomography (CT) and magnetic resonance imaging (MRI) has been augmented by the use of recent technologies such as 3D printing, virtual reality, augmented reality, computational modelling, and artificial intelligence because of insufficient information available with these standard imaging techniques. This has created potential opportunities of incorporating these technologies into routine clinical practice to achieve the best outcomes through delivery of personalized medicine. In this review, we provide an overview of these evolving technologies and a new approach enabling physicians to better understand their real-world application in adult CHD as a prelude to clinical workflow implementation.

AEPC recommendations for training in interventional catheterisation for CHD.

To reflect new developments in imaging, indications, and techniques in catheterisation of patients with CHD, the training recommendations of the Association for European Paediatric and Congenital Cardiology (AEPC) for the training in interventional catheterisation for CHD have been reviewed and updated after more than 7 years. They include detailed information about knowledge, skills, and approach to clinical practice expected from trainees at basic, intermediate, and advanced level.

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.

Development and validation of the parents' healthcare needs scale for adolescents with congenital heart disease.

PURPOSE: The healthcare needs of parents of adolescents with congenital heart disease (CHD) have been under-investigated as no valid and reliable tools have been developed for assessing their needs. Therefore, this study aims to develop and validate the Parents' Healthcare Needs Scale for adolescents with CHD (PHNS-CHD). DESIGN AND METHODS: A multi-method approach and multi-phase design were employed. Phase one referred to generating scale items based on emerging themes in the literature, and phase two showed the validation process, divided into three steps. Step one tested the content and face validity of the first version of the PHNS-CHD. After that, step two described the initial psychometric validation process of scale using an exploratory factorial analysis (EFA). Then, step three confirmed the PHNS-CHD factorial structure and assessed its internal consistency. RESULTS: The PHNS-CHD showed evidence of face and content validity, adequate construct, and internal consistency and stability. Specifically, it had 22 items grouped into five domains, labeled as follows: Healthcare education to the child; to be supported as a parent, clinical support to the child, the continuum of care to the child; emotional support to the child. CONCLUSIONS: The PHNS-CHD is a psychometrically robust measure for assessing the healthcare needs of parents of adolescents with CHD. PRACTICE IMPLICATIONS: The PHNS-CHD might help clinicians, especially pediatric nurses, assess the healthcare needs of parents of adolescents with CHD and design adequate care plans for the whole family.

The Effect of Holographic Heart Models and Mixed Reality for Anatomy Learning in Congenital Heart Disease: An Exploratory Study.

In this paper, we present an exploratory study on the potential impact of holographic heart models and mixed reality technology on medical training, and in particular in teaching complex Congenital Heart Diseases (CHD) to medical students. Fifty-nine medical students were randomly allocated into three groups. Each participant in each group received a 30-minute lecture on a CHD condition interpretation and transcatheter treatment with different instructional tools. The participants of the first group attended a lecture in which traditional slides were projected onto a flat screen (group "regular slideware", RS). The second group was shown slides incorporating videos of holographic anatomical models (group "holographic videos", HV). Finally, those in the third group wore immersive, head-mounted devices (HMD) to interact directly with holographic anatomical models (group "mixed reality", MR). At the end of the lecture, the members of each group were asked to fill in a multiple-choice questionnaire aimed at evaluating their topic proficiency, as a proxy to evaluate the effectiveness of the training session (in terms of acquired notions); participants from group MR were also asked to fill in a questionnaire regarding the recommendability and usability of the MS Hololens HMDs, as a proxy of satisfaction regarding its use experience (UX). The findings show promising results for usability and user acceptance.

Coronavirus disease 2019 in adults with congenital heart disease: a position paper from the ESC working group of adult congenital heart disease, and the International Society for Adult Congenital Heart Disease.

We are witnessing an unparalleled pandemic caused by the novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) associated with coronavirus disease 2019 (COVID-19). Current data show that SARS-CoV-2 results in mild flu-like symptoms in the majority of healthy and young patients affected. Nevertheless, the severity of COVID-19 respiratory syndrome and the risk of adverse or catastrophic outcomes are increased in patients with pre-existing cardiovascular disease. Patients with adult congenital heart disease (ACHD)-by definition-have underlying cardiovascular disease. Many patients with ACHD are also afflicted with residual haemodynamic lesions such as valve dysfunction, diminished ventricular function, arrhythmias or cyanosis, have extracardiac comorbidities, and face additional challenges regarding pregnancy. Currently, there are emerging data of the effect of COVID-19 on ACHD patients, but many aspects, especially risk stratification and treatment considerations, remain unclear. In this article, we aim to discuss the broad impact of COVID-19 on ACHD patients, focusing specifically on pathophysiology, risk stratification for work, self-isolation, hospitalization, impact on pregnancy, psychosocial health, and longer-term implications for the provision of ACHD care.

Emergency department management of patients with adult congenital heart disease: a consensus paper from the ESC Working Group on Adult Congenital Heart Disease, the European Society for Emergency Medicine (EUSEM), the European Association for Cardio-Thoracic Surgery (EACTS), and the Association for Acute Cardiovascular Care (ACVC).

Adult congenital heart disease (ACHD) patients represent a growing population with increasing use of acute emergency department (ED) care. Providing comprehensive ED care necessitates an understanding of the most common clinical scenarios to improve morbidity and mortality in this population. The aim of this position document is to provide a consensus regarding the management of the most common clinical scenarios of ACHD patients presenting to the ED.

European position paper on the management of patients with patent foramen ovale. Part II - Decompression sickness, migraine, arterial deoxygenation syndromes and select high-risk clinical conditions.

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.

Urinary Metabolomics Study of Patients with Bicuspid Aortic Valve Disease.

Bicuspid aortic valve (BAV) is the most common congenital heart defect responsible for valvular and aortic complications in affected patients. Causes and mechanisms of this pathology are still elusive and thus the lack of early detection biomarkers leads to challenges in its diagnosis and prevention of associated cardiovascular anomalies. The aim of this study was to explore the potential use of urine Nuclear Magnetic Resonance (NMR) metabolomics to evaluate a molecular fingerprint of BAV. Both multivariate and univariate statistical analyses were performed to compare the urinary metabolome of 20 patients with BAV with that of 24 matched controls. Orthogonal partial least squared discriminant analysis (OPLS-DA) showed statistically significant discrimination between cases and controls, suggesting seven metabolites (3-hydroxybutyrate, alanine, betaine, creatine, glycine, hippurate, and taurine) as potential biomarkers. Among these, glycine, hippurate and taurine individually displayed medium sensitivity and specificity by receiver operating characteristic (ROC) analysis. Pathway analysis indicated two metabolic pathways likely perturbed in BAV subjects. Possible contributions of gut microbiota activity and energy imbalance are also discussed. These results constitute encouraging preliminary findings in favor of the use of urine-based metabolomics for early diagnosis of BAV.

European position paper on the management of patients with patent foramen ovale. General approach and left circulation thromboembolism.

The presence of a patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions; however, the subject remains controversial and no official statements have been published. This interdisciplinary paper, prepared with involvement of eight European scientific societies, aims to review the available trial evidence and to define the principles needed to guide decision making in patients with PFO. In order to guarantee a strict process, position statements were developed with the use of a modified grading of recommendations assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements of particular management options were weighed and graded according to predefined scales. Despite being based often on limited and non-randomised data, while waiting for more conclusive evidence, it was possible to conclude on a number of position statements regarding a rational general approach to PFO management and to specific considerations regarding left circulation thromboembolism. For some therapeutic aspects, it was possible to express stricter position statements based on randomised trials. This position paper provides the first largely shared, interdisciplinary approach for a rational PFO management based on the best available evidence.

Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital heart disease, endorsed by HRS, PACES, APHRS, and SOLAECE.

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

The Challenging Pathway Toward Heart Transplant Listing for Adult Congenital Heart Disease Patients.

Adult congenital heart disease (ACHD) patients are at risk for end-stage heart failure; heart transplantation (Htx) represents the only definitive therapy available although not easily achievable for all patients. The study aims to assess the pathway difficulties and outcomes of ACHD patients with end-stage heart failure referred for Htx evaluation. This is a single center retrospective study on ACHD patients with end-stage heart failure referred to Htx evaluation from 2004 to 2015. Demographic data, medical history, failure modality, and follow-up were obtained from patient charts. End-points were Htx list enrollment, transplant, and survival. Statistical analysis was performed comparing patients listed and not listed. There were 21 ACHD patients with end-stage heart failure referred to Htx evaluation. Transplant listing was declined for 12 (57%) meanwhile 9 patients were listed. Htx was successfully achieved in 3 patients after 24 and 36 months, respectively. Three patients are still on the wait list and three died while waiting, with a listed group mortality of 33.3% (3/9). Mortality occurred in first 18 months after Htx list enrollment. Not listed group mortality was 50% (6/12) and occurred after a median time of 17.5 months (IQR: 9-23 months). There was no difference in survival (P = 0.574) between listed and not listed (89, 63, and 63% vs. 83, 56, and 47% at 12-24-48 months). Follow-up median duration was 27 months (IQR: 14-56 months). Heart transplant listing for ACHD patients with end-stage heart failure is hard to obtain. Almost 2/3 of the patients were declined. Survival for these patients is reduced severely either in waiting list for transplant or excluded indicating the potential need of mechanical circulatory support as bridge to transplant or as destination therapy to improve survival likelihood.

Management of balloon rupture during a percutaneous pulmonary valve implantation procedure.

Percutaneous pulmonary valve implantation is increasingly adopted as an alternative procedure to surgery in dysfunctional homograft, and in patients with "native" or wide right ventricle outflow tract dysfunction. Pre-stenting is mandatory in this category of patients for many reasons, one of which is to create an adequate landing zone for the bioprosthesis. Here we report on a tricky situation that occurred during pre-stenting, and we describe how we successfully overcame it.

Adverse outcome of coarctation stenting in patients with Turner syndrome.

OBJECTIVES: This study examines the outcome and procedural outcomes of percutaneous stent angioplasty for aortic coarctation in patients with Turner syndrome (TS). BACKGROUND: TS occurs in 1 in 2,500 live-born females and is associated with aortic coarctation. METHODS: In this multicenter, retrospective cohort study, all patients with TS and a coarctation of the aorta, treated with percutaneous stent implantation were included. The procedural strategies were dictated by local protocols. Adverse events at short- and long-term follow-up and qualitative parameters concerning the stent implantation were assessed. RESULTS: In the largest study to date of TS patients receiving aortic stents, a total of 19 patients from 10 centers were included. Twelve patients were treated for native and 7 for recurrent coarctation. Age at intervention was 16.9 (7-60) years (median; min-max). The coarctation diameter increased significantly from 8.0 mm (2-12) pre-intervention to 15.0 mm (10-19) post-intervention (P < 0.001). Three (15.8%) adverse events occurred within 30 days of the procedure, including two dissections despite the use of covered stents, one resulting in death. At long-term follow-up (6.5 years, min-max: 1-16), two additional deaths occurred not known to be stent-related. CONCLUSIONS: Though percutaneous treatment of aortic coarctation in TS patients is effective, it is associated with serious morbidity and mortality. These risks suggest that alternative treatment options should be carefully weighed against percutaneous stenting strategies. © 2016 Wiley Periodicals, Inc.

Surgery for supraventricular tachycardia and congenital heart defects: long-term efficacy of the combined approach in adult patients.

AIMS: Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. METHODS AND RESULTS: Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures. Significant clinical predictors of arrhythmia recurrence were determined by univariate analysis. We performed 47 right-sided Maze procedures, and 33 Cox-Maze III procedures. In 75 survivors, the ablation was effective immediately. Over an average follow-up period of 72 months (12-155 months), arrhythmias recurred in nine (20%) patients after right-sided Maze, and in six (19%) patients after Cox-Maze III. Eleven patients were controlled with medical therapy, three underwent catheter ablation of the arrhythmia, and one required a permanent pacemaker. Preoperative arrhythmia length ≥3 years (P ≤ 0.001), tetralogy of Fallot (P ≤ 0.006), and preoperative atrial fibrillation (P ≤ 0.016) were associated with recurrence arrhythmia. Conversely, NYHA class <3 (P ≤ 0.047) was associated with a lower risk of recurrence. CONCLUSION: Surgical treatment of unresponsive supraventricular arrhythmia during concomitant cardiac surgery in ACHD is a safe and effective procedure. Freedom from arrhythmias recurrence is 75% after 6 years of follow-up. Long-term recurrence of arrhythmia in these patients seems to be strongly correlated to preoperative arrhythmia duration.