Efficacy of a Transition Program in Adolescents and Young Adults With Congenital Heart Disease: The TRANSITION-CHD Randomized Controlled Trial.

PURPOSE: Currently, nearly 90% of patients with congenital heart disease (CHD) reach adulthood in relatively good health. Structured transition programs have emerged to support adolescents and young adults in transitioning to adult care structures, improve their autonomy, and limit healthcare ruptures. The TRANSITION-CHD randomized controlled trial aimed to assess the impact of a transition program on health-related quality of life (HRQoL) in adolescents and young adults with CHD. METHODS: From January 2017 to February 2020, 200 subjects with a CHD, aged 13-25 years, were enrolled in a prospective, controlled, multicenter study and randomized in two balanced groups (transition program vs. standard of care). The primary outcome was the change in PedsQL self-reported HRQoL score between baseline and 12-month follow-up, using an intention-to-treat analysis. The secondary outcomes were the change in disease knowledge, physical health (cardiopulmonary fitness, physical activity), and mental health (anxiety, depression). RESULTS: The change in HRQoL differed significantly between the transition group and the control group (mean difference = 3.03, 95% confidence interval (CI) = [0.08; 5.98]; p = .044; effect size = 0.30), in favor of the intervention group. A significant increase was also observed in the self-reported psychosocial HRQoL (mean difference = 3.33, 95% CI = [0.01; 6.64]; p = .049; effect size = 0.29), in the proxy-reported physical HRQoL (mean difference = 9.18, 95% CI = [1.86; 16.51]; p = .015; effect size = 0.53), and in disease knowledge (mean difference = 3.13, 95% CI = [1.54; 4.72]; p < .001; effect size = 0.64). DISCUSSION: The TRANSITION-CHD program improved HRQoL and disease knowledge in adolescents and young adults with CHD, supporting the generalization and systematization of similar preventive interventions in pediatric and congenital cardiology.

Usefulness of perioperative transoesophageal echocardiography during paediatric cardiac surgery.

BACKGROUND: Paediatric transoesophageal echocardiography probes allow perioperative evaluation during paediatric congenital heart disease surgery. AIM: To assess the usefulness of perioperative transoesophageal echocardiography in evaluating the severity of residual lesions, based on the type of congenital heart disease repaired in paediatric patients. METHODS: A retrospective analysis was conducted on paediatric patients who underwent open-heart surgery at our tertiary centre over a four-year period. Perioperative transoesophageal echocardiography studies were performed, and residual lesions were classified as mild, moderate or severe. RESULTS: Overall, 323 procedures involving 310 patients with a median age of 13.8 (0.07-214.4) months and a median weight of 8.2 (2-96) kg at intervention were enrolled in the study. Twenty-one (6.5%) residual lesions led to immediate reintervention: severe right ventricular outflow tract obstruction (n=12); severe aortic regurgitation (n=3); superior vena cava stenosis (n=2); moderate residual ventricular septal defect (n=2); severe mitral regurgitation (n=1); and severe mitral stenosis (n=1). Three (0.9%) neonates had ventilation difficulties caused by the transoesophageal echocardiography probe having to be removed, but experienced no sequelae. CONCLUSION: Perioperative transoesophageal echocardiography is a safe procedure, providing information on severe residual lesions, leading to the immediate revision of several paediatric congenital heart disease cases.

Advancing paediatric cardiac imaging: a comprehensive analysis of the feasibility and accuracy of a novel 3D paediatric transoesophageal probe.

Aims: Pediatric transoesophageal echocardiography (TOE) probes have remained two-dimensional (2D) limiting their use compared to adults. While critical in pediatrics for interventions and post-surgery assessments, technological advancements introduced a three-dimensional (3D) pediatric TOE probe. This study assessed the new 3D pediatric TOE probe (GE 9VT-D) for feasibility, handling, and imaging quality. Methods and results: At Children's Hospital of Toulouse, 2-month prospective study enrolled children undergoing TOE with the new probe. All imaging modalities were rated by 2 operators using a 5-point Likert-type scale from 1 (very poor) to 5 (very good) quality. Forty-five children, median age 3.7 (range: 2 months-14.7 years) median weight 7.8 kg (range: 4.3-48 kg) underwent 60 TOEs: 25% pre-surgery, 45% post-surgery, 28% during percutaneous procedures, and 2% in intensive care. Probe handling was "very easy" in all cases without adverse events. The median score of 2D, 2D colour, pulsed Doppler and 3D were noted 5 out of 5 and continuous Doppler and 3D colour 4 out of 5. The 3D image quality remained consistent irrespective of the patient weighing above or below 7.8 kg (p = 0.72). Postoperative TOEs identified two cases needing further interventions, emphasizing its value in evaluating surgical outcomes and also for guiding percutaneous interventions. Conclusion: Our comprehensive evaluation demonstrates that the new 3D pediatric TOE probe is feasible and provides high-quality imaging in pediatric patients. The successful integration of this novel probe into clinical practice has the potential to enhance diagnostic accuracy and procedural planning, ultimately optimizing patient outcomes in pediatric cardiac care.

Splenic Subcapsular Hematoma After Endoscopic Retrograde Cholangiopancreatography.

The complications of endoscopic retrograde cholangiopancreatography (ERCP) are numerous and mainly intraluminal. We present a unique case of a patient who developed splenic hematoma after ERCP. A 41-year-old woman was hospitalized for evaluation of chronic abdominal pain, for which she underwent an ERCP. The next day, the patient developed hemorrhagic shock. She was found to have a large ruptured subcapsular splenic bleed. Splenic artery embolization was performed, and the patient was stabilized. In conclusion, a high index of suspicion should be kept when managing patients presenting with unstable vital signs and/or acute anemia after ERCP.

Assessment of Radiology Artificial Intelligence Software: A Validation and Evaluation Framework.

Artificial intelligence (AI) software in radiology is becoming increasingly prevalent and performance is improving rapidly with new applications for given use cases being developed continuously, oftentimes with development and validation occurring in parallel. Several guidelines have provided reporting standards for publications of AI-based research in medicine and radiology. Yet, there is an unmet need for recommendations on the assessment of AI software before adoption and after commercialization. As the radiology AI ecosystem continues to grow and mature, a formalization of system assessment and evaluation is paramount to ensure patient safety, relevance and support to clinical workflows, and optimal allocation of limited AI development and validation resources before broader implementation into clinical practice. To fulfil these needs, we provide a glossary for AI software types, use cases and roles within the clinical workflow; list healthcare needs, key performance indicators and required information about software prior to assessment; and lay out examples of software performance metrics per software category. This conceptual framework is intended to streamline communication with the AI software industry and provide healthcare decision makers and radiologists with tools to assess the potential use of these software. The proposed software evaluation framework lays the foundation for a radiologist-led prospective validation network of radiology AI software. Learning Points: The rapid expansion of AI applications in radiology requires standardization of AI software specification, classification, and evaluation. The Canadian Association of Radiologists' AI Tech & Apps Working Group Proposes an AI Specification document format and supports the implementation of a clinical expert evaluation process for Radiology AI software.

Radiation dose during catheter ablation in children using a low fluoroscopy frame rate.

BACKGROUND: Catheter ablation (CA) in children using fluoroscopy carries risks inherent to ionizing radiation exposure. AIMS: The objective of this study was to demonstrate the feasibility of using low frames rate during ablation in children to maximally decrease radiation dose. METHODS: Hundred sixty eight successive patients<18 years of age undergoing CA performed under a 3.75 frames/second rate were retrospectively included. Demographics, procedural and dosimetry data were analysed. The effective dose (ED) was evaluated in a subgroup of 14 patients. RESULTS: Median age and weight were 15 years and 54kg, 72% had WPW, 10% AV node reentrant tachycardia, 10% ventricular tachycardia (atrial tachycardia, flutter and atrial fibrillation for the other cases). Acute success was achieved in 98.5% without any complication. Median procedure and fluoroscopy duration were 120 and 16minutes. Median Dose Area Product (DAP) and Air Kerma were 2.46Gy.cm2 and 18 mGy respectively (2Gy.cm2 and 15 mGy for WPW ablation). A significant reduction of median DAP was noted over the years for WPW, from 3.1Gy.cm2 in 2011 to 1.4 in 2019. Median estimated ED was 0.19 mSv (0.03 to 1.64), falling into the range of yearly normal natural irradiation or caused by leasure or professional activity. CONCLUSION: Low frame rate fluoroscopy is a highly effective and safe approach in decreasing radiation exposure during CA in children without altering the success rate of the procedure. ED is low, similar to natural/leisure irradiation. This approach can be considered a good alternative to 3D-based procedures in terms of efficiency and radiation issues, at least for WPW ablations.

Impact of 3D-printed models in meetings with parents of children undergoing interventional cardiac catheterisation.

Background: Paediatric interventional catheterisation has consistently improved in recent decades, with often highly successful outcomes. However, progress is still required in terms of the information delivered to parents and how parental anxiety is managed. Aim: To investigate the impact of cardiac printed models on improving parental understanding and alleviating anxiety before interventional catheterisation. Methods: The parents of children undergoing interventional cardiac catheterisation were prospectively enrolled in the study. A questionnaire highlighting knowledge and understanding of the condition and cardiac catheterisation per se was scored on a scale of 1-30. The State-Trait Anxiety Inventory (STAI), which generates current anxiety scores, was also used before and after the pre-catheterisation meeting. The "printing group" received an explanation of catheterisation using the device and a three-dimensional (3D) model, while the "control group" received an explanation using only the device and a manual drawing. Results: In total, 76 parents of 50 children were randomly assigned to a "control group" (n = 38) or "printing group" (n = 38). The groups were comparable at baseline. The level of understanding and knowledge improved after the "control group" and "printing group" meetings (+5.5±0.8 and +10.2±0.8; p < 0.0001 and p < 0.0001, respectively). A greater improvement was documented in the "printing group" compared to the "control group" (p < 0.0001). The STAI score also improved after the explanation was given to both groups (-1.8±0.6 and -5.6±1.0; p < 0.0001 and p < 0.0001). The greatest improvement was noted in the "printing group" (p = 0.0025). Most of the parents (35/38 from the "printing group") found the models to be extremely useful. Conclusion: 3D-printed models improve parental knowledge and understanding of paediatric cardiac catheterisation, thereby reducing anxiety levels.

The usefulness of 3D printed heart models for medical student education in congenital heart disease.

BACKGROUND: Three-dimensional (3D) printing technology enables the translation of 2-dimensional (2D) medical imaging into a physical replica of a patient's individual anatomy and may enhance the understanding of congenital heart defects (CHD). We aimed to evaluate the usefulness of a spectrum of 3D-printed models in teaching CHD to medical students. RESULTS: We performed a prospective, randomized educational procedure to teach fifth year medical students four CHDs (atrial septal defect (ASD, n = 74), ventricular septal defect (VSD, n = 50), coarctation of aorta (CoA, n = 118) and tetralogy of Fallot (ToF, n = 105)). Students were randomized into printing groups or control groups. All students received the same 20 min lecture with projected digital 2D images. The printing groups also manipulated 3D printed models during the lecture. Both groups answered an objective survey (Multiple-choice questionnaire) twice, pre- and post-test, and completed a post-lecture subjective survey. Three hundred forty-seven students were included and both teaching groups for each CHD were comparable in age, sex and pre-test score. Overall, objective knowledge improved after the lecture and was higher in the printing group compared to the control group (16.3 ± 2.6 vs 14.8 ± 2.8 out of 20, p < 0.0001). Similar results were observed for each CHD (p = 0.0001 ASD group; p = 0.002 VSD group; p = 0.0005 CoA group; p = 0.003 ToF group). Students' opinion of their understanding of CHDs was higher in the printing group compared to the control group (respectively 4.2 ± 0.5 vs 3.8 ± 0.4 out of 5, p < 0.0001). CONCLUSION: The use of 3D printed models in CHD lectures improve both objective knowledge and learner satisfaction for medical students. The practice should be mainstreamed.

Feasibility and accuracy of printed models of complex cardiac defects in small infants from cardiac computed tomography.

BACKGROUND: Three-dimensional (3-D) printed models are increasingly used to enhance understanding of complex anatomy in congenital heart disease. OBJECTIVE: To assess feasibility and accuracy of 3-D printed models obtained from cardiac CT scans in young children with complex congenital heart diseases. MATERIALS AND METHODS: We included children with conotruncal heart anomalies who were younger than 2 years and had a cardiac CT scan in the course of their follow-up. We used cardiac CT scan datasets to generate 3-D models. To assess the models' accuracy, we compared four diameters for each child between the CT images and the printed models, including the largest diameters (Dmax) of ventricular septal defects and aortic annulus and their minimal diameters (Dmin). RESULTS: We obtained images from 14 children with a mean age of 5.5 months (range 1-24 months) and a mean weight of 6.7 kg (range 3.4-14.5 kg). We generated 3-D models for all children. Mean measurement difference between CT images and 3-D models was 0.13 mm for Dmin and 0.12 mm for Dmax for ventricular septal defect diameters, and it was 0.16 mm for Dmin and -0.13 mm for Dmax for aortic annulus diameter, indicating a non-clinically significant difference. CONCLUSION: Three-dimensional printed models could be feasibly generated from cardiac CT scans in a small pediatric population with complex congenital heart diseases. This technique is highly accurate and reliably reflects the same structural dimensions when compared to CT source images.

Case Report: Late Successful Thrombectomy for Ischemic Stroke in a 2-Year-Old Child.

Despite extensive evidence of benefit of thrombectomy in adult ischemic stroke due to large-vessel occlusion in the 6-h window, its role remains uncertain in very young children. We describe hereafter the case of a 2-year-old female child who had a successful thrombectomy 9 h after stroke onset. The patient presented with right hemiplegia, central facial palsy, a normal level of consciousness, and speech difficulties. The PedNIHS score was 11. CT scan without contrast injection displayed spontaneous hyperdensity of the middle cerebral artery (MCA), with only limited early signs of ischemia (ASPECTS 8). CT angiography demonstrated occlusion of the proximal MCA with good collaterals. Thrombectomy was realized. Complete recanalization (TICI 3) was obtained under general anesthesia after two passes of a stent retriever. Time from symptoms onset to full recanalization was 9 h. The acute ischemic stroke was caused by embolic thrombus from a congenital heart disease. Clinical recovery was complete. Three months after the thrombectomy, the young patient was doing well without any neurological sequelae (PedNIHSS 0; modified Rankin Scale: 0). This case report is an example of a decision-making process to perform thrombectomy in a very young child, which included cardio-embolic etiology as a parameter that potentially might have participated to the successful outcome of the therapeutic procedure.

Impact of a transition education program on health-related quality of life in pediatric patients with congenital heart disease: study design for a randomised controlled trial.

BACKGROUND: Recent advances in the field of congenital heart disease (CHD) led to an improved prognosis of the patients and in consequence the growth of a new population: the grown up with congenital heart disease. Until recently, more than 50% of these patients were lost to follow up because of the lack of specialized structures. The critical moment is the transition between paediatric and adult unit. Therapeutic education is crucial to solve this issue by helping patients to become independent and responsible. The TRANSITION-CHD randomized trial aims to assess the impact of a transition education program on health-related quality of life (HRQoL) of adolescents and young adults with CHD. METHODS: Multicentre, randomised, controlled, parallel arm study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (education program vs. no intervention). The primary outcome is the change in self-reported HRQoL between baseline and 12-month follow-up. A total of 100 patients in each group is required to observe a significant increase of the overall HRQoL score of 7 ± 13.5 points (on 100) with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (peak VO2, VAT, VE/VCO2 slope), level of knowledge of the disease using the Leuven knowledge questionnaire for CHD, physical and psychological status. DISCUSSION: As the current research is opening on patient related outcomes, and as the level of proof in therapeutic education is still low, we sought to assess the efficacy of a therapeutic education program on HRQoL of CHD patients with a randomized trial. TRIAL REGISTRATION: This study was approved by the National Ethics Committee (South-Mediterranean IV 2016-A01681-50) and was registered on Clinicaltrials.gov (NCT03005626).

Position paper concerning the competence, performance and environment required for the practice of ablation in children and in congenital heart disease.

The population of patients with congenital heart disease (CHD) is continuously increasing, and a significant proportion of these patients will experience arrhythmias because of the underlying congenital heart defect itself or as a consequence of interventional or surgical treatment. Arrhythmias are a leading cause of mortality, morbidity and impaired quality of life in adults with CHD. Arrhythmias may also occur in children with or without CHD. In light of the unique issues, challenges and considerations involved in managing arrhythmias in this growing, ageing and heterogeneous patient population and in children, it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The introduction of catheter ablation techniques has greatly improved the treatment of cardiac arrhythmias. However, catheter ablation in adults or children with CHD and in children without CHD is more technically demanding, potentially causing various complications, and thus requires a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding required technical competence and equipment are lacking in this situation, the Working Group of Pacing and Electrophysiology of the French Society of Cardiology and the Affiliate Group of Paediatric and Adult Congenital Cardiology have decided to produce a common position paper compiled from expert opinions from cardiac electrophysiology and paediatric cardiology. The paper details the features of an interventional cardiac electrophysiology centre that are required for ablation procedures in adults with CHD and in children, the importance of being able to diagnose, monitor and manage complications associated with ablations in these patients and the supplemental hospital-based resources required, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Lastly, the need for quality evaluations and French registries of ablations in these populations is discussed. The purpose of this consensus statement is therefore to define optimal conditions for the delivery of invasive care regarding ablation of arrhythmias in adults with CHD and in children, and to provide expert and - when possible - evidence-based recommendations on best practice for catheter-based ablation procedures in these specific populations.

Impact of Sophrology on cardiopulmonary fitness in teenagers and young adults with a congenital heart disease: The SOPHROCARE study rationale, design and methods.

BACKGROUND: Recent advances in the field of congenital heart disease (CHD) have significantly improved the overall prognosis. Now more attention is being given to health-related quality of life (HRQoL) and promotion of physical activity. Non-invasive relaxation therapy may be effective in cardiac patients concerned with exercise-induced dyspnoea. The SOPHROCARE randomised trial aims to assess the impact of Caycedian Sophrology on cardiopulmonary fitness in adolescents and young adults with CHD. METHODS: The SOPHROCARE trial is a nationwide, multicentre, randomised, controlled study in CHD patients aged from 13 to 25 years old. Patients will be randomised into 2 groups (8 Sophrology group sessions vs. no intervention). The primary outcome is the change in percent predicted maximum oxygen uptake (VO2max) between baseline and 12-month follow-up. A total of 94 patients in each group is required to observe a significant increase of 10% in VO2max with a power of 80% and an alpha risk of 5%. The secondary outcomes are: clinical outcomes, cardiopulmonary exercise test parameters (VE/VCO2 slope, ventilatory anaerobic threshold, oxygen pulse, respiratory response to hypercapnia), health-related quality of life score (PedsQL), physical and psychological status. CONCLUSION: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. We sought to assess in the SOPHROCARE trial, if a Sophrology program, could improve exercise capacity and quality of life in youth with CHD. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03999320).

Transcatheter closure of a perimembranous ventricular septal defect with Nit-Occlud Lê VSD Coil: A French multicentre study.

BACKGROUND: Transcatheter perimembranous ventricular septal defect (pmVSD) closure remains challenging and is seldom used in France given the risk of atrioventricular block (AVB). pmVSD closure with the Nit-Occlud Lê VSD coil was recently introduced in France as an alternative to occluder devices. AIMS: To study the safety and feasibility of pmVSD closure with the Nit-Occlud Lê VSD coil. METHODS: All consecutives cases of pmVSD closure with the Nit-Occlud Lê VSD coil in 20 tertiary French centres were included between January 2015 and December 2018. RESULTS: Among 46 procedures in five centres, indications for pmVSD closure were left ventricle overload (76.1%), exertional dyspnoea (17.4%), history of infective endocarditis (4.3%) and mild pulmonary hypertension (2.2%). The median (interquartile [IQR]) age of the patients was 13.9 (5.7-31.8) years. Aneurismal tissue was identified in 91.3% of patients. VSD median (IQR) size was 8 (7-10) mm on the left ventricle side and 5 (4-6) mm on the right ventricle side. Implantation was successful in 40 patients (87.0%; 95% confidence interval [CI] 73.7-95.1%). Severe complications occurred in six patients (13.0%, 95% CI 4.9-26.3%), mainly severe haemolysis (8.7%, 95% CI 2.4-20.8%). One aortic valve lesion required surgical aortic valvuloplasty. Occurrence of severe complications was significantly related to the presence of haemolysis (P=0.001), residual shunt (P=0.007) and multi-exit VSD (P=0.005). Residual shunt was observed in 40% of cases with the implanted device shortly after closure and 15% after a median follow-up of 27 months. No immediate or delayed device embolization or complete AVB was recorded. CONCLUSION: pmVSD closure with the Nit-Occlud Lê VSD Coil is feasible in older children and adults. However, residual shunting (leading to haemolysis) is a dreaded complication that should not be tolerated. pmVSD closure with the Nit-Occlud Lê VSD as a therapeutic strategy remains controversial and is limited to selected patients.

Incidence of cardiovascular events and risk markers in a prospective study of children diagnosed with Marfan syndrome.

BACKGROUND: Little is known about the incidence of cardiovascular events (CVEs) and their associated risk markers in children with Marfan syndrome (MFS). AIMS: To assess the incidence of CVEs and determine risk markers in a cohort diagnosed with Marfan syndrome during childhood and followed for several years. METHODS: From a French multicentre nationwide database, 462 patients with MFS diagnosed during childhood were included prospectively. Patients' files were screened for a period of 20 years (1993-2013). CVEs (e.g. death, aortic dissection, cardiac valve or aortic root surgery) were assessed during the prospective follow-up. RESULTS: Median (interquartile range) age at the end of follow-up was 17.2 (11.1-21.3) years. CVEs were reported for 35 participants (7.6%; 95% confidence interval [CI] 5.3-10.4%). First CVEs were prophylactic aortic root surgery (n=29), aortic dissection (n=4; two aged <18 years) and death (n=2). Kaplan-Meier cumulative incidence of CVEs was 5.3% (95% CI 3.3-8.7%) during childhood (aged≤18 years) and 19.4% (95% CI 13.3-27.9%) at 25years of age. The cumulative rate of CVEs was higher in case of Valsalva sinus Z-score increase of≥0.1 per year (P=0.0003), maximal Valsalva sinus diameter growth speed ≥5mm per year (P=0.03), aortic regurgitation≥2 (P=0.0005) and maximal Valsalva sinus Z-score≥3 before 16 years of age (P<0.0001). In a multivariable Cox proportional analysis, the Valsalva sinus Z-score remained significantly related to outcome. Considering aortic root evolution, aortic regurgitation, age at diagnosis and beta-blocker therapy were related to Valsalva sinus Z-score evolution during follow-up. CONCLUSIONS: CVEs in children with MFS are mainly related to prophylactic aortic root surgery. Aortic dissections are rarely observed in children. The Valsalva sinus Z-score is a strong indicator of subsequent CVEs in children with MFS. Attention to follow-up and beta-blocker observance may be warranted in high-risk children.