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The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.
Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.
Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.
We report our experience of using the Bentley BeGraft Aortic stent in patients with severe or near-atretic aortic coarctation and small femoral arterial access.
Use of covered stent is recommended in some settings such as aortic coarctation with associated aneurysm, Turner syndrome, and coarctation with aortic atresia. However, currently available covered stents need larger sheaths that may limit their use in children and patients with smaller arterial access. Newer stents may overcome this limitation.
Single-centre retrospective study of patients with severe or near-atretic aortic coarctation and small femoral arterial access.
Between July and October, 2019, five patients (median age 15 years) with near-atretic or severe coarctation were treated with a Bentley BeGraft Aortic stent. Long sheaths between 9 and 11 Fr were used to implant stents, which were dilated up to 12–16 mm. None of the patients had residual coarctation (gradient >20 mm of mercury) after stenting. None of the patients developed acute vascular injuries or local access related complications at the end of the procedure or during follow-up (range 6–10 months).
Bentley BeGraft aortic stents are important to consider in patients with severe coarctation and provided acute procedural success in patients with small femoral arterial access and widen the applicability in this patient population.
Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.
Methods and results:
Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.
A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.
The range and number of educational and networking events that are available for fellows, trainees, and junior faculty to attend grows every year. Each meeting useful in its own way; each adding value to the development and the growth of an interventionist. Within paediatric, congenital, and structural heart disease, three of the standout meetings are: Pediatric and Interventional Cardiac Symposium (PICS-AICS), Congenital and Structural Interventions (CSI), and International Workshop on Interventional Pediatric and Adult Congenital Cardiology (IPC). All of these were started by leaders in our field; people known to be passionate educators and innovators. International congresses focusing more broadly on congenital cardiac disease in children and adults are rare. These forums allow more interdisciplinary discussions between the interventionist, surgeon, and non-invasive specialists. Purely interventional meetings are essential to allow colleagues to debate and explore the nuances and intricacies of technique and approach, developing concepts to be challenged in wider forums. During the recent 21st PICS-AICS meeting Prof. Ziyad M. Hijazi, Shakeel A. Qureshi, Mario Carminati, and Dr Damien Kenny shared their time to engage in frank, recorded conversations which provide a unique insight in to the process and concepts behind three of our most important educational congresses.
We describe percutaneous repair of severe pulmonary regurgitation and a right ventricular outflow tract pseudoaneurysm in a 19-year-old patient after repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. A 3D printed model of his heart was used to simulate percutaneous repair with a closure device in the aneurysm neck and a Venus P-valve in the right ventricular outflow tract. The encouraging findings from the simulation allowed us to plan the complex procedure effectively with a successful outcome and avoidance of surgery.
Pulmonary valve stenosis is common in patients with Noonan’s syndrome. The response to balloon valvoplasty varies.
We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention.
This is a retrospective study conducted from 1995 to 2014.
Of 14 patients identified, seven had re-intervention 28±54 months (range 3–149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups.
We could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan’s patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.
We sought to evaluate the first-in-man use of a new system for implantation of covered stents in patients with complex structural and CHD.
Methods and results
Retrospective data were collected of the first 13 NuDEL™ delivery systems used in patients. The NuDEL™ comprises a covered Cheatham-Platinum stent mounted on a balloon-in-balloon and pre-loaded in a long delivery sheath. Data were collected from three centres in the United Kingdom and Ireland. A total of 13 covered stents were delivered via 12 NuDEL™ delivery systems in 12 patients. Among them, six patients had coarctation of the aorta, five patients had right ventricular outflow tract stenosis, and one patient had severe stenosis of a Mustard systemic venous baffle. There were no complications, and all the stents were deployed in the desired position with satisfactory haemodynamic results.
The development of a bespoke system of a pre-mounted, pre-loaded covered stent may negate some of the technical challenges that complicate large-calibre stent deployment. Our preliminary results suggest that the NuDEL™ system is a safe and effective means of covered stent deployment in challenging anatomy.
We aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.
The lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.
We carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.
In total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.
Hybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.
In patients with large patent arterial ducts and severe pulmonary hypertension, the natural history of progression of pulmonary hypertension is very variable. Whether to close or not to close is often a difficult decision, as there are no established haemodynamic parameters predicting reversibility.
The objectives of this study were to evaluate the results of device closure of large patent arterial ducts with severe pulmonary hypertension after 2 years of age and to determine haemodynamic variables associated with its regression during long-term follow-up.
A total of 45 patients, with median age of 10 (2–27) years, with large patent arterial ducts and severe pulmonary hypertension, were considered. Haemodynamic variables were assessed in air, oxygen, and after occlusion. The follow-up was performed to assess regression of pulmonary hypertension.
Device closure was successful in 43 (96%) patients. Pulmonary artery systolic and mean pressures decreased from 79 to 67 mmHg and from 59 to 50 mmHg, respectively (p<0.001). At a median follow-up of 80 (41–151) months, severe pulmonary hypertension persisted in four (9.7%) patients. Multivariate analysis showed pulmonary vascular resistance index ⩽6 WU m2 and pulmonary artery systolic and mean pressures ⩽75 and ⩽55 mmHg (all in oxygen), having 97.8% predictive value for regression of pulmonary hypertension (p<0.001) in the long term. In 24 patients with catheterisation-based criteria, regression of pulmonary hypertension was associated with pulmonary vascular resistance index <8 WU m2 (p=0.001) and its fall of >25% (both in oxygen) (p=0.007).
Device closure of large patent arterial ducts with severe pulmonary hypertension is safe and effective. Pulmonary vascular resistance index and systolic and mean pulmonary artery pressures in oxygen are the key prognostic variables predicting regression of pulmonary hypertension.
The aim of this study was to describe the clinical characteristics, progression, treatment, and outcomes in isolated coronary artery fistula cases diagnosed prenatally.
We carried out a retrospective review of babies diagnosed prenatally with coronary artery fistulas between January, 2000 and December, 2013; five fetuses were included. Echocardiographic features and measurements were noted during pregnancy and after birth. Treatment and outcome were noted.
Gestational age at initial diagnosis was between 19 and 22 weeks; four coronary artery fistulas originated from the right and one from the left circumflex coronary artery. Drainage was into the right atrium in four cases and into the left ventricle in one case. There was cardiomegaly in two cases at the initial scan. The size of the fistulas increased during pregnancy in all except one. All prenatal diagnoses were confirmed postnatally. Among all, two patients developed congestive cardiac failure soon after birth and required transcatheter closure of the coronary artery fistula, 5 and 17 days after birth, respectively; three patients remained asymptomatic, and all of them showed progressive dilation of the feeding artery and had closure of the fistula at 20 months, 4 years, and 7 years of age, respectively. During the follow-up period, which ranged 2–14 years, all the patients were alive and well.
Coronary artery fistulas can be diagnosed accurately during fetal life. Some babies may develop congestive cardiac failure soon after birth requiring early treatment. Those treated conservatively should be kept under review as intervention may be required later.
At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract.
To report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valveTM, implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts.
There were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years.
The Venus P-valveTM was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m2 (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure.
The Venus P-valveTM can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.
We report on a 6-year-old boy with a huge right coronary artery to the right ventricle fistula, who had previously been treated by device closure at the right ventricular exit point. However, 3 years later, the right coronary artery aneurysm showed progressively dilation and compressed the right ventricle. To prevent further complications related to the aneurysm, the proximal part of the aneurysm was successfully occluded by a vascular plug.
Background: Most children in the Third World do not have access to treatment for heart diseases, as the priorities of health care are different from the developed countries. Materials and methods: Since 2009, teams supported by the Chain of Hope and Spanish medical volunteers have travelled twice a year to help develop paediatric cardiac services in the Cardiac Center in Ethiopia, undertaking four missions each year. As of December 2012, 296 procedures were performed on 287 patients. The procedures included 128 duct occlusions, 55 pulmonary valve dilations, 25 atrial septal defect closures, 14 mitral valve dilations, and others. The local staff were trained to perform a majority of these cases. Results: Procedural success was achieved in 264 (89.2%). There were three deaths, five device embolisations, and three complications in mitral valve dilation. During the visits, the local staff were trained including one cardiologist, six nurses, and two technicians. The local team performed percutaneous interventions on its own after a couple of years. The goal is also to enable the local team to perform interventions independently. Conclusion: Training of an interventional cardiology team in a sub-Saharan setting is challenging but achievable. It may be difficult for a single centre to commit to sending frequent missions to a developing country to make a meaningful contribution to the training of local teams. In our case, coordination between the teams from the two countries helped to achieve our goals.
Isolation of the subclavian artery is associated with “steal” of blood from the carotid circulation to the arm, through the circle of Willis and the vertebral artery. When associated with a patent arterial duct, there is an additional “steal” of blood from the arm to the lungs, through the arterial duct because of the lower pulmonary vascular resistance. When this combination manifests clinically with arm ischaemia on the side of the isolated subclavian artery, closure of the arterial duct will prevent the “steal” of blood from the subclavian artery to the pulmonary artery and may improve the blood flow to the arm. We report three patients with this unusual combination of the “steal” phenomenon that improved after interventional closure of the arterial duct. This report discusses the embryological basis of the defect, clinical and echocardiographic clues to diagnose this unusual anomaly, angiographic findings, and transcatheter management options.
The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications.
Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment.
Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation.
Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries.
Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.
In order to optimise care of the adult patients with complex congenital heart disease, there is a need to develop recommendations for interventions. This document is the work of representatives of the three relevant societies and provides recommendations for institutions and operators performing cardiac interventions in these patients.