Search

AbstractObjective:This study aimed to explore relationships between parental stress, coping, and outcomes for parents of infants with CHD, via observational approach reflecting domains of the Parental Stress and Resilience in CHD (PSRCHD) model.Methods:Fifty-five parents of 45 infants with CHD completed questionnaires with measures of parental stress, Problem-Focused Coping (PFC), Emotion-Focused Coping (EFC), Avoidant Coping (AC), mental health (symptoms of anxiety and symptoms of depression), post-traumatic growth (PTG) and quality of life (QoL). Demographic and infant clinical data were obtained.Results:Parental stress showed significant small to medium positive correlations with MH and PTG, but no significant correlations with QoL. EFC and AC showed significant small to medium positive correlations with MH, and medium negative correlations with parental QoL. EFC and PFC had significant small to medium correlations with PTG. PFC and AC had significant small to medium correlations with infant QoL. Hierarchical multiple regression analyses indicated that parental symptoms of anxiety, PTG, parental QoL, infant QoL were significantly predicted by models comprising of parental stress, coping styles, and clinical controls (adjusted R2= 13.0–47.9%, prange < 0.001–.048), with results for parental symptoms of depression falling marginally above significance (adjusted R2= 12.3%, p= .056).Conclusions:Parental stress, coping styles, and length of hospital stay are related to psychological outcomes in parents of infants with CHD. Future research may use the PSRCHD framework to assess mechanisms underlying CHD parents’ stress and coping experiences and investigate longitudinal relationships between parental factors and parent and child outcomes.

Human gene replacement therapies such as onasemnogene abeparvovec (OA) use recombinant adeno-associated virus (rAAV) vectors to treat monogenic disorders. The heart and liver are known target organs of toxicity in animals; with cardiac and hepatic monitoring recommended in humans after OA dosing. This manuscript provides a comprehensive description of cardiac data from preclinical studies and clinical sources including clinical trials, managed access programs and the post-marketing setting following intravenous OA administration through 23 May 2022. Single dose mouse GLP-Toxicology studies revealed dose-dependent cardiac findings including thrombi, myocardial inflammation and degeneration/regeneration, which were associated with early mortality (4-7 weeks) in the high dose groups. No such findings were documented in non-human primates (NHP) after 6 weeks or 6 months post-dose. No electrocardiogram or echocardiogram abnormalities were noted in NHP or humans. After OA dosing, some patients developed isolated elevations in troponin without associated signs/symptoms; the reported cardiac adverse events in patients were considered of secondary etiology (e.g. respiratory dysfunction or sepsis leading to cardiac events). Clinical data indicate cardiac toxicity observed in mice does not translate to humans. Cardiac abnormalities have been associated with SMA. Healthcare professionals should use medical judgment when evaluating the etiology and assessment of cardiac events post OA dosing so as to consider all possibilities and manage the patient accordingly.

AbstractBackground:No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice.Aims:To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice.Methods:Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events.Results:Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1–3).Conclusion:The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.jtcvs.2009.12.055 Byline: Mohamed T. Ghorbel, Myriam Cherif, Emma Jenkins, Amir Mokhtari, Damien Kenny, Gianni D. Angelini, Massimo Caputo Abbreviations: BAX, Bcl-2-associated X protein; CPB, cardiopulmonary bypass; ELISA, enzyme-linked immunosorbent assay; ERK, extracellular signal-regulated kinase; FDR, false discovery rate; MAPK, mitogen-activated protein kinase; RVH, right ventricular hypertrophy; TOF, tetralogy of Fallot; VEGF, vascular endothelial growth factor Abstract: In cyanotic patients undergoing repair of heart defects, chronic hypoxia is thought to lead to greater susceptibility to ischemia and reoxygenation injury. We sought to find an explanation to such a hypothesis by investigating the cardiac gene expression in patients with tetralogy of Fallot undergoing cardiac surgery. Author Affiliation: Bristol Heart Institute, University of Bristol, Bristol Royal Infirmary, Bristol, United Kingdom Article History: Received 1 August 2009; Revised 19 November 2009; Accepted 19 December 2009 Article Note: (footnote) This work was funded by the British Heart Foundation, the Garfield Weston Trust and the Bristol NIHR BRU in Cardiovascular Medicine. M. T. Ghorbel was supported by an Intermediate Research Fellowship from the British Heart Foundation., Disclosures: None.

AbstractBackground:Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate.Methods:We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus–Kaye–Stansel procedure over the past decade at a single national cardiology centre.Results:Of 114 children who underwent Norwood procedure or Damus–Kaye–Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2–82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10–70mmHg) to 2mmHg (range 0–20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2–7.9 mm) to 8.6 mm (range 6.2–10.9 mm). The median coarctation index increased by 0.49 (range = 0.24–0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications.Conclusions:The prevalence of recurrent aortic arch obstruction following Norwood/Damus–Kaye–Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.

AbstractThe 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.

AbstractCHD affects millions of patients worldwide. Interventional therapies for CHD goes back to the mid-1960s when Bill Rashkind performed balloon atrial septostomy on a cyanotic baby with transposition of the great vessels. This was followed by development of balloon catheters to perform balloon valvuloplasties and angioplasties in the early to late 1980s. Although King and Mills performed the first transcatheter closure of secundum atrial septal defect in the mid-1970s, this procedure was better realised in the mid-1990s. More intracardiac defect closures were performed in the late 1990s and early 2000. This brings us to the current era of percutaneous valve implantation as developed by Bonhoeffer. In this paper, we will discuss the past, present, and future of interventional cardiac catheterisation for CHD patients.

AbstractThe interventional cardiology track at the 7th World Congress of Pediatric Cardiology and Cardiac Surgery reflects the pivotal position of transcatheter interventions in the future of our speciality. The highlights of the week are outlined in this review.

AbstractAimsWe 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 resultsRetrospective 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.ConclusionsThe 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.

AbstractWe retrospectively reviewed all the children with right ventricular outflow tract obstruction, hypoplastic pulmonary annulus, and pulmonary arteries who underwent stenting of the right ventricular outflow tract for hypercyanotic spells at our institution between January, 2008 and December, 2013; nine patients who underwent cardiac catheterisation at a median age of 39 days (range 12–60 days) and weight of 3.6 kg (range 2.6–4.3 kg) were identified. The median number of stents placed was one stent (range 1–4). The median oxygen saturation increased from 60% to 96%. The median right pulmonary artery size increased from 3.3 to 5.5 mm (−2.68 to −0.92 Z-score), and the median left pulmonary artery size increased from 3.4 to 5.5 mm (−1.93 to 0 Z-scores). Among all, one patient developed transient pulmonary haemorrhage, and one patient had pericardial tamponade requiring drainage. Complete repair of tetralogy of Fallot +/− atrioventricular septal defect or double-outlet right ventricle was achieved in all nine patients. Transcatheter stent alleviation of the right ventricular outflow tract obstruction resolves hypercyanotic spells and allows reasonable growth of the pulmonary arteries to facilitate successful surgical repair. This represents a viable alternative to placement of a systemic-to-pulmonary artery shunt, particularly in small neonates.

Supplemental Digital Content is available in the text.

Supplemental Digital Content is available in the text.

Although established, transcatheter pulmonary valve replacement is in its infancy compared with surgical pulmonary valve replacement. Extended clinical experience and follow-up have identified new challenges; however, careful evaluation of data through clinical trials has facilitated effective evolution of responses to these challenges. The limited patient population has resulted in less interest in new valve design, but having been the older sibling to transcatheter aortic valve replacement, transcatheter pulmonary valve replacement is likely to benefit in the future from design modifications to the more popular and commercially viable transcatheter aortic valve revolution. Improving valve longevity and applying the technology to native outflow tracts remain the short-to-medium term goals.

Transcatheter pulmonary valve replacement (tPVR) has evolved into a viable alternative to surgical conduit or bioprosthetic valve replacement. This procedure has paved the way for a more advanced approach to congenital and structural interventional cardiology. Although many successes have been noted, there are still a number of challenges with this procedure, including large delivery systems, the need for a conduit or a bioprosthetic valve as a landing zone for the valve, optimal timing of the procedure to prevent right ventricular failure, arrhythmias, and possible death. Research is ongoing to broaden the use of this technology when treating patients with dilated right ventricular outflow tracts, and early experience with a self-expanding valve model has been reported. Affordability is an important factor that must be considered especially in developing nations. The aim of this review is to emphasize the advancement of tPVR, the benefits and challenges of valve implantation, the current state, and the future innovations associated with this approach.Transcatheter pulmonary valve replacement (tPVR) has evolved into a viable alternative to surgical conduit or bioprosthetic valve replacement. This procedure has paved the way for a more advanced approach to congenital and structural interventional cardiology. Although many successes have been noted, there are still a number of challenges with this procedure, including large delivery systems, the need for a conduit or a bioprosthetic valve as a landing zone for the valve, optimal timing of the procedure to prevent right ventricular failure, arrhythmias, and possible death. Research is ongoing to broaden the use of this technology when treating patients with dilated right ventricular outflow tracts, and early experience with a self-expanding valve model has been reported. Affordability is an important factor that must be considered especially in developing nations. The aim of this review is to emphasize the advancement of tPVR, the benefits and challenges of valve implantation, the current state, and the future innovations associated with this approach.

In all cases of congenital valvar aortic stenosis (AS), reduced effective orifice area leads to obstruction to flow, usually resulting from thickening and reduced motion of the valve leaflets. The most severe cases of valvar AS present soon after birth, with low cardiac output secondary to left ventricular dysfunction. Interventional treatment options consist of open surgical valvotomy or balloon valvuloplasty, with both therapies providing excellent but usually only temporary relief of stenosis. This article focuses on balloon aortic valvuloplasty as a therapy for congenital valvar AS in infants and children, focusing on established techniques, outcomes, and future challenges.

We report on a 3‐year‐old male who underwent transcatheter stent fenestration of the inferior portion of an extracardiac total cavopulmonary connection in the setting of hypoplastic left heart syndrome. Transhepatic approach, following an unsuccessful attempt from the femoral vein facilitated delivery of a diabolo‐shaped stent.

Aims. To outline the etiology, clinical course, short‐term survival to discharge and neurological outcome of infants (<1 yr) with new cardiac diagnoses presenting to a pediatric intensive care (PICU) unit with acute cardiac compromise.

Ni Ainle: Leo Pharma: Research Funding; Actelion: Research Funding; Bayer: Research Funding; Bayer: Membership on an entity's Board of Directors or advisory committees; BMS: Membership on an entity's Board of Directors or advisory committees; Daiichi Sankyo: Membership on an entity's Board of Directors or advisory committees; Boehringer: Membership on an entity's Board of Directors or advisory committees.

Walsh: Actelion UK: Research Funding. Maguire:Actelion UK: Research Funding. Ní Áinle:Actelion UK: Research Funding.