Cardiac Rhabdomyomas Presenting with Critical Cardiac Obstruction in Neonates and Infants: Treatment Strategies and Outcome, A Single-Center Experience.

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.

Aspirin Responsiveness in a Cohort of Pediatric Patients with Right Ventricle to Pulmonary Artery Conduits and Transcatheter Valve Replacement Systems.

The aim of this study was to determine the rate of aspirin responsiveness in a cohort of pediatric patients with in situ xenograft valved right ventricle to pulmonary artery (RV-PA) conduits and/or transcatheter valve replacements (TVR). Aspirin is routinely prescribed to these patients. Optimizing anti-platelet therapy could promote valve longevity and reduce the risk of infective endocarditis in this at-risk group. This was a prospective, observational study. Patients were recruited from both ward and outpatient settings. Patients were eligible if under 18 years and taking aspirin. Non-response to aspirin was defined as > 20% platelet aggregation using light transmission platelet aggregometry (LTA) and < 50% platelet inhibition by thromboelastography with platelet mapping (TEGPM). Participants were invited to provide a confirmatory sample in cases of aspirin resistance and dose adjustments were made. Thirty patients participated. Median age was 9 years (2 months to 18 years). The majority (93%) had complex right ventricular outflow tract pathology. 13 (43%) had an RV-PA conduit and 24 (80%) had a TVR, with valve situated in conduit in 7 (23%) cases. Rate of aspirin non-response on initial testing was 23% (n = 7/30) with median LTA 74.55% (60-76%) and TEG 13.25% (0-44%) in non-responders. Non-responders were more likely to be under 1 year. Two patients required dose increases and one patient non-adherence to dose was identified. Four patients on repeat testing were responsive to aspirin by laboratory tests. The rate of aspirin non-response on laboratory testing in this cohort of patients was 23% and resulted in therapeutic intervention in 10%.

Assessing the State of Training in Congenital Interventional Cardiology: A Global Survey of Program Directors.

This study aimed to evaluate the current state of congenital interventional cardiology training worldwide, with a focus on case volumes, competency assessment, and the need for ongoing mentorship during early career stages. A survey was conducted among program directors (PDs) of congenital interventional training programs across the globe. The survey gathered data on training pathways, case volumes, types of procedures performed, trainee competency assessment, and the role of ongoing mentorship. Of the 79 PDs who completed the survey, it was observed that training pathways and case volumes varied significantly, particularly between the United States and other countries. Most PDs reported an annual laboratory case volume of >500 congenital cardiac cases, with most cases being interventional. While trainees demonstrated competency in simple procedures (diagnostic cases, simple ASD closure), complex interventions (such as patent ductus arteriosus closure in premature infants) require ongoing mentorship for graduates. PDs recommended a minimum case volume of 400 total cases for trainees, including 250 interventional cases. In addition to case volumes, assessing trainee competency was deemed important, with clinical reasoning, judgment, skillset, teamwork, and complication management being key areas of evaluation. The study highlights the variability in congenital interventional cardiology training and the need for ongoing mentorship during the early career years. External mentorship programs, facilitated by national and international societies, are proposed to provide critical support for early career interventionalists thus enhancing patient care for congenital heart disease. Ultimately, the findings of this survey may serve as a framework for future training standards and guidelines in this specialized field.

Exploring relationships between parental stress, coping, and psychological outcomes for parents of infants with CHD.

OBJECTIVE: 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%, p range < 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.

Review of cardiac safety in onasemnogene abeparvovec gene replacement therapy: translation from preclinical to clinical findings.

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.

European expert recommendations on clinical investigation and evaluation of high-risk medical devices for children.

Several high-risk medical devices for children have become unavailable in the European Union (EU), since requirements and costs for device certification increased markedly due to the EU Medical Device Regulation. The EU-funded CORE-MD project held a workshop in January 2023 with experts from various child health specialties, representatives of European paediatric associations, a regulatory authority and the European Commission Directorate General Health and Food Safety. A virtual follow-up meeting took place in March 2023. We developed recommendations for investigation of high-risk medical devices for children building on participants' expertise and results of a scoping review of clinical trials on high-risk medical devices in children. Approaches for evaluating and certifying high-risk medical devices for market introduction are proposed.

Transfemoral Perimembranous Ventricular Septal Defect Device Closure in Infants Weighing ≤ 10 kg.

Transcatheter closure of Perimembranous VSDs (PMVSD) remains challenging particularly in infants. The aim of this study is to evaluate the efficacy and safety of transfemoral PMVSD device closure in infants weighing ≤ 10 kg in a single centre. Retrospective review of departmental databases and medical charts to define patient cohort and collect demographic, procedural and follow-up data. Between July 2014 and March 2021, 16 patients underwent attempted transfemoral PMVSD device closure (12 retrograde) at a median age of 11 months (interquartile range [IQR] 9-15.5) and a median weight of 8.3 kg (IQR 7.2-9.5). All patients were either symptomatic, had progressive left heart dilation or had VSD associated valve regurgitation. Median defect size on pre-procedural transoesophageal echocardiography was 6.8 mm (IQR 6-8.5). Median device waist size was 6 mm (IQR 4.5-8). Successful device placement was achieved in 14 patients (88%). One patient developed moderate aortic and tricuspid valve regurgitation upon retrograde and antegrade device deployment, respectively, and subsequently underwent surgical closure. The second patient developed progressive aortic regurgitation (AR) 2 days post procedure, and also underwent surgical removal with no residual AR. There was no cases of device embolization and no femoral arterial compromise. On median follow-up of 40.5 months (IQR 25-64), none of the patients developed complete heart block. Three patients (18.75%) had small residual shunts at latest follow-up which have not required any further intervention. Device closure of PMVSD's in children weighing ≤ 10 kg is feasible and safe with good procedural success rates. Use of both the antegrade and retrograde approaches may be necessary depending on anatomical variances.

Planning Transition of Care for Adolescents Affected by Congenital Heart Disease: The Irish National Pathway.

At some point in their life, adolescent patients with a congenital heart disease (CHD) transition from paediatric services to adult care facilities. The process is not without any risks, as it is often linked with a significantly progressive deterioration in adolescents' health and loss of follow-up. In fact, transition patients often encounter troubles in finding a care giver who is comfortable managing their condition, or in re-establishing trust with the new care provider. Planning the rules of transition is pivotal in preventing these risks. Unfortunately, the American and European guidelines on CHD provide just generic statements about transition. In a recently published worldwide inter-societies consensus document, a hybrid model of transition, which should be adapted for use in high- and low- resource settings, has been suggested. Currently, in literature there are a few models of transition for CHD patients, but they are by far local models and cannot be generalized to other regions or countries. This paper describes the Irish model for transition of care of CHD patients. Due to the peculiarity of the healthcare organization in the Republic of Ireland, which is centralized with one main referral centre for paediatric cardiology (in Dublin, with a few smaller satellite centres all around, according to the "hub and spoke" model) and one centre for adult with CHD (in Dublin), the model can be considered as a national one and the first to be released in the old continent.

Use of the Medtronic Microvascular Plug 7Q for transcatheter closure of large patent ductus arteriosus in infants weighing less than 2.5 kg.

BACKGROUND: The sole Food and Drug Administration-approved device for transcatheter closure of the patent arterial duct in premature infants is indicated for patent ductus arteriosus (PDAs) ≤ 4 mm in diameter. We report a two-center experience with transcatheter closure of large PDAs (>4 mm) in infants weighing <2.5 kg using the Microvascular Plug 7Q (MVP-7Q) device. METHODS: This is a retrospective review of departmental databases and medical charts to define patient cohort and report demographic, procedural, and follow-up data. RESULTS: Twenty-two patients (12 male) with a median gestational age and birthweight of 25.5 weeks (interquartile range [IQR] = 24-28) and 800 g (572-1075), respectively, underwent attempted PDA occlusion with the MVP-7Q using a transvenous approach. The median age and weight at the time of PDA occlusion was 32 days (IQR = 24-28) and 1100 g (IQR = 960-1700), respectively. The median PDA length was 12 mm (IQR = 11-12.65). The median PDA diameters at the aortic and pulmonary ends were 5.1 (IQR = 4.9-5.5) and 4.8 mm (IQR = 4.6-5.3), respectively. Successful device occlusion was achieved in 20 patients (91%). There were two failed attempts: One due to inappropriate sizing, and the other secondary to left pulmonary artery stenosis. There were no procedural complications and no residual shunting on follow-up. CONCLUSIONS: The MVP-7Q is safe and effective for transcatheter closure of large (>4 mm) PDAs in infants <2.5 kg. The lack of retention disks may help with avoiding impingement on surrounding vessels.

Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.

BACKGROUND: Morbidity with surgical systemic-to-pulmonary artery shunting (SPS) in infants ≤2.5 kg has remained high. Patent ductus arteriosus (PDA) stenting may be a valid alternative. The objective of this study is to evaluate outcomes following PDA stenting in patients ≤2.5 kg from four large tertiary centers. METHODS: Retrospective review of all neonates ≤2.5 kg with duct-dependent pulmonary circulation who underwent PDA stenting. Procedural details, pulmonary arterial growth, reinterventions, surgery type, and outcomes were assessed. RESULTS: PDA stents were implanted in 37 of 38 patients attempted (18 female) at a median procedural weight of 2.2 kg (interquartile range [IQR], 2-2.4 kg). Seven patients (18%) had a genetic abnormality and 16 (42%) had associated comorbidities. The median intensive care unit stay was 4 days (IQR, 2-6.75 days), and the median hospital stay was 20 days (IQR, 16-57.25). One patient required a rescue shunt procedure, with three others requiring early SPS (<30 days postprocedure). Twenty patients (54%) required reintervention with either balloon angioplasty, restenting, or both. At 6-month follow-up, right pulmonary artery growth (median z-score -1.16 to 0.01, p = 0.05) was greater than the left pulmonary artery (median z-score -0.9 to -0.64, p = 0.35). Serious adverse effects (SAEs) were seen in 18% (N = 7) of our cohort. One patient developed an SAE during planned reintervention There were no intraprocedural deaths, with one early procedure-related mortality, and three interstage mortalities not directly related to PDA stenting. CONCLUSIONS: PDA stenting in infants ≤2.5 kg is feasible and effective, promoting pulmonary artery growth. Reintervention rates are relatively high, though many are planned to allow for optimal growth before a definitive operation.

Right ventricular outflow tract stenting in symptomatic infants without the use of a long delivery sheath.

BACKGROUND AND OBJECTIVES: Right ventricular outflow tract (RVOT) stenting improves systemic oxygenation and facilitates pulmonary arterial growth in symptomatic infants prior to repair of tetralogy of Fallot. The aim of this study was to evaluate the safety and efficacy of RVOT stenting without the use of a long delivery sheath. METHODS: Retrospective data analysis of patients under 1 year of age undergoing RVOT stenting from January 2010 to January 2020 at a single tertiary pediatric cardiology center. RESULTS: Sixty-three RVOT stents were deployed during 53 procedures into 44 patients. The median age and weight at insertion were 41 days (range 2-204) and 3.6 kg (range 1.59-7) respectively. Thirty-one procedures were semi-elective and 22 were emergencies. Stent positioning was guided by transthoracic echocardiogram and/or RV angiography from a pigtail micro-catheter placed via the aorta. The median total procedure and fluoroscopy times were 67.5 (range 15-145) and 19 min (1-107), respectively. The median length of hospital stay was 7 days (range 1-258). Twenty-one patients were admitted to ICU post-procedure with a median ICU length of stay of 3 days (range 3-11). There were three major complications including two deaths within 30 days of the procedure. A patient with Cornelia de Lange Syndrome (1.8 kg) died following stent migration and inability to wean from emergency cardiopulmonary bypass and the second infant had an unexplained asystolic arrest post-procedure while awaiting transfer to ICU. CONCLUSIONS: RVOT stenting is technically possible with minimal complications without the need for a long delivery sheath. Additional imaging with transthoracic echocardiography can facilitate the safe deployment of the stent.

Correction of sinus venosus atrial septal defects with the 10 zig covered Cheatham-platinum stent - An international registry.

BACKGROUND: Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. METHODS: An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. RESULTS: Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p < .001) and RVEDVi from 149.1 ± 35.4 to 95.6 ± 21.43 ml/m2 (p < .001). CONCLUSIONS: Ten-zig CCPs of 7-8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.

Transcarotid Approach to Ventricular Septal Defect Closure in Small Infants.

Perimembranous ventricular septal defect closure in small infants has traditionally been a surgically treated defect, although alternative hybrid strategies are emerging. We aim to describe a novel approach to retrograde device closure of clinically relevant perimembranous ventricular septal defects in small infants via carotid cutdown. A retrospective review of all patients managed with attempted carotid cutdown for device closure of a perimembranous ventricular septal defect was recorded at a single tertiary cardiac centre. We summarized data on successful device deployment, conversion to open repair, complications, and length of stay. Eighteen infants with median (IQR) age of 7 months (5-9 months) and weight of 7.1 kg (6.5-7.8 kg) with clinically relevant PMVSD underwent attempted retrograde closure via carotid cutdown. Median (IQR) defect size was 8 mm (7-9 mm). Successful device deployment without significant aortic or tricuspid valve interference occurred in 15 (83%) patients. Three patients were converted to open repair, one following damage to the tricuspid valve apparatus. Median (IQR) hospital stay was 1 day (1-3 days). There were no complications related to carotid cutdown. Retrograde device closure of hemodynamically significant PMVSD is feasible and effective in small infants. Decision to convert to surgical repair should be made early if suboptimal device placement occurs. Carotid evaluation should be performed to rule out any access-related complications.

Hybrid Subxiphoid Perventricular Approach as an Alternative Access in Neonates and Small Children Undergoing Complex Congenital Heart Interventions.

The hybrid subxiphoid perventricular approach provides direct access through the heart and may alleviate the technical limitations of complex percutaneous interventions particularly in infants with low body weight. We present the outcomes from a tertiary cardiology center using this approach. We performed a retrospective review of all patients less than 15 kg who underwent a hybrid perventricular approach via a small subxiphoid incision. Medical records were reviewed to obtain clinical, demographic and outcome data. Seventeen patients underwent 18 hybrid perventricular procedures using a subxiphoid approach. Median age at time of procedure was 4.6 months (IQR = 1.6 to 18 months) and median weight was 6.2 kgs (IQR = 3.4 to 8.6 kgs). Six patients underwent hybrid pulmonary valve replacement (PVR), 5 patients underwent pulmonary outflow stenting, and 5 infants underwent hybrid ventricular septal defect (VSD) device closure. One patient with a single ventricle who did not tolerate a percutaneous approach underwent left pulmonary artery (LPA) stenting for severe LPA coarctation with subsequent right ventricular outflow tract (RVOT) stenting. One further patient underwent implantation of a larger diameter stent for pulmonary artery bifurcation stenosis. Procedure success rate was 89% with two of the VSD cases reverted to open surgical repair. There were no intra-procedural complications; however, one patient died within 72 h. Minor adverse events occurred in 2 patients including a wound infection in one patient with an immunodeficiency syndrome. Hybrid subxiphoid perventricular approach provides an excellent alternative access to the heart especially in low birth weight infants to prevent hemodynamic instability or in small children requiring large delivery sheaths.

Validating a risk assessment tool in United Kingdom and Irish paediatric cardiac catheterisation practice.

BACKGROUND: 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.

Balloon expandable covered stents as primary therapy for hemodynamically stable traumatic aortic injuries in children.

OBJECTIVES: To expand on the limited available literature regarding the use of balloon expandable covered stents for the treatment of traumatic aortic injuries (TAI) in the pediatric population. BACKGROUND: Although endovascular grafts have largely replaced surgery for TAI repair, there are significant limitations to the use of these grafts in pediatric patients. METHODS: Multicenter, retrospective chart review of pediatric patients with TAI following blunt chest wall trauma. Procedural characteristics, follow-up, and reinterventions are described. RESULTS: Six covered stents implanted in five patients. Median patient age was 12 years (11-13 years) and median weight 50 kg (44-54 kg). Procedural success was achieved in all cases. No procedural or postprocedural complications were noted. Median follow-up time was 24 months (11-36 months). CONCLUSIONS: Balloon expandable covered stent treatment of pediatric patients with TAI is a feasible alternative to open surgical repair, and preferred over endovascular grafts due to graft size limitations and the large delivery systems.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

Preclinical comparative assessment of a dedicated pediatric poly-L-lactic-acid-based bioresorbable scaffold with a low-profile bare metal stent.

BACKGROUND: Polymer-based bioresorbable scaffolds (PBBS) have been assessed for coronary revascularization with mixed outcomes. Few studies have targeted pediatric-specific scaffolds. We sought to assess safety, efficacy, and short-term performance of a dedicated drug-free PBBS pediatric scaffold compared to a standard low-profile bare metal stent (BMS) in central and peripheral arteries of weaned piglets. METHODS: Forty-two devices (22 Elixir poly-L-lactic-acid-based pediatric bioresorbable scaffolds [BRS] [6 × 18 mm] and 20 control BMS Cook Formula 418 [6 × 20 mm]) were implanted in the descending aorta and pulmonary arteries (PAs) of 14 female Yucatan piglets. Quantitative measurements were collected on the day of device deployment and 30 and 90 days postimplantation to compare device patency and integrity. RESULTS: The BRS has a comparable safety profile to the BMS in the acute setting. Late lumen loss (LLL) and percent diameter stenosis (%DS) were not significantly different between BRS and BMS in the PA at 30 days. LLL and %DS were greater for BRS versus BMS in the aorta at 30 days postimplantation (LLL difference: 0.96 ± 0.26; %DS difference: 16.15 ± 4.51; p < .05). At 90 days, %DS in the aortic BRS was less, and PA BRS LLL was also less than BMS. Histomorphometric data showed greater intimal proliferation and area stenosis in the BRS at all time points and in all vessels. CONCLUSIONS: A dedicated PBBS pediatric BRS has a favorable safety profile in the acute/subacute setting and demonstrates characteristics that are consistent with adult BRSs.

A randomized, controlled, multi-center trial of the efficacy and safety of the Occlutech Figulla Flex-II Occluder compared to the Amplatzer Septal Occluder for transcatheter closure of secundum atrial septal defects.

AIMS: The aim of this study was to compare the efficacy and safety of the Occlutech Figulla Flex II Occluder (OFFII) with the Amplatzer Septal Occluder (ASO) in patients > 8kg undergoing transcatheter ASD closure. METHODS AND RESULTS: Randomized, controlled, multi-center prospective clinical trial with randomization 2:1 in favor of the OFFII. Primary efficacy endpoint was the rate of successful device placement and defect closure without major complications at hospital discharge. All data were assessed through a core laboratory. Interim analysis was performed when 70% of the patients were treated to evaluate for noninferiority. From a total of 176 randomized subjects, interim analysis was performed on the first 158 patients (65.2% female) (107 OFFII/51 ASO) undergoing device closure at a median weight of 42 kg (range 13-125 kg). Seventy-six percent (120 patients) completed 6-month follow-up. Successful device placement (first attempt) was achieved in 99.1% of the OFF group vs 90.2% of the ASO group (P < 0.05). Early efficacy success was achieved in 94.4% of the OFFII group vs 90.2% of the ASO group (P < 0.001). The incidence of major complications was 5.6% for the OFFII group compared to 9.8% for the ASO. CONCLUSIONS: The OFFII device was not inferior to the ASO with less complications and greater efficacy than the ASO.

Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts.

BACKGROUND: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT). METHODS: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath. RESULTS: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access. CONCLUSIONS: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs.