Early brain magnetic resonance imaging findings and neurodevelopmental outcome in children with congenital heart disease: A systematic review.

AIM: To investigate the association between early brain magnetic resonance imaging (MRI) findings and neurodevelopmental outcome (NDO) in children with congenital heart disease (CHD). METHOD: A search for studies was conducted in Embase, Medline, Web of Science, Cochrane Central, PsycINFO, and Google Scholar. Observational and interventional studies were included, in which patients with CHD underwent surgery before 2 months of age, a brain MRI scan in the first year of life, and neurodevelopmental assessment beyond the age of 1 year. RESULTS: Eighteen studies were included. Thirteen found an association between either quantitative or qualitative brain metrics and NDO: 5 out of 7 studies showed decreased brain volume was significantly associated with worse NDO, as did 7 out of 10 studies on brain injury. Scanning protocols and neurodevelopmental tests varied strongly. INTERPRETATION: Reduced brain volume and brain injury in patients with CHD can be associated with impaired NDO, yet standardized scanning protocols and neurodevelopmental assessment are needed to further unravel trajectories of impaired brain development and its effects on outcome.

"Absent" unilateral pulmonary arteries aren't absent, but disconnected. Review of the current literature based on 4 cases.

BACKGROUND: Disconnected unilateral pulmonary arteries are frequently misdiagnosed as "absent". They typically arise from the base of the innominate artery and are fed by an aberrant arterial duct. If diagnosed early enough, they can be reconnected with catheter techniques even after closure of this aberrant duct. Consecutive surgical anatomical correction at a later stage is possible. METHODS: Four cases illustrate the anatomical findings on computed tomography and angiography, all show an outpouching at the base of the brachiocephalic artery. RESULTS: The therapeutic approach consisted of stenting of the aberrant ductus and consecutive surgery. In the oldest patient, 13 years, such an approach was impossible. CONCLUSION: If identified early in life, disconnected pulmonary arteries can be recruited with catheter techniques, and reconnected surgically at a later stage. It is not yet known if this approach prevents pulmonary damage, which is frequently seen in older untreated patients.

Staged correction of pulmonary atresia, ventricular septal defect, and collateral arteries.

OBJECTIVES: Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed. METHODS: Since 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto-pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow-up. RESULTS: In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow-up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II. CONCLUSIONS: After complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

The First Evaluation of Remote Magnetic Navigation-Guided Pediatric Ventricular Arrhythmia Ablation.

Catheter ablation (CA) is an important treatment option for ventricular arrhythmias (VA) in pediatric cardiology. Currently, various CA techniques are available, including remote magnetic navigation (RMN)-guided radiofrequency (RF) ablation. However, no studies evaluate RMN-guided ablative therapy outcomes in children with VA yet. This study aimed to compare procedural and long-term outcomes between RMN-guided and manual (MAN)-guided VA ablation in children. This single-center, retrospective study included all CA procedures for VA performed in children with or without structural heart disease from 2008 until 2020. Two study groups were defined by CA technique: RMN or MAN. Primary outcome was recurrence of VA. Baseline clinical, procedural and safety data were also evaluated. This study included 22 patients, who underwent 30 procedures, with a median age of 15 (IQR 14-17; range 1-17) years and a mean weight of 57 ± 20 kg. In total, 14 procedures were performed using RMN and 16 using MAN (22 first and 8 redo procedures). Regarding first procedures, recurrence rates were significantly lower in RMN compared to MAN (20% versus 67%, P = 0.029), at a mean follow-up of 5.2 ± 3.0 years. Moreover, fluoroscopy dosages were significantly lower in RMN compared to MAN [20 (IQR 14-54) versus 48 (IQR 38-62) mGy, P = 0.043]. In total, 20 patients (91%) were free of VA following their final ablation procedure. This is the first study to investigate the use of RMN in pediatric VA ablation. RMN showed improved outcomes compared to MAN, resulting in lower VA recurrence and reduced fluoroscopy exposure.

Development of a Ductal Aneurysm in a Patient with Williams Syndrome, and Subsequent Interventional Closure.

Whilst stenosis of systemic and pulmonary arteries in Williams syndrome is frequently described, aneurysm formation is uncommon. We provide the first description of a Williams patient with development of an aneurysm of the arterial duct. This aneurysm developed concomitantly with supravalvar aortic, and peripheral pulmonary stenosis. The duct was closed interventionally to reduce the risk of rupture.

Transthoracic access for pulmonary artery stenting.

Pulmonary artery stenting may not be possible transcutaneously because of anatomic features. Although intraoperative stenting has been well described, we present a case in which stenting of the left pulmonary artery was performed transthoracically in a separate procedure. Unusual anatomic conditions may require a multi-disciplinary hybrid approach to achieve the desired results.

CHIP-Family intervention to improve the psychosocial well-being of young children with congenital heart disease and their families: results of a randomised controlled trial.

OBJECTIVE: Children with congenital heart disease and their families are at risk of psychosocial problems. Emotional and behavioural problems, impaired school functioning, and reduced exercise capacity often occur. To prevent and decrease these problems, we modified and extended the previously established Congenital Heart Disease Intervention Program (CHIP)-School, thereby creating CHIP-Family. CHIP-Family is the first psychosocial intervention with a module for children with congenital heart disease. Through a randomised controlled trial, we examined the effectiveness of CHIP-Family. METHODS: Ninety-three children with congenital heart disease (age M = 5.34 years, SD = 1.27) were randomised to CHIP-Family (n = 49) or care as usual (no psychosocial care; n = 44). CHIP-Family consisted of a 1-day group workshop for parents, children, and siblings and an individual follow-up session for parents. CHIP-Family was delivered by psychologists, paediatric cardiologists, and physiotherapists. At baseline and 6-month follow-up, mothers, fathers, teachers, and the child completed questionnaires to assess psychosocial problems, school functioning, and sports enjoyment. Moreover, at 6-month follow-up, parents completed program satisfaction assessments. RESULTS: Although small improvements in child outcomes were observed in the CHIP-Family group, no statistically significant differences were found between outcomes of the CHIP-Family and care-as-usual group. Mean parent satisfaction ratings ranged from 7.4 to 8.1 (range 0-10). CONCLUSIONS: CHIP-Family yielded high program acceptability ratings. However, compared to care as usual, CHIP-Family did not find the same extent of statistically significant outcomes as CHIP-School. Replication of promising psychological interventions, and examination of when different outcomes are found, is recommended for refining interventions in the future. TRIAL REGISTRY: Dutch Trial Registry number NTR6063, https://www.trialregister.nl/trial/5780.

Dilating and fracturing side struts of open cell stents frequently used in pediatric cardiac interventions-An in vitro study.

BACKGROUND: Open cell stents are frequently used in interventional therapy of congenital heart disease. Overstenting of vessel branches may necessitate strut dilation. METHODS AND RESULTS: The strut size achievable in Bard Valeo and Cook Formula stents, and the pressure necessary to fracture struts was assessed. In addition, a self expanding stent (Optimed SinusflexDS) was also tested. With the original balloon at nominal pressure, in Valeo stents side struts could be dilated to approximately 90% of the nominal stent diameter, in Formula stents to approximately 80%. With larger high pressure woven balloons, strut size increased to approximately 125% in Valeo stents, and to approximately 105% in the Formula. Strut fracture can connect two adjoining struts. Pressures were dependent on the balloon utilized. Sidestruts of the Sinusflex could lastingly overdilated with large balloons only. CONCLUSION: Dilation and overdilation of side struts in open cell stents can be achieved. Dependent on the clinical context, the original balloon used to place the premounted stent can be used to achieve strut dilation, but woven high pressure balloons maybe safer for patients. Should a larger diameter be required, these high pressure woven balloons can achieve bigger diameters and even strut fracture.

The CHIP-Family study to improve the psychosocial wellbeing of young children with congenital heart disease and their families: design of a randomized controlled trial.

BACKGROUND: Children with congenital heart disease (CHD) are at increased risk for behavioral, emotional, and cognitive problems. They often have reduced exercise capacity and participate less in sports, which is associated with a lower quality of life. Starting school may present more challenges for children with CHD and their families than for families with healthy children. Moreover, parents of children with CHD are at risk for psychosocial problems. Therefore, a family-centered psychosocial intervention for children with CHD when starting school is needed. Until now, the 'Congenital Heart Disease Intervention Program (CHIP) - School' is the only evidence-based intervention in this field. However, CHIP-School targeted parents only and resulted in non-significant, though positive, effects as to child psychosocial wellbeing. Hence, we expanded CHIP by adding a specific child module and including siblings, creating the CHIP-Family intervention. The CHIP-Family study aims to (1) test the effects of CHIP-Family on parental mental health and psychosocial wellbeing of CHD-children and to (2) identify baseline psychosocial and medical predictors for the effectiveness of CHIP-Family. METHODS: We will conduct a single-blinded randomized controlled trial comparing the effects of CHIP-Family with care as usual (no psychosocial intervention). Children with CHD (4-7 years old) who are starting or attending kindergarten or primary school (first or second year) at the time of first assessment and their families are eligible. CHIP-Family consists of a separate one-day workshop for parents and children. The child workshop consists of psychological exercises based on the evidence-based cognitive behavioral therapy Fun FRIENDS protocol and sports exercises. The parent workshop focuses on problem prevention therapy, psychoeducation, general parenting skills, skills specific to parenting a child with CHD, and medical issues. Approximately 4 weeks after the workshop, parents receive an individual follow-up session. The baseline (T1) and follow-up assessment (T2 = 6 months after T1) consist of online questionnaires filled out by the child, parents, and teacher (T2 only). Primary outcome measures are the CBCL for children and the SCL-90-R for parents. DISCUSSION: This trial aims to test the effects of an early family-centered psychosocial intervention to meet the compelling need of young children with CHD and their families to prevent (further) problems. If CHIP-Family proves to be effective, it should be structurally implemented in standard care. TRIAL REGISTRATION: Dutch Trial Registry; NTR6063 on 23 August, 2016.

Endocarditis of a congenital coronary fistula in a child.

Endocarditis of congenital coronary fistulas in the cardiac chambers is rare, especially in the paediatric age group. We describe the case of a 9-year-old boy with a fistula from the dilated right coronary artery to the junction of the superior caval vein to the right atrium, complicated by endocarditis. Treatment consisted of 6 weeks of antibiotics and interventional closure of the fistula 3 months later with an Amplatzer vascular plug.

Limited effects of intravenous paracetamol on patent ductus arteriosus in very low birth weight infants with contraindications for ibuprofen or after ibuprofen failure.

UNLABELLED: Finding the optimal pharmacological treatment of a patent ductus arteriosus (PDA) in preterm neonates remains challenging. There is a growing interest in paracetamol as a new drug for PDA closure. In this prospective observational cohort study, we evaluated the effectiveness of intravenous paracetamol in closing a PDA in very low birth weight infants with a hemodynamically significant PDA who either did not respond to ibuprofen or had a contraindication for ibuprofen. They received high-dose paracetamol therapy (15 mg/kg/6 h intravenous) for 3-7 days. Cardiac ultrasounds were performed before and 3 and 7 days after treatment. Thirty-three patients were included with a median gestational age of 25(1/7) weeks (IQR 1.66), a median birth weight of 750 g (IQR 327), and a median postnatal age of 14 days (IQR 12). Paracetamol was ineffective in 27/33 patients (82 %). Even more, after previous exposure to ibuprofen, this was even 100 %. CONCLUSION: In this study, paracetamol after ibuprofen treatment failure was not effective for PDA closure in VLBW infants. From the findings of this study, paracetamol treatment for PDA closure cannot be recommended for infants with a postnatal age >2 weeks. Earlier treatment with paracetamol for PDA might be more effective.

CHD7 mutations are not a major cause of atrioventricular septal and conotruncal heart defects.

Since 2004, CHD7 mutations have been a known cause of CHARGE (Coloboma, Heart defects, Atresia of choane, Retardation of growth and development, Genital hypoplasia, Ear anomalies) syndrome, but the full clinical spectrum of CHD7 mutations is only now gradually emerging. CHD7 mutations have been identified in patients who do not fulfill the clinical criteria for CHARGE syndrome and in patients with overlapping syndromes. Variable congenital heart defects occur in the majority of patients with CHD7 mutations, with an overrepresentation of atrioventricular septal defects and conotruncal heart defects. This prompted us to study CHD7 in 46 patients with these heart defects and one other feature of CHARGE syndrome. We identified two CHD7 variants that were inherited from a healthy parent (c.3778 + 17C > T, c.7294G > A), but no pathogenic CHD7 mutations. We conclude that CHD7 mutations are not a major cause of the atrioventricular septal defects and conotruncal heart defects, not even if one extra phenotypic feature of CHARGE syndrome is present. Therefore, CHD7 analysis should not be performed routinely in this group of patients. However, we do recommend adding CHD7 to massive parallel sequencing gene panels for diagnostic work in patients with syndromic heart defects.

CeRebrUm and CardIac Protection with ALlopurinol in Neonates with Critical Congenital Heart Disease Requiring Cardiac Surgery with Cardiopulmonary Bypass (CRUCIAL): study protocol of a phase III, randomized, quadruple-blinded, placebo-controlled, Dutch multicenter trial.

BACKGROUND: Neonates with critical congenital heart disease (CCHD) undergoing cardiac surgery with cardiopulmonary bypass (CPB) are at risk of brain injury that may result in adverse neurodevelopment. To date, no therapy is available to improve long-term neurodevelopmental outcomes of CCHD neonates. Allopurinol, a xanthine oxidase inhibitor, prevents the formation of reactive oxygen and nitrogen species, thereby limiting cell damage during reperfusion and reoxygenation to the brain and heart. Animal and neonatal studies suggest that allopurinol reduces hypoxic-ischemic brain injury and is cardioprotective and safe. This trial aims to test the hypothesis that allopurinol administration in CCHD neonates will result in a 20% reduction in moderate to severe ischemic and hemorrhagic brain injury. METHODS: This is a phase III, randomized, quadruple-blinded, placebo-controlled, multicenter trial. Neonates with a prenatal or postnatal CCHD diagnosis requiring cardiac surgery with CPB in the first 4 weeks after birth are eligible to participate. Allopurinol or mannitol-placebo will be administered intravenously in 2 doses early postnatally in neonates diagnosed antenatally and 3 doses perioperatively of 20 mg/kg each in all neonates. The primary outcome is a composite endpoint of moderate/severe ischemic or hemorrhagic brain injury on early postoperative MRI, being too unstable for postoperative MRI, or mortality within 1 month following CPB. A total of 236 patients (n = 188 with prenatal diagnosis) is required to demonstrate a reduction of the primary outcome incidence by 20% in the prenatal group and by 9% in the postnatal group (power 80%; overall type 1 error controlled at 5%, two-sided), including 1 interim analysis at n = 118 (n = 94 with prenatal diagnosis) with the option to stop early for efficacy. Secondary outcomes include preoperative and postoperative brain injury severity, white matter injury volume (MRI), and cardiac function (echocardiography); postnatal and postoperative seizure activity (aEEG) and regional cerebral oxygen saturation (NIRS); neurodevelopment at 3 months (general movements); motor, cognitive, and language development and quality of life at 24 months; and safety and cost-effectiveness of allopurinol. DISCUSSION: This trial will investigate whether allopurinol administered directly after birth and around cardiac surgery reduces moderate/severe ischemic and hemorrhagic brain injury and improves cardiac function and neurodevelopmental outcome in CCHD neonates. TRIAL REGISTRATION: EudraCT 2017-004596-31. Registered on November 14, 2017. ClinicalTrials.gov NCT04217421. Registered on January 3, 2020.

Propranolol, a very promising treatment for ulceration in infantile hemangiomas: a study of 20 cases with matched historical controls.

BACKGROUND: Ulceration is a common but poorly understood complication of infantile hemangiomas (IH) that is difficult to control. OBJECTIVE: To investigate the possible role of monotherapy with propranolol for ulcerating IH. METHODS: Propranolol was given to 20 patients with IH, who suffered from ulceration at the start of treatment (mean age at onset of treatment, 3.5 months; standard error of the mean: 0.4). After cardiac screening, propranolol was administered in a progressive schedule to 2 to 2.5 mg/kg per day, divided in 3 doses. Blood pressure, heart rate, and fasting glucose levels were monitored during the first 3 days in hospital and, in the absence of complications, treatment was continued at home until the age of approximately 1 year. The 20 propranolol-treated patients were matched to patients from a historical control group, seen before the 'propranolol era'. These matches were randomly made by using clinical pictures based on type, location and size of the IH, extent of ulceration, and age at the start of ulceration. RESULTS: The time to complete healing from the onset of ulceration was significantly shorter for the propranolol-treated patients, compared with the control group (8.7 vs 22.4 weeks; t test: P < .015). In the propranolol group, a tendency to shorter ulceration duration was seen in patients starting propranolol at an earlier stage of disease. LIMITATIONS: The study was limited by the partially retrospective design and the small number of patients. CONCLUSION: Propranolol reduces the duration of ulceration in IH and seems to be more effective when started in an early phase. We propose propranolol as the treatment of first choice for ulcerating IH.

Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment.

Kaposiform hemangioendothelioma is a rare vascular tumor in children. Especially, in association with the Kasabach-Merritt Phenomenon it can be life threatening. The management of these patients is very difficult and an aggressive treatment regime is required. Several multimodality and chemotherapeutic regimens have been described but with variable success and many side effects. We present a 6-week-old boy with Kaposiform hemangioendothelioma and Kasabach-Merritt Phenomenon. Ongoing propranolol treatment with only 4 initial courses of vincristine resulted in a remission that lasted at least 1 year.

Protective effect of periconceptional folic acid supplements on the risk of congenital heart defects: a registry-based case-control study in the northern Netherlands.

AIMS: To investigate the potentially protective of periconceptional folic acid use on the risk of congenital heart defects (CHDs) relative to other non-folate related malformations. METHODS AND RESULTS: We analysed data from a large regional register of birth defects (EUROCAT-Northern Netherlands), over a 10 year period (1996-2005) for a case-control study. The cases were mothers who had delivered infants with isolated or complex heart defects, without any related syndrome or genetic abnormality (n = 611). We used two control groups; one from the EUROCAT database and another from the general population. The registry controls consisted of mothers of children with a known chromosomal or genetic defect, and with infants with other non-folate related congenital malformations (n = 2401). Additional folic acid was taken as a single supplement or as a multivitamin containing folic acid in a dose of >or=400 microg daily. Mothers who had used folate antagonists or who had diabetes, and mothers of children with oral clefts, hypospadias, limb reduction- or neural tube defects, were excluded from both groups. Potentially confounding factors of periconceptional folic acid use in relation to CHD were explored, including baby's birth year, maternal body mass index, education, maternal age at delivery of index baby, smoking behaviour, and alcohol use during pregnancy. Periconceptional folic acid use revealed an odds ratio (OR) of 0.82 (95% CI 0.68-0.98) for all types of CHD relative to other malformations. The estimated relative risk for CHDs of additional folic acid use compared with the general population was comparable [OR 0.74 (95%CI 0.62-0.88)]. Subgroup analysis showed an OR of 0.62 (95% CI 0.47-0.82) for isolated septal defects. The proportions of the potential confounders between mothers of case and control infants did not differ significantly. CONCLUSION: Our results support the hypothesis that additional periconceptional folic acid use reduces CHD risk in infants. Use of periconceptional folic acid supplements was related to approximately 20% reduction in the prevalence of any CHD. Given the relatively high prevalence of CHD worldwide, our findings are important for public health.

Homograft durability after correction of pulmonary atresia and ventricular septal defect with or without systemic pulmonary collateral arteries.

Background: Pulmonary atresia and ventricular septal defect (PA-VSD), with or without systemic pulmonary collateral arteries (SPCAs), represents a complex anatomic and surgical spectrum of congenital heart disease. Currently, there is limited evidence on homograft durability after complete correction, which potentially could be affected by anatomic differences in pulmonary vasculature. Methods: This retrospective single-center study included all 69 consecutive PA-VSD patients (46 with SPCAs, 23 without SPCAs) operated on between 1978 and 2018. The primary interest was in homograft durability after complete repair. Longitudinal echocardiographic homograft function and right ventricular systolic pressure were analyzed with linear mixed-effects models. Results: The median duration of follow-up was 20 years. Of the 46 patients with SPCAs, 37 (80.4%) underwent biventricular correction at a median age of 2.7 years (interquartile range [IQR], 1.8-6.3 years). Two patients are currently awaiting unifocalization and correction. All 23 patients without SPCAs underwent successful complete correction at a median age of 1.6 years (IQR, 1.1-3.6 years). Freedom from any reintervention after 20 years was 15%. When a homograft was used during correction, freedom from homograft replacement after 20 years was comparable in the 2 groups (P = .925), at 32 ± 11% in the SPCA group and 32 ± 13% in the non-SPCA group. Indications for homograft replacement were isolated stenosis (n = 7; 46.7%), isolated regurgitation (n = 3; 20.0%), and mixed stenosis and regurgitation (n = 5; 33.3%) in the SPCA group and isolated stenosis (n = 8; 88.9%) and stenosis and regurgitation (n = 1; 11.1%) in the non-SPCA group. Peak homograft gradient was significantly (P = .0003) higher in patients without SPCA, with a comparable rate of progression in the 2 groups. However, the prevalence of severe pulmonary regurgitation (PR) was higher in patients with SPCAs, estimated at 35% at 10 years, compared with 15% in patients without SPCAs. Conclusions: Homografts used for right ventricular outflow tract reconstruction in patients with PA-VSD, either with or without SPCAs, have similar limited durability. Repeated reintervention is common, and careful follow-up with attention to severe PR is warranted.

Reconstruction of the Aortic Arch in Neonates and Infants: The Importance of Patch Material.

OBJECTIVES: Restenosis after aortic arch reconstruction is a known complication in neonates and infants. Homograft is the most commonly used patch material for aortic arch reconstructions in our center. Since 2014, tissue-engineered bovine pericardium (CardioCel) has been used as an alternative. The aim of our study was to determine whether the choice of material affected the development of restenosis in these patients. METHODS: Data of all neonates and infants who underwent aortic arch reconstruction with the use of any patch material between 2005 and 2016 were analyzed. Restenosis was defined by the need for reintervention, either percutaneous or surgical. RESULTS: Forty-one patients underwent aortic arch repair. Excluding the 30-day mortality, 36 patients represented the study population. At primary repair, the aortic arch was reconstructed with homograft (n = 26) or CardioCel (n = 10). Restenosis was documented during the first year of life in 13 patients: Six (23%) patients in the homograft group and seven (70%) patients in the CardioCel group (P = .01). In the homograft group, the median time from operation to first intervention for restenosis was 22.0 (range: 14-32) weeks, as compared to 14.0 (range: 7-21) weeks in the CardioCel group (P = .04). CONCLUSION: We conclude that choice of patch material is likely to be an important determinant for the risk of restenosis needing reintervention following reconstruction of the aortic arch in neonates and infants.

Remote magnetic navigation shows superior long-term outcomes in pediatric atrioventricular (nodal) tachycardia ablation compared to manual radiofrequency and cryoablation.

BACKGROUND: Catheter ablation (CA) is the first-choice treatment for tachyarrhythmia in children. Currently available CA techniques differ in mechanism of catheter navigation and energy sources. There are no large studies comparing long-term outcomes between available CA techniques in a pediatric population with atrioventricular reentry tachycardia (AVRT) or atrioventricular nodal reentry tachycardia (AVNRT) mechanisms. OBJECTIVE: This study aimed to compare procedural and long-term outcomes of remote magnetic navigation-guided radiofrequency (RF) ablation (RMN), manual-guided RF ablation (MAN) and manual-guided cryoablation (CRYO). METHODS: This single-center, retrospective study included all first consecutive CA procedures for AVRT or AVNRT performed in children without structural heart disease from 2008 to 2019. Three study groups were defined by the ablation technique used: RMN, MAN or CRYO. Primary outcome was long-term recurrence of tachyarrhythmia. RESULTS: In total, we included 223 patients, aged 14 (IQR 12-16) years; weighting 56 (IQR 47-65) kilograms. In total, 108 procedures were performed using RMN, 76 using MAN and 39 using CRYO. RMN had significantly lower recurrence rates compared to MAN and CRYO at mean follow-up of 5.5 ± 2.9 years (AVRT: 4.3% versus 15.6% versus 54.5%, P < 0.001; AVNRT: 7.7% versus 8.3% versus 35.7%, P = 0.008; for RMN versus MAN versus CRYO respectively). In AVNRT ablation, RMN had significantly lower fluoroscopy doses compared to CRYO [30 (IQR 20-41) versus 45 (IQR 29-65) mGy, P = 0.040). CONCLUSION: In pediatric patients without structural heart disease who underwent their first AV(N)RT ablation, RMN has superior long-term outcomes compared to MAN and CRYO, in addition to favorable fluoroscopy doses.

Ibuprofen treatment after the first days of life in preterm neonates with patent ductus arteriosus.

AIM: Patent ductus arteriosus (PDA) is treated with ibuprofen and it is known that the clearance of ibuprofen increases with postnatal age. We aimed to study whether postnatal age-adjusted ibuprofen dosages improve the effectiveness of treatment compared to standard ibuprofen dosages after the first days of life. METHODS: A historical cohort of 207 preterm neonates treated with standard ibuprofen dosages (Group A; 2011-2015) was compared to a prospective cohort of 66 preterm neonates treated with postnatal age-adjusted ibuprofen dosages (Group B; 2015-2016). RESULTS: Both groups had comparable background characteristics. Treatment was started after median 6 (25-75th percentile: 4-11) and 5 (25-75th percentile: 4-11) days and effectiveness was 33.2 and 44.7% (p = .17) in groups A and B, respectively. No hemodynamically significant PDA was found in 23/49 (46.9%) of the patients born before 28 weeks after adjusted ibuprofen dosages compared to 48/162 (29.6%) after standard ibuprofen dosages (p = .04). There were significantly more reversible side effects with the postnatal age-adjusted ibuprofen dosages (p = .04). CONCLUSIONS: There seems to be a trend to higher effectiveness with the adjusted ibuprofen dosages in preterm neonates before 28 weeks, but it is associated with more reversible side effects.