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Objective: Postinfarction ventricular septal defect (PIVSD) is a rare and life-threatening complication with high risk of both surgical and medical treatment. Another option available now is transcatheter closure. The purpose of this paper is to report the results of such treatment with Amplatzer occluders. Method: Seven patients aged from 51 to 71 years were included. The procedure was performed between 2 and 10 weeks after myocardial infarction. One patient had double residual VSD (2 months after previous surgery) and another, coexisting critical stenosis of right coronary artery (RCA). All patients were in III/IV NYHA class, on intropes, one patient on aortic balloon counterpulsation. Venous jugular approach was used to close the VSD in six patients, venous transfemoral in one patient. Implantation of six Ampaltzer atrial septal occluders (ASO) and one muscular Amplatzer VSD occluder (VSO) were performed. Results: All procedures but two were finished successfully. In one patient, the defect could not be entered neither from the venous nor the arterial side due to unusual oblique course (which was confirmed during subsequent operation). In the second patient (2 weeks after MI), the reason was unstable position of 24 mm ASO (probably due to necrotic borders of VSD). Immediate significant clinical improvement was achieved in all patients, in whom PIVSD was closed with Amplatzer occluders. In one postsurgical patient, two ASO were used; in another patient, prior to VSD closure, PTCA and stent implantation to RCA was performed. The stretched diameter of PIVSD ranged from 8 to 22 mm, the size of implanted Amplatzer occluders from 12 to 24 mm. Fluoroscopy time was 60 min (18–120). During the procedure, ventricular fibrillation requiring defibrillation was observed in three patients. One patient died 1 week after the procedure because of multiorgan failure and increasing mitral incompetence (MI). Conclusions: Despite some technical problems, implantation of Amplatzer occluders, is an attractive option of treatment of patients with subacute PIVSD. [Copyright &y& Elsevier]

Background: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD)., Methods: Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point., Results: A total of 375 children (mean age, 8.1±5.6 years; 52% female) were randomized to sacubitril/valsartan (n=187) or enalapril (n=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P =0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children., Conclusions: In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks., Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02678312.

The clinical significance of B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in pediatric patients remains an area of evolving understanding, particularly regarding their utility in the presence or absence of pre-existing heart conditions. While clear cutoff values and established roles in heart failure are understood in adult patients, pediatric norms vary with age, complicating interpretation. Notably, the emergence of multi-system inflammatory syndrome in children (MIS-C) has highlighted the importance of these markers not only in the detection of acute heart failure but also as a marker of disease severity and even as a differential diagnosis tool. This review summarizes current knowledge on the utility of BNP and NT-proBNP in pediatric patients. Their unique physiology, including circulation and compensation mechanisms, likely influence BNP and NT-proBNP release, potentially even in non-heart failure states. Factors such as dynamic volemic changes accompanying inflammatory diseases in children may contribute. Thus, understanding the nuanced roles of BNP and NT-proBNP in pediatric populations is crucial for the accurate diagnosis, management, and differentiation of cardiac and non-cardiac conditions.

Background: Radiofrequency catheter ablation (RFCA) of the slow pathway in atrioventricular nodal reentry tachycardia (AVNRT) is associated with high efficacy and low risk of total perioperative or late atrioventricular block. This study aimed to evaluate the efficacy, safety, and feasibility of slow-pathway RFCA for AVNRT using a zero-fluoroscopy approach., Methods: Data were obtained from a prospective multicenter registry of catheter ablation from January 2012 to February 2018. Consecutive unselected patients with the final diagnosis of AVNRT were recruited. Electrophysiological and 3-dimensional (3D) electroanatomical mapping systems were used to create 3D maps and to navigate only 2 catheters from the femoral access. Acute procedural efficacy was evaluated using the isoproterenol and/or atropine test, with 15-min observation after ablation. Each case of recurrence or complication was consulted at an outpatient clinic during long-term follow-up., Results: Of the 1032 procedures, 1007 (97.5%) were completed without fluoroscopy. Conversion to fluoroscopy was required in 25 patients (2.5%), mainly due to an atypical location of the coronary sinus (n = 7) and catheter instability (n = 7). The mean radiation exposure time was 1.95 ± 1.3 min for these cases. The mean fluoroscopy time for the entire study cohort was 0.05 ± 0.4 min. The mean total procedure time was 44.8 ± 18.6 min. There were no significant in-hospital complications. The total success rate was 96.1% (n = 992), and the recurrence rate was 3.9% (n = 40)., Conclusion: Slow-pathway RFCA can be safely performed without fluoroscopy, with a minimal risk of complications and a high success rate., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers., Aims: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey., Methods: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points., Results: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy)., Conclusions: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

Cases of severe heart damage in patients presenting with multisystem inflammatory syndrome in children are one of the most intriguing phenomena during the coronavirus disease of 2019 pandemic. The pathophysiology of myocardial changes in the course of this syndrome has not been fully understood yet. We present a case of a child with multisystem inflammatory syndrome in children and with cardiac changes corresponding to Takotsubo syndrome.

Riociguat, a soluble guanylate cyclase stimulator, is approved for treatment of adults with pulmonary arterial hypertension (PAH). The safety, tolerability, and pharmacokinetics (PK) of oral riociguat in a pediatric population with PAH was assessed in PATENT-CHILD (NCT02562235), a multicenter, single-arm, 24-week, open-label, Phase 3 study. Patients aged 6-17 years in World Health Organization functional class (WHO-FC) I-III treated with stable endothelin receptor antagonists and/or prostacyclin analogs received riociguat equivalent to 0.5-2.5 mg three times daily in adults, as either oral pediatric suspension or tablets, based on bodyweight. Primary outcomes were safety, tolerability, and PK of riociguat. Twenty-four patients (mean age 12.8 years), 18 of whom were in WHO-FC II, were enrolled. Adverse events (AEs), mostly mild or moderate, were reported in 20 patients (83%). Four patients (17%) experienced a serious AE; all resolved by study end and two (8%) were considered study-drug related. Hypotension was reported in three patients and hemoptysis in one (all mild/moderate intensity). Riociguat plasma concentrations in pediatric patients were consistent with those published in adult patients. From baseline to Week 24, mean ± standard deviation increase in 6-minute walking distance was 23 ± 69 m ( n  = 19), and mean decrease in NT-proBNP was -66 ± 585 pg/ml ( n  = 14). There was no change in WHO-FC. Two patients experienced clinical worsening events of hospitalization for right heart failure. PK results confirmed a suitable riociguat dosing strategy for pediatric patients with PAH. The data suggest an acceptable safety profile with potential efficacy signals., Competing Interests: H. G. A. reports fees for lectures and member of advisory boards for Bayer and Janssen. D. D. I. reports the University of Colorado contracts with Acceleron, Actelion/Janssen, Altavant, Bayer, Eli Lilly, GlaxoSmithKline, and United Therapeutics for D. D. I. to be a consultant and perform clinical trials. S. M. reports consultancy from Actelion/Janssen and GlaxoSmithKline. J. P. reports fees for lectures and/or consultancy from Actelion/Janssen. T. P. reports fees for lectures, member of advisory boards, and research grants from Actelion/Janssen, Bayer, GlaxoSmithKline, and Pfizer. H. Y. reports research grants and lecture fees from Actelion, AbbVie, and Teijin Pharma; research grants from Tanabe‐Mitsubishi; lecture fees from Bayer, GE Healthcare Japan, Merck Sharp and Dohme, Novo Nordisk, Nihon Seiyaku, Nihon Shinyaku, Pfizer, and Termo. G. C., M. G., S. L., A. R., S. S., S. W., G. W. are employees and stockholders of Bayer AG. D. B. is a steering committee member for Eli Lilly and Company, and steering committee and advisory board member for Actelion Pharmaceuticals, Bayer, and Novartis. M. B. reports fees for lectures and/or consultancy from Acceleron, Actelion/Janssen, AOP, Bayer, Eli Lilly, GlaxoSmithKline, and Gossamer; unrestricted research grants from Actelion and Bayer. The remaining authors declare no conflict of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

During the winter months of 2020/2021 a wave of multisystem inflammatory syndrome in children (MIS-C) emerged in Poland. We present the results of a nationwide register aiming to capture and characterise MIS-C with a focus on severity determinants. The first MIS-C wave in Poland was notably high, hence our analysis involved 274 children. The group was 62.8% boys, with a median age of 8.8 years. Besides one Asian, all were White. Overall, the disease course was not as severe as in previous reports, however. Pediatric intensive care treatment was required for merely 23 (8.4%) of children, who were older and exhibited a distinguished clinical picture at hospital admission. We have also identified sex-dependent differences; teenage boys more often had cardiac involvement (decreased ejection fraction in 25.9% vs. 14.7%) and fulfilled macrophage activation syndrome definition (31.0% vs. 15.2%). Among all boys, those hospitalized in pediatric intensive care unit were significantly older (median 11.2 vs. 9.1 years). Henceforth, while ethnicity and sex may affect MIS-C phenotype, management protocols might be not universally applicable, and should rather be adjusted to the specific population., (© 2021. The Author(s).)

Competing Interests: The authors declare no conflict of interest.