Steven E. Lipshultz , Thomas D. Ryan , Leah C. Mumm , Teresa M. Lee
{"title":"The first six international conferences on cardiomyopathy in children: Beginning with the end in mind","authors":"Steven E. Lipshultz , Thomas D. Ryan , Leah C. Mumm , Teresa M. Lee","doi":"10.1016/j.ppedcard.2025.101900","DOIUrl":"10.1016/j.ppedcard.2025.101900","url":null,"abstract":"","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"80 ","pages":"Article 101900"},"PeriodicalIF":0.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145790509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Khalifah A. Aldawsari , Claudia Mattos , Becky Li , Mubeena Abdulkarim , Michael Lopez
{"title":"The prevalence of QRS fragmentation in children with acute myocarditis: Single center experience","authors":"Khalifah A. Aldawsari , Claudia Mattos , Becky Li , Mubeena Abdulkarim , Michael Lopez","doi":"10.1016/j.ppedcard.2026.101904","DOIUrl":"10.1016/j.ppedcard.2026.101904","url":null,"abstract":"<div><h3>Background</h3><div>Acute myocarditis in children presents with a broad spectrum of clinical features, often delaying diagnosis. Fragmented QRS (fQRS) is an emerging EKG finding associated with various cardiac conditions.</div></div><div><h3>Objectives</h3><div>This study aimed to investigate the prevalence and significance of fQRS in children with acute myocarditis.</div></div><div><h3>Methods</h3><div>Patients less than 18 years of age who presented to our institution between April 2012 and January 2023 with a discharge diagnosis of acute myocarditis were included. Demographic and clinical data, as well as EKG and imaging results, were retrospectively reviewed. fQRS was defined as the presence of an additional R wave (R’) or notching in the nadir of the S wave or the presence of more than 1 R’ in at least 2 contiguous leads corresponding to the cardiac regions.</div></div><div><h3>Results</h3><div>99 patients (median age 13 years; 76% male). Chest pain was the most common presenting symptom (74.7%). At presentation, 20.2% (95% CI: 12.8–29.5) exhibited fQRS, predominantly in inferior leads. Median follow-up was 403 days, during which 3 patients (15% of those with positive fQRS) had persistent fQRS despite being clinically asymptomatic. On univariate regression, younger age was significantly associated with fQRS at presentation (<em>p</em> = 0.043). No associations were found with biomarkers, echocardiographic parameters, or outcomes.</div></div><div><h3>Conclusion</h3><div>fQRS is an additional EKG finding that may assist in diagnosing acute myocarditis, particularly in younger children. While fQRS may support the diagnosis of myocarditis, it was not associated with disease severity or prognosis. Larger studies are warranted to clarify its role.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"80 ","pages":"Article 101904"},"PeriodicalIF":0.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145976794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of SMAD6 gene variant and descriptive brief insights into genetics of aortic valve disease","authors":"Snehal Mallakmir , Bhushan Chavan , Shilpa Kamble","doi":"10.1016/j.ppedcard.2025.101889","DOIUrl":"10.1016/j.ppedcard.2025.101889","url":null,"abstract":"<div><h3>Aim</h3><div>Indian reports on the genetic etiology of aortopathy are scarce, and insights into this subject are needed.</div></div><div><h3>Method</h3><div>We report a case of a 12-year-old boy with a bicuspid valve and a dilated ascending aorta with a heterozygous pathogenic variation in the <em>SMAD6</em> gene. We conducted a PUBMED and OMIM literature search on genetic evaluation in aortopathy and/or aortic valve disease.</div></div><div><h3>Results</h3><div>Heterozygous indel in the <em>SMAD6</em> gene, resulting in a frameshift-insertion and premature truncation of the protein, was detected in our patient, which has not been reported before. <em>SMAD6</em> has emerged as an important contributor to aortic disease recently, along with other genes, and a brief review is presented here.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"80 ","pages":"Article 101889"},"PeriodicalIF":0.8,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145692021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sadik K.C. , Chetan Kumar Nanjegowda , Anand P. Subramanian
{"title":"Percutaneous ventricular septal defect device closure in a child with Ebstein's anomaly – Hemodynamics and rationale in a rare combination of lesions","authors":"Sadik K.C. , Chetan Kumar Nanjegowda , Anand P. Subramanian","doi":"10.1016/j.ppedcard.2025.101871","DOIUrl":"10.1016/j.ppedcard.2025.101871","url":null,"abstract":"<div><div>Ebstein's anomaly is only rarely associated with a ventricular septal defect. We report device closure of a moderate-sized perimembranous ventricular septal defect in a child with Ebstein's anomaly and discuss the potential hemodynamic advantages of shunt elimination in this scenario.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101871"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145219530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alex Ismael Catalán Cabrera , Edwin Martin Bedoya Rivera , Alfredo Ernesto Homes Linfa , Mónica Medina Durand , Karen del Rosario Condori Alvino , Geovanna Geraldine Gutiérrez Iparraguirre , Nelly Tamayo Huiza
{"title":"Congenital tricuspid valve calcification: A diagnostic and surgical challenge. Case report","authors":"Alex Ismael Catalán Cabrera , Edwin Martin Bedoya Rivera , Alfredo Ernesto Homes Linfa , Mónica Medina Durand , Karen del Rosario Condori Alvino , Geovanna Geraldine Gutiérrez Iparraguirre , Nelly Tamayo Huiza","doi":"10.1016/j.ppedcard.2025.101876","DOIUrl":"10.1016/j.ppedcard.2025.101876","url":null,"abstract":"<div><div>Among congenital anomalies of the tricuspid valve, Ebstein’s anomaly is the most frequent, followed by tricuspid valve dysplasia[1], and, more rarely, congenital tricuspid valve calcification. All these conditions may present with tricuspid regurgitation, which can become severe and lead to early hemodynamic compromise.</div><div>We present the case of a one-month-old infant with persistent cyanosis and hemodynamic instability. Prostaglandin infusion was initiated to maintain ductal patency and ensure pulmonary blood flow, achieving hemodynamic stabilization. Echocardiography revealed severe tricuspid regurgitation with a markedly echogenic anterior leaflet, without apical displacement of the valve leaflets. Cardiac tomography further suggested calcification of the tricuspid valve.</div><div>This case represented a diagnostic and therapeutic challenge. Intraoperative findings confirmed congenital tricuspid valve calcification, which precluded repair. The patient underwent surgical intervention with a favorable postoperative course. Pathological examination corroborated the diagnosis. The patient was discharged and continues follow-up in outpatient pediatric cardiology.</div><div>This case underscores that surgical management should be guided by tricuspid valve morphology and clinical presentation [2], with some patients requiring early intervention.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101876"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145362087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Hybrid valve-in-valve implantation for bioprosthetic antrioventricular valve stenosis in a failing fontan circulation: A case report","authors":"Ahmed Afifi , Ikenna Omeje , Suhair Shebani , Imran Saeed , Marinos Kantzis","doi":"10.1016/j.ppedcard.2025.101883","DOIUrl":"10.1016/j.ppedcard.2025.101883","url":null,"abstract":"<div><div>Children with single-ventricle physiology palliated with the Fontan circulation are prone to late complications, including atrioventricular valve (AVV) dysfunction. We present the case of a 9-year-old boy with left atrial isomerism, unbalanced atrioventricular septal defect, and an extracardiac Fontan who developed severe stenosis of a previously implanted bioprosthetic Magna Mitral Ease atrioventricular (AV) valve leading to Fontan circulation failure. On presentation with AVV stenosis, both surgical and catheter-based interventions were deemed high risk due to multiple previous sternotomies and complex anatomy. Cardiac transplant was not considered, as he did not meet the criteria for listing. A hybrid surgical-transcatheter approach was undertaken, allowing safe deployment of a Myval Octacor valve under direct vision. The procedure was successful, improving hemodynamic status with gradient reduced to 2.5 mmHg with no regurgitation. This case illustrates the feasibility of a novel hybrid atrioventricular valve-in-valve implantation in a Fontan patient, offering an alternative when conventional surgical or transcatheter approaches are unsuitable.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101883"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145415564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Global trends in obesity and cardiovascular disease research among children and adolescents: A bibliometric analysis (1984–2024)","authors":"Deepanshi Saxena , Sudip Bhattacharya , Akanksha Singh , Alok Singh","doi":"10.1016/j.ppedcard.2025.101867","DOIUrl":"10.1016/j.ppedcard.2025.101867","url":null,"abstract":"<div><h3>Background</h3><div>Childhood and adolescent obesity have emerged as significant public health concerns, contributing to an increased risk of cardiovascular disease (CVD) later in life. Over the past four decades, extensive research has examined the relationship between obesity and CVD among young populations, highlighting the need for early intervention strategies. Bibliometric analysis offers a comprehensive overview of research trends, influential studies, and key thematic areas within this field.</div></div><div><h3>Aim of review</h3><div>This study aims to map the scientific landscape of global research on obesity and cardiovascular disease among children and adolescents from 1984 to 2024. By employing bibliometric methods, we identify prolific authors, leading institutions, impactful publications, and emerging research themes, providing insights into the evolution of this critical area of study.</div></div><div><h3>Key scientific concepts of review</h3><div>The bibliometric analysis was conducted using the Scopus database, focusing on publication trends, citation networks, and keyword co-occurrence patterns. The study identifies research hotspots, including metabolic risk factors, lifestyle interventions, genetic predisposition, and public health policies targeting obesity-related CVD in young populations. Additionally, the review highlights collaborative networks among global researchers and suggests future directions for advancing knowledge and policy-making in this domain.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101867"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144988374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Małgorzata Kowalczyk , Maria Kordybach-Prokopiuk , Piotr Hoffman , Mirosław Kowalski
{"title":"Systolic function of a single left ventricle and exercise capacity in adults after Fontan palliation: A preliminary study using exercise echocardiography","authors":"Małgorzata Kowalczyk , Maria Kordybach-Prokopiuk , Piotr Hoffman , Mirosław Kowalski","doi":"10.1016/j.ppedcard.2025.101872","DOIUrl":"10.1016/j.ppedcard.2025.101872","url":null,"abstract":"<div><h3>Background</h3><div>Patients with functionally single ventricle after Fontan palliation are an increasingly large group in adult congenital heart disease departments. This cohort is also among the most complex to manage. However, many patients face a range of complications. Due to several reasons, patients following Fontan palliation experience significant reductions in exercise capacity. Cardiopulmonary exercise testing (CPET) provides means to assess exercise tolerance with objective parameters.</div></div><div><h3>Objectives</h3><div>The aim of the study is comparison of Stress Echocardiography (SE) parameters and CPET data in order to discover the potential relationship between them.</div></div><div><h3>Methods</h3><div>We enrolled 30 patients with a functionally single ventricle of left ventricle morphology who had undergone Fontan palliation and were hospitalized at the Department of Congenital Heart Defects between 2019 and 2021. The study was conducted using a bicycle ergometer in a semi-recumbent position. The parameters measured at rest and at peak exercise included the ejection fraction (EF) of the single ventricle and global longitudinal strain (GLS). The results of the current CPET were also incorporated. Demographic data, previous interventions, pharmacological treatment, and comorbidities were extracted from the patients' medical records.</div></div><div><h3>Results</h3><div>GLS measured at rest and EF measured at peak exercise correlated with Predicted Peak Oxygen Consumption (pVO2%pred).</div></div><div><h3>Conclusion</h3><div>GLS of the single left ventricle shows a meaningful association with pVO₂%pred and EF measured at peak exercise during stress echocardiography also correlates with pVO₂%pred, suggesting that both parameters provide complementary insights into the functional capacity and contractile reserve of the single ventricle in Fontan patients.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101872"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145266618","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Apnea mediated transient complete atrioventricular block in a pre-term neonate","authors":"Sakthi Saravanan , Navaneetha Sasikumar , Raman Krishna Kumar","doi":"10.1016/j.ppedcard.2025.101866","DOIUrl":"10.1016/j.ppedcard.2025.101866","url":null,"abstract":"<div><div>We present a previously unrecognized, reversible cause for complete atrioventricular block in the sick neonate. An 18 day old 33 weeker with Tetralogy of Fallot was admitted with sinus rhythm on ECG. Soon after, the baby developed episodes of apnea during which ECG showed complete atrioventricular block. This was unresponsive to atropine and isoprenaline, and heart rate continued to deteriorate. Mechanical ventilation was initiated in view of the critical condition and in preparation for an emergency temporary pacing wire insertion. Remarkably, ventilation restored sinus rhythm. The probable mechanisms by which apnea and acute hypoxia can cause atrioventricular block include carotid chemoreceptor mediated reflex vagal stimulation, depression of atrioventricular nodal conduction, and increase in endogenous adenosine levels. It is prudent to consider this reversible mechanism in the differential diagnosis of new onset atrioventricular block in the sick neonate, as early recognition may help avoid unnecessary invasive interventions.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"79 ","pages":"Article 101866"},"PeriodicalIF":0.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144893022","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}