PROGRESS IN PEDIATRIC CARDIOLOGY最新文献

{"title":"Energy drink exposures and trends in children and young adults reported to the National Poison Data System","authors":"Steven E. Lipshultz ,&nbsp;Stacy D. Fisher ,&nbsp;Vivian I. Franco ,&nbsp;Brandon J. Warrick ,&nbsp;Sebastian M. Seifert ,&nbsp;Alvin C. Bronstein","doi":"10.1016/j.ppedcard.2025.101819","DOIUrl":"10.1016/j.ppedcard.2025.101819","url":null,"abstract":"<div><h3>Background</h3><div>Energy drinks (EDs) may contain caffeine, amino acids, vitamins, and other ingredients that have been associated with serious adverse effects, primarily in children and young adults.</div></div><div><h3>Objectives</h3><div>We sought to understand ED exposures, including demographic trends, clinical effects, and outcomes in the US Poison Centers' National Poison Data System (NPDS), and compared similar reports of caffeinated beverages.</div></div><div><h3>Methods</h3><div>We analyzed all NPDS closed human exposures to single-use EDs reported to NPDS between October 1, 2010, and September 30, 2013.</div></div><div><h3>Results</h3><div>NPDS recorded 10,588 cases of ED exposure. Active ingredients were identified in 5139 (49 %) cases. Of the 4803 (93 %) exposures to alcohol-free EDs, 51 % were in children 5 years old or younger, 10 % were in children 6 to 12 years old, 16 % were in adolescents 13 to 19 years old, and 23 % were adults at least 20 years old. Unintentional exposures were highest in children 5 years old or younger (75 %). Intentional exposures were highest in adolescents (45 %). Moderate or major adverse outcomes from EDs containing multiple caffeine ingredients were more common than single ingredient caffeine products (23 % vs. 15 %; <em>P</em> &lt; 0.001). Exposures associated with ethanol EDs had worse outcomes than those without (42 % vs. 19 %, respectively; <em>P</em> &lt; 0.001). The most common clinical effects associated with ED exposures were neurologic (<em>N</em> = 1042, 22 %), gastrointestinal (<em>N</em> = 792, 17 %), and cardiovascular (<em>N</em> = 567, 12 %); 14 cases were life-threatening, and 1 adolescent girl with vascular Ehlers Danlos syndrome died. Recent follow up from January 1, 2020, to December 31, 2021, shows consistent trends in use and a clear difference between EDs and caffeinated beverage case numbers and medical outcomes. The number of cases was similar in each year except for a notable increase mid-2020, believed to be related to the pandemic, with our query returning 4367 reported ED cases between 2020 and 2021.</div></div><div><h3>Conclusion</h3><div>A substantial proportion of ED calls to poison centers involve children, some of whom experience severe neurologic and cardiac toxicity, among other symptoms. ED exposure calls are more common and have more medical severity than exposures to caffeine or coffee beans alone. The number and severity of adverse ED events warrant efforts to educate the public about the risks, especially in children.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101819"},"PeriodicalIF":0.6,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144481039","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":"Redefining success in pediatric clinical trials","authors":"Humera Ahmed,&nbsp;Joseph W. Rossano","doi":"10.1016/j.ppedcard.2025.101820","DOIUrl":"10.1016/j.ppedcard.2025.101820","url":null,"abstract":"<div><h3>Background</h3><div>Historically, there have been limited clinical trials in pediatric patients with cardiomyopathy or heart failure. Although the number of these studies has increased in recent years, many have failed to meet their primary endpoints. However, these “negative” trials have been invaluable to the field of pediatric cardiology.</div></div><div><h3>Aim of review</h3><div>This review aims to highlight the importance of negative clinical trials in pediatric cardiology. It focuses on how these trials, despite not meeting their primary objectives, have provided critical insights into safety, drug metabolism, biomarkers, and have contributed to refining trial design for future studies.</div></div><div><h3>Key scientific concepts of review</h3><div>Negative trials have played a key role in advancing pediatric heart failure treatment by revealing essential data on drug metabolism, particularly across different age groups, and by identifying novel biomarkers for monitoring treatment efficacy. These trials have also led to improvements in trial design, ensuring better patient selection and more accurate evaluation of therapeutic interventions. Despite not meeting their primary endpoints, these studies have provided a foundation for future innovations and have helped shape treatment strategies in pediatric cardiology.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101820"},"PeriodicalIF":0.6,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143704159","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":"Multi center experience with Cocoon Duct Occluder for closure of different types of patent ductus arteriosus (PDA)","authors":"Muhammad Ayyaz,&nbsp;Abdul Sattar Shaikh,&nbsp;Salahudin Kakar,&nbsp;Hussain Bux Korejo,&nbsp;Ram Chand,&nbsp;Aliya Kemal Ahsan,&nbsp;Rumana Sangi,&nbsp;Sanam Khan,&nbsp;Veena Kumari","doi":"10.1016/j.ppedcard.2025.101818","DOIUrl":"10.1016/j.ppedcard.2025.101818","url":null,"abstract":"<div><h3>Background</h3><div>Transcatheter closure of patent ductus arteriosus (PDA) is a widely practiced procedure using various duct occluder devices.</div></div><div><h3>Objective</h3><div>To assess the safety and efficacy of the Cocoon Duct Occluder for PDA closure.</div></div><div><h3>Methods</h3><div>This single-arm cohort study, conducted in the Pediatric Cardiology Departments of NICVD Karachi, TMK, and Sukkur, included both prospective and retrospective recruitment. Using non-probability consecutive sampling, a minimum sample size of 35 was calculated to achieve a 90 % success rate with a 95 % confidence level. A total of 195 patients met the inclusion criteria over 2.5 years. Comprehensive preprocedural assessments were performed, including medical history, clinical examination, electrocardiography, chest X-ray, full blood count, and detailed echocardiographic measurements of PDA dimensions and left ventricular function. Procedures were performed by experienced pediatric cardiologists.</div></div><div><h3>Results</h3><div>PDA closure was successfully achieved in 186 of 195 patients (95.3 %). The most common PDA type was Type A (86.6 %), followed by Type B (4.6 %), Type C (2.6 %), Type D (3 %), and Type E (3 %) per Krichenko's classification. Minor complications included peripheral vascular injury (6.6 %), arterial thrombosis (4 patients), venous thrombosis (7 patients), and arrhythmias (5 patients: 4 with supra-ventricular tachycardia [SVT] and 1 with ventricular tachycardia [VT], of which 2 SVT cases required adenosine therapy). One major complication, i.e. infective endocarditis, and no other major complications, such as device embolization or vascular injury, were observed.</div></div><div><h3>Conclusion</h3><div>The Cocoon Duct Occluder is a safe and effective device for PDA closure across all anatomical types. The procedure's success is enhanced through meticulous device sizing, proper technique, and operator experience, minimizing the risk of major complications.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101818"},"PeriodicalIF":0.6,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143643271","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":"Corrigendum to “Prognostic biomarkers and clinical parameters in adults with atrial septal defect-related pulmonary arterial hypertension treated with sildenafil and beraprost combination therapy” [Prog. Pediadtr. Cardiol. 2024 vol 73 article number PPC_PPC-D-23-00087]","authors":"Anudya Kartika Ratri ,&nbsp;I. Gde Rurus Suryawan ,&nbsp;Meity Ardiana ,&nbsp;Andrianto ,&nbsp;Stavros G. Drakos","doi":"10.1016/j.ppedcard.2025.101817","DOIUrl":"10.1016/j.ppedcard.2025.101817","url":null,"abstract":"","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101817"},"PeriodicalIF":0.6,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143637545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The diagnostic yield of cardiac screening in first-degree relatives of sudden arrhythmic death syndrome or unexplained cardiac arrest probands: A systematic review of the literature","authors":"Jennifer Tollit ,&nbsp;I-Ting Tu ,&nbsp;Gabrielle Norrish ,&nbsp;Ella Field ,&nbsp;Jo Wray ,&nbsp;Juan Pablo Kaski","doi":"10.1016/j.ppedcard.2025.101816","DOIUrl":"10.1016/j.ppedcard.2025.101816","url":null,"abstract":"<div><h3>Background</h3><div>First-degree relatives of Sudden Arrhythmic Death Syndrome (SADS) or Unexplained Cardiac Arrest (UCA) are recommended to undergo clinical evaluation for potential inherited cardiac conditions (ICC). However, data on the yield of family screening in these populations remains scarce.</div></div><div><h3>Aim of review</h3><div>This systematic review aimed to explore the diagnostic yield of clinical screening of first-degree relatives of SADS or UCA probands. A secondary aim was to compare the diagnostic yield of adult-aged and pediatric-aged relatives.</div></div><div><h3>Key scientific concepts of review</h3><div>Included studies described the clinical cardiac screening and yield of first-degree relatives of SADS and UCA probands. Quality of selected studies was assessed using a modified Joanna Briggs Institute checklist.</div><div>14 studies met inclusion criteria for this review, together including 1646 first-degree relatives of SADS probands and 656 first-degree relatives of UCA probands. Overall diagnostic yield described ranged from 0 to 32 %. The combined mean diagnostic yield of SADS relatives did not differ significantly from that of relatives of UCA probands. Three studies described outcomes of clinical screening in pediatric relatives, with an overall reported yield of 9.4 % ± 3.4 %, not significantly different from adult populations. Whilst there is a clear indication for clinical screening of first-degree relatives following SADS or an UCA, a lack of well-designed large population-based studies means that the evidence base is not robust. The yield in reported literature varies considerably, with no difference between SADS and UCA cohorts and a similar yield in pediatric and adult relatives. This supports screening for all first-degree relatives regardless of age.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101816"},"PeriodicalIF":0.6,"publicationDate":"2025-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144231774","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":"Patterns and outcomes of 975 patients with congenital heart disease from the Sudan Congenital Heart Disease Registry- (Sudan CHEER)","authors":"Selma M.A. Ahmed ,&nbsp;Eintsar Ali ,&nbsp;Sara F.E. Mohammed ,&nbsp;Samah A.M. Taha ,&nbsp;Wafa M.A. Yousif ,&nbsp;Farida A.A. Nimir ,&nbsp;Hassan O.A. Mohammed ,&nbsp;Abdelmoneim Adam ,&nbsp;Mohmmed A. Mohmmed Ahmed ,&nbsp;Sulafa K.M. Ali","doi":"10.1016/j.ppedcard.2025.101814","DOIUrl":"10.1016/j.ppedcard.2025.101814","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) constitutes an important cause of mortality and morbidity in children. In limited resource settings, data regarding patterns and outcomes of CHD are deficient.</div></div><div><h3>Objectives</h3><div>To describe the clinical and echocardiographic (echo) features as well as outcomes of patients with CHD seen at Sudan Heart Center.</div></div><div><h3>Methods</h3><div>This is a study reporting results of the <u>Sudan C</u>ongenital <u>He</u>art Dis<u>e</u>ase <u>R</u>egistry (<u>Sudan CHEER</u>) from January 2021–September 2022. Clinical, echo, and interventional management data were collected from hospital records as well as through telephone calls.</div></div><div><h3>Results</h3><div>975 patients with CHD were included (males 53 %). The most common age group was 1–12 months (40 %), and only 7 % were neonates. Acyanotic CHD constituted 71 % of patients. Eisenmenger's syndrome was present in 37 patients (3.8 %). Surgery was indicated in 472 patients (48 %) and performed in 167 (35 % of those in need). The Risk Adjustment Scale for CHD surgery was 2 for 70 % of cases. Interventional cardiac catheterization was indicated in 226 patients and done in 70 % of these. Only 56 % of patients were accessible for follow up period, mean of 12 months. Of unoperated patients, 25 died (6 %), mostly (43 %) having left to right shunts. The surgical operative mortality was 12 %; 80 % of those who underwent surgery did not have residual lesions. Interventional catheterization mortality was 0.6 %, and all survivors were well on follow up. Of those who had palliative interventional procedures, 70 % are still awaiting corrective surgery.</div></div><div><h3>Conclusion</h3><div>There are huge gaps in early diagnosis and access to interventions that need to be addressed to improve outcomes of patients with CHD in Sudan and similarly low-income countries.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101814"},"PeriodicalIF":0.6,"publicationDate":"2025-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143465012","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":"Pediatric myocarditis: Current concepts review","authors":"Caroline M. Kinchler ,&nbsp;Katharine L. Lightfoot ,&nbsp;Nathaniel S. Olliff ,&nbsp;Erin K. Powell ,&nbsp;Emily R. Ribeiro ,&nbsp;David Saulino ,&nbsp;Brian Stewart ,&nbsp;Supatida Tengsupakul ,&nbsp;Omar Sanchez Villanueva ,&nbsp;Nita Davis","doi":"10.1016/j.ppedcard.2025.101815","DOIUrl":"10.1016/j.ppedcard.2025.101815","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric myocarditis is an inflammatory condition of the heart muscle typically caused by viral infections, drugs, toxins, bacteria, or autoimmune disease.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review pediatric myocarditis, including epidemiology, pathophysiology, clinical features, diagnosis, treatment, and prognosis.</div></div><div><h3>Key scientific concepts of review</h3><div>The incidence of myocarditis in children is 0.2 to 2 cases per 100,000 children aged &lt;18 years. Myocarditis is most often caused by a virus such as coxsackievirus, adenovirus, parvovirus B19, human herpesvirus 6, or SARS-CoV-2 entering cardiomyocytes via virus-specific receptors. Pediatric myocarditis may be acute or chronic. A key feature of acute myocarditis is a viral prodrome that may occur one to four weeks before the onset of symptoms associated with cardiac pathology. The criterion standard for diagnosis is histology from an endomyocardial biopsy. Cardiac magnetic resonance imaging changes suggestive of myocarditis may be considered confirmatory, but access to this study is not readily available. Echocardiography is typically the first imaging study used in the diagnosis of pediatric myocarditis, given its ubiquity. Chest radiographic findings in pediatric myocarditis may include cardiomegaly, pulmonary venous congestion, and pleural effusion. Electrocardiography in patients who have pediatric myocarditis may show nonspecific ST-segment changes, T wave inversion, atrioventricular block, ST-segment elevation, and low-voltage QRS complexes in aVR, aVL, and aVF leads. Treatment includes anti-inflammatory medications and hemodynamic support. Patients who are hemodynamically unstable may require heart failure treatment in an intensive care unit. In rapidly deteriorating patients, early mechanical circulatory support may be important, including extracorporeal membrane oxygenation, a ventricular assist device, an implantable cardioverter-defibrillator, or heart transplant. Noval therapies, including use of mesenchymal stem cells, are also under investigation. Myocarditis may resolve, and ventricular function may improve with supportive therapy, but some patients may develop complications associated with major morbidity and mortality. Regular cardiology follow-up may be advised after diagnosis to monitor changes to the myocardium.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101815"},"PeriodicalIF":0.6,"publicationDate":"2025-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143621309","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":"Endothelial dysfunction in children with obesity-induced metabolic dysfunction-associated liver disease: Relationship to plasminogen activator inhibitor-1","authors":"Hekma Saad Farghaly ,&nbsp;Kotb Abbass Metwalley ,&nbsp;Edrees Zaki ,&nbsp;Shimaa Khalaf ,&nbsp;Omima Abdel-Rahman ,&nbsp;Ghada Mohamed Saied ,&nbsp;Magdy Algowhary ,&nbsp;Shimaa Kamal Mohamed","doi":"10.1016/j.ppedcard.2024.101797","DOIUrl":"10.1016/j.ppedcard.2024.101797","url":null,"abstract":"<div><h3>Background</h3><div>Metabolic dysfunction-associated fatty liver disease (MAFLD), formerly known as non-alcoholic fatty liver disease (NAFLD), and cardiovascular abnormalities are closely related in adulthood, but few studies were performed in childhood to prove this relationship, and its pathophysiologic mechanisms have not fully studied yet.</div></div><div><h3>Objectives</h3><div>This study aims to assess vascular endothelial dysfunction in children with obesity- related MAFLD and to investigate its relationship to the plasminogen activator inhibitor-1 (PAI-1) levels.</div></div><div><h3>Methods</h3><div>In this case-control study, we assessed both brachial flow-mediated dilation (FMD) and carotid intima-media thickness (CA-IMT), in addition to liver profile, lipid profile, glucose, insulin, insulin resistance, fasting C-peptide, high-sensitivity C-reactive protein (hs-CRP), and PAI-1 in 200 children with obesity, 80 with and 120 without MAFLD, respectively.</div></div><div><h3>Results</h3><div>Compared with non-MAFLD, children with MAFLD had lower brachial FMD% (3.94 ± 1.42 versus 8.16 ± 2.32 <em>p</em> = 0.001), similar CA-IMT (0.45 ± 0.06 versus 0.45 ± 0.07, <em>p</em> = 0.09) and higher PAI-1 (55.16 ± 14.42 versus 43.32 ± 10.34 <em>p</em> = 0.000). Moreover, FMD% was significantly correlated with PAI-1 (<em>r</em> = −0.57, <em>p</em> = 0.001), hs-CRP (<em>r</em> = −0.474, p = 0.001), Homeostatic Model Assessment for Insulin Resistance (HOMA-IR) (<em>r</em> = −0.306, <em>p</em> = 0.01) and CA-IMT (<em>r</em> = −0.223, <em>p</em> &lt;0.05). Using linear regression analysis, PAI-1 was the most significant independent predictor of FMD (p=0.001) in children with obesity-related MAFLD.</div></div><div><h3>Conclusions</h3><div>Children with obesity who have MAFLD showed impairment of FMD %, which was independently associated with elevated PAI-1 levels. Thus, the diagnosis of MAFLD in a child should raise urgent attention to its cardiovascular sequelae. In the management of MAFLD, the prevention of cardiovascular disease is crucial, along with the prevention of end-stage liver disease.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101797"},"PeriodicalIF":0.6,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143508500","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":"Heart murmurs in pediatric practice","authors":"Tyler Tolleson ,&nbsp;Juan Hernandez ,&nbsp;Kamryn Caroll ,&nbsp;Anna McVay ,&nbsp;Jennifer Cole ,&nbsp;Myria Mack-Williams ,&nbsp;Myrtle Delgado ,&nbsp;Nicole Weidow ,&nbsp;Jane Messemer","doi":"10.1016/j.ppedcard.2025.101813","DOIUrl":"10.1016/j.ppedcard.2025.101813","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric heart murmurs are common and may be innocent or associated with cardiac pathology and risk of sudden cardiac arrest.</div></div><div><h3>Aim of review</h3><div>The aim of this article is to review the common pediatric heart murmurs including evaluation, indications for pediatric cardiology referral, and patient and family education.</div></div><div><h3>Key scientific concepts of review</h3><div>The first step in the evaluation of a heart murmur is to take a thorough and systematic history, including history of present illness, symptoms and signs of cardiovascular disease, and prenatal, past medical, family, and social history. The systematic cardiovascular examination includes vital signs, inspection, palpation, and auscultation. The seven characteristics of a heart murmur include timing, shape, location, radiation, intensity, pitch, and quality. The normal first heart sound S₁ occurs from mitral and tricuspid valve closure. The normal second heart sound S₂ occurs from aortic and pulmonary valve closure. Innocent murmurs such as the Still, pulmonary flow, supraclavicular murmurs, and venous hum do not require treatment. Pathologic murmurs are caused by structural heart disease and necessitate referral to a pediatric cardiologist. Systolic ejection murmurs may be caused by aortic stenosis, pulmonary stenosis, hypertrophic obstructive cardiomyopathy, or coarctation of the aorta. Holosystolic murmurs may be caused by ventricular septal defect, mitral regurgitation, mitral valve prolapse, or tricuspid regurgitation. Diastolic murmurs are pathologic and include murmurs caused by aortic regurgitation, pulmonary regurgitation, mitral stenosis, or tricuspid stenosis. The most common pathologic continuous murmur is caused by patent ductus arteriosus. Referral of a patient with a murmur to pediatric cardiology is indicated by age, murmur characteristics, associated symptoms and findings consistent with possible cardiovascular disease, and family and genetic history. Parental anxiety about the upcoming cardiology visit may be decreased through discussion with the clinician.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101813"},"PeriodicalIF":0.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143422132","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Everolimus for Cardiac Rhabdomyomas in Neonate with Tuberous Sclerosis Complex and Significant Arrhythmias","authors":"Marisa Pereira ,&nbsp;Tiago Magalhães ,&nbsp;Ana Vilan ,&nbsp;Joana Pimenta ,&nbsp;João Antunes Sarmento","doi":"10.1016/j.ppedcard.2025.101812","DOIUrl":"10.1016/j.ppedcard.2025.101812","url":null,"abstract":"<div><div>Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in the TSC1 or TSC2 genes, characterized by benign tumors in multiple organs, including the heart. Cardiac rhabdomyomas are the most frequent neonatal primary cardiac tumors in TSC, often resolving spontaneously but sometimes necessitating intervention due to arrhythmias or flow obstruction.</div><div>This case report describes a term male neonate with TSC alternating between baseline bradycardia and supraventricular tachycardia (SVT) episodes. Imaging revealed multiple cardiac rhabdomyomas and cortical tuberomas, confirming the TSC diagnosis with a TSC2 gene mutation. Initial anti-arrhythmic therapy titration was not possible due to baseline bradycardia and failed to control SVT. Everolimus, an mTOR inhibitor, was introduced, leading to complete regression of cardiac rhabdomyomas and stabilization of baseline heart rate, which allowed adjustment of anti-arrhythmic therapy and heart rate control. The patient, now 12 months old, is clinically stable, off anti-arrhythmic drugs, and continues preventive anti-epileptic therapy and Everolimus with no significant adverse effects.</div><div>This case underscores the potential efficacy and safety of Everolimus in treating TSC-associated cardiac rhabdomyomas, advocating for further research to refine therapeutic strategies for TSC's cardiac manifestations.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101812"},"PeriodicalIF":0.6,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143207579","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}