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Patterns and outcomes of 975 patients with congenital heart disease from the Sudan Congenital Heart Disease Registry- (Sudan CHEER)
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-02-11 DOI: 10.1016/j.ppedcard.2025.101814
Selma M.A. Ahmed , Eintsar Ali , Sara F.E. Mohammed , Samah A.M. Taha , Wafa M.A. Yousif , Farida A.A. Nimir , Hassan O.A. Mohammed , Abdelmoneim Adam , Mohmmed A. Mohmmed Ahmed , Sulafa K.M. Ali
{"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}
引用次数: 0
Endothelial dysfunction in children with obesity-induced metabolic dysfunction-associated liver disease: Relationship to plasminogen activator inhibitor-1
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-02-04 DOI: 10.1016/j.ppedcard.2024.101797
Hekma Saad Farghaly , Kotb Abbass Metwalley , Edrees Zaki , Shimaa Khalaf , Omima Abdel-Rahman , Ghada Mohamed Saied , Magdy Algowhary , Shimaa Kamal Mohamed
{"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}
引用次数: 0
Heart murmurs in pediatric practice
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-02-01 DOI: 10.1016/j.ppedcard.2025.101813
Tyler Tolleson , Juan Hernandez , Kamryn Caroll , Anna McVay , Jennifer Cole , Myria Mack-Williams , Myrtle Delgado , Nicole Weidow , Jane Messemer
{"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 S<sub>1</sub> occurs from mitral and tricuspid valve closure. The normal second heart sound S<sub>2</sub> 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}
引用次数: 0
Everolimus for Cardiac Rhabdomyomas in Neonate with Tuberous Sclerosis Complex and Significant Arrhythmias
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-28 DOI: 10.1016/j.ppedcard.2025.101812
Marisa Pereira , Tiago Magalhães , Ana Vilan , Joana Pimenta , João Antunes Sarmento
{"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}
引用次数: 0
Pediatric palliative care in patients with cardiovascular disease: The basics of why, when, where, who and how
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-15 DOI: 10.1016/j.ppedcard.2025.101810
Brandy Merritt , Kara Nix , Laney Smith , Hope Lund , Meagan Jones , Rene Sprague , Lynn A. Batten
{"title":"Pediatric palliative care in patients with cardiovascular disease: The basics of why, when, where, who and how","authors":"Brandy Merritt ,&nbsp;Kara Nix ,&nbsp;Laney Smith ,&nbsp;Hope Lund ,&nbsp;Meagan Jones ,&nbsp;Rene Sprague ,&nbsp;Lynn A. Batten","doi":"10.1016/j.ppedcard.2025.101810","DOIUrl":"10.1016/j.ppedcard.2025.101810","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric Palliative Care (PPC) has been shown to offer a wide berth of supportive services for families as they navigate life with pediatric heart disease. PPC assists families in medical decision making, symptom management, emotional and spiritual resources, clarifying goals, educating families on medical and community resources, and advanced care planning.</div></div><div><h3>Aim of review</h3><div>The aim of this review is to examine the data that shows early PPC consultation at diagnosis, often even prior to birth, gives the best opportunity to access the multitude of services. Building early relationships during times of wellness allows the PPC team to be a strong and crucial support for families when critical illness or surgical needs arise. Many barriers prevent pediatric cardiologists and pediatric cardiovascular surgeons from incorporating these benefits for their patients. There is a national lack of access to PPC programs. Therefore, it is important to educate providers on the services PPC can provide so children with pediatric heart conditions will be able to live longer and better lives.</div></div><div><h3>Key scientific concepts of review</h3><div>We reviewed the current literature to summarize benefits of early introduction of PPC in complex cardiac conditions.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101810"},"PeriodicalIF":0.6,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176836","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}
引用次数: 0
Viral myocarditis and cardiogenic shock in surgically palliated hypoplastic left heart syndrome
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-09 DOI: 10.1016/j.ppedcard.2025.101809
Samantha I. Druckman , Brandon Dudeck , Priya Nigam , Adrian J. Holloway
{"title":"Viral myocarditis and cardiogenic shock in surgically palliated hypoplastic left heart syndrome","authors":"Samantha I. Druckman ,&nbsp;Brandon Dudeck ,&nbsp;Priya Nigam ,&nbsp;Adrian J. Holloway","doi":"10.1016/j.ppedcard.2025.101809","DOIUrl":"10.1016/j.ppedcard.2025.101809","url":null,"abstract":"<div><div>In children with physiologically normal hearts, myocarditis is difficult to diagnose and manage due to variable clinical presentation and lack of consensus regarding effective supportive care measures. In those with palliated single ventricle physiology, baseline cardiac function is often altered, and thus, most children receive supportive therapy for congestive heart failure. We theorize that these children are at increased risk of clinically significant impairment in cardiac function in the setting of viral myocarditis.</div><div>We present three cases of children diagnosed with hypoplastic left heart syndrome (HLHS) who underwent multi-step surgical palliation. All three children subsequently developed fulminant viral myocarditis later in life, manifesting as symptomatic heart failure and cardiogenic shock. As a result, one child ultimately successfully completed orthotopic heart transplantation, one developed multiple system organ failure, and their care was withdrawn, and one recovered.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101809"},"PeriodicalIF":0.6,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176835","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}
引用次数: 0
Electrolyte imbalance in pediatric patients following cardiac surgery with CPB: Experience from a single institution in Afghanistan
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-06 DOI: 10.1016/j.ppedcard.2024.101807
Atefa Ahmadi , Brishna Dawlaty , Ahmed Maseh Haidary
{"title":"Electrolyte imbalance in pediatric patients following cardiac surgery with CPB: Experience from a single institution in Afghanistan","authors":"Atefa Ahmadi ,&nbsp;Brishna Dawlaty ,&nbsp;Ahmed Maseh Haidary","doi":"10.1016/j.ppedcard.2024.101807","DOIUrl":"10.1016/j.ppedcard.2024.101807","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Open cardiac surgery with cardiopulmonary bypass (CPB) contributes to postoperative electrolyte imbalances, which increases the risk of complications like arrhythmias. These imbalances stem from fluid shifts, hemodilution, acid-base disturbances, and factors such as cardioplegic solutions, hypothermia, elevated catecholamine levels, and antidiuretic hormone release, as well as non-pulsatile blood flow. Early monitoring and targeted prevention are crucial to managing these risks and improving postoperative outcomes.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Objective&lt;/h3&gt;&lt;div&gt;This study aimed to investigate the prevalence of electrolyte imbalances in pediatric patients undergoing cardiac surgery while considering potential influencing factors such as age, gender, body mass index (BMI), type of cardiac surgery, and cardiopulmonary bypass (CPB) duration.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Materials and methods&lt;/h3&gt;&lt;div&gt;A retrospective cross-sectional study was conducted at the French Medical Institute for Mothers and Children in Kabul, Afghanistan, from January 1, 2021, to September 30, 2023. A total of 393 pediatric patients aged 0–18 years undergoing open cardiac surgery were included, excluding those with closed cardiac surgery, incomplete data, chronic renal disease, or gastrointestinal disorders. Data collected included patient demographics, surgery type (cyanotic vs. acyanotic), primary diagnoses (e.g., ASD, VSD, TOF), RBC transfusion amounts, CPB duration, and electrolyte levels measured before surgery, immediately after surgery, and on the first and second postoperative days. Data were analyzed using SPSS version 22, employing descriptive statistics and various statistical tests (Kolmogorov-Smirnov, Wilcoxon signed rank, Kruskal-Wallis, binomial regression, Spearman correlation), with significance set at p &lt; 0.05.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;Participants ranged from 0 to 18 years, with a mean age of 5.8 years (SD = 4.10), and 55.5 % (n = 218) were male. Most patients (93.4 %, n = 367) were classified as underweight, with a mean body mass index (BMI) of 15.8 kg/m&lt;sup&gt;2&lt;/sup&gt; (SD = 2.31). VSD closure was performed in 41.3 % (n = 162) of cases, and 76 % (n = 299) had a bypass duration exceeding 60 min. Electrolyte imbalances were common among the patients: hyponatremia occurred in 18.6 % (n = 72) preoperatively, rising to 60.3 % (n = 199) by the second postoperative day. Hypokalemia increased from 6.9 % (n = 27) preoperatively to 64 % (n = 251) post-surgery, while hypocalcemia was noted in 31 % (n = 122) on the first postoperative day. Sodium levels rose significantly after packed cell transfusions (p &lt; 0.001), and magnesium levels increased in toddlers and adolescents (p &lt; 0.001). Notably, postoperative potassium levels were 0.6 times lower in females compared to males (p = 0.026), and patients undergoing surgery for over 2 h had 2.6 times higher odds of sodium imbalances (p &lt; 0.001).&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Conclusion&lt;/h3&gt;&lt;div&gt;Pediat","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101807"},"PeriodicalIF":0.6,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176837","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}
引用次数: 0
Surgical timing and mortality in nonsevere paediatric congenital heart disease: A EUROlinkCAT cohort analysis across Europe
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-02 DOI: 10.1016/j.ppedcard.2024.101808
Mads Damkjær , Ester Garne , Maria Loane , Stine K. Urhoj , Elisa Ballardini , Clara Cavero-Carbonell , Alessio Coi , Laura García-Villodre , Joanne Given , Mika Gissler , Anna Heino , Sue Jordan , Elizabeth Limb , Amanda J. Neville , Anna Pierini , Anke Rissmann , Joachim Tan , Ieuan Scanlon , Joan K. Morris
{"title":"Surgical timing and mortality in nonsevere paediatric congenital heart disease: A EUROlinkCAT cohort analysis across Europe","authors":"Mads Damkjær ,&nbsp;Ester Garne ,&nbsp;Maria Loane ,&nbsp;Stine K. Urhoj ,&nbsp;Elisa Ballardini ,&nbsp;Clara Cavero-Carbonell ,&nbsp;Alessio Coi ,&nbsp;Laura García-Villodre ,&nbsp;Joanne Given ,&nbsp;Mika Gissler ,&nbsp;Anna Heino ,&nbsp;Sue Jordan ,&nbsp;Elizabeth Limb ,&nbsp;Amanda J. Neville ,&nbsp;Anna Pierini ,&nbsp;Anke Rissmann ,&nbsp;Joachim Tan ,&nbsp;Ieuan Scanlon ,&nbsp;Joan K. Morris","doi":"10.1016/j.ppedcard.2024.101808","DOIUrl":"10.1016/j.ppedcard.2024.101808","url":null,"abstract":"<div><h3>Background</h3><div>Management and outcomes amongst children with congenital heart defects (CHD) are relatively heterogeneous. Traditionally, the highest-risk conditions and procedures have received the most attention, with less focus on outcome for less severe CHDs.</div></div><div><h3>Objectives</h3><div>Evaluate the management of children born with certain less severe CHDs across Europe.</div></div><div><h3>Methods</h3><div>This population-based linkage cohort study included children diagnosed with ventricular septal defect (VSD), atrial septal defect (ASD), pulmonary valve stenosis (PS), and patent arterial duct (PDA) from nine European regions. Data on surgery/intervention and survival from 1995 to the age of 10 years or until the end of 2014 were collected through electronic linkage to mortality and hospital databases. Kaplan-Meier survival estimates and meta-analyses were used to analyze the proportion of children undergoing surgery/intervention and survival rates.</div></div><div><h3>Results</h3><div>The study included 20,608 children with the less severe CHDs. Surgical correction rates for the anomaly within the first year of life varied significantly, particularly for VSD. The median age for initial surgery/intervention was consistent for VSD but varied for other conditions. Most conditions were corrected in a single procedure, except for PS, which required a median of 1.7 (95 % CI; 1.4–2.0) interventions. Postoperative mortality was highest for neonates undergoing VSD surgery/intervention, 8.7 (95 % CI; 4.0–15.8) deaths per 100 surgeries. The overall 5-year survival rate for all conditions was approximately 98 %.</div></div><div><h3>Conclusion</h3><div>There was considerable variation in the management of less severe CHDs across Europe, particularly in the timing of surgical interventions. Despite these differences, the overall 5-year survival rate is comparable and high.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101808"},"PeriodicalIF":0.6,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143176838","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}
引用次数: 0
Transition to a non-invasive rejection surveillance protocol with donor-derived cell-free DNA in pediatric heart transplant recipients
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2025-01-02 DOI: 10.1016/j.ppedcard.2024.101806
Stephen A. Bravo , Leor H. Akabas , Oliver M. Barry , Wendy C. Hsiao , Marc E. Richmond , Irene D. Lytrivi
{"title":"Transition to a non-invasive rejection surveillance protocol with donor-derived cell-free DNA in pediatric heart transplant recipients","authors":"Stephen A. Bravo ,&nbsp;Leor H. Akabas ,&nbsp;Oliver M. Barry ,&nbsp;Wendy C. Hsiao ,&nbsp;Marc E. Richmond ,&nbsp;Irene D. Lytrivi","doi":"10.1016/j.ppedcard.2024.101806","DOIUrl":"10.1016/j.ppedcard.2024.101806","url":null,"abstract":"<div><h3>Background</h3><div>While endomyocardial biopsy (EMB) remains the gold standard for detection of acute allograft rejection (AR), its limitations have led many pediatric programs to utilize adjunctive non-invasive methods of rejection surveillance.</div></div><div><h3>Objectives</h3><div>This study compares the procedural burden, detection of AR, and severity of AR episodes between an EMB-based protocol and a surveillance protocol utilizing donor-derived cell free DNA (dd-cfDNA).</div></div><div><h3>Methods</h3><div>Patients who underwent orthotopic heart transplantation (OHT) before age 18 followed at our center from 2019 to 2023 were retrospectively reviewed. Two protocols and time periods were compared: Era 1 (01/2019–03/2020, EMB-based) and Era 2 (07/2022–06/2023, dd-cfDNA based). Patients with multi-organ transplants, post-transplant lymphoproliferative disorder, and bone marrow transplants were excluded.</div></div><div><h3>Results</h3><div>Biopsies, anesthesia, and intubations had a statistically significant decrease in Era 2, with a 46 % reduction in biopsies per patient-year in Era 2. When screening with dd-cfDNA in Era 2, there was a significant increase in the number of positive EMB (32/312, 10.1 %), compared to 19/719 (2.6 %) positive EMB found in Era 1 (<em>p</em> &lt; 0.001). Cases of rejection were significantly more likely to be associated with a positive EMB (72.9 % in Era 2 vs. 45.9 % in Era 1) and less likely to present as clinical rejection (<em>p</em> = 0.017).</div></div><div><h3>Conclusion</h3><div>dd-cfDNA surveillance is associated with a significant decrease in surveillance EMB and related procedures without a change in clinical allograft rejection. Our limited experience suggests that integrating dd-cfDNA may also lead to earlier detection and lesser severity of rejection in pediatric populations.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101806"},"PeriodicalIF":0.6,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143175024","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}
引用次数: 0
Cardiovascular causes of pediatric chest pain: Case-based review
IF 0.6
PROGRESS IN PEDIATRIC CARDIOLOGY Pub Date : 2024-12-26 DOI: 10.1016/j.ppedcard.2024.101805
Bijay Shrestha , Avery Thomas , Hency Patel , Eru Sujakhu , Elena Rueda de Leon , Emily Ribeiro , Gul H. Dadlani
{"title":"Cardiovascular causes of pediatric chest pain: Case-based review","authors":"Bijay Shrestha ,&nbsp;Avery Thomas ,&nbsp;Hency Patel ,&nbsp;Eru Sujakhu ,&nbsp;Elena Rueda de Leon ,&nbsp;Emily Ribeiro ,&nbsp;Gul H. Dadlani","doi":"10.1016/j.ppedcard.2024.101805","DOIUrl":"10.1016/j.ppedcard.2024.101805","url":null,"abstract":"<div><h3>Background</h3><div>Chest pain is a common presenting symptom in pediatric emergency rooms and clinics. The wide spectrum of causes may create a diagnostic conundrum for clinicians.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review cardiovascular causes of pediatric chest pain with illustrative case histories and discussion of pathophysiology, diagnosis, and treatment.</div></div><div><h3>Key scientific concepts of review</h3><div>Guidelines for cardiovascular screening include the American College of Cardiology and American Heart Association 14-element checklist, American Academy of Pediatrics recommendations with four questions about cardiac symptoms, signs, and family history, and international guidelines for electrocardiogram interpretation. Noncardiac chest pain may be caused by various conditions, such as exercise-induced asthma. Pericarditis may be acute or chronic and caused by various conditions, including viral infection. Sudden cardiac arrest may occur without previous chest pain and most commonly is caused by hypertrophic cardiomyopathy. Anomalous coronary artery is a rare congenital defect that may cause exertional chest pain or sudden cardiac arrest and may require multidisciplinary care for diagnosis and treatment. Myocarditis may be caused by viral infection, may present with tachycardia, chest pain, difficulty breathing, and cardiovascular instability, and may be diagnosed with endomyocardial biopsy or cardiac magnetic resonance imaging. Spontaneous pneumothorax may present with sharp chest pain in tall, thin adolescent boys, and treatment may include close observation with serial chest radiographs, oxygen therapy, simple aspiration, or chest tube placement. Aortic dissection is a rare, life-threatening condition associated with connective tissue disorders and may necessitate percutaneous endovascular grafting or aortic replacement surgery. Pulmonary embolism may occur in pediatric patients, and risk factors include sepsis, current oral contraceptive use, and recent surgery. Prompt diagnosis and treatment of conditions causing chest pain may be facilitated with a comprehensive history, detailed physical examination, and ancillary studies.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101805"},"PeriodicalIF":0.6,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143175458","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}
引用次数: 0
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