{"title":"Sotalol: A new alternative in medically-resistant pediatric POTS-a case series","authors":"Neel Dayal , William A. Suarez , Blair Grubb","doi":"10.1016/j.ppedcard.2025.101842","DOIUrl":"10.1016/j.ppedcard.2025.101842","url":null,"abstract":"<div><div>Postural orthostatic tachycardia syndrome (POTS) causes an increase in heart rate of >40 beats per minute with positional change. We describe 3 interesting cases of “treatment-resistant” pediatric POTS patients with profound symptoms managed with sotalol. Treatment led to heart rate control and symptom improvement with no signs of toxicity.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101842"},"PeriodicalIF":0.6,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144196039","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}
Michelle Rybka , Arzu Cetin , Aaron DeWitt , Heather Griffis , Maryam Naim , Jennifer Walter
{"title":"Caring for chronically critically ill children in the pediatric cardiac intensive care unit: Correlations of a primary palliative care intervention with clinical outcomes","authors":"Michelle Rybka , Arzu Cetin , Aaron DeWitt , Heather Griffis , Maryam Naim , Jennifer Walter","doi":"10.1016/j.ppedcard.2025.101839","DOIUrl":"10.1016/j.ppedcard.2025.101839","url":null,"abstract":"<div><h3>Background</h3><div>A growing number of pediatric patients admitted to the cardiac intensive care unit (CICU) meet the criteria of chronically critically ill (CCI), but there is little consensus on how to meet their distinct needs. A novel primary palliative care intervention, Chronic Care Rounds (CCR), was used to assess whether CCI patients with higher risk for mortality are being identified correctly.</div></div><div><h3>Objectives</h3><div>Exposure to primary palliative care intervention (CCR) will be associated with clinical characteristics and worse health outcomes of CCI patients in the CICU.</div></div><div><h3>Methods</h3><div>This retrospective cohort study evaluated patients admitted to a pediatric CICU for >14 days between 2014 and 2019. Demographics, clinical data, and whether patients received the primary palliative care intervention of interest, Chronic Care Rounds (CCR), were collected. CCR is a weekly interprofessional meeting to discuss a CCI patient, followed by a family meeting. Associations between clinical characteristics and CCR were analyzed using Chi-square/Fisher's exact and Wilcoxon rank sum tests.</div></div><div><h3>Results</h3><div>A total of 692 hospitalizations were identified, with 253 (37 %) receiving CCR. CCR patients were significantly more likely to have a syndrome diagnosis (42 % vs 32 %, p 0.007), extracardiac anomalies (47 % vs 39 %, p 0.042), chromosomal abnormalities (54 % v 36 %, <em>p</em> < 0.001), or list ‘Other’ as race compared to Caucasian (33 % v 23 %, p 0.001). The CCR group had a higher mortality rate (34 % vs 16 %, <em>p</em> < 0.001). The CCR group had higher rates of DNR in place compared to the non-CCR group (19 % vs 7 %, <em>p</em> < 0.001) as well as higher rates of sub-specialty palliative care involvement (49 % vs 23 %, p < 0.001). The two cohorts had similar rates of withdrawal of life sustaining technology (45 % vs 42 %, p 0.202).</div></div><div><h3>Conclusions</h3><div>This study showed that CCR successfully identified patients at higher risk for mortality and therefore greater need for communication with family.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101839"},"PeriodicalIF":0.6,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144116168","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}
Wendy Wang , Savannah Her , Em Long-Mills , Dmitry Tumin
{"title":"School performance in children with acquired heart disease","authors":"Wendy Wang , Savannah Her , Em Long-Mills , Dmitry Tumin","doi":"10.1016/j.ppedcard.2025.101840","DOIUrl":"10.1016/j.ppedcard.2025.101840","url":null,"abstract":"<div><h3>Background</h3><div>Children with congenital heart diseases (CHD) experience many challenges to school performance; however, no study has examined school performance in children with acquired heart diseases (AHD).</div></div><div><h3>Objective</h3><div>To evaluate school performance among children with AHD and compare their outcomes to peers with CHD and peers without heart disease.</div></div><div><h3>Methods</h3><div>We used the 2020–2022 National Survey of Children's Health database to identify children aged 6–17 years who were enrolled in school. Caregiver-reported heart conditions present at the time of the survey were classified as congenital or acquired. Outcomes included school absenteeism, school engagement, school-reported problems, and special education placement.</div></div><div><h3>Results</h3><div>Based on a sample of 87,730 children meeting inclusion criteria, we estimated 2.1 % had CHD, 0.3 % had AHD, and 97.6 % did not have a heart condition. Children with AHD exhibited comparable rates of school absenteeism, school engagement, and school-reported problems as children with CHD. Compared to children with no heart disease, AHD was associated with higher odds of school absenteeism (OR: 4.55; 95 % CI: 2.49, 8.30; <em>p</em> < 0.001) and school-reported problems (OR: 1.98; 95 % CI: 1.17, 2.22; <em>p</em> = 0.011).</div></div><div><h3>Conclusion</h3><div>Children with AHD face similar educational challenges as those with CHD when compared to peers without current heart conditions. Tailored academic interventions may be needed to better support children with AHD.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101840"},"PeriodicalIF":0.6,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105097","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}
Lauren Hirth , Connor Smith , Daniel McAree , Mary Kay Olive , Jonathon Hagel
{"title":"Untreated congenital heart disease leading to Eisenmenger syndrome: Cultural Implications and barriers to care","authors":"Lauren Hirth , Connor Smith , Daniel McAree , Mary Kay Olive , Jonathon Hagel","doi":"10.1016/j.ppedcard.2025.101837","DOIUrl":"10.1016/j.ppedcard.2025.101837","url":null,"abstract":"<div><div>Pulmonary arterial hypertension in children can occur from many causes, but when secondary to excessive pulmonary blood flow due to congenital heart disease, timely management is essential. Excessive pulmonary blood flow can occur in ventricular septal defects (VSDs) and in patent ductus arteriosus (PDA), among other defects, and if longstanding, can lead to irreversible changes to the pulmonary vasculature and development of Eisenmenger syndrome (ES). We present the case of a pediatric patient who developed ES due to a hemodynamically significant PDA and VSD, as well as a sizable atrial septal defect, that were not evaluated for many years in the setting of both language and cultural barriers to accessing care in the Amish community. While demonstrating slight improvements in oxygen saturations with medical therapies tailored toward the family's cultural needs, her prognosis remains guarded. We review the essential need for the medical community to proactively provide care to patient populations with identifiable barriers and to appropriately intervene to prevent morbidity and mortality.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101837"},"PeriodicalIF":0.6,"publicationDate":"2025-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144105098","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":"Staged biventricular repair for D-transposition of the great arteries with a hypoplastic right ventricle","authors":"Khaled Shams Abdelmagid , Nirbhay Parashar , Brian Reemtsen , Amna Qasim","doi":"10.1016/j.ppedcard.2025.101838","DOIUrl":"10.1016/j.ppedcard.2025.101838","url":null,"abstract":"<div><div>Right ventricular hypoplasia with dextro-transposition of great arteries (D-TGA) is a rare association that precludes arterial switch operation in the neonatal period. Given its rarity, a standardized pathway for surgical decision-making (single vs biventricular repair) does not exist. We report a case of D-TGA-hypoplastic-RV that underwent successful staged biventricular repair.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101838"},"PeriodicalIF":0.6,"publicationDate":"2025-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144070798","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":"Social determinants and dilated cardiomyopathy in children","authors":"Alfred Asante-Korang","doi":"10.1016/j.ppedcard.2025.101834","DOIUrl":"10.1016/j.ppedcard.2025.101834","url":null,"abstract":"<div><h3>Background</h3><div>Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy. The Pediatric Cardiomyopathy registry data show a yearly incidence of 0.57 per 100,000 children. The incidence was higher in Blacks than in Whites, a finding that has broad implications in terms of outcomes.</div></div><div><h3>Aims of review</h3><div>The aims of this manuscript are to 1) Review available evidence of the impact of social determinants of health (SDOH) on outcomes of pediatric DCM, 2) Discuss mitigating strategies for improving the care and outcomes of DCM in the children affected by the negative effects of SDOH, and finally 3) Discuss how we can incorporate some of these strategies in our daily pediatric practice as advocates for children with DCM who struggle with SDOH.</div></div><div><h3>Key scientific concepts and methodology</h3><div>We performed a comprehensive literature review to determine the scope of the problem of SDOH in pediatric patients with DCM, heart failure, and mechanical circulatory support. We reviewed potential strategies that have been recommended to help mitigate the problem.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101834"},"PeriodicalIF":0.6,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143916618","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":"Review of cardiovascular complications in children with sickle cell disease","authors":"Saswat Kumar Jha , Linhan Jasmine Ha , Maxon Bassett , Avery Silar , Brianna L. Gilner , Induja Gajendran , Preethi Marri","doi":"10.1016/j.ppedcard.2025.101836","DOIUrl":"10.1016/j.ppedcard.2025.101836","url":null,"abstract":"<div><h3>Background</h3><div>Cardiovascular complications in pediatric patients with sickle cell disease may be life-threatening.</div></div><div><h3>Aim of review</h3><div>The purpose of this article is to review cardiovascular problems associated with sickle cell disease in children and adolescents, including pathophysiology, clinical presentation, diagnosis, and treatment.</div></div><div><h3>Key scientific concepts of review</h3><div>Sickle cell disease is a hemoglobinopathy that causes red blood cell sickling and vaso-occlusive crises. Chronic anemia may cause high-output cardiac failure. Other cardiovascular abnormalities include pulmonary hypertension, increased tricuspid regurgitation velocity, increased right ventricular pressure, cardiomyopathy with left ventricular hypertrophy and dysfunction, and increased risk of myocardial infarction, dysrhythmia, and sudden cardiac death. Patients may have chronic hemolytic anemia, red blood cell sickling, vaso-occlusive crises, and cardiovascular symptoms including chest pain, palpitations, shortness of breath, and fatigue. Cardiovascular complications also may include stroke, acute renal failure, and priapism. Diagnosis includes family history and diagnostic tests such as complete blood count, electrocardiography, echocardiography, chest radiography, and pulmonary function tests. Treatment of sickle cell disease may include hydroxyurea and treatment of complications, including pain from vaso-occlusive crises and cardiovascular disease. Early detection and intervention are important to limit sequelae of cardiovascular complications, and regular cardiovascular screening in pediatric patients with sickle cell disease is recommended.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101836"},"PeriodicalIF":0.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144116169","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":"Aortic arch dimensions in neonates with Down syndrome","authors":"Abhishek Chakraborty , Mohanageetha Ardhanari , Marjorie Gayanilo , Hanah Walker , Lindsey Bird , Nao Sasaki , Sethuraman Swaminathan","doi":"10.1016/j.ppedcard.2025.101835","DOIUrl":"10.1016/j.ppedcard.2025.101835","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) is a major cause of morbidity and mortality in patients with Down syndrome.</div></div><div><h3>Objectives</h3><div>The objective of this study is to identify the prevalence of abnormal aortic arch dimensions in patients with Down syndrome.</div></div><div><h3>Methods</h3><div>This was a retrospective chart review of patients with Down syndrome who were born at Jackson Health System over a 14-year period. The initial neonatal echocardiograms were reviewed for presence of CHD along with the dimensions of aortic annulus, distal transverse arch, and aortic isthmus, and then compared with age and sex matched cohorts.</div></div><div><h3>Results</h3><div>A total of 138 patients were included in this study. The overall prevalence of congenital heart disease in our cohort was 70.7 % (<em>n</em> = 97). Among those identified, the most common CHDs were ventricular septal defect (VSD) (23.2 %), atrial septal defect (ASD) (24.6 %), and atrioventricular septal defect (AVSD) (34.8 %) Fourteen patients (10.1 %) had aortic isthmus hypoplasia and 9 (6.5 %) had hypoplasia of the distal transverse arch.</div></div><div><h3>Conclusion</h3><div>Mild aortic arch hypoplasia without coarctation is found in 13.8 % of patients with Down syndrome. Specific nomograms may be necessary for measuring aortic arch dimensions in patients with Trisomy 21.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101835"},"PeriodicalIF":0.6,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923458","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":"Risk of neurodevelopmental delay in children with congenital heart defects: Hospital based prospective cross-sectional study","authors":"Tesfaye Taye Gelaw, Shitahun Fentie Tilahun","doi":"10.1016/j.ppedcard.2025.101832","DOIUrl":"10.1016/j.ppedcard.2025.101832","url":null,"abstract":"<div><h3>Background</h3><div>The effect of congenital heart defects (CHDs) on adverse neurodevelopment has been reduced with modern medical services in high-income countries. This is in contrast to low-and-middle-income countries where the benefits of modern medical advantages are practically non-existent.</div></div><div><h3>Objective</h3><div>Determine the risk of developmental delay in children with CHD and examine associated factors.</div></div><div><h3>Method</h3><div>A hospital-based prospective cross-sectional study was conducted on 288 children from 2 to 60 months of age with CHDs. Data were collected prospectively from August 15, 2024 – January 15, 2025. Development assessment was conducted using the “CDC's Milestone Moments booklet.” Logistic regression was performed to see the association of independent variables with the dependent variable – development delay. A <em>P</em>-value of ≤0.05 was considered significant. SPSS version 27 was used for analysis.</div></div><div><h3>Result</h3><div>Of the studied children with CHDs, 51 % were females. The mean (SD) age was 18 months (16). Delayed development was seen in 46 % of the studied children. The motor domain is the most affected development domain (42 %). Rural residency {AOR = 2.21, 95 % CI = (1.08, 4.53)}, suspected genetic/syndromic comorbidity {AOR = 18.92, 95 % CI = (7.91, 45.24)}, severe stunting {AOR = 5.91, 95 % CI = (2.67, 13.11)} and dextro-transposition of great arteries (d-TGA) {AOR = 58.56, 95 % CI = (4.97, 689.6)} were statistically significant associated factors.</div></div><div><h3>Conclusion</h3><div>The risk of neurodevelopmental delay is a common problem in children with CHD. Suspected genetic/syndromic comorbidities, severe stunting, rural residency, and d-TGA are statistically significantly associated factors with neurodevelopmental delay in a developing nation.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"78 ","pages":"Article 101832"},"PeriodicalIF":0.6,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144633311","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":"Performance of first-trimester fetal nuchal translucency, ductus venosus, tricuspid regurgitation in diagnosing congenital heart defects: A systematic review and meta-analysis","authors":"Behnaz Moradi , Payam Jannatdoust , Paria Shafiekhani , Mohamadreza Tahamtan , Gita Manzari Tavakoli , Parya Valizadeh , Pouya Ebrahimi , Niloofarsadaat Eshaghhosseiny , Farzan Moodi , Hamed Ghorani , Parisa Kohnepoushi , Mahshad Razaghi , Morteza Sanei Taheri , Delaram J. Ghadimi","doi":"10.1016/j.ppedcard.2025.101833","DOIUrl":"10.1016/j.ppedcard.2025.101833","url":null,"abstract":"<div><h3>Background</h3><div>Congenital heart disease (CHD) is the main cause of perinatal morbidity and mortality. Nuchal Translucency (NT), Ductus Venosus (DV), and Tricuspid Regurgitation (TR) have shown potential in CHD detection.</div></div><div><h3>Aim of review</h3><div>We evaluated the pooled diagnostic test accuracy of these markers during the first-trimester screening.</div></div><div><h3>Key scientific concepts of review</h3><div>PubMed, Scopus, Web of Science, and Embase were searched. A bivariate random effects model created Summary Receiver Operating Characteristic (SROC) curves and the pooled sensitivities and specificities. Forty-two studies were included. For major CHDs, the pooled sensitivities and specificities were 43.1 % (95 % CI: 35.0 %–51.6 %) and 95.5 % (95 % CI: 93.5 %–96.9 %) for A/R DV a wave, 57.8 % (95 % CI: 43.3 %–71.0 %) and 88.8 % (95 % CI: 77.7 %–94.7 %) for abnormal DV-PIV, 37.0 % (95 % CI: 26.6 %–48.6 %) and 97.7 % (95 % CI: 94.6 %–99.1 %) for TR, 41.4 % (95 % CI: 23.2 %–62.2 %) and 93.7 % (95 % CI: 92.7 %–94.6 %) for NT > 95th percentile, and 26.6 % (95 % CI: 11.0 %–51.7 %) and 98.3 % (95 % CI: 97.5 %–98.9 %) for NT > 99th percentile. For the combined models in detecting major CHDs, the highest specificity of 97.8 % (95 % CI: 93.9 %–99.2 %) belonged to NT > 95th percentile and A/R DV a wave. The most sensitive tests were the combination of NT > 95th percentile or A/R DV a wave or TR 61.4 % (95 % CI: 49.7 %–71.9 %). Combining increased NT with the presence of A/R a-wave can help diagnose CHD, while normal NT, A/R DV a wave, and TR indicate lower CHD risk.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101833"},"PeriodicalIF":0.6,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143912627","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}