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":"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}
Ryan Kimball , Isaac J. Jacobs , Matthew W. Sorensen , Jeffrey A. Robinson
{"title":"Differences in school preparedness for sudden cardiac arrest based on automated external defibrillator availability and staff training between schools of different enrollment size","authors":"Ryan Kimball , Isaac J. Jacobs , Matthew W. Sorensen , Jeffrey A. Robinson","doi":"10.1016/j.ppedcard.2025.101831","DOIUrl":"10.1016/j.ppedcard.2025.101831","url":null,"abstract":"<div><h3>Background</h3><div>A school has a 1–2 % chance of having a sudden cardiac arrest (SCA) on its campus in any given year. Schools can be prepared by having automated external defibrillators (AEDs) readily accessible with staff trained in their use. The overall survival rate of SCA is less than 10 %. However, those who suffer SCA at a school that has an AED on-site have more than a 60 % chance of surviving to discharge. Currently, AEDs are not required in schools across all 50 states in the US.</div></div><div><h3>Objective</h3><div>Investigate the availability of AEDs in high schools across a state prior to any formal mandate and explore discrepancies among schools of varying enrollment sizes.</div></div><div><h3>Methods</h3><div>A 16-question survey about AED availability, accessibility, and percentage of staff trained for its use was distributed to every high school in Nebraska in July 2022.</div></div><div><h3>Results</h3><div>Schools with smaller enrollments were more likely to have AEDs accessible to the public (<em>p</em> = 0.047), available at all events (<em>p</em> = 0.0002), and over 50 % of staff trained for its use (<em>p</em> = 0.00019) than schools with larger enrollments. There was no correlation between number of AEDs and enrollment (R<sup>2</sup> = 0.172).</div></div><div><h3>Conclusions</h3><div>Most high schools have automated external defibrillators (AEDs), even without statewide mandates; however, continuing efforts are needed to ensure that AEDs at all schools are accessible to the public and that staff are appropriately trained. This study highlights the need for increased advocacy and targeted resource allocation, particularly for larger schools, to ensure appropriate/adequate AED distribution and emergency preparedness across all high schools.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101831"},"PeriodicalIF":0.6,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143839302","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}
Chelsea Miller, Michael J. Shorofsky, Michael Hainstock
{"title":"Transcatheter occlusion of the neoaorta to augment mechanical circulatory support after stage 1 single ventricle palliation","authors":"Chelsea Miller, Michael J. Shorofsky, Michael Hainstock","doi":"10.1016/j.ppedcard.2025.101830","DOIUrl":"10.1016/j.ppedcard.2025.101830","url":null,"abstract":"<div><div>Aortic insufficiency can be a complication of continuous mechanical circulatory support that leads to inefficient circulation and decreased coronary perfusion. In adults, this has been addressed through both surgical and transcatheter approaches. However, this has rarely been reported in children with congenital heart disease. We present the case of a neonate who developed severe neoaortic insufficiency after stage one single ventricle palliation while on central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) that was managed with transcatheter neoaortic occlusion. We used an Amplatzer Vascular Plug II 10 mm device (Abbott Cardiovascular) to successfully improve neoaortic insufficiency and overall hemodynamics. This procedure was technically feasible and may be considered in rare cases to address aortic insufficiency while on mechanical circulatory support.</div></div>","PeriodicalId":46028,"journal":{"name":"PROGRESS IN PEDIATRIC CARDIOLOGY","volume":"77 ","pages":"Article 101830"},"PeriodicalIF":0.6,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143815538","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}