Pediatric Cardiology最新文献

{"title":"The Inadequate Oxygen Delivery Index and Its Correlation with Venous Saturation in the Pediatric Cardiac Intensive Care Unit.","authors":"Rohit S Loomba, Enrique G Villarreal, Saul Flores, Juan S Farias, Alex Constas","doi":"10.1007/s00246-023-03302-x","DOIUrl":"10.1007/s00246-023-03302-x","url":null,"abstract":"<p><p>Continuous monitoring software, T3, has an integrated index called the inadequate oxygen delivery index 50% (IDO2-50) which displays a probability that the mixed venous saturation is below a user-selected threshold of 30-50%. The primary aim of this study was to determine the correlation of the IDO2-50 with a measured venous saturation. The secondary aim of this study was to characterize the hemodynamic factors that contributed to the IDO2-50. This single-center, retrospective study aimed to characterize the correlation between IDO2-50 and inferior vena cava (IVC) saturation. A Bayesian Pearson correlation was conducted to assess the correlation between the collected variables of interest, with a particular interest in the correlation between the IDO2-50 and the IVC saturation. Receiver operator curve (ROC) analysis to assess the ability of the IDO2-50 to identify when the venous saturation was less than 50%. Bayesian linear regression was done with the IDO2-50 (dependent variable) and other independent variables. A total of 113 datasets were collected across 15 unique patients. IDO2-50 had moderate correlation with the IVC saturation (correlation coefficient - 0.569). The IDO2-50 had a weak but significant correlation with cerebral near-infrared spectroscopy (NIRS) values, a weak but significant correlation with heart rate, and a moderate and significant correlation with arterial saturation. ROC analysis demonstrated that the IDO2-50 had a good ability to identify a venous saturation below 50%, with an area under the curve of 0.797, cutoff point of 24.5 with a sensitivity of 81%, specificity of 66%, positive predictive value of 44%, and negative predictive value of 91%. Bayesian linear regression analysis yielded the following model: 237.82 + (1.18 × age in months) - (3.31 × arterial saturation) - (1.92 × cerebral NIRS) + (0.84 × heart rate). The IDO2 index has moderate correlation with IVC saturation. It has good sensitive and negative predictive value. Cerebral NIRS does appear to correlate better with the underlying venous saturation than the IDO2 index.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"39-45"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41160223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Experience with SGLT2 Inhibitors in Patients with Single Ventricle Congenital Heart Disease and Fontan Circulatory Failure.","authors":"Anusha Konduri, Caroline West, Ray Lowery, Tiffany Hunter, Audrey Jarosz, Sunkyung Yu, Heang M Lim, Amanda D McCormick, Kurt R Schumacher, David M Peng","doi":"10.1007/s00246-023-03332-5","DOIUrl":"10.1007/s00246-023-03332-5","url":null,"abstract":"<p><p>Heart failure is the leading cause of morbidity and mortality in patients with Fontan circulation. Sodium-glucose-cotransporter 2 inhibitors (SGLT2i) have become a mainstay of heart failure therapy in adult patients, however, there remains a paucity of literature to describe its use in pediatric heart failure patients, especially those with single ventricle physiology. We describe our early experience using SGLT2i in patients with single ventricle congenital heart disease surgically palliated to the Fontan circulation. We conducted a single-center retrospective chart review of all patients with Fontan circulation who were initiated on an SGLT2i from January 1, 2022 to March 1, 2023. Patient demographics, diagnoses, clinical status, and other therapies were collected from the electronic medical record. During the study period, 14 patients (median age 14.5 years, range 2.0-26.4 years) with Fontan circulation were started on a SGLT2i. Mean weight was 54 kg (range 11.6-80.4 kg). Median follow-up since SGLT2i initiation was 4.1 months (range 13 days-7.7 months). Four patients had a systemic left ventricle and 10 had a systemic right ventricle. Half the patients had Fontan Circulatory Failure with reduced Ejection Fraction (FCFrEF) of the systemic ventricle and the other half had Fontan Circulatory Failure with preserved Ejection Fraction (FCFpEF) of the systemic ventricle. In addition, 3 patients experienced Protein Losing Enteropathy (PLE) and 2 patients had plastic bronchitis, one of whom also was diagnosed with chylothorax. There were no genitourinary infections, hypoglycemia, ketoacidosis, hypotension or other significant adverse effects noted in our patient population. One patient experienced significant diuresis and transient acute kidney injury. Patients with FCFrEF showed a decrease in natriuretic peptide levels. Given the lack of proven therapies, demonstrated benefits of SGLT2i in other populations, and some suggestion of efficacy in Fontan circulation, further study of SGTLT2i in patients with Fontan circulation is warranted.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"81-88"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71425837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mitral Valve Abnormalities Associated with Single-Ventricle Palliation, Cardiac Death or Transplant in Fetuses with Postnatally Confirmed Coarctation of the Aorta.","authors":"Alex J Foy, Jonathan H Soslow, Ann L Kavanaugh-McHugh, Stacy A S Killen","doi":"10.1007/s00246-023-03335-2","DOIUrl":"10.1007/s00246-023-03335-2","url":null,"abstract":"<p><p>Predicting if a fetus with borderline left heart structures and coarctation of the aorta (CoA) will require single ventricle palliation (SVP) is challenging, partly due to the limitations of fetal echocardiography in defining valvar abnormalities. Fetal echocardiographic findings predictive of SVP, particularly in relation to the mitral valve (MV), are not well defined. We performed a retrospective review of fetuses with postnatally confirmed CoA from 2010 to 2020. Fetuses with complex congenital heart disease or unequivocal hypoplastic left heart syndrome were excluded. Data were compared between those who underwent biventricular repair (BVR) versus SVP, cardiac death or orthotopic heart transplant (OHT) to determine differences in fetal echocardiograms. Of 67 fetuses with 131 total echocardiograms, 62 (93%) underwent BVR and 5 (7%) experienced SVP, cardiac death or OHT. Fetuses with confirmed CoA who experienced SVP, cardiac death, or OHT, had fetal MV z-scores that were 2.03 lower, on average, than those who underwent BVR (z-score =  - 3.98 vs. - 1.94, 95% CI - 2.93, - 1.13). The incidences of MV anomalies and left to right flow across the foramen ovale were higher in the SVP, cardiac death and OHT group. SVP, cardiac death or OHT in fetuses with confirmed CoA were associated with severe fetal MV hypoplasia, MV anomalies and left to right flow across the foramen ovale. These findings may help guide prenatal counseling about the likelihood of SVP, cardiac death or OHT in fetuses with CoA and borderline left heart structures.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"98-106"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"107591962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Enhancing Quality of Congenital Heart Care Within Resource-Limited Settings.","authors":"Shazia Mohsin, Babar Hasan, Bistra Zheleva, Raman Krishna Kumar","doi":"10.1007/s00246-023-03351-2","DOIUrl":"10.1007/s00246-023-03351-2","url":null,"abstract":"<p><p>Over 90% of the world's children with congenital heart disease (CHD) are born in the resources poor settings of low- to middle-income countries (LMICs). The shortfall in human and material resources and dysfunctional health systems leads to poor quality of care (QoC) which contributes substantially to suboptimal outcomes of patients with CHD in LMICs. Notwithstanding these challenges, it is possible to develop a quality improvement (QI) framework that can have a significant impact on outcomes and prevent a number of deaths. In this review, we examine the common barriers to implementing effective QI processes in LMICs. Using examples of successful QI initiatives in LMIC, we propose a broad framework that focuses on simple, yet effective measures involving cohesive efforts of all key participants guided and nurtured by a leadership that strongly values QoC.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"27-38"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138830903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aspirin response testing: time for patient-specific tailored care.","authors":"Irene Regan, Sean Kelleher, Dermot Cox, Colin J McMahon","doi":"10.1007/s00246-024-03677-5","DOIUrl":"10.1007/s00246-024-03677-5","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"237-238"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Expression Profile of TGFB1 Gene in Pediatric Patients with Isolated Bicuspid Aortic Valve.","authors":"Nazan Eras, Ayşegül Çetinkaya, Dilek Giray, Olgu Hallıoglu, Nurcan Aras","doi":"10.1007/s00246-024-03733-0","DOIUrl":"10.1007/s00246-024-03733-0","url":null,"abstract":"<p><p>Studies on the genetic basis of bicuspid aortic valve (BAV), characterized by a configuration of the aortic valve with two leaflets instead of three, are insufficient. This study aimed to elucidate the possible relationship between BAV and TGF-β1 gene expression levels. Forty-eight pediatric patients diagnosed with isolated BAV and 50 healthy children with innocent heart murmurs were included in the study. A comprehensive transthoracic echocardiographic examination was performed using the Vivid S5 Pro Ultrasound for all children. TGF-β1 gene expression levels were detected by RT-PCR method. The mean age of the patients was 8.54 ± 5.3 years, and the mean age of the children in the control group was 7.07 ± 5.34 years. It was noted that TGF-β1 gene expression level increased 2.91 times in the patient group compared to the control group (p = 0.03). TGF-β1 gene expression levels of patients with BAV decreased with age. This is the first study to show TGF-β1 gene expression levels in children with BAV. According to the results of our study, TGF-β1 gene expression levels in children with BAV decrease with age. Further studies are needed in all age groups to use TGF-β1 as a prognostic marker in patients with BAV.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"207-212"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142837967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy of a Longitudinal Project-Based Quality Improvement Curriculum in Pediatric Cardiology Fellowship.","authors":"Cara M Bird, Sonia Kaushal, Mira K Trivedi, Judson A Moore, Shreya S Sheth","doi":"10.1007/s00246-023-03340-5","DOIUrl":"10.1007/s00246-023-03340-5","url":null,"abstract":"<p><p>Quality improvement knowledge is a requirement of fellowship training. Our goal was to evaluate the efficacy of a 3-year quality improvement and patient safety (QI/PS) curriculum that gives fellows both didactic knowledge and first-hand experience with improvement science, and meets Clinical Learning Environment Review (CLER) requirements. Knowledge assessment is obtained through pre- and post-surveys. A secondary measure of success is academic products resulting from fellows' QI/PS work, and future participation in QI/PS efforts. Since 2019, 51 pre-tests and 36 post-tests were completed, showing improvement across all competencies. Fellows have produced one published manuscript, two poster presentations, and two oral presentations describing their improvement work. Additionally, mentoring faculty members have gone on to lead other QI work throughout the division. This longitudinal QI/PS curriculum provides both knowledge and experience in QI/PS work. It also creates opportunities for academic publications and presentations, builds faculty expertise, and most importantly, works to improve multiple aspects of patient care. This curriculum can serve as a model for other cardiology fellowships working to meet CLER requirements.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"127-133"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"107591960","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cross or Not to Cross-Dilemma of the Pulmonary Arteries.","authors":"Derya Bako, Yasemin Nuran Dönmez, Ulaş Yalım Uncu, Ezgi Yangın Ergon, Sinan Genç, Zerrin Karakuş Epçaçan, Şafak Alpat, Serdar Epçaçan","doi":"10.1007/s00246-023-03393-6","DOIUrl":"10.1007/s00246-023-03393-6","url":null,"abstract":"<p><p>We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0-18 years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients' images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly (p = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"198-206"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139040351","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Lead Extraction in Children and Young Adults: When is the Best Time for Lead/System Replacement?","authors":"Andrzej Kutarski, Maria Miszczak-Knecht, Monika Brzezinska, Mariusz Birbach, Wojciech Lipiński, Wojciech Jacheć, Bettina Ziaja, Anna Polewczyk, Łukasz Tułecki, Marek Czajkowski, Dorota Nowosielecka, Katarzyna Bieganowska","doi":"10.1007/s00246-023-03320-9","DOIUrl":"10.1007/s00246-023-03320-9","url":null,"abstract":"<p><p>The best strategy for lead management in children is a matter of debate, and our experiences are limited. This is a retrospective single-center study comparing difficulties and outcomes of transvenous lead extraction (TLE) implanted ich childhood and at age < 19 years (childhood-implanted-childhood-extracted, CICE) and at age < 19 (childhood-implanted-adulthood-extracted, CIAE). CICE patients-71 children (mean age 15.1 years) as compared to CIAE patients (114 adults (mean age 28.61 years) were more likely to have VVI than DDD pacemakers. Differences in implant duration (7.96 vs 14.08 years) appeared to be most important, but procedure complexity and outcomes also differed between the groups. Young adults with cardiac implantable electronic device implanted in childhood had more risk factors for major complications and underwent more complex procedures compared to children. Implant duration was significantly longer in CIAE patients than in children, being the most important factor that had an impact on patient safety and procedure complexity. CIAE patients were more likely to have prolonged operative duration and more complex procedures due to technical problems, and they were 2-3 times more likely to require second-line or advanced tools compared to children, but the rates of clinical and procedural success were comparable in both groups. The difference between the incidence of major complications between CICE and CIAE patients is very clear (MC 2.9 vs 7.0%, hemopericardium 1.4 vs 5.3% etc.), although statistically insignificant. Delay of lead extraction to adulthood seems to be a riskier option than planned TLE in children before growing up.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"61-71"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11753338/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"66784203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gut Microbiome in Children with Congenital Heart Disease After Cardiopulmonary Bypass Surgery (GuMiBear Study).","authors":"Shahida Bibi, Kanwal Majeed, Zarmeen Rukhman","doi":"10.1007/s00246-024-03670-y","DOIUrl":"10.1007/s00246-024-03670-y","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"231-232"},"PeriodicalIF":1.5,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378073","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}