Pediatric Cardiology最新文献

{"title":"Predictive Role of Atrial Strain, Aortic Stiffness, and Muscle-Related Factors for Maximal and Submaximal Exercise Capacity in Fontan Patients.","authors":"Haluk Tekerlek, Hayrettin Hakan Aykan, Naciye Vardar-Yagli, Sinem Nur Selcuk, Merve Basol-Goksuluk, Tevfik Karagoz, Melda Saglam","doi":"10.1007/s00246-024-03687-3","DOIUrl":"https://doi.org/10.1007/s00246-024-03687-3","url":null,"abstract":"<p><p>Fontan patients' exercise capacity and associated risks of morbidity and mortality necessitate a comprehensive study. We aim to explore maximal and submaximal exercise capacity, assessing the impact of cardiovascular and peripheral factors, including atrial strain, aortic stiffness, and muscle-related factors. Fontan patients and matched controls were examined. Clinical details were recorded, and atrial strain and aortic stiffness were measured using echocardiography. Hand grip and knee extensor strength were assessed, and muscle oxygenation was recorded. Cardiopulmonary exercise test determined exercise capacity, with peak oxygen uptake/kg (VO₂/kg) and oxygen uptake efficiency slope/kg (OUES/kg) as markers for maximal and submaximal capacity, respectively. Thirty-one Fontan patients (median age = 18, range = 7-31 years) and 30 controls (median age = 18, range = 7-32 years) were studied. No significant differences in body composition and muscle strength were observed between Fontan patients and controls (p > 0.05). Fontan patients exhibited lower peak VO₂/kg and OUES/kg (p < 0.001 for both), along with decreased atrial strain and increased aortic stiffness compared to controls (p < 0.001). Aortic pulse wave velocity (PWV), left atrial strain contractile phase (LASct), and knee extensor strength independently predicted peak VO₂/kg (R² = 0.514) and OUES/kg (R² = 0.486) in Fontan patients; age was a predictor for peak VO₂/kg. Atrial contractile strain, aortic stiffness, and knee extensor muscle strength are predictors of submaximal exercise capacity; furthermore, age, along with these variables, serves as predictors of maximal exercise capacity in Fontan patients. Evaluating hemodynamic, vascular, and muscular parameters, in conjunction with assessing both maximal and submaximal exercise capacities, is crucial for optimizing disease management in Fontan patients. Clinicaltrials.gov registration: NCT05011565.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142546717","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":"Pulmonary Valve Aneurysm Associated with a Patent Ductus Arteriosus.","authors":"W Wyatt Lindsey, Catherine E Tomasulo, Alan E Benheim","doi":"10.1007/s00246-024-03695-3","DOIUrl":"10.1007/s00246-024-03695-3","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142546718","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 Annular Disjunction Associated with Ventricular Dilation in Pediatric Marfan Syndrome: A Cardiovascular Magnetic Resonance Study.","authors":"Ryan S Bishop, Tam T Doan, Natalie K Craik, Sara B Stephens, Alejandra Iturralde Chavez, Shagun Sachdeva, Justin D Weigand","doi":"10.1007/s00246-024-03678-4","DOIUrl":"https://doi.org/10.1007/s00246-024-03678-4","url":null,"abstract":"<p><strong>Background: </strong>Mitral annular disjunction (MAD) has increasingly been recognized as a marker for adverse cardiovascular events in Marfan syndrome (MFS). As recent adult data links MFS with left ventricular (LV) dilation and reduced ejection fraction (LVEF), we hypothesized that MAD may be associated with LV dilation in pediatric MFS patients.</p><p><strong>Methods: </strong>A retrospective analysis was performed among MFS patients < 19 years old at initial cardiac MRI (CMR). MAD and mitral valve prolapse (MVP) were assessed by CMR or most proximate echo. CMR-derived left ventricular end-diastolic (LVEDV) and end-systolic (LVESV) volumes were measured. Indexed volumes, absolute and indexed z-scores, and LVEF were calculated. The combined volume load from mitral and aortic regurgitation was indexed to LV stroke volume, allowing exclusion of patients with greater than mild volume load or prior MV intervention. MAD association with LV volumes and z-scores was then assessed.</p><p><strong>Results: </strong>Forty-two patients were analyzed (median age 13.5 years old, IQR [10.9, 15.3]). MAD was present in 28 patients (66.7%), and MVP was present in 13 patients (31.0%). Absolute LVEDV z-score was > 2 in 35.7% of patients, LVESV z-score was > 2 in 42.9%, and LVEF was < 55% in 45.2%. In multivariable analysis including MVP, MAD remained independently associated with elevated absolute LVESV z-score > 2 (RR 3.88, 95% CI 1.02-14.69, p = 0.046).</p><p><strong>Conclusion: </strong>MAD was associated with CMR-derived volume-load-independent LV dilation among pediatric MFS patients. Prospective studies are needed to further understand this association and its relationship with LV dilation over time.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505505","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":"Reply to the Comments on \"Biomarkers and Related Factors for the Diagnosis, Risk of Coronary Artery Lesions, and Resistance to Intravenous Immunoglobulin in Kawasaki Disease: An Umbrella Review of Meta-Analyses\".","authors":"Tae Hyeon Kim, Yejun Son, Jiseung Kang, Dong Keon Yon","doi":"10.1007/s00246-024-03693-5","DOIUrl":"https://doi.org/10.1007/s00246-024-03693-5","url":null,"abstract":"<p><p>The article \"Biomarkers and related factors for the diagnosis, risk of coronary artery lesions, and resistance to intravenous immunoglobulin in Kawasaki disease: an umbrella review of meta-analyses\" offers valuable insights into diagnostic and therapeutic settings by overviewing various biomarkers associated with Kawasaki disease. There are some comments related to our umbrella review methodology, particularly PRIOR guidelines and GRADE framework. We clarify our choice of the PRISMA 2020 guidelines over PRIOR and explain the rigorous methodology used, which aligns with GRADE principles, ensuring robust and reliable findings. In addition, we address the suggestion for sensitivity analysis, noting that potential biases were managed through AMSTAR2 assessments, heterogeneity testing, and publication bias analysis.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505506","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":"Screening for Life-Threatening Arrhythmia in Asymptomatic Patients After Paediatric Cardiac Surgery: A Single-Centre Retrospective Analysis of 790 Pre-hospital-discharge 24-h Holter Electocardiogram Recordings.","authors":"Evangelia Blana, Matthias Gass, Florian Berger, Hitendu Dave, Christian Balmer","doi":"10.1007/s00246-024-03691-7","DOIUrl":"10.1007/s00246-024-03691-7","url":null,"abstract":"<p><p>Severe arrhythmias may occur early after open heart surgery. Because younger patients do not usually show any specific symptoms, presently Holter monitoring is routinely performed for 24 h predischarge at our centre to prevent adverse outcomes. It is unknown whether this test is truly justified in this patient population. Retrospective single-centre analysis of all consecutive patients younger than 19 years old after open heart surgery 2013-2019 who underwent routine Holter monitoring before hospital discharge. Patients with permanent pacemakers and patients who died during this hospital stay were excluded. The cohort was divided into two groups depending on whether severe arrhythmia occurred or not. The study includes 790 Holter recordings from 666 patients with a median age of 0.5 years (IQR 0.23-3.08), performed at a median time of 8 days (IQR 6-15) postoperatively. Postoperative arrhythmia was detected in 554 of 790 24-h Holter recordings (70%); in 47 of 790 (6%), this arrhythmia was classified as severe. The most common severe arrhythmias were premature ventricular contractions (n = 26/47) and long pauses (n = 14/47). A longer aortic cross-clamp time (mean 94.5 (SD ± 53.0) versus 68.1 (SD ± 51.9) min, p = 0.001) was associated with the occurrence of severe postoperative arrhythmia. Severe arrhythmias are rare in predischarge assessments after open heart surgery in children. In current postoperative monitoring at our centre, the diagnostic yield of ECG Holter monitoring for 24 h is too low to justify routine screening in all paediatric patients after open heart surgery.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505507","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":"Palliative Care Referral Patterns and Implications for Standardization in Cardiac ICU.","authors":"Arshia Madni, Jocelyn Matheson, Amanda Linz, Austin Dalgo, Rumana Siddique, Anthony Merlocco","doi":"10.1007/s00246-024-03681-9","DOIUrl":"https://doi.org/10.1007/s00246-024-03681-9","url":null,"abstract":"<p><p>Evidence suggests that pediatric palliative care involvement (PPC) is beneficial to medically complex patients. Historically, PPC involvement has been overlooked or delayed and varies by institution but PPC awareness has increased in cardiovascular ICUs (CVICU) and so we investigated frequency and timeliness of PPC referral for patients dying in ICU. Retrospective study of pediatric cardiac patients experiencing death in ICU to review PPC presence and timing of initial PPC, most recent PPC, and interventions, therapies, CPR, and presence of do-not-resuscitate DNR discussion. Fifty-four patients died during a 5-year period aged 11d-17y (54% male). PPC involvement occurred in 40/54 (74%). Of those patients without PPC, the Center to Advance Palliative Care (CAPC) guidelines would have supported PPC in 11/14 (79%). DNR discussion was more likely in PPC patients (63% vs 14%; p = 0.0011), though often only on DOD. Comparing prior to DOD, PPC patients were still more likely to have DNR discussion (55% vs 0%; p = 0.0003). PPC patients were no less likely to have CPR on DOD (28% vs 43%, p = 0.29). PPC occurred frequently in patients experiencing death in CVICU. However, frequently the initial PPC occurred within a week or day of death. Patients without PPC would often qualify under published guidelines. Standardization, timing, and patient identification for PPC will expand efficacy in CVICU.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472003","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":"Comparative Analysis of Respiratory and Functional Outcomes in Children Post-Fontan Procedure Versus Healthy Peers.","authors":"João Bruno Piantino Dias Moura Silveira, Aida Luiza Ribeiro Turquetto, Luciana Patrick Amato, Daniela Regina Agostinho, Luiz Fernando Caneo, Maria Angelica Binotto, Maria Isabel da Costa Soares Lopes, Joaquim Carlos Rodrigues, Marcus Vinicius Barbosa Santos, Patricia Alves Oliveira, Ana Cristina Sayuri TanaKa, Marcelo Biscegli Jatene","doi":"10.1007/s00246-024-03666-8","DOIUrl":"https://doi.org/10.1007/s00246-024-03666-8","url":null,"abstract":"<p><p>Studies have shown that respiratory muscle training enhances functional capacity and pulmonary function in Fontan patients. However, diaphragm muscle characteristics in Fontan children have not been fully elucidated. The aim of this study was to compare respiratory function, maximal and submaximal functional capacities, and quality of life, as well as to assess diaphragm mobility and thickness, between Fontan patients aged 8 to 12 years and healthy individuals. This cross-sectional study included 45 children-27 Fontan patients, Fontan Group (FG) and 18 healthy control Group (CG) subjects. Different examinations were performed: spirometry was used to analyze pulmonary volume and capacity; manovacuometry was used to determine respiratory muscle strength; chest ultrasound was used to determine diaphragm muscle characteristics; cardiopulmonary exercise and the six-minute walk test (6MWT) were used to determine functional capacity; the AUQEI questionnaire was used to determine quality of life. Descriptive analysis and intergroup comparisons were performed for all the data. Compared with the CG, the FG exhibited impaired pulmonary function and reduced functional capacity. Significant differences in median values were noted for forced expiratory volume in one second (FEV₁): 2.39 L/min, p = 0.002; forced vital capacity (FVC): 1.73 vs. 3.06 L/min, p = 0.002; maximal inspiratory pressure: - 73 vs. - 117 cmH₂O, p = 0.007; absolute peak VO₂: 1.09 vs. 1.51 L/min, p < 0.001; relative peak VO₂: 31.9 vs. 42.5 mL/kg/min, p = 0.003; and 6MWT distance: 420 vs. 586 m, p < 0.001. Diaphragmatic thickness, mobility and quality of life were similar between the groups. Despite the reduced functional capacity, impaired pulmonary volume and capacity, and respiratory muscle weakness of the FG compared to those of the CG, the diaphragm characteristics and quality of life were similar between the groups.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471997","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":"Correction: Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative.","authors":"George T Nicholson, Jeffrey D Zampi, Andrew C Glatz, Bryan H Goldstein, Christopher J Petit, Yun Zhang, Courtney E McCracken, Athar M Qureshi, Caren S Goldberg, Jennifer C Romano, Mark A Law, Jeffery J Meadows, Shabana Shahanavaz, Sarosh P Batlivala, Shiraz A Maskatia, Asaad Beshish, Michael L O'Byrne, R Allen Ligon, Kathryn O Stack, Hala Q Khan, Shalin Parekh, Dawn L Ilardi","doi":"10.1007/s00246-024-03680-w","DOIUrl":"https://doi.org/10.1007/s00246-024-03680-w","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471999","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":"Using Quality Improvement to Optimize Blood Product Utilization in a Pediatric Cardiac Catheterization Laboratory.","authors":"Mira Trivedi, Shreya Sheth, Felicia Cooper, Justin Elhoff, Miranda A Rodrigues, Gary Stapleton","doi":"10.1007/s00246-024-03682-8","DOIUrl":"https://doi.org/10.1007/s00246-024-03682-8","url":null,"abstract":"<p><p>Packed red blood cells (PRBC) are frequently ordered for cardiac catheterization procedures, which increases resource utilization and patient charges. We applied the Plan-Do-Study-Act (PDSA) principle in order to optimize the ordering of PRBC for pediatric cardiac procedures and reducing charges. Our primary aim was to increase adherence to ordering guidelines to greater than 97%, with a global aim to reduce resource utilization. The existing PRBC ordering guidelines were revised and procedure reports were updated to include administration of PRBC. The rate of pre-procedure PRBC orders, adherence to the new protocol guidelines, presence of documentation and rate of blood transfusion within 24 h post-procedure, frequency of emergency release blood during a procedure were monitored. During our study period, there was an increased adherence from 86 to 100% adherence following implementation of the updated guidelines. With improved adherence, we decreased PRBC ordering and hospital charges to the patient in low-risk cardiac catheterization procedures, without an increase in blood transfusions.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472017","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":"The Influence of Arrhythmia on the Outcomes of Pediatric Patients with Idiopathic Dilated Cardiomyopathy.","authors":"Mohammad Dalili, Marieh Dastafshan, Mohammad Mahdavi, Hossein Shahzadi, Golnar Mortaz Hejri, Fariba Rashidighader, Pedro Brugada","doi":"10.1007/s00246-024-03684-6","DOIUrl":"https://doi.org/10.1007/s00246-024-03684-6","url":null,"abstract":"<p><p>Children diagnosed with dilated cardiomyopathy (DCM) are at an increased risk of developing arrhythmias, which can significantly affect their overall prognosis. However, the implantation and effectiveness of implantable cardioverter-defibrillators (ICDs) pose challenges in the pediatric population. Therefore, it is crucial to determine the prevalence of arrhythmia-related death in pediatric patients with DCM. This study investigated the correlation between arrhythmias and mortality in pediatric patients with DCM. This retrospective cohort study examined children with idiopathic DCM referred to Rajaie Cardiovascular, Medical, and Research Institute in Tehran, Iran, from 2017 to 2023. All patients underwent a standard 12-lead electrocardiogram, 24-h Holter monitoring, cardiac magnetic resonance imaging, and echocardiography. Patient outcomes, including death, heart transplantation, and survival, were evaluated within one year of Holter monitoring. A total of 178 children (79 males) with a mean age of 83.06 ± 56.41 months were included in the study. Overall, 72 deaths (40.4%) and 46 heart transplants (25.8%) were recorded and 60 patients (33.7%) survived. There was no significant association between patients' outcomes with ventricular arrhythmias, late gadolinium enhancement, corrected QT interval, and QRS interval prolongation; however, a significantly higher proportion of dead patients had abnormal heart rate variability (P = 0.034). Heart failure was the leading cause of death, with ventricular arrhythmia accounting for two out of 72 deaths. Both cases of ventricular arrhythmia were associated with severe cardiac dysfunction. Arrhythmia-related mortality is rare in children with idiopathic DCM, with severe heart failure being the primary cause of death in this population. Life-threatening arrhythmias primarily occur in DCM children with more compromised cardiac function.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472016","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}