Pediatric Cardiology最新文献

{"title":"Can Real-Time Three-Dimensional (Four-Dimensional) and Speckle-Tracking Echocardiography Predict Cardiac Involvement Detected by Magnetic Resonance Imaging in Children with Duchenne Muscular Dystrophy?","authors":"Ayşe Güler Eroğlu, Enes Kaynak, Nujin Uluğ Murt, Emine Şebnem Durmaz, Seda Sönmez Kurukaya, İbrahim Adaletli, Ayşe Sultan Kaya, Elif Yüksel Karatoprak, Sema Saltık","doi":"10.1007/s00246-026-04164-9","DOIUrl":"10.1007/s00246-026-04164-9","url":null,"abstract":"<p><p>This study aimed to evaluate usefulness of real-time three-dimensional (four-dimensional) and three-dimensional speckle-tracking echocardiography in detecting cardiac involvement identified by cardiac magnetic resonance imaging in children with Duchenne muscular dystrophy. This cross-sectional study included 18 boys with a median age of 11.9 years (10.5-13.2 years). Left ventricular ejection fraction, global and regional myocardial strains were assessed using real-time three-dimensional echocardiography and three-dimensional speckle-tracking echocardiography as well as magnetic resonance imaging. Late gadolinium enhancement was evaluated by cardiac magnetic resonance imaging. Moderately positive correlations were found for ejection fraction (r = 0.669, p = 0.003), global longitudinal strain (r = 0.618, p = 0.008), global circumferential strain (r = 0.513, p = 0.035), and global radial strain (r = 0.605, p = 0.01) measured by three-dimensional speckle-tracking echocardiography and cardiac magnetic resonance imaging. In addition, longitudinal and circumferential strain values measured by three-dimensional speckle-tracking echocardiography showed correlations with cardiac magnetic resonance imaging in the same segment and/or adjacent segments in 13 of 16 regions , with correlation coefficients between 0.487 and 0.776 (p < 0.05). Radial strain values demostrated correlations in 25 regions, with correlation coefficients between 0.487 and 0.690 (p < 0.05). Real-time three-dimensional echocardiography and three-dimensional speckle-tracking echocardiography provided valuable information about left ventricular function as well as global and regional myocardial strain compared with magnetic resonance imaging in our patients with Duchenne muscular dystrophy. Further studies including larger patient populations and a broader range of disease severity are needed to confirm these findings.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3581-3588"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146041113","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 and Tolerability of Selexipag in Pediatric Patients with Pulmonary Arterial Hypertension: An Updated Systematic Review and Meta-analysis.","authors":"Vasiliki Patsiou, Triantafyllia Grantza, Thomas Chrysochoidis-Trantas, Maria Kavga, Nikolaos Fragakis, Antonios Ziakas, Alexandra Arvanitaki, George Giannakoulas","doi":"10.1007/s00246-025-04094-y","DOIUrl":"10.1007/s00246-025-04094-y","url":null,"abstract":"<p><p>Evidence supporting selexipag in pediatric pulmonary arterial hypertension (PAH) is sparse, mainly from small, heterogeneous studies. This meta-analysis assessed its efficacy and tolerability in this population. A systematic search of PubMed, Embase, Web of Science, Cochrane Library, and Scopus was conducted until April 2025. Eligible studies included patients under 18 years diagnosed with PAH and treated with selexipag, reporting clinical outcomes. Two reviewers independently screened, extracted data, and evaluated study quality. Pooled analyses of efficacy and safety outcomes were performed, with subgroup comparisons for add-on therapy versus transition from parenteral prostacyclin analogs. Twelve studies involving 344 pediatric patients (90.6% diagnosed with PAH) were included. Selexipag significantly improved 6-minute walk distance (SMD: - 0.75; 95% CI: - 1.22 to - 0.29; P = 0.002), but did not affect pulmonary vascular resistance index, mean pulmonary artery pressure (mPAP), or NT-proBNP. Sensitivity analysis revealed reduced mPAP in PAH-only studies (SMD: - 0.30; P = 0.03). Adverse events occurred in 62.8%, predominantly mild. Functional improvement was greater in the add-on therapy group, which also experienced more adverse events. Selexipag improved functional capacity and mPAP in pediatric PAH and was generally well tolerated. Further prospective studies are needed to confirm long-term benefits.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3233-3243"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145541613","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":"Coronary Anomalies and Early Surgical Outcomes in Transposition of the Great Arteries and Taussig-Bing Anomaly: Insights from 251 Consecutive Arterial Switch Cases.","authors":"Ensar Duras, Samet Paksoy, Okan Yildiz, Hacer Kamali, Aslihan Karaman, Ismihan Selen Onan, Alper Guzeltas, Sertac Haydin","doi":"10.1007/s00246-025-04119-6","DOIUrl":"10.1007/s00246-025-04119-6","url":null,"abstract":"<p><p>Coronary artery anomalies (CAAs) are a critical factor in the arterial switch operation (ASO), yet their prognostic significance remains a matter of debate. This study aimed to evaluate the impact of CAAs on early outcomes, including mortality and postoperative recovery, in patients with transposition of the great arteries (TGA) and Taussig-Bing anomaly (TBA). We retrospectively analyzed 251 consecutive patients who underwent an ASO between 2010 and 2022. Coronary anatomy was classified intraoperatively according to the Leiden convention: Group I (usual, n = 181) and Group II (anomalous, n = 70). Preoperative, operative, and postoperative variables were compared. The primary outcome was early mortality (≤ 30 days or during hospitalization), and secondary outcomes included intensive care unit (ICU) and hospital stay, need for extracorporeal membrane oxygenation (ECMO), and major complications. Statistical analysis involved the Mann-Whitney U and Chi-squared tests; logistic regression was used to identify predictors of mortality. Coronary anomalies were identified in 70 patients (27.9%). Group II had higher median age (17.5 vs. 8 days, p = 0.003) and more associated anomalies (17.1% vs. 6.1%, p = 0.008), but mortality (11.4% vs. 8.3%, p = 0.43), ICU stay (11.5 vs. 11 days, p = 0.90), and hospital stay (21.5 vs. 20 days, p = 0.94) were comparable. In multivariable analysis, ECMO requirement (OR 31.0; 95% CI 10.0-95.9, p < 0.01) and prolonged ICU stay (OR 0.94; 95% CI 0.90-0.99, p = 0.04) were independent predictors of mortality, while CAAs were not. These findings suggest that coronary anatomy does not independently affect early outcomes after ASO, although complex variants may still increase technical difficulty and require individualized surgical strategies.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3323-3331"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582434","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":"Child Opportunity, Race, and Language in Congenital Cardiac Surgery Outcomes.","authors":"Kenneth M Coca, Jamie L Fierstein, Joseph Manipadam, Vera Ignjatovic, Joyce Johnson, Michael Puchalski, John M Morrison, Benton Ng","doi":"10.1007/s00246-026-04159-6","DOIUrl":"10.1007/s00246-026-04159-6","url":null,"abstract":"<p><p>Racial and ethnic disparities and language barriers coexist with inequities in a child's educational, environmental, and economic opportunity. We evaluated the association between surgical outcomes and a composite child neighborhood opportunity index (COI), race and ethnicity, and language barriers among children undergoing congenital heart surgery. Utilizing the Society of Thoracic Surgeons database, we conducted a single-center retrospective cohort study of patients who underwent congenital cardiac surgery from 2010 to 2023. Patients were classified by quintile COI scores based on their reported address. Outcomes included in-hospital mortality, length of stay (LOS), and major complications. Adjusted analyses were performed using generalized linear mixed models. Among 1,568 patients, 51.2% were non-Hispanic White and 64.1% had public insurance. 57.4% lived in very low or low COI neighborhoods. Race, ethnicity, primary caregiver language, and insurance payer were significantly associated with COI (p < 0.0001). After adjustment, COI was not associated with mortality, LOS, or complications. Compared with non-Hispanic White patients, non-Hispanic Black patients had higher odds of in-hospital mortality (OR 2.19, 95% CI 1.07-5.30) and longer LOS (β = 0.25, 95% CI 0.09-0.42). Patients with non-English-speaking caregivers had longer LOS (β = 0.30, 95% CI 0.10-0.51). Neighborhood opportunity was not independently associated with surgical outcomes. However, disparities by race and ethnicity and language persisted, suggesting that COI may not fully capture structural racism or communication barriers in pediatric cardiac care.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3551-3560"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019144","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":"Cor Triatriatum Dexter: Embryology, Presentation and Management.","authors":"Sarah Kerr, Amy Hong, Aarti Bhat","doi":"10.1007/s00246-025-04147-2","DOIUrl":"10.1007/s00246-025-04147-2","url":null,"abstract":"<p><strong>Background: </strong>Cor triatriatum dexter (CTD) is a rare congenital cardiac anomaly resulting from persistence of the right valve of the sinus venosus. Clinical presentation varies from neonatal hypoxemia to incidental diagnosis in adulthood. Optimal management strategies remain poorly defined due to limited published literature.</p><p><strong>Methods: </strong>We report a single-center series of four patients diagnosed with CTD. Clinical presentation, echocardiographic findings, surgical management, and postoperative outcomes were retrospectively reviewed.</p><p><strong>Results: </strong>All four patients presented in the neonatal period with hypoxemia and failed critical congenital heart disease (CCHD) pulse oximetry screening, ultimately resulting in echocardiographic diagnosis of a redundant membrane within the right atrium consistent with CTD. All four patients underwent surgical repair at an average of 21 days-of-life (5-41 DOL) and 4 kg (3.4-4.5 kg). Mean cardiopulmonary bypass time was 33 minutes (14-44 min). Mean postoperative length of stay was 6 days (3-9 days). Follow-up was available in 3 patients, all of whom were doing well with no cardiac symptoms and on no cardiac medications up to 7 years postoperatively.</p><p><strong>Conclusions: </strong>CTD repair can be performed with a relatively short cardiopulmonary bypass time, limited postoperative hospital stay, and no cardiac concerns at medium term follow-up. We advocate for an individualized approach when deciding surgical intervention vs. watchful waiting but generally err on the side of early surgical repair.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3593-3599"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145998721","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":"Transapical Mitral Valve-in-Valve Implantation in Less than 10 kg Children.","authors":"Lisa Bianco, Celine Grunenwald, Guido Michielon, Mohamed Ly, Alban-Elouen Baruteau, Alain Fraisse","doi":"10.1007/s00246-025-04142-7","DOIUrl":"10.1007/s00246-025-04142-7","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3589-3592"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145857459","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":"Tricuspid Regurgitation and Impact of Surgical Valve Intervention in the Single Ventricle Reconstruction Trial.","authors":"J F Cnota, S M Chowdhury, A Floh, R Gongwer, B H Goot, J P Jacobs, M A Jolley, S Kirmani, D J LaPar, J C Levine, A B Lewis, R H Pignatelli, C Pizarro, T C Slesnick, T Thorsson, F Trachtenberg, D T Truong, J W Newburger, P C Frommelt","doi":"10.1007/s00246-025-04122-x","DOIUrl":"10.1007/s00246-025-04122-x","url":null,"abstract":"<p><p>Tricuspid regurgitation (TR) is common in single right ventricle (RV) anomalies and affects outcomes during staged surgical palliations. The Pediatric Heart Network's (PHN) Single Ventricle Reconstruction trial followed children with single RV to age 6 years. This study compared serial echocardiographic measures of TR during staged palliations and determine the impact of TR and tricuspid valve (TV) intervention on transplant-free survival in the PHN cohort. Study echocardiograms were performed at 6 pre-determined times: baseline, post-Norwood, pre-stage II, age 14 months, pre-Fontan, and age 6 years. TR was categorized as ≤ mild or > mild. McNemar's test compared TR across time; t-tests compared RV size/function by TR category; and survival analysis assessed the effect of > mild TR on transplant-free survival. TR was assessed in 543 participants prior to Norwood. From baseline to post-Norwood, those with > mild TR increased from 12 to 24% (p < 0.001). Thereafter, the proportion of surviving participants with > mild TR was stable. Participants with > mild TR had increased baseline TV annular area, and increased RV volume but not decreased function. Transplant-free survival at 6 years was lower with > mild TR at any time except pre-Fontan. 78 TV interventions occurred in 66 participants. Those with TV intervention at the Norwood and/or Stage II had a higher rate of death/transplant than those without or those who had a later intervention. At baseline, post Norwood and pre-stage II, > mild TR is associated with decreased transplant-free survival. TV intervention at the Norwood or Stage II operations was not associated with improved survival.Clinical Trial Registration: NCT00115934.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3354-3361"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13687309/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146220767","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":"Nicorandil Attenuates Global Myocardial Ischemia-Reperfusion Injury and Systemic Inflammation Through Inhibition of TLR-4 Signaling in a Neonatal Piglet Model of Cardiopulmonary Bypass and Cardioplegic Arrest.","authors":"Yun-Wen Peng, Alvaro Rojas-Pena, Bailey Schneider, John Toomasian, Lise Tchouta, Matthew Johnson, Hayes McKenzie, John R Charpie","doi":"10.1007/s00246-025-04125-8","DOIUrl":"10.1007/s00246-025-04125-8","url":null,"abstract":"<p><p>Pediatric cardiac surgery with cardiopulmonary bypass (CPB) and cardioplegic arrest is associated with global myocardial ischemia-reperfusion (IR) injury, leukocyte activation, and a systemic inflammatory response that contributes to early postoperative morbidity and mortality. The mechanisms for acute myocardial dysfunction and end-organ injury after pediatric heart surgery are complex and incompletely understood. This study aimed to: (1) investigate the contributions of the toll-like receptor-4 (TLR-4) pro-inflammatory and the pro-survival hypoxia-inducible factor-1α/phosphatidylinositol 3-kinase (HIF-1α/PI3K) pathways to global myocardial IR injury and inflammation, and (2) evaluate the impact of nicorandil, a coronary vasodilator and anti-ischemic agent, on TLR-4 and HIF-1α/PI3K signaling and end-organ dysfunction in a neonatal piglet model of CPB and cardioplegic arrest. Piglets (4-5 weeks old, N = 12) were randomly assigned to IR or IR + nicorandil. After initiation of CPB with cardioplegic arrest, hearts were reperfused on partial CPB, followed by complete separation from CPB. Left ventricular (LV) systolic and diastolic pressures were continuously recorded, and serial blood and terminal tissue samples were collected to measure inflammation, oxidant stress (OS), and apoptosis. CPB with cardioplegic arrest transiently and significantly impaired both LV contractility (p = 0.01) and diastolic relaxation (p = 0.001) associated with a 39-fold increase in cardiac TLR-4 expression (p = 0.034), a 3-fold increase in IL-6 production (p = 0.01), and significant increases in plasma markers for end-organ injury, but without the expected upregulation in the HIF-1α/PI3K pro-survival pathway. Nicorandil pre-treatment significantly augmented LV systolic (p = 0.004) and diastolic (p = 0.002) functional recovery associated with attenuated upregulation of TLR-4 signaling pathways, augmented HIF-1α/PI3K pro-survival signaling, and reduced inflammation, OS, apoptosis, and end-organ injury. Our piglet model of CPB with cardioplegic arrest demonstrates that the TLR-4 pro-inflammatory signaling pathway plays an essential role in myocardial IR-induced cardiac dysfunction, inflammation, OS, apoptosis, and multi-organ injury. Nicorandil pre-treatment attenuates these effects. Nicorandil also upregulates the HIF-1α/PI3K pro-survival signaling pathway. These results suggest that nicorandil should be studied further to assess its potential therapeutic effects after CPB in children.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3382-3391"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145637231","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":"Prevalence of Congenital Aortic Valve Regurgitation in a Newborn Cohort.","authors":"Aysha F Farooqui, Jakob B Norsk, Anna A Raja, Vibeke Hjortdal, Julian K Bjerrekaer, Marie Reindahl Fagerlund, R Ottilia Vøgg, Christian Pihl, Henning Bundgaard, Kasper Iversen, Anne-Sophie Sillesen","doi":"10.1007/s00246-025-04149-0","DOIUrl":"10.1007/s00246-025-04149-0","url":null,"abstract":"<p><p>Congenital aortic valve regurgitation is seen in approximately 1 in 4,000 live-births globally. This study investigated the prevalence and early impacts of aortic regurgitation in a newborn cohort. Between April 2016 and October 2018, all newborns in the Copenhagen Area were offered systematic transthoracic echocardiography in a prospective, multicenter population-based study. Aortic valve function was categorized as no, trivial (glimpse of regurgitant flow and/or vena contracta < 0.1 cm), or non-trivial aortic regurgitation (vena contracta ≥ 0.1 cm). Newborns with non-trivial regurgitation were matched 1:4 with controls (no regurgitation). Among 25,590 newborns, aortic regurgitation was detected in 329 newborns (52% male, median age 11 days), i.e. a prevalence of 1.3% (95% CI, 1.15%-1.43%), with 260 (79%) classified as trivial and 69 (21%) as non-trivial. Bicuspid aortic valve was more common in newborns with non-trivial regurgitation (13.0%) than controls (1.1%) (p < 0.001). Newborns with non-trivial regurgitation had larger aortic dimensions compared to controls (mean ± SD): aortic valve annulus (7.3 ± 0.7 mm vs. 7.0 ± 0.6 mm), Sinuses of Valsalva (10.3 ± 1.0 mm vs. 9.7 ± 1.0 mm), sino-tubular junction (8.4 ± 0.8 mm vs. 8.0 ± 0.8 mm), and ascending aorta diameter (10.0 ± 0.9 mm vs. 9.4 ± 1.1 mm) (all p < 0.006). Left ventricular dimensions and function did not differ between groups. Congenital aortic regurgitation was present in 1.3% of all newborns. Non-trivial regurgitation (0.3%) was associated with larger aortic dimensions and higher prevalence of bicuspid aortic valve (13%), but no early signs of left ventricular impairment. Follow-up will help identify those at risk of progression, potentially due to valvular and/or aortic pathology, who may benefit from regular surveillance, and those who can be safely discontinued.Trial registration ClinicalTrials.gov NCT02753348 (registered April 27th 2016).</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3484-3491"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13687415/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146003850","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":"Epicardial Fat is Associated with Ventricular Dilation in Patients After the Fontan Operation.","authors":"Meghan D Ryan, Nicole St Clair, Christine K Lee, Rahul H Rathod","doi":"10.1007/s00246-025-04148-1","DOIUrl":"10.1007/s00246-025-04148-1","url":null,"abstract":"<p><p>Epicardial and hepatic fat are suspected to play a role in cardiac remodeling. The primary objective of this study was to identify the cardiac MRI (CMR) parameters that are associated with indexed epicardial fat mass (EFM_i) and hepatic steatosis in patients after the Fontan operation. This was a single-center, retrospective analysis of Fontan patients. Epicardial and subcutaneous fat were analyzed with CMR post-processing software, cvi42 (Circle Cardiovascular Imaging, Calgary, Alberta, Canada). Hepatic steatosis was measured with controlled attenuation parameter (CAP) scores via vibration controlled transient elastography. The cohort included 82 patients (65% male, median age 16 years). On univariate analysis, EFM_i correlated with BMI (ρ = 0.52, p < 0.001), age (ρ = 0.48, p < 0.001), ESV_i (ρ = 0.44, p < 0.001), EDV_i (ρ = 0.42, p < 0.001), ventricular mass_i (ρ = 0.41, p < 0.01), subcutaneous fat thickness (ρ = 0.32, p < 0.01), ejection fraction (ρ=-0.37, p < 0.001), and inferior vena cava (IVC) flow_i (ρ=-0.26, p < 0.05). On multivariable regression analysis, BMI (β 2.21, p < 0.001) and EDV_i (β 0.33, p < 0.001) were independently associated with EFM_i (R² = 0.51). On univariate analysis, CAP scores correlated with BMI (ρ = 0.54, p < 0.001), subcutaneous fat thickness (ρ = 0.49, p < 0.001), subcutaneous fat mass_i (ρ = 0.44, p < 0.001), age (ρ = 0.36, p < 0.01), EFM_i (ρ = 0.36, p < 0.001), and ventricular mass_i (ρ = 0.24, p < 0.05). On linear regression analysis, BMI (β 6.6, p < 0.001) was independently associated with CAP scores (R² = 0.27). Neither EFM_i nor CAP was significantly associated with adverse clinical events. BMI is strongly associated with epicardial and hepatic fat distribution. Epicardial fat mass is also associated with ventricular dilation.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3476-3483"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13687122/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145952623","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}