Pediatric Cardiology最新文献

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Left Ventricular Functional Changes after Ross Procedure in Children and Adolescents. 儿童和青少年罗斯手术后左心室功能的改变。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2025-11-27 DOI: 10.1007/s00246-025-04124-9
Michal Schäfer, Aaron Eckhauser, Reilly Hobbs, Eric Griffiths, Vikas Sharma, Horacio Carvajal, Sanja Dzelebdzic, Edem Binka, S Adil Husain
{"title":"Left Ventricular Functional Changes after Ross Procedure in Children and Adolescents.","authors":"Michal Schäfer, Aaron Eckhauser, Reilly Hobbs, Eric Griffiths, Vikas Sharma, Horacio Carvajal, Sanja Dzelebdzic, Edem Binka, S Adil Husain","doi":"10.1007/s00246-025-04124-9","DOIUrl":"10.1007/s00246-025-04124-9","url":null,"abstract":"<p><p>The Ross procedure is a well-established method for aortic valve replacement (AVR) in pediatric and adolescent patients. Prior studies investigating morbidity outcomes have focused on autograft and homograft functionality, but no studies have investigated the effect of Ross AVR on early myocardial remodeling. This study investigates early global myocardial function using strain deformation indices in children and adolescents after the Ross AVR. Children and adolescents receiving the Ross procedure underwent pre-surgery, 3-6 month and 1-year follow-up echocardiography involving comprehensive myocardial strain analysis. Specifically, LV global circumferential strain (LV GCS) and LV global longitudinal strain (LV GLS), which is considered to be most susceptible to myocardial injury were assessed. Paired comparison at different stages were assessed along the with the predictive role of strain metrics to in-hospital outcomes. 58 patients who underwent Ross procedure (median age 12.5 years) underwent comprehensive echocardiographic analysis. At the 3-6 month post-operative period, there was a reduction in systolic function as demonstrated by significantly reduced LV GCS (P < 0.001) with no difference in LV GLS (P = 0.186) when compared to pre-operative function. At 1-year follow-up, the LV systolic function as assessed by LV GLS improved when compared to 3-6 month findings (P < 0.001) and trended toward improvement compared to the baseline (P = 0.124). The ratio of GLS / GCS was progressively increased post-operatively (3-6 month, P < 0.001) and (1-year, P < 0.001). Patients with worse pre-operative GLS (>-16%) had higher median hospital stay (median: 6 vs. 8 days, P = 0.007). Initially, after the Ross procedure, GCS is reduced without significant change in GLS. By one year, the function of the longitudinal sub-endocardial fibers is improved, and mid-wall circumferential fiber contractile function remains decreased. Overall, these findings are suggestive of possible beneficial LV remodeling as early as one year after the Ross procedure.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3374-3381"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145637195","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}
引用次数: 0
Physician Preparedness for Managing Juvenile Obesity in the MENA Region: A Cross-Sectional Analysis of Perceptions, Knowledge, Practices, and Barriers. 医生准备管理青少年肥胖在中东和北非地区:观念,知识,实践和障碍的横断面分析。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2025-11-28 DOI: 10.1007/s00246-025-04121-y
Ala Daoud, Eyad A Qunaibi, Shahem Raies, Ula Y Altorman, Ahmed Salim, Saad M Wali, Taliaa M Al-Yafeai, Tarek Alsaied
{"title":"Physician Preparedness for Managing Juvenile Obesity in the MENA Region: A Cross-Sectional Analysis of Perceptions, Knowledge, Practices, and Barriers.","authors":"Ala Daoud, Eyad A Qunaibi, Shahem Raies, Ula Y Altorman, Ahmed Salim, Saad M Wali, Taliaa M Al-Yafeai, Tarek Alsaied","doi":"10.1007/s00246-025-04121-y","DOIUrl":"10.1007/s00246-025-04121-y","url":null,"abstract":"<p><p>This study aimed to evaluate physicians' preparedness to manage juvenile obesity (JO) in the Middle East and North Africa (MENA) region by assessing their attitudes, knowledge, clinical practices, confidence, and perceived barriers. A 38-question cross-sectional survey was created, validated, and distributed in December 2024 across MENA countries using a skip-logic-tailored, bilingual (Arabic/English) online platform. It assessed physicians' attitudes, familiarity with JO-management guidelines and concepts, screening and treatment practices, prior JO-training, confidence, and barriers. Data were analyzed using both descriptive and inferential statistics. The study included 400 physicians (54% female, 57% younger than 35, 65% primary care physicians). 90% considered JO a routine checkup priority. Two-thirds reported no JO-training; half were unfamiliar with JO-management guidelines, and 45-65% were unfamiliar with JO-management concepts. Screening was suboptimal, with 25% relying exclusively on visual assessment. Behavioral therapy (33%) and pharmacologic treatments (17%) were underutilized. Most (73%) reported low to moderate confidence in managing JO. Confidence was significantly associated with guideline awareness and recent JO-training. Guideline awareness was also associated with recent JO-training. Physicians aware of guidelines were more likely to applying evidence-based practices (p < 0.001). Majoy selected barriers included lack of JO-training, institutional limitations, and socio-cultural factors. Physicians in MENA face deficits in JO-training, JO-management guidelines awareness, and systemic support that limit confidence in JO management. Expanding JO-training, disseminating regionally-suitable guidelines, and enhancing institutional support are essential to improving physician preparedness for JO management.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3342-3353"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145637374","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}
引用次数: 0
Pulmonary Atresia -Critical Stenosis with Intact Ventricular Septum: An International Survey. 肺动脉闭锁-完全性室间隔狭窄:一项国际调查。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2025-12-29 DOI: 10.1007/s00246-025-04136-5
Cecilia Villalaín, Anita Moon-Grady, Mats Mellander, Shiraz A Maskatia, Shabnam Peyvandi, Leticia Albert, Enery Gómez-Montes, Alberto Galindo
{"title":"Pulmonary Atresia -Critical Stenosis with Intact Ventricular Septum: An International Survey.","authors":"Cecilia Villalaín, Anita Moon-Grady, Mats Mellander, Shiraz A Maskatia, Shabnam Peyvandi, Leticia Albert, Enery Gómez-Montes, Alberto Galindo","doi":"10.1007/s00246-025-04136-5","DOIUrl":"10.1007/s00246-025-04136-5","url":null,"abstract":"<p><p>Pulmonary atresia/critical stenosis with intact ventricular septum is a heterogeneous disease in which perinatal management is not exempt from controversies. We aim to understand which areas are the ones with the highest and least consensus. An online questionnaire including 30 questions was administered through a REDCap link. Invitations were sent out through the Fetal Heart Society and personal emails to the heads of fetal cardiology programs. Results were analyzed in a descriptive manner and compared among the regions with most responses (North America and Europe). A total of 72 specialists in fetal and pediatric cardiology responded, most from North-America (n = 31) and Europe (n = 29). Antenatal counselling was mainly based on the tricuspid valve z-score (43.7%), although among European responders, multivariable models were preferred (44.8%). There was a wide regional variation in the nomograms used for prenatal assessment of the tricuspid valve. More than half did not consider prenatal valvuloplasty (54.3%), and those who did diverged in selection criteria. Postnatally, in mild-moderately hypoplastic right ventricle, most opted to first assess the tricuspid valve z-score (60%) whereas others would always attempt a biventricular pathway (30.8%). The majority opted to perform cardiac catheterization in all cases to assess ventriculo-coronary connections (60.6%). If right ventricle-dependent coronary circulation was confirmed, 66.7% would opt for single ventricle palliation, whereas 15.9% would directly list for transplant. There is great heterogeneity in the management of pulmonary atresia-critical stenosis with intact ventricular septum, particularly in prenatal assessment and consideration for prenatal intervention.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3410-3417"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145850247","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}
引用次数: 0
The Influence of Pre-operative Mitral Regurgitation in Post Operative Recovery of Ventricular Function in Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA). 术前二尖瓣反流对左冠状动脉离肺动脉异常(ALCAPA)患者术后心室功能恢复的影响
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2026-01-19 DOI: 10.1007/s00246-026-04160-z
Zarin Rangwala, Deepa Sasikumar, Sowmya Ramanan, Baiju S Dharan
{"title":"The Influence of Pre-operative Mitral Regurgitation in Post Operative Recovery of Ventricular Function in Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA).","authors":"Zarin Rangwala, Deepa Sasikumar, Sowmya Ramanan, Baiju S Dharan","doi":"10.1007/s00246-026-04160-z","DOIUrl":"10.1007/s00246-026-04160-z","url":null,"abstract":"<p><p>We attempted to study the influence of pre-operative mitral regurgitation on the recovery of ventricular function in paediatric patients with repaired anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Eighty-eight patients under 18 years who underwent ALCAPA repair from 2003 to 2022 were included. Baseline clinical and echocardiographic data, including MR and left ventricular ejection fraction (LVEF), were recorded pre-operatively and at multiple post-operative follow-ups (discharge, 1 month, 6 months, 1 year, and annually thereafter). Friedman's Test and correlation/regression analyses were used to assess the impact of pre-operative MR on ventricular function recovery. The median age at surgery was 6 months(range 1-197), and the median hospital stay was 17 days. 56.81% of patients had moderate to severe MR pre-operatively, while only 25% had moderate to severe MR at 1-year follow-up. Mitral valve repair was performed in 44% of cases with significant MR. Pre-operative MR grades positively correlated with post-operative MR (ρ = 0.574, p < 0.001) and pre-operative LVEF correlated with post-operative LVEF (ρ = 0.698, p < 0.001). Linear regression analysis showed that patients with lesser MR had more change in LVEF (38 to 63.66%) than the moderate to severe MR group (42.22% to 66.38%, odd's ratio=-3.073, p = 0.024). Irrespective of severity of pre-operative MR, left ventricular function improved significantly after the surgical repair. Patients with pre-operative mild MR tended to experience more pronounced gains in LVEF following repair.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3561-3568"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145998749","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}
引用次数: 0
Pediatric Cardiology Echocardiography Curriculum and Workshop for Pediatric Residents in Mwanza, Tanzania-Using Deliberate Practice for Skill Acquisition. 小儿心脏病超声心动图课程和工作坊为儿科住院医师在姆万扎,坦桑尼亚-使用刻意练习技能习得。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2026-01-09 DOI: 10.1007/s00246-025-04140-9
Shivani Govind Patel, Jennifer Acevedo, Angira Patel, Colleen Fant, Theophylly Mushi, Julieth Kabrigi, Tulla Masoza
{"title":"Pediatric Cardiology Echocardiography Curriculum and Workshop for Pediatric Residents in Mwanza, Tanzania-Using Deliberate Practice for Skill Acquisition.","authors":"Shivani Govind Patel, Jennifer Acevedo, Angira Patel, Colleen Fant, Theophylly Mushi, Julieth Kabrigi, Tulla Masoza","doi":"10.1007/s00246-025-04140-9","DOIUrl":"10.1007/s00246-025-04140-9","url":null,"abstract":"<p><p>Bugando Medical Center (BMC) in Mwanza, Tanzania, with two hundred pediatric beds, has a large burden of pediatric heart disease. The general pediatric residents are front-line cardiology providers. At the time of this study, there was a single pediatrician with echocardiography training, performing all echocardiograms and providing trainee education, teaching residents on-the-job with limited access to continued education. Our aim was to build pediatric residents' cardiac ultrasound capacity at BMC through asynchronous learning and a hands-on workshop. The target learner group was BMC pediatric residents participating in a pediatric echocardiographic workshop. Baseline data was collected through a comfort survey, knowledge test, and skills test in acquisition/interpretation of echocardiographic images. An in-person, five-day workshop was conducted for acquisition of basic 2-dimensional images and recognizing abnormal images. At the end of the workshop, learners completed a knowledge post-test, an assessment of normal vs. abnormal images, and a skills test to determine ability to obtain standard images. 29 pediatric residents (15 PGY-2, 13 PGY-3, 1 registrar) participated in the workshop. The percentage of trainees who felt comfortable using an echocardiography machine increased from 60% to 96%. Knowledge test scores increased from 58% (n = 28) to 84% (n = 25) after intervention (p < 0.001). Skills assessment exhibited a significant improvement, from 10% to 83% of trainees' ability to acquire images from all five imaging planes. Trainees were able to identify abnormal echocardiography images with an average score of 16 ± 3/20. An intense five-day echocardiography workshop significantly increased knowledge and skill in performing and interpreting basic pediatric echocardiograms for novice trainees and served as an excellent starting point for longitudinal future pediatric echocardiography training.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3432-3439"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13687231/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145934638","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}
引用次数: 0
Predictors of Superior Exercise Performance in Patients Following Fontan Palliation. Fontan姑息治疗后患者卓越运动表现的预测因素。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2025-12-06 DOI: 10.1007/s00246-025-04129-4
Jason Bobbak Meschin, Alicia Mai, Elizabeth Wang-Giuffre
{"title":"Predictors of Superior Exercise Performance in Patients Following Fontan Palliation.","authors":"Jason Bobbak Meschin, Alicia Mai, Elizabeth Wang-Giuffre","doi":"10.1007/s00246-025-04129-4","DOIUrl":"10.1007/s00246-025-04129-4","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3392-3399"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145687808","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}
引用次数: 0
Cardiac Magnetic Resonance Imaging in Pediatric Heart Transplant Recipients: Correlation with Cardiopulmonary Exercise Testing. 儿童心脏移植受者的心脏磁共振成像:与心肺运动试验的相关性。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2025-11-22 DOI: 10.1007/s00246-025-04110-1
Sarah Tucker, Alan Wang, Garett Griffith, Kendra Ward, Lajja Desai, Katheryn Gambetta, Nazia Husain
{"title":"Cardiac Magnetic Resonance Imaging in Pediatric Heart Transplant Recipients: Correlation with Cardiopulmonary Exercise Testing.","authors":"Sarah Tucker, Alan Wang, Garett Griffith, Kendra Ward, Lajja Desai, Katheryn Gambetta, Nazia Husain","doi":"10.1007/s00246-025-04110-1","DOIUrl":"10.1007/s00246-025-04110-1","url":null,"abstract":"<p><p>Multiparametric cardiac magnetic resonance (CMR) is increasingly used for rejection and coronary artery vasculopathy (CAV) surveillance in pediatric heart transplant recipients (PHTR). There is limited data regarding how graft assessment by multiparametric CMR may reflect functional capacity in PHTR. To explore the relationship between multiparametric CMR and markers of exercise capacity in PHTR. PHTR who underwent CMR within 1 year of cardiopulmonary exercise testing (CPET) were retrospectively reviewed. Those with submaximal effort on CPET (respiratory exchange ratio < 1.10), depressed function (left ventricular ejection fraction (LVEF) < 50% and/or right ventricular ejection fraction (RVEF) < 45%), or significant clinical events (rejection, new or worsening CAV, cardiac hospitalizations) between CMR and CPET were excluded. CMR variables included biventricular volumes, ejection fraction, cardiac index (CI), myocardial T2, T1/extracellular volume fraction (ECV), and myocardial perfusion reserve index (MPRI). CPET variables were VO<sub>2peak</sub>, O<sub>2</sub> pulse, percent age-predicted maximum heart rate (APMHR), HR reserve, and exercise duration. Relationships between variables were studied using correlations and regression. Forty-seven PHTR were included. Time between CPET and CMR was 5.5 ± 3.4 months. CI correlated positively with O<sub>2</sub> pulse (R = 0.642, p < 0.001). Global T1 correlated negatively with both APMHR (R = - 0.538, p < 0.001) and HR reserve (R = - 0.598, p < 0.001). Global T2 correlated negatively with APMHR (R = - 0.335, p = 0.049), HR reserve (R = - 0.488, p = 0.003), and VO<sub>2peak</sub> (R = - 0.327, p = 0.045). In PHTR with normal LV function, CMR-derived tissue characteristics have correlations with exercise capacity. Larger studies are needed to understand the role of multiparametric CMR in the functional surveillance of PHTR.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3287-3294"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145582327","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}
引用次数: 0
Response to: Comment on "Prediction of Symptoms and Evaluation of Surgical Indications After Birth Based on Tracheal Morphology of Double Aortic Arch". 对《基于双主动脉弓气管形态的出生后症状预测及手术指征评价》的评论。
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2026-03-10 DOI: 10.1007/s00246-026-04222-2
Takeshi Ikegawa, Akio Kato, Motoyoshi Kawataki, Yoshinori Inagaki, Katsuaki Toyoshima, Hideaki Ueda
{"title":"Response to: Comment on \"Prediction of Symptoms and Evaluation of Surgical Indications After Birth Based on Tracheal Morphology of Double Aortic Arch\".","authors":"Takeshi Ikegawa, Akio Kato, Motoyoshi Kawataki, Yoshinori Inagaki, Katsuaki Toyoshima, Hideaki Ueda","doi":"10.1007/s00246-026-04222-2","DOIUrl":"10.1007/s00246-026-04222-2","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3613-3614"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147434627","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}
引用次数: 0
Survival by Heart Failure Phenotype in a Fontan Surveillance Population, 2010-2023. 2010-2023年Fontan监测人群心衰表型生存率
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-12-01 Epub Date: 2026-01-08 DOI: 10.1007/s00246-025-04145-4
Lindsey C Ivey, Cheryl L Raskind-Hood, Fred H Rodriguez, Giancarlo Licitra, Ryan O Sheets, Mollie R Westrick, Yuting Guo, Abeed Sarker, Wendy M Book
{"title":"Survival by Heart Failure Phenotype in a Fontan Surveillance Population, 2010-2023.","authors":"Lindsey C Ivey, Cheryl L Raskind-Hood, Fred H Rodriguez, Giancarlo Licitra, Ryan O Sheets, Mollie R Westrick, Yuting Guo, Abeed Sarker, Wendy M Book","doi":"10.1007/s00246-025-04145-4","DOIUrl":"10.1007/s00246-025-04145-4","url":null,"abstract":"<p><strong>Background: </strong>The Fontan operation palliates single-ventricle defects but is associated with late morbidity, heart failure (HF), and premature mortality. Long-term survival among Fontan patients by HF phenotype and transplant status are evaluated.</p><p><strong>Methods: </strong>We identified 722 Fontan patients (median age 10.2 years at first encounter; 57.3% male; 41.1% Black) with healthcare encounters between 2010 and 2023. The cohort was linked to death certificates. HF was categorized by ICD codes as systolic (SHF) or non-systolic (NSHF). Transplant evaluation and procedures were validated by chart review. The first HF-coded encounter, transplant evaluation and transplant date were recorded. Survival was analyzed using Kaplan-Meier methods.</p><p><strong>Results: </strong>Patients without HF were younger [median 6.3 years (5.0-56.0)] than those with NSHF [21.1 years (5.0-44.7)], SHF [16.1 years (5.0-53.0)], transplant [17.3 years (5.3-47.3)], or evaluation without transplant [18.0 years (5.4-50.2)]. Fifty-seven patients died (7.9%, median age 24.6 years). Mortality occurred in 2.8% (no HF), 6.4% (NSHF), 14.0% (SHF), 28.6% (evaluated, not transplanted), and 30.6% (transplanted; p < 0.001). Post-transplant 1-, 5-, and 10-year survival was 91.7%, 74.4%, and 61.8%, compared to 96.2%, 91.9%, and 77.2% for SHF. NSHF and no HF groups had similar 5-year survival (97.3%). Among 28 patients evaluated but not transplanted, 1-year mortality was 26.6%.</p><p><strong>Conclusion: </strong>The SHF Fontan phenotype was associated with significantly worse survival than Fontan patients without SHF, who have favorable long-term outcomes. Although transplant offers potential benefit, long-term post-transplant survival remains limited. Improved risk stratification to guide optimal transplant timing is essential for this high-risk population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"3459-3466"},"PeriodicalIF":1.3,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145934691","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}
引用次数: 0
Clinical Effects of Sodium-Glucose Cotransporter 2 Inhibitors on Children with Single Ventricle Congenital Heart Disease: A Single-Center Experience. 钠-葡萄糖共转运蛋白2抑制剂治疗儿童单心室先天性心脏病的临床效果:一项单中心研究
IF 1.3 4区 医学
Pediatric Cardiology Pub Date : 2026-10-27 DOI: 10.1007/s00246-026-04532-5
Alexander O Canova, David M Newland, Erin L Albers, Erin S Bouska, Christina L Hartje-Dunn, Mehreen S Iqbal, Mariska S Kemna, Yuk M Law, Sarah M Kerr, Bethany L Wisotzkey, Borah J Hong
{"title":"Clinical Effects of Sodium-Glucose Cotransporter 2 Inhibitors on Children with Single Ventricle Congenital Heart Disease: A Single-Center Experience.","authors":"Alexander O Canova, David M Newland, Erin L Albers, Erin S Bouska, Christina L Hartje-Dunn, Mehreen S Iqbal, Mariska S Kemna, Yuk M Law, Sarah M Kerr, Bethany L Wisotzkey, Borah J Hong","doi":"10.1007/s00246-026-04532-5","DOIUrl":"https://doi.org/10.1007/s00246-026-04532-5","url":null,"abstract":"<p><p>This study evaluates the impact of sodium-glucose cotransporter 2 inhibitors (SGLT2i) on pediatric patients with single ventricle (SV) heart disease, using invasive and non-invasive markers of cardiac function. We reviewed patients (< 21 years) with SV physiology who received ≥ 90 days SGLT2i at our center from 2020 to 2025. Baseline laboratory, echocardiographic, and hemodynamic data were recorded and compared to follow up data after ≥ 90 days on SGLT2i. Hospitalizations before and after SGLT2i were also analyzed. Seventy-seven patients with median age 10.5 years (range 47 days-19.9 years) had median treatment duration of 386 days (IQR 218-853). Among 62 patients (81%) with echocardiographic data (Fontan [39], Glenn [18], Stage 1 [5]) there was a significant decrease in median qualitative AVVR score from mild-moderate to mild (p = 0.01). Median BNP decreased from 84 pg/mL (IQR 18-274) to 42 pg/mL (IQR 18-105) (p = 0.02) and mean hemoglobin increased from 14.4 ± 2.5 g/dL to 15.5 ± 2.4 g/dL (p < 0.01). Ten patients had hemodynamic data without significant change in mean pulmonary artery pressure from 17 to 16 mmHg (p = 0.168) or ventricular end-diastolic pressure from 10 to 11 mmHg (p = 0.275). More patients had > 1 hospitalizations for heart failure during the 6-months prior to SGLT2i compared to the 6-months following initiation, with an odds ratio of 4.5 (95% CI: 1.6-13.7, p < 0.01). Overall, we identified significant reductions in AVVR, BNP, and hospitalizations for heart failure after ≥ 90 days of therapy. Further larger scale studies are needed to determine if SGLT2i will affect clinical outcomes in the pediatric SV population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2026-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149288186","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}
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