Pediatric Cardiology最新文献

{"title":"Self-expandable Venus P-valve in Patient with Transposition of Great Arteries and Arterial Switch Operation.","authors":"Stéphanie Douchin, Helene Bouvaist, Sebastien Hascoet, Phuoc Duong, Alain Fraisse","doi":"10.1007/s00246-025-04036-8","DOIUrl":"https://doi.org/10.1007/s00246-025-04036-8","url":null,"abstract":"<p><p>We report the successfull transcatheter pulmonary implantation of a self-expandable Venus P-valve in a patient with transposition of the great arteries, status post arterial switch operation in infancy. She had free pulmonary regurgitation, severely dilated right ventricle, and was in NYHA class II. After confirmation with the sizing balloon of the distensibility of the \"choke point\" at the main pulmonary anastomosis, the valve was implanted and became wedged, without compromising the final result. Follow-up confirmed the excellent result. This suggests that a self-expandable valve is an interesting alternative to balloon expandable valve for selected patients with such anatomy.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145186463","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":"Management of a Large Rhabdomyoma with Sirolimus and Antiarrhythmic Agents.","authors":"Mats Steffi Jennifer Masilamani, Talha Niaz, Philip L Wackel, Justin M Horner, Frank Cetta, Patrick W O'Leary, M Yasir Qureshi","doi":"10.1007/s00246-025-04030-0","DOIUrl":"https://doi.org/10.1007/s00246-025-04030-0","url":null,"abstract":"<p><p>Cardiac rhabdomyomas are the most common pediatric cardiac tumors and are frequently associated with tuberous sclerosis complex (TSC). Large fetal cardiac rhabdomyomas causing inflow or outflow obstruction can lead to hemodynamic compromise and arrhythmias, sometimes necessitating emergent perinatal interventions. We present the fetus of a 32-year-old with multiple intracardiac rhabdomyomas identified at 23 weeks gestation. The largest, located in the left atrium (LA), enlarged by 32 weeks, causing mitral inflow obstruction and moderate mitral regurgitation (MR). Maternal sirolimus therapy was initiated, leading to tumor regression and permitting delivery at 39 weeks without the need for fetal or neonatal surgery. Postnatally, the infant developed hemodynamically significant refractory arrhythmias, requiring antiarrhythmics and inotropic support. Continued sirolimus therapy further reduced tumor burden and improved MR. This case adds to the growing evidence supporting transplacental sirolimus therapy (mTOR inhibition) for managing large, hemodynamically significant fetal cardiac rhabdomyomas. It also underscores the importance of anticipating and aggressively managing associated arrhythmias.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182085","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":"Outcomes After Stenting of Right Ventricular Outflow Tract for Tetralogy of Fallot and Its Variants.","authors":"Nisanth Selvam, Navaneetha Sasikumar, Raman Krishna Kumar","doi":"10.1007/s00246-025-04039-5","DOIUrl":"https://doi.org/10.1007/s00246-025-04039-5","url":null,"abstract":"<p><p>We reviewed our institutional database to examine the effect of right ventricular outflow tract (RVOT) stenting on branch pulmonary artery (PA) sizes and their outcomes. RVOT stenting was performed following standard technique. Since 2021, a conscious effort was made to spare the pulmonary valve (PV) whenever feasible. PA annulus and branch PA sizes were serially monitored by echocardiography. Sixty patients, (4.5 [1.75-24] months, weight 6.4 [3.5-9 kg]), were included (Time period:2012-2025). Commonest indications included cyanotic spell (65%) and hypoplastic branch PAs (30%). Pulmonary valve annulus measured 5.1 mm (4.0-7.7); Z score - 3.8 (- 5.1 to - 2.6), and MPA 4.35 mm (3.34-5.7); Z score - 4.9 (- 7.0 to [- 3.1]). At follow-up of 8(25-102) months, branch PAs grew in all, albeit at different paces. RPA z-scores improved from a baseline of - 2.48 (- 5.05 to [- 0.85]) to - 1.55 (- 3.07 to 0.46) and LPA z-scores from - 2.5 (- 4.38 to [- 0.78]) to - 1.08 (- 2.41 to 0.63) in hypoplastic PA group over 1-year period (p value < 0.001). Pulmonary valve was spared in 57% of patients in the cyanotic spell group and 22% of hypoplastic PA group. 29 (48%) patients underwent corrective surgery at 9 (6-14) months from RVOTS. Requirement of transannular patch (TAP) was 38% (11/29) and conduit was 34% (10/29). Valve sparing ICR was feasible in 67% (8/12) when the stent was placed below the valve. In addition to effective palliation, RVOT stenting promotes growth of branch pulmonary arteries even in those with inherently hypoplastic branch PAs. It is possible to effectively palliate selected patients without stenting across the pulmonary valve thereby potentially enabling valve-sparing TOF correction in those with suitable anatomy.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145177218","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 Impact of Bronchopulmonary Dysplasia on the Maturation of Preterm Infants' Right Ventricular Mechanics Over the First Year of Life: A Prospective Strain-Echocardiography Cohort.","authors":"Karen Saori Shiraishi Sawamura, Lilian Dos Santos Rodrigues Sadeck, Antonildes Nascimento Assunção Junior, Nara Yuri Yamada Kushikawa, Alessandro Cavalcanti Lianza, Maria de Fatima Rodrigues Diniz, Carolina Rocha Brito Menezes, Isabela de Souza Lobo da Silva, Gabriela Nunes Leal","doi":"10.1007/s00246-025-04043-9","DOIUrl":"https://doi.org/10.1007/s00246-025-04043-9","url":null,"abstract":"<p><p>Bronchopulmonary dysplasia (BPD) is frequent in very-preterm infants, yet its effects on right-ventricular (RV) maturation during the first post-natal year remains unclear. We aimed to investigate RV mechanics among preterm with and without BPD, using conventional echocardiogram and speckle-tracking derived strain, throughout this period. We prospectively enrolled 118 infants born < 32 weeks' gestation who were scanned at 36 weeks post-menstrual age (PMA), as well as at 1-month and 1-year corrected age (CA). Seventy-two infants met BPD criteria and 46 served as controls. Pattern-mixture models, adjusted for sex, birth-weight z-score, and respiratory support, analyzed trajectories of RV global longitudinal strain (RVGLS). Conventional RV dimensions and functional measurements were comparable between groups at all visits. RVGLS increased in controls (+ 4.4 ± 1.2%) but remained unchanged in BPD (- 0.2 ± 1.3%), yielding a significant group-by-time interaction (β = - 5.8%, 95% CI - 9.3 to - 2.4; p = 0.001). At 1-year CA, mean RVGLS was lower in BPD than controls (24.3 ± 4.1% vs 28.7 ± 4.6%; p < 0.001). Oxygen dependency at 36 weeks PMA (β = - 5.8%; p = 0.001) and invasive ventilation > 2 days (β = - 2.8%; p = 0.012) independently predicted worse RVGLS, whereas high-frequency oscillatory ventilation was associated with improvement (β =  + 4.9%; p = 0.043). BPD may disrupt the normal maturation of RV mechanics during the first postnatal year. RVGLS identifies subclinical dysfunction undetectable by conventional echocardiography and may serve as an early biomarker to guide cardiopulmonary interventions in this high-risk population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150004","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":"Neonatal Portal Vein Thrombosis (PVT): A Case Series from a Tertiary NICU.","authors":"Yakup Karakurt, Emrah Can","doi":"10.1007/s00246-025-04044-8","DOIUrl":"https://doi.org/10.1007/s00246-025-04044-8","url":null,"abstract":"<p><p>Neonatal portal vein thrombosis (PVT) is an uncommon but clinically important vascular complication, often associated with umbilical venous catheter (UVC) use. The optimal management strategy, including the role of anticoagulation, remains uncertain. This retrospective case series included 13 neonates with PVT diagnosed in a level III NICU between January 2021 and December 2024. Clinical and imaging data were compared between infants with spontaneous thrombus resolution and those with persistent thrombosis. The median gestational age was 30.1 weeks, and the median birth weight was 875 g. All infants had UVC placement; every thrombus involved the left portal vein with intrahepatic extension, and all extended into the IVC. Two neonates (15.4%) received anticoagulation; the remainder were managed conservatively. Spontaneous resolution occurred in 6 of 13 cases (46.2%). Earlier diagnosis and higher birth weight were more frequent in the resolution group, although not statistically significant. No thrombus-related acute complications occurred during a median follow-up of 6 months. In this case series, nearly half of neonates with non-occlusive PVT showed spontaneous resolution without anticoagulation. These findings suggest that conservative management can be considered in clinically stable infants, but the short follow-up precludes firm conclusions regarding long-term safety. Ongoing surveillance is essential to detect late complications such as portal hypertension or portosystemic shunting.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145176750","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":"Impact of Nonlinear Heart Rate Variability in Postoperative Complex Congenital Heart Disease: Insights From Repaired Tetralogy of Fallot and Fontan Palliation.","authors":"Azusa Akiya, Ken Takahashi, Kanato Iwahara, Yusuke Akatsuka, Hiroyuki Sato, Keiya Sato, Hirohisa Kago, Sachie Shigemitsu, Hideo Fukunaga, Katsumi Akimoto, Masahiko Kishiro, Keisuke Nakanishi, Hiromichi Shoji","doi":"10.1007/s00246-025-04020-2","DOIUrl":"https://doi.org/10.1007/s00246-025-04020-2","url":null,"abstract":"<p><p>Postoperative patients with complex congenital heart disease (CHD), such as those who have undergone Fontan procedures or Tetralogy of Fallot (TOF) repairs, often experience complications, including arrhythmias and heart failure. Heart rate variability (HRV) analysis assesses autonomic function and predicts cardiac outcomes; however, conventional linear methods have limitations, particularly in pediatric populations with variable heart rates. Nonlinear HRV analysis offers a robust alternative. This study evaluated the utility of nonlinear HRV indices to identify early autonomic dysfunction in postoperative patients with CHD. A total of 41 postoperative patients with CHD (22 Fontan, 19 TOF) and 34 age-matched healthy controls underwent 24-h electrocardiogram recordings during sleep. Frequency- and time-domain HRV indices were calculated, alongside nonlinear measures such as Poincaré plot analysis and Higuchi fractal dimension (HFD). Statistical analyses compared the groups and assessed correlations between HRV indices and clinical parameters. Patients who underwent TOF repairs exhibited significantly lower low frequency, high frequency, standard deviation of all normal R-R intervals, and the root mean square of successive differences between R-R intervals values than those in the control group. Nonlinear indices, particularly HFD, were unaffected by heart rate, enhancing reliability in pediatric populations. Age-related declines in fractal indices in postoperative patients with TOF repairs suggest progressive autonomic dysfunction. Nonlinear HRV analysis offers valuable insights into autonomic dysregulation in postoperative patients with CHD, surpassing conventional methods in reliability. These approaches may enable early detection of autonomic deterioration, improving risk stratification and guiding management. Further validation in larger cohorts is warranted.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145149843","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":"In-hospital Outcomes of Pediatric Cardiac Surgery Admissions for Acute Infective Endocarditis in Congenital Heart Disease.","authors":"Mohamed F Elsisy, Tammy Ruth, Garrett Coyan, Daniel E Dulek, David Parra","doi":"10.1007/s00246-025-04037-7","DOIUrl":"https://doi.org/10.1007/s00246-025-04037-7","url":null,"abstract":"<p><p>Infective endocarditis (IE) is uncommon in children with congenital heart disease (CHD), and limited data exist regarding in-hospital outcomes of those requiring cardiac surgery. We retrospectively reviewed hospitalizations from the pediatric health information system from January 2016 to November 2024. Among 357,031 unique pediatric patients with CHD (≤ 21 years), 2108 patients (0.5%) were hospitalized for IE. The index hospitalizations during which cardiac surgery was performed were included. We aimed to characterize in-hospital outcomes among CHD children undergoing cardiac surgery for IE, with sub-analysis of those who required surgery versus medical therapy. The incidence of IE in CHD was 5.6 cases per 1000. Microorganism coding was unavailable in 41.3%; Staphylococcus (24.1%) and Streptococcus (22.3%) were the most common. Stroke occurred in 10.7%. Prosthetic valve endocarditis was present in 9.4%. Overall, 30% of patients required cardiac surgery, most commonly pulmonary valve or conduit procedures (36.6%) and tricuspid valve surgery (16.7%). Two-valve surgery was performed in 13.3%. In-hospital mortality was comparable between surgical and medical patients (10.1% vs 9.6%, p = 0.7). Permanent pacemaker implantation was more common after surgery (6.6% vs 2.2%, p < 0.01). Length of hospital stay and hospitalization costs were higher in the surgical group. On multivariable analysis, older age, heart failure, stroke, and extracorporeal membrane oxygenation (ECMO) were associated with in-hospital mortality. IE in children with CHD remains associated with substantial morbidity and mortality. In-hospital mortality remains high despite cardiac surgery. Older age, heart failure, stroke, and ECMO requirement are independent factors of in-hospital mortality.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145176726","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":"Aerobic Fitness is Related to Sports Participation in Children with Congenital Heart Disease.","authors":"Astrid-Marie De Souza, Kathryn R Armstrong, Kimberly Hoskins, Nicholas Tran, Kaelan C Naylor, Hilary V Romans, James E Potts, Martin C Hosking, David C Clarke","doi":"10.1007/s00246-025-04014-0","DOIUrl":"https://doi.org/10.1007/s00246-025-04014-0","url":null,"abstract":"<p><p>Anatomical and physiological differences in structural heart lesions may affect peak aerobic capacity ( <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak) and influence sports participation in children with congenital heart disease (CHD). We hypothesized that a higher frequency of sports participation would be associated with higher <math> <mrow><mover><mi>V</mi> <mo>˙</mo></mover> <msub><mi>O</mi> <mrow><mn>2</mn> <mi>p</mi> <mi>e</mi> <mi>a</mi> <mi>k</mi></mrow> </msub> </mrow> </math> in these patients. A two-year, single-center, retrospective review (May 2016-November 2018) was conducted in CHD patients who had a maximal cardiopulmonary exercise test (CPET) and documented sport participation. Sports participation was categorized into 3 groups: 0-1 days/week; 2-3 days/week; and ≥ 4 days/week. <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak z-scores, % <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O₂ at gas exchange threshold ( <math> <mrow><mover><mi>V</mi> <mo>˙</mo></mover> <msub><mi>O</mi> <mrow><mn>2</mn> <mi>G</mi> <mi>E</mi> <mi>T</mi></mrow> </msub> </mrow> </math> ), and O₂ pulse were calculated. Z-scores were calculated based on a reference population. Means and standard deviation (SD) are reported. p < 0.05 was considered statistically significant. In our study cohort (n = 56), <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak z-score was - 1.01 ± 0.95; 83% had a z-score within ± 2 SD, while 59% were within one SD. The overall regression for sport participation with <math> <mrow><mover><mi>V</mi> <mo>˙</mo></mover> <msub><mi>O</mi> <mrow><mn>2</mn> <mi>p</mi> <mi>e</mi> <mi>a</mi> <mi>k</mi></mrow> </msub> </mrow> </math> and <math> <mrow><mover><mi>V</mi> <mo>˙</mo></mover> <msub><mi>O</mi> <mrow><mn>2</mn> <mi>G</mi> <mi>E</mi> <mi>T</mi></mrow> </msub> </mrow> </math> was statistically significant (R² = 0.40, F(4, 54) = 11.44, p =  < .0001) and (R² = 0.17, F(4, 54) = 3.46, p = 0.0227), respectively. There was a significant main effect for O₂ pulse (R² = 0.41, F(4, 52) = 11.91, p < 0.0001) but not for HR_peak (p = 0.86), SBP_peak (p = 0.74) or DBP_peak (p = 0.94). <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak is higher in those who participate in sports compared to those who do not. It is unclear whether those with a higher <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak are more inclined to participate in sports or whether sports participation leads to a higher <math><mover><mi>V</mi> <mo>˙</mo></mover> </math> O_2peak.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150468","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":"Acquired Conduction Disease in Pediatric Myocarditis: A Review of the Literature and Case Series.","authors":"Gayathri Plant, Robin Clegg, Deborah Fruitman","doi":"10.1007/s00246-025-04017-x","DOIUrl":"https://doi.org/10.1007/s00246-025-04017-x","url":null,"abstract":"<p><p>Myocarditis is an important cause of mortality, and particularly sudden death, in children. Arrhythmias, particularly tachyarrhythmias, are a known clinical sequelae of myocarditis. Bradyarrhythmias and conduction disease in pediatric myocarditis are a less common, but potentially critical complication of myocarditis, which may be associated with increased morbidity and mortality. Pediatric myocarditis with effects on atrioventricular conduction and ventricular conduction represents an underexplored and clinically significant field. We conducted a systematic review of the published literature and present two cases of significant conduction disease. Literature searches were performed as per PRISMA guidelines in Medline, EMBASE, Web of Science, and Cochrane Systematic Reviews. We analyzed 53 articles and identified 175 published cases of atrioventricular (AV) conduction disease and bradyarrhythmias in pediatric myocarditis. Diagnostic approaches were varied among the studies, with heterogeneity in both serum investigations, cardiac imaging, and management of the conduction issues. There were 91 cases of complete heart block, 53 cases of first-degree heart block, 11 cases of second-degree heart block, and 10 reported cases of clinically significant sinus bradycardia. Of the 175 reported cases, 31 required temporary pacing, with 10 having a pacemaker implanted, and the remaining were treated with medical therapy alone and did not require pacemaker placement. In this review, there were 13 deaths among the 175 reported cases, all of whom had complete heart block. AV conduction disease in pediatric myocarditis, although less common, can be clinically significant and have severe outcomes. The variation in presentation, treatment, outcome, and diversity in pathogens warrants further study in order to improve our understanding of this potential complication of myocarditis.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145137955","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":"Use of SGLT2 inhibitors in pediatric heart failure: a multi-center study.","authors":"Ryan Butts, Deipanjan Nandi, Borah Hong, Thomas D Ryan, Juli Sublett-Smith, Humera Ahmed, David M Peng, Victor Benvenuto, Gabrielle Vaughn, Kathleen Simpson, Benjamin Kroslowitz, Joseph Spinner","doi":"10.1007/s00246-025-04025-x","DOIUrl":"10.1007/s00246-025-04025-x","url":null,"abstract":"<p><strong>Introduction: </strong>Sodium-glucose cotransporter 2 inhibitors (SGLT2is) are utilized in pediatric heart failure (HF) with little data on dosing or safety profile. Our aim is to report on dosing and adverse events associated with SGLT2i use in pediatric HF.</p><p><strong>Methods: </strong>A retrospective study was performed utilizing the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) pediatric heart failure registry. Patient demographics, medical regimen, echocardiographic data, laboratory data, adverse events, and relevant heart failure outcomes were collected at SGLT2i initiation and last follow-up.</p><p><strong>Results: </strong>At time of database query, data from 278 patients from 19 institutions were common. The most common SGLT2i prescribed was dapagliflozin (244) followed by empagliflozin (34). Median age at initiation was 15.1 years (IQR 10.7-18.2), 106 had DCM, 54 had Fontan physiology, and 67% of patients were initiated in the outpatient setting. For all patients prescribed dapagliflozin, the median mg/kg/dose at initiation was 0.11 (IQR 0.08-0.14). The median follow-up was 195 days (IQR 90-450, n = 180). In the follow-up cohort, 32 patients discontinued SGLT2i with 15 due to drug intolerance. 28 patients had a total of 34 adverse events (AE) reported. The most common AE was UTI (11) followed by AKI (10). After SGLT2i initiation, 13% of patients had a subsequent HF admission, 5% had a VAD, and 9% underwent heart transplantation.</p><p><strong>Conclusion: </strong>In pediatric HF, SGLT2is are being utilized in a diverse patient population. AKI and UTI were the most common reported AE. Typical initiation dose is approximately 0.1mg/kg/dose. Prospective studies are needed to help determine efficacy.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145138127","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}