Pediatric Cardiology最新文献_第3页

Gaps in Pediatric Interventional Cardiology Technology: A Survey Assessment. 儿童介入心脏病技术的差距：一项调查评估。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-03 DOI: 10.1007/s00246-025-04046-6

Daniel Levi, Nicholas Sharawy, Tim Moran

{"title":"Gaps in Pediatric Interventional Cardiology Technology: A Survey Assessment.","authors":"Daniel Levi, Nicholas Sharawy, Tim Moran","doi":"10.1007/s00246-025-04046-6","DOIUrl":"https://doi.org/10.1007/s00246-025-04046-6","url":null,"abstract":"As a follow-up to the device needs survey conducted and published in 2015, an updated survey was repeated in 2024 to evaluate gaps in device and equipment availability for pediatric congenital interventional cardiologists. Over 200 US pediatric interventional cardiologists were contacted directly via email. The Congenital Cardiovascular Interventional Study Consortium (CCISC) and the Pediatric and Congenital Interventional Cardiovascular Society (PICS) distributed the survey to its membership in the United States (US). Respondents cited a need for new devices which differed significantly from the 2015 survey. \"Biodegradable Stents\" (37%), \"PDA Stents\" (patent ductus arteriosus, 21%), and \"Pulmonary Artery Flow Restrictors\" (21%) were ranked as the top three most needed devices. Devices that were suspected to offer the greatest potential reduction in mortality were \"Flow Restrictors\" (30%), \"PDA Stents\" (18%), and \"Biodegradable Stents\" (6%). The respondents also prioritized the needs for devices not available in the US or removed from the market. With many startups and larger companies interested in the congenital interventional space, the results of this survey are intended to help guide tailored device development for the most pressing needs of the congenital interventional cardiology community.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145213333","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

Evaluation of Cardiac Function in Children Undergoing Liver Transplantation. 评估接受肝移植的儿童的心功能。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-11-22 DOI: 10.1007/s00246-024-03673-9

Neha Bansal, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Michelle Ovchinsky, Kayla Ganzburg, Nadine Choueiter

{"title":"Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.","authors":"Neha Bansal, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Michelle Ovchinsky, Kayla Ganzburg, Nadine Choueiter","doi":"10.1007/s00246-024-03673-9","DOIUrl":"10.1007/s00246-024-03673-9","url":null,"abstract":"Cirrhotic cardiomyopathy is a complication of cirrhosis resulting in cardiac dysfunction. It remains poorly characterized in children. The aim of this study was to assess relationship of pre-liver transplant (LT) conventional and novel parameters of biventricular function with post-LT clinical course. This is a retrospective study of pre-LT echocardiograms performed on patients < 18 years of age with cirrhosis at a single center, who received a LT. Demographic, clinical, and echocardiographic data were collected. Speckle tracking echocardiography (STE) analysis was performed by a single observer using TomTec system. Descriptive data were expressed as mean (SD) and number (%). The relationship between clinical data and echocardiographic variables were assessed using Spearman correlation coefficient. Significance was set at < 0.05. Thirty-five patients (median age 6.5; IQR 14.2 years) underwent LT between 2010 and 2020. Pre-LT diagnosis was biliary atresia in 14 (40%) patients and 7 (20%) patients were listed as status 1A/1B. Their median natural pediatric/model end-stage liver disease score was 13 (IQR 9). Their pre-LT echocardiogram showed normal left ventricular systolic (LV) function by ejection fraction and strain parameters. Right ventricular (RV) function was abnormal in 74% of patients as measured by RV GLS (23 ± 3%). There was correlation between echocardiographic parameters with pre-transplant clinical disease and post-operative LT course (length of stay and duration of mechanical ventilation). Children undergoing liver transplant have RV dysfunction as evidenced by abnormal RV GLS on STE. There is echocardiographic parameter correlation between clinical liver disease and post-LT clinical course. This evidence highlights the importance of using novel technology like STE in assessment of children undergoing evaluation for liver transplant.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"2019-2026"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12496272/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142688495","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

VSD Surgical Closure in Colombia in Children with Secondary Pulmonary Hypertension. Does Altitude Influence Postoperative Pulmonary Pressure? 哥伦比亚继发性肺动脉高压患儿的 VSD 手术关闭术。海拔高度会影响术后肺压吗？

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-11-15 DOI: 10.1007/s00246-024-03697-1

Linibeth Cruz-Baquero, Nicolas Molano-Gonzalez, Daniel García-Vargas, Alberto García Torres

{"title":"VSD Surgical Closure in Colombia in Children with Secondary Pulmonary Hypertension. Does Altitude Influence Postoperative Pulmonary Pressure?","authors":"Linibeth Cruz-Baquero, Nicolas Molano-Gonzalez, Daniel García-Vargas, Alberto García Torres","doi":"10.1007/s00246-024-03697-1","DOIUrl":"10.1007/s00246-024-03697-1","url":null,"abstract":"A retrospective, cross-sectional, descriptive, observational study was carried out to describe the history of pulmonary hypertension in pediatric patients living at different altitudes following surgical correction of ventricular septal defect. Data from 40 patients who underwent surgery in La Fundacion Cardioinfantil was collected and used for our analysis. Bivariate analysis showed no significant relationship between altitude and pulmonary hypertension after ventricular septal defect closure. Unrelated to the main objective of our study, our investigation revealed that our population underwent surgical correction of VSD at older ages than expected. While previous publications demonstrate the benefit of intervention at 4 years of age or younger (19, 20), the average age in our studied population was found to be 7.8 years old. These patients had unfavorable hemodynamic parameters for ventricular septal defect closure, but our study showed that our patients benefited from surgery with an immediate satisfactory postoperative result. Patients transitioned from parameters indicating severe PH to mild PH within the first 24-48 h after surgery.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1968-1973"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12496296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142638879","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

Nutrition Provision in Children with Heart Disease on Extracorporeal Membrane Oxygenation (ECMO). 为接受体外膜氧合（ECMO）治疗的心脏病患儿提供营养。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-08-26 DOI: 10.1007/s00246-024-03628-0

Jason S Kerstein, Caroline R Pane, Lynn A Sleeper, Emily Finnan, Ravi R Thiagarajan, Nilesh M Mehta, Kimberly I Mills

{"title":"Nutrition Provision in Children with Heart Disease on Extracorporeal Membrane Oxygenation (ECMO).","authors":"Jason S Kerstein, Caroline R Pane, Lynn A Sleeper, Emily Finnan, Ravi R Thiagarajan, Nilesh M Mehta, Kimberly I Mills","doi":"10.1007/s00246-024-03628-0","DOIUrl":"10.1007/s00246-024-03628-0","url":null,"abstract":"Nutrition provision for children with heart disease supported with extracorporeal membrane oxygenation (ECMO) involves nuanced decision making. We examined nutrition provision while on ECMO in the CICU and the relationship between energy and protein adequacy and end organ function as assessed by pediatric sequential organ failure assessment (pSOFA) scores in children with heart disease supported with ECMO. Children (≤ 21 years-old) with congenital or acquired heart disease who received ECMO in the cardiac intensive care unit were included. There were 259 ECMO runs in 252 patients over an 8-year study period (2013-2020). Median energy delivery and adequacy were 26.1 [8.4, 45.9] kcal/kg/day and 58.3 [19.8, 94.6]%, respectively. Median protein delivery and adequacy were 0.98 [0.36, 1.64] g/kg/day and 35.7 [13.4, 60.3]%, respectively. pSOFA increased by a median of four points during the ECMO run. Change in pSOFA score was not associated with energy or protein adequacy (p = 0.46 and p = 0.72, respectively). Higher energy and protein adequacy-from parenteral nutrition-correlated with increased hospital-acquired infections (HAIs, p = 0.031 and p = 0.003, respectively). Achieving nutritional adequacy was dependent on the use of parenteral nutrition. Similar clinical outcomes with regard to end organ function but with an increased incidence of HAIs suggests the need to explore the role of optimal enteral nutrition delivery on ECMO.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"2097-2106"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142056249","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

Analysis and Visualization of Inter-/Intra-surgeon Variability Using Surgical Simulation for Partial Anomalous Pulmonary Venous Connection Repair. 利用部分异常肺静脉连接修复手术模拟，分析和直观显示医生间/医生内的变异性。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-11-01 DOI: 10.1007/s00246-024-03699-z

Yuki Nakamura, Elizabeth Joy Trimble, Aswathy Kumar Vaikom House, Harold MacDonald Burkhart

{"title":"Analysis and Visualization of Inter-/Intra-surgeon Variability Using Surgical Simulation for Partial Anomalous Pulmonary Venous Connection Repair.","authors":"Yuki Nakamura, Elizabeth Joy Trimble, Aswathy Kumar Vaikom House, Harold MacDonald Burkhart","doi":"10.1007/s00246-024-03699-z","DOIUrl":"10.1007/s00246-024-03699-z","url":null,"abstract":"There may be inter-/intra-surgeon variability in the repair of congenital heart defects. The objective was to analyze and visualize inter-/intra-surgeon variability in the level of patch suture lines and in the shape and size of patches developed through surgical simulation for partial anomalous pulmonary venous connection (PAPVC) repair using a patient-specific 3D-printed heart model. A patient with PAPVC and preoperative computed tomography data were selected, and a patient-specific heart model was obtained. Two different exposures on the model were tested. Two surgeons were enrolled. Both surgeons performed a single-patch repair on four heart models with one exposure and four models with the other. On the potential suture line, 20 points common in each model were allocated. The level of patch suture lines was represented as a deviation from the 20 points. The shape and size of the patches were analyzed and visualized using geometric morphometrics approaches, using the 20 points as landmarks to represent the patches. There was inter-surgeon variability in the level of patch suture lines, and the variability was higher in particular locations. There was inter-surgeon variability in the shape and size of patches, which was not affected by the exposures. The inter-surgeon variability in the shape of patches was higher in particular locations. There was intra-surgeon variability in the shape of patches. Inter-/intra-surgeon variability was analyzed and visualized by the surgical simulation using geometric morphometrics approaches. Further studies are needed to scrutinize whether the variability affects postoperative hemodynamics in actual surgery.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"2027-2036"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564478","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

ECG Abnormalities, Diastolic Blood Pressure, and Adverse Events After Systemic to Pulmonary Artery Shunt in Infants with Congenital Heart Disease. 先天性心脏病婴儿系统性肺动脉分流术后的心电图异常、舒张压和不良事件。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-09-24 DOI: 10.1007/s00246-024-03645-z

Tighe N Marrone, Venu Amula, Mary C Niu, Zhining Ou, Eric R Griffiths

{"title":"ECG Abnormalities, Diastolic Blood Pressure, and Adverse Events After Systemic to Pulmonary Artery Shunt in Infants with Congenital Heart Disease.","authors":"Tighe N Marrone, Venu Amula, Mary C Niu, Zhining Ou, Eric R Griffiths","doi":"10.1007/s00246-024-03645-z","DOIUrl":"10.1007/s00246-024-03645-z","url":null,"abstract":"All patients with Systemic to Pulmonary Artery (SPA) shunt as the index surgical procedure at a single center were studied to determine the association between post-operative ECG repolarization abnormalities, diastolic blood pressure (DBP), and adverse outcomes. Postoperative ECGs were categorized into three grades, Grade 2 defined as ST elevation/depression ≥ 2 mm in ≥ 2 precordial or ≥ 1 mm in ≥ 2 limb leads; Grade 1-T-wave inversion or flattening in ≥ 3 leads; and Grade 0-no criteria for grades 1 or 2. For each patient, time with invasive DBP below 25, 25-29, 30-34, or above 34 mmHg in the first 24 h was calculated. The primary outcome was a pre-discharge composite of death, cardiac arrest, ECMO, unplanned shunt reintervention, and necrotizing enterocolitis after 24 h of surgery. Of the 109 patients included in final analysis, 17 (15.6%) had the composite outcome. Grade 2 ECG abnormality occurred in 12%, and Grade 1 in 37%. There was no association between ECG abnormalities and adverse events. Increasing time with DBP < 30 was not associated with adverse outcomes, while increasing time with DBP 30-34 was associated with decreased odds, and increasing time with DBP > 34 mmHg was associated with increased odds of adverse outcomes on multivariable analysis accounting for indexed shunt size and chromosomal abnormalities. In conclusion, after SPA shunt placement, ECG repolarization abnormalities and low DBP within 24 h were common and not associated with adverse outcomes. Sustained elevation of DBP above 34 mmHg was not protective, especially in patients with high indexed shunt size and chromosomal abnormalities.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1916-1924"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308318","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

Congenital Heart Defects and Concurrent Diagnoses in Influenza Hospitalization in the Pediatric Health Information System Study, 2004-2019. 2004-2019年儿科健康信息系统研究中的先天性心脏缺陷和流感住院并发症诊断。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-08-05 DOI: 10.1007/s00246-024-03613-7

Sara B Stephens, Rocky Tsang, Ruosha Li, Cecilia Cazaban-Ganduglia, A J Agopian, Shaine A Morris

{"title":"Congenital Heart Defects and Concurrent Diagnoses in Influenza Hospitalization in the Pediatric Health Information System Study, 2004-2019.","authors":"Sara B Stephens, Rocky Tsang, Ruosha Li, Cecilia Cazaban-Ganduglia, A J Agopian, Shaine A Morris","doi":"10.1007/s00246-024-03613-7","DOIUrl":"10.1007/s00246-024-03613-7","url":null,"abstract":"Influenza is associated with adverse outcomes in children, although modification by additional medical conditions is not well-documented. We aimed to compare outcomes in children with versus without congenital heart defects (CHDs) who were hospitalized for influenza. We retrospectively evaluated patients 1-18y hospitalized for influenza in the Pediatric Health Information (PHIS) database from 2004 to 2019. Outcomes were compared by CHD presence and then by CHD severity (minor biventricular, major biventricular, and single ventricle disease) using log-binomial regression adjusted for propensity scores accounting for age at admission, sex, and history of asthma. Outcomes included inpatient mortality, intensive care unit (ICU) admission, mechanical ventilation, and length of stay (LOS) > 12 days. To evaluate for effect modification by genetic diagnoses, analyses were repeated stratified by CHD and genetic diagnosis. Among 55,161 children hospitalized for influenza, 2369 (4.3%) had CHDs, including 963 with minor biventricular, 938 with major biventricular, and 468 with single ventricle CHDs. Adjusting for propensity scores, children with CHDs had higher mortality (4.1% versus 0.9%) compared to those without CHDs (risk ratio [RR] 2.5, 95% confidence interval [CI] 1.9-3.4). Children with CHDs were at higher risk of mechanical ventilation (RR 1.6, 95% CI 1.6-1.7), ICU admission (RR 1.9, 95% CI 1.8-2.1), and LOS > 12 days (RR 2.2, 95% CI 2.0-2.3). Compared to those with neither CHD nor genetic condition, children with both had significantly higher risk of all outcomes, with the largest difference for LOS > 12 days (RR 2.3, 95% CI 2.0-2.7). Children with CHDs hospitalized for influenza are particularly susceptible to adverse outcomes compared to those without CHDs. Future studies are needed to corroborate findings in light of influenza vaccination.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"2070-2077"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141893995","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

Efficacy and Safety of Colchicine in Pediatric Pericarditis: A Systematic Review and Future Directions. 秋水仙碱治疗小儿心包炎的有效性和安全性：系统回顾与未来方向

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-07-30 DOI: 10.1007/s00246-024-03606-6

Mohammed Alsabri, Sarah Makram Elsayed, Ahmed Bostamy Elsnhory, Khaled Abouelmagd, Mohammed Ayyad, Basel F Alqeeq, Abdelmonem Siddiq, Yousef Soliman, Muhammad Azan Shahid

{"title":"Efficacy and Safety of Colchicine in Pediatric Pericarditis: A Systematic Review and Future Directions.","authors":"Mohammed Alsabri, Sarah Makram Elsayed, Ahmed Bostamy Elsnhory, Khaled Abouelmagd, Mohammed Ayyad, Basel F Alqeeq, Abdelmonem Siddiq, Yousef Soliman, Muhammad Azan Shahid","doi":"10.1007/s00246-024-03606-6","DOIUrl":"10.1007/s00246-024-03606-6","url":null,"abstract":"Pediatric pericarditis presents challenges in its management, necessitating effective therapeutic interventions. Colchicine, known for its efficacy in adults, requires further investigation for its application and safety in pediatric cohorts. A systematic search across renowned databases identified relevant literature on colchicine use in pediatric pericarditis. Twenty-nine articles underwent rigorous screening, with 18 studies meeting inclusion criteria. Data extraction, quality assessment, and synthesis were conducted meticulously. Included studies comprised case reports, case series, and retrospective cohort studies. Colchicine demonstrated efficacy in reducing recurrence rates and symptom burden, with doses ranging from 0.25 mg/day to 2 mg/day. Adverse events were minimal, predominantly gastrointestinal. Notably, nausea was the most common side effect reported. The safety profile of colchicine was favorable, with rare instances of hepatic and hematologic toxicity. Colchicine emerges as a promising therapeutic option for pediatric pericarditis, demonstrating efficacy in reducing recurrence rates and alleviating symptoms. Its favorable safety profile suggests potential as a preferred long-term therapy. However, further research, including randomized controlled trials, is warranted to confirm its efficacy and safety and explore potential combination therapies.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1790-1805"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141856152","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 Comorbidity and Exercise Intolerance in Bilateral Lung Transplant Recipients Followed at a Pediatric Center. 儿科中心随访的双侧肺移植受者的心脏合并症和运动不耐受性

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 Epub Date: 2024-10-17 DOI: 10.1007/s00246-024-03674-8

Christina M Mangano, Levent Midyat, Dawn Freiberger, Kimberlee Gauvreau, Debra Boyer, Gary Visner, Ming Hui Chen

{"title":"Cardiac Comorbidity and Exercise Intolerance in Bilateral Lung Transplant Recipients Followed at a Pediatric Center.","authors":"Christina M Mangano, Levent Midyat, Dawn Freiberger, Kimberlee Gauvreau, Debra Boyer, Gary Visner, Ming Hui Chen","doi":"10.1007/s00246-024-03674-8","DOIUrl":"10.1007/s00246-024-03674-8","url":null,"abstract":"Reduced exercise capacity is common in young bilateral lung transplantation (Bi-LTx) recipients, but longer-term data on cardiac comorbidities are limited. We evaluate potential cardiac contributions to long-term exercise intolerance in this population. All Bi-LTx recipients at a single pediatric center, who completed routine clinical post-transplant cardiac assessment, including echocardiogram, cardiac exam, and cardiopulmonary exercise testing (CPET), were included. Cardiac risk factors (CRFs) were assessed by history and laboratory tests. CPET-derived peak and percent-predicted peak myocardial oxygen consumption (VO2 peak, ppVO2 peak) were used to quantitate exercise capacity. Percent-predicted peak oxygen pulse (pp peak O2 pulse) assessed stroke volume. 15 patients (67% M; median age 21.6 years, median follow-up from Bi-LTx 7.0 years) were included. Almost all patients (14, 93%) had multiple CRFs; hypertension and hyperlipidemia/dyslipidemia were the most common. On CPET, 93% (n = 14) had abnormal (≤ 85%) ppVO2 peak (median 59%). 73% (n = 11) had abnormal pp peak O2 pulse (median 74%). Ten had blunted heart rate response to exercise. Nine had left ventricular diastolic dysfunction (LV-DD) on echocardiogram. Median percent-predicted forced expiratory volume in one second was 70%. One had severe chronic lung allograft dysfunction. Cardiac risk factors and exercise intolerance are common among young Bi-LTx recipients years post-transplant, even among those without significant pulmonary dysfunction. High prevalence of multiple CRFs, LV-DD, chronotropic dysfunction, and abnormal stroke volume suggest cardiac comorbidities may contribute to intolerance. Medical management of CRFs and tailored exercise may decrease cardiac risk and improve functional capacity for Bi-LTx survivors.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1906-1915"},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471988","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

Systemic Sirolimus Monitoring in Pediatric Pulmonary Vein Stenosis. 儿童肺静脉狭窄的全身西罗莫司监测。

IF 1.4 4区医学

Pediatric Cardiology Pub Date : 2025-10-01 DOI: 10.1007/s00246-025-04038-6

Heather Meluskey, Bridget Blowey, Anna B O'Brien, Michael L O'Byrne, Jonathan J Rome, David B Frank, Catherine M Avitabile, Mudit Gupta, Jessica Tang, Kimberly L Butler, Constantine D Mavroudis, Stephanie Fuller, Ryan Callahan

{"title":"Systemic Sirolimus Monitoring in Pediatric Pulmonary Vein Stenosis.","authors":"Heather Meluskey, Bridget Blowey, Anna B O'Brien, Michael L O'Byrne, Jonathan J Rome, David B Frank, Catherine M Avitabile, Mudit Gupta, Jessica Tang, Kimberly L Butler, Constantine D Mavroudis, Stephanie Fuller, Ryan Callahan","doi":"10.1007/s00246-025-04038-6","DOIUrl":"https://doi.org/10.1007/s00246-025-04038-6","url":null,"abstract":"Systemic sirolimus (SS) is an mTOR inhibitor used in the management of pediatric intraluminal pulmonary vein stenosis (PVS). SS initiation, monitoring, including patient compliance with toxicity surveillance, and potential adverse events (AE) in PVS patients are under reported. A single-center retrospective cohort study of consecutive patients who were initiated on SS for PVS from January 1, 2020 to December 31, 2024 was performed. Fifty patients with a median age of 7 months (range 2-165) received SS for PVS (median number of stenotic veins; n = 3 (1-4)) for a median duration of 18 months (1-60). The median time to therapeutic level was 9 days [IQR 3, 20] with two never achieving therapeutic values. In patients who received SS for at least 6 months (n = 39), the median number of blood draws and number of dose adjustments in the first 6 months were 14 [IQR 5, 27] and 3 [1, 7], respectively. Most levels among patients (75%; [IQR 64, 84]) did not require a dose adjustment. Toxicity surveillance compliance increased from 58% [IQR 42, 83] to 79% [IQR 62.5, 92] (p = 0.22) following transitioning ownership of SS management to a dedicated PVS team. Eighteen percent (9/50) of patients had an AE potentially related to SS; SS was discontinued in three. PVS patients receiving SS have high, but variable rates of therapeutic levels and SS discontinuation due to AEs is uncommon. Compliance with safety labs may improve with ownership by a dedicated monitoring team.","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207372","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