Acute myocarditis (AM) is an inflammatory disease of the heart muscle that can progress to fulminant myocarditis (FM), a severe and life-threatening condition. The cytokine profile of myocarditis in children, especially in relation to fulminant myocarditis, is not well understood. This study aims to evaluate the cytokine profiles of acute and fulminant myocarditis in children. Pediatric patients diagnosed with myocarditis were included in the study. Cytokine levels were measured using a multiplexed fluorescent bead-based immunoassay. Statistical analysis was performed to compare patient characteristics and cytokine levels between FM, AM, and healthy control (HC) groups. Principal component analysis (PCA) was applied to cytokine groups that were independent among the FM, AM, and HC groups. The study included 22 patients with FM and 14 with AM patients. We identified four cytokines that were significantly higher in the FM group compared to the AM group: IL1-RA (p = 0.002), IL-8 (p = 0.005), IL-10 (p = 0.011), and IL-15 (p = 0.005). IL-4 was significantly higher in the AM group compared to FM and HC groups (p = 0.006 and 0.0015). PDGF-AA, and VEGF-A were significantly lower in the FM group than in the AM group (p = 0.013 and <0.001). Similar results were obtained in PCA. Cytokine profiles might be used to differentiate pediatric FM from AM, stratify severity, and predict prognosis. The targeted therapy that works individual cytokines might provide a potential treatment for reducing the onset of the FM and calming the condition, and further studies are needed.
急性心肌炎(AM)是一种心肌炎性疾病,可发展为危及生命的重症暴发性心肌炎(FM)。儿童心肌炎的细胞因子谱,尤其是与暴发性心肌炎相关的细胞因子谱尚不十分清楚。本研究旨在评估儿童急性和暴发性心肌炎的细胞因子谱。研究对象包括确诊为心肌炎的儿童患者。使用基于荧光珠的多重免疫测定法测定细胞因子水平。研究人员进行了统计分析,以比较 FM 组、AM 组和健康对照组(HC)的患者特征和细胞因子水平。主成分分析(PCA)适用于 FM、AM 和 HC 组间独立的细胞因子组。研究对象包括 22 名 FM 患者和 14 名 AM 患者。我们发现,与 AM 组相比,FM 组的四种细胞因子含量明显更高:IL1-RA (p = 0.002)、IL-8 (p = 0.005)、IL-10 (p = 0.011) 和 IL-15 (p = 0.005)。AM 组的 IL-4 明显高于 FM 组和 HC 组(p = 0.006 和 0.0015)。FM 组的 PDGF-AA 和 VEGF-A 明显低于 AM 组(p = 0.013 和 0.0015)。
{"title":"Analysis of Cytokine Profiles in Pediatric Myocarditis Multicenter Study.","authors":"Yoji Nomura, Takanori Suzuki, Katsuyuki Kunida, Hidetoshi Uchida, Ryoichi Ito, Yasunori Oshima, Machiko Kito, Yuki Imai, Satoru Kawai, Kei Kozawa, Kazuyoshi Saito, Tadayoshi Hata, Junichiro Yoshimoto, Tetsushi Yoshikawa, Kazushi Yasuda","doi":"10.1007/s00246-024-03452-6","DOIUrl":"10.1007/s00246-024-03452-6","url":null,"abstract":"<p><p>Acute myocarditis (AM) is an inflammatory disease of the heart muscle that can progress to fulminant myocarditis (FM), a severe and life-threatening condition. The cytokine profile of myocarditis in children, especially in relation to fulminant myocarditis, is not well understood. This study aims to evaluate the cytokine profiles of acute and fulminant myocarditis in children. Pediatric patients diagnosed with myocarditis were included in the study. Cytokine levels were measured using a multiplexed fluorescent bead-based immunoassay. Statistical analysis was performed to compare patient characteristics and cytokine levels between FM, AM, and healthy control (HC) groups. Principal component analysis (PCA) was applied to cytokine groups that were independent among the FM, AM, and HC groups. The study included 22 patients with FM and 14 with AM patients. We identified four cytokines that were significantly higher in the FM group compared to the AM group: IL1-RA (p = 0.002), IL-8 (p = 0.005), IL-10 (p = 0.011), and IL-15 (p = 0.005). IL-4 was significantly higher in the AM group compared to FM and HC groups (p = 0.006 and 0.0015). PDGF-AA, and VEGF-A were significantly lower in the FM group than in the AM group (p = 0.013 and <0.001). Similar results were obtained in PCA. Cytokine profiles might be used to differentiate pediatric FM from AM, stratify severity, and predict prognosis. The targeted therapy that works individual cytokines might provide a potential treatment for reducing the onset of the FM and calming the condition, and further studies are needed.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"544-552"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140120282","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}
Pub Date : 2025-03-01Epub Date: 2024-10-05DOI: 10.1007/s00246-024-03668-6
Kenji Baba
{"title":"The Advantages of Balloon Angioplasty over Stent Implantation for Some Lesions.","authors":"Kenji Baba","doi":"10.1007/s00246-024-03668-6","DOIUrl":"10.1007/s00246-024-03668-6","url":null,"abstract":"","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"746"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378076","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}
Pub Date : 2025-03-01Epub Date: 2024-03-24DOI: 10.1007/s00246-024-03462-4
Joshua A Daily, Stephen Dalby, Lawrence Greiten
Extensive research has consistently demonstrated that humans frequently diverge from rational decision-making processes due to the pervasive influence of cognitive biases. This paper conducts an examination of the impact of cognitive biases on high-stakes decision-making within the context of the joint pediatric cardiology and cardiothoracic surgery conference, offering practical recommendations for mitigating their effects. Recognized biases such as confirmation bias, availability bias, outcome bias, overconfidence bias, sunk cost fallacy, loss aversion, planning fallacy, authority bias, and illusion of agreement are analyzed concerning their specific implications within this conference setting. To counteract these biases and enhance the quality of decision-making, practical strategies are proposed, including the implementation of a no-interruption policy until all data is reviewed, leaders refraining from immediate input, requiring participants to formulate independent judgments prior to sharing recommendations, explicit probability estimations grounded in base rates, seeking external opinions, and promoting an environment that encourages dissenting perspectives.
{"title":"Cognitive Biases in High-Stakes Decision-Making: Implications for Joint Pediatric Cardiology and Cardiothoracic Surgery Conference.","authors":"Joshua A Daily, Stephen Dalby, Lawrence Greiten","doi":"10.1007/s00246-024-03462-4","DOIUrl":"10.1007/s00246-024-03462-4","url":null,"abstract":"<p><p>Extensive research has consistently demonstrated that humans frequently diverge from rational decision-making processes due to the pervasive influence of cognitive biases. This paper conducts an examination of the impact of cognitive biases on high-stakes decision-making within the context of the joint pediatric cardiology and cardiothoracic surgery conference, offering practical recommendations for mitigating their effects. Recognized biases such as confirmation bias, availability bias, outcome bias, overconfidence bias, sunk cost fallacy, loss aversion, planning fallacy, authority bias, and illusion of agreement are analyzed concerning their specific implications within this conference setting. To counteract these biases and enhance the quality of decision-making, practical strategies are proposed, including the implementation of a no-interruption policy until all data is reviewed, leaders refraining from immediate input, requiring participants to formulate independent judgments prior to sharing recommendations, explicit probability estimations grounded in base rates, seeking external opinions, and promoting an environment that encourages dissenting perspectives.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"536-543"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140194382","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}
Pub Date : 2025-03-01Epub Date: 2024-06-06DOI: 10.1007/s00246-024-03527-4
Ahmed Deniwar, Mohammad Alnoor, Ahmadreza Ghasemiesfe, Frank F Ing, Timothy J Pirolli, Ralf J Holzer
This is a case of an infant with duct-dependent pulmonary circulation, who required 6 stents delivered over three procedures to fully stent the arterial duct, which originated in a very unusual fashion. The attainable angiographic projections were unable to profile its origin, and only a CT scan was ultimately able to delineate the (stenotic) ductal origin from the aorta.
{"title":"PDA Stent Placement *6: High-Impact Limitations of Angiography to Delineate a Complex PDA.","authors":"Ahmed Deniwar, Mohammad Alnoor, Ahmadreza Ghasemiesfe, Frank F Ing, Timothy J Pirolli, Ralf J Holzer","doi":"10.1007/s00246-024-03527-4","DOIUrl":"10.1007/s00246-024-03527-4","url":null,"abstract":"<p><p>This is a case of an infant with duct-dependent pulmonary circulation, who required 6 stents delivered over three procedures to fully stent the arterial duct, which originated in a very unusual fashion. The attainable angiographic projections were unable to profile its origin, and only a CT scan was ultimately able to delineate the (stenotic) ductal origin from the aorta.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"736-739"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141262522","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}
Pub Date : 2025-03-01Epub Date: 2024-10-05DOI: 10.1007/s00246-024-03667-7
Mukesh Kumar, F N U Nandni, Sangeeta Davi, F N U Venjhraj, Falak Rehan
The article "Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome" offers valuable insights into Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome. This letter commends the study for its relevance and potential to improve patient care but highlights several limitations. Notably, the study overlooks the impact of Balloon angioplasty is a useful treatment for recoarctation in individuals with HLHS, but it frequently necessitates further operations. Following Norwood or Fontan palliation, stenting is safe, adaptable, and can be used to control uncommon neointimal growth. The risk of pulmonary artery stenosis may be decreased by the hemi-Fontan the technique. BA is better for recoarctation because it has fewer risks, whereas endovascular stenting helps high-risk patients avoid surgery.
{"title":"Response to \"Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome\".","authors":"Mukesh Kumar, F N U Nandni, Sangeeta Davi, F N U Venjhraj, Falak Rehan","doi":"10.1007/s00246-024-03667-7","DOIUrl":"10.1007/s00246-024-03667-7","url":null,"abstract":"<p><p>The article \"Pulmonary Flow Management by Combination Therapy of Hemostatic Clipping and Balloon Angioplasty for Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome\" offers valuable insights into Right Ventricular-Pulmonary Artery Shunt in Hypoplastic Left Heart Syndrome. This letter commends the study for its relevance and potential to improve patient care but highlights several limitations. Notably, the study overlooks the impact of Balloon angioplasty is a useful treatment for recoarctation in individuals with HLHS, but it frequently necessitates further operations. Following Norwood or Fontan palliation, stenting is safe, adaptable, and can be used to control uncommon neointimal growth. The risk of pulmonary artery stenosis may be decreased by the hemi-Fontan the technique. BA is better for recoarctation because it has fewer risks, whereas endovascular stenting helps high-risk patients avoid surgery.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"744-745"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378075","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}
Pub Date : 2025-03-01Epub Date: 2024-11-12DOI: 10.1007/s00246-024-03710-7
Huzafa Ali
In response to the article "Early Postoperative ECG Changes as a Predictor of Post-Pericardiotomy Syndrome Following Atrial Septal Defect Repair" by Hyberg et al. I commend the authors for their valuable insights into early ECG changes predicting postoperative complications. However, I raise several concerns regarding their study's methodology. Specifically, the use of European Society of Cardiology (ESC) criteria without incorporating pediatric-specific criteria, such as those proposed by Heching et al. potentially limits the study's applicability to younger patients. Additionally, reliance solely on ST-segment elevation and PR segment depression for assessing pericardial inflammation overlooks the significance of Spodick's sign. The lack of a standardized timing for postoperative ECGs and the omission of independent risk factors for Post-Pericardiotomy Syndrome (PPS), including history of pericarditis and pneumonia, further limit the study's comprehensiveness. I suggest that future research should address these aspects to refine diagnostic and monitoring strategies.
{"title":"Comment on: Early Post-operative ECG Changes as a Predictor of Post-pericardiotomy Syndrome Following Atrial Septal Defect Repair.","authors":"Huzafa Ali","doi":"10.1007/s00246-024-03710-7","DOIUrl":"10.1007/s00246-024-03710-7","url":null,"abstract":"<p><p>In response to the article \"Early Postoperative ECG Changes as a Predictor of Post-Pericardiotomy Syndrome Following Atrial Septal Defect Repair\" by Hyberg et al. I commend the authors for their valuable insights into early ECG changes predicting postoperative complications. However, I raise several concerns regarding their study's methodology. Specifically, the use of European Society of Cardiology (ESC) criteria without incorporating pediatric-specific criteria, such as those proposed by Heching et al. potentially limits the study's applicability to younger patients. Additionally, reliance solely on ST-segment elevation and PR segment depression for assessing pericardial inflammation overlooks the significance of Spodick's sign. The lack of a standardized timing for postoperative ECGs and the omission of independent risk factors for Post-Pericardiotomy Syndrome (PPS), including history of pericarditis and pneumonia, further limit the study's comprehensiveness. I suggest that future research should address these aspects to refine diagnostic and monitoring strategies.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"747-748"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142625525","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}
Pub Date : 2025-03-01Epub Date: 2024-05-18DOI: 10.1007/s00246-024-03491-z
Andrew A Lawson, Paul Tannous, Christina Laternser, Karl Migally
Cardiac index (CI) may be derived from the Fick method, using measured or estimated oxygen consumption (VO2), or from thermodilution. In children, LaFarge VO2 estimates correlate poorly with measured VO2 values. In a large adult cohort, there was only modest correlation between estimated Fick CI (eFick CI) and thermodilution CI (TDCI). We evaluated the extent of agreement between eFick CI using LaFarge estimates of VO2 and TDCI in a pediatric cohort. A retrospective, single-center chart review of patients 3-18 years of age who underwent cardiac catheterization with documented eFick CI and TDCI from 2004 to 2020 included 201 catheterizations from 161 unique patients. The mean patient age at catheterization was 12.2y (SD 4.4y). The most frequent diagnosis was cardiomyopathy, followed by congenital heart disease and pulmonary hypertension. TDCI and eFick CI differed by > 20% in 49% of catheterizations. eFick CI systematically exceeded TDCI by a mean percentage difference of 24% (SD 31%). Higher mean CI ((eFick CI + TDCI)/2) and older age were predictive of greater percent difference between eFick CI and TDCI. For each increase in mean CI by 1.0 L/min/m2, the expected percent difference in CI increased by 9.9% (p < 0.001). In pediatric patients undergoing cardiac catheterization, eFick CI with LaFarge VO2 systematically exceeds TDCI. The difference between methods is frequently > 20%, which may have clinically significant implications. Discrepancies between eFick CI and TDCI increase at higher mean CI.
心脏指数(CI)可通过菲克法、测量或估算的耗氧量(VO2)或热稀释法得出。在儿童中,LaFarge VO2 估计值与测量的 VO2 值相关性较差。在一个大型成人队列中,估计的菲克 CI(eFick CI)与热稀释 CI(TDCI)之间的相关性不大。我们评估了儿科队列中使用 LaFarge 估算 VO2 的 eFick CI 与 TDCI 之间的一致程度。我们对 2004 年至 2020 年期间接受心导管检查并记录有 eFick CI 和 TDCI 的 3-18 岁患者进行了回顾性单中心病历审查,其中包括 161 名患者的 201 次心导管检查。患者接受导管检查时的平均年龄为 12.2 岁(标清 4.4 岁)。最常见的诊断是心肌病,其次是先天性心脏病和肺动脉高压。在 49% 的导管检查中,TDCI 和 eFick CI 的差异大于 20%。较高的平均 CI((eFick CI + TDCI)/2)和较大的年龄可预测 eFick CI 与 TDCI 之间较大的百分比差异。平均 CI 每增加 1.0 升/分钟/平方米,CI 的预期差异百分比就会增加 9.9%(p 2 系统性地超过 TDCI)。不同方法之间的差异经常大于 20%,这可能会对临床产生重大影响。平均 CI 越高,eFick CI 与 TDCI 之间的差异就越大。
{"title":"The Estimated Fick Method Systematically Over-Estimates Cardiac Index Compared to Thermodilution in Children.","authors":"Andrew A Lawson, Paul Tannous, Christina Laternser, Karl Migally","doi":"10.1007/s00246-024-03491-z","DOIUrl":"10.1007/s00246-024-03491-z","url":null,"abstract":"<p><p>Cardiac index (CI) may be derived from the Fick method, using measured or estimated oxygen consumption (VO<sub>2</sub>), or from thermodilution. In children, LaFarge VO<sub>2</sub> estimates correlate poorly with measured VO<sub>2</sub> values. In a large adult cohort, there was only modest correlation between estimated Fick CI (eFick CI) and thermodilution CI (TDCI). We evaluated the extent of agreement between eFick CI using LaFarge estimates of VO<sub>2</sub> and TDCI in a pediatric cohort. A retrospective, single-center chart review of patients 3-18 years of age who underwent cardiac catheterization with documented eFick CI and TDCI from 2004 to 2020 included 201 catheterizations from 161 unique patients. The mean patient age at catheterization was 12.2y (SD 4.4y). The most frequent diagnosis was cardiomyopathy, followed by congenital heart disease and pulmonary hypertension. TDCI and eFick CI differed by > 20% in 49% of catheterizations. eFick CI systematically exceeded TDCI by a mean percentage difference of 24% (SD 31%). Higher mean CI ((eFick CI + TDCI)/2) and older age were predictive of greater percent difference between eFick CI and TDCI. For each increase in mean CI by 1.0 L/min/m<sup>2</sup>, the expected percent difference in CI increased by 9.9% (p < 0.001). In pediatric patients undergoing cardiac catheterization, eFick CI with LaFarge VO<sub>2</sub> systematically exceeds TDCI. The difference between methods is frequently > 20%, which may have clinically significant implications. Discrepancies between eFick CI and TDCI increase at higher mean CI.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"713-718"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140958743","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}
Duct-dependent pulmonary circulation has traditionally been addressed by the Blalock-Taussig-Thomas shunts (BTTS). Recently, catheter-based alternatives such as ductal stenting have emerged as a particularly advantageous option, especially in resource-constrained settings. This article delves into the nuances of ductal stenting within low-resource environments, highlighting its relative ease of application, reduced morbidity, and cost-effectiveness as key factors in its favor. Comparisons in mortality between the two procedures are however likely to be confounded by selection biases. Ductal stenting appears to be particularly beneficial for palliating older infants and children with cyanotic congenital heart disease and diminished pulmonary blood flow who present late. Additionally, it serves as a valuable tool for left ventricular training in late-presenting transposition with an intact ventricular septum. A meticulous pre-procedure echocardiographic assessment of anatomy plays a pivotal role in planning access and hardware, with additional imaging seldom required for this purpose. The adaptation of adult coronary hardware has significantly enhanced the technical feasibility of ductal stenting. However, challenges such as low birth weight and sepsis specifically impact the performance of ductal stenting and patient recovery in low-resource environments. There is potential for systematic application of quality improvement processes to optimize immediate and long-term outcomes of ductal stenting. There is also a need to prospectively examine the application of ductal stenting in low-resource environments through multi-center registries.
{"title":"Ductal Stenting in Low-Resource Environments.","authors":"Navaneetha Sasikumar, Pranoti Toshniwal, Praveen Reddy Bayya, Abish Sudhakar, Raman Krishna Kumar","doi":"10.1007/s00246-024-03496-8","DOIUrl":"10.1007/s00246-024-03496-8","url":null,"abstract":"<p><p>Duct-dependent pulmonary circulation has traditionally been addressed by the Blalock-Taussig-Thomas shunts (BTTS). Recently, catheter-based alternatives such as ductal stenting have emerged as a particularly advantageous option, especially in resource-constrained settings. This article delves into the nuances of ductal stenting within low-resource environments, highlighting its relative ease of application, reduced morbidity, and cost-effectiveness as key factors in its favor. Comparisons in mortality between the two procedures are however likely to be confounded by selection biases. Ductal stenting appears to be particularly beneficial for palliating older infants and children with cyanotic congenital heart disease and diminished pulmonary blood flow who present late. Additionally, it serves as a valuable tool for left ventricular training in late-presenting transposition with an intact ventricular septum. A meticulous pre-procedure echocardiographic assessment of anatomy plays a pivotal role in planning access and hardware, with additional imaging seldom required for this purpose. The adaptation of adult coronary hardware has significantly enhanced the technical feasibility of ductal stenting. However, challenges such as low birth weight and sepsis specifically impact the performance of ductal stenting and patient recovery in low-resource environments. There is potential for systematic application of quality improvement processes to optimize immediate and long-term outcomes of ductal stenting. There is also a need to prospectively examine the application of ductal stenting in low-resource environments through multi-center registries.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"525-535"},"PeriodicalIF":1.5,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140870802","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}
Pub Date : 2025-02-25DOI: 10.1007/s00246-025-03812-w
Joseph Andreola, Svetlana Shugh, Robert Winchester, Fredrick Fricker, Maryanne Chrisant, Laura D'Addese
Noonan syndrome is an autosomal dominant genetic condition associated with cardiac manifestations that may necessitate heart transplantation. This case series describes the short- and medium-term outcomes in five patients with Noonan syndrome status-post heart transplant followed at our institution. Retrospective, single center chart review of the electronic medical record in post-heart transplant patients with a diagnosis of Noonan syndrome. Five out of 88 heart transplant patients at our institution had genetically confirmed Noonan syndrome with either RAF1 or PTPN11 mutations. All patients were noted to have hypertrophic cardiomyopathy. Severe outflow tract obstruction, in conjunction with comorbidities such as intractable arrhythmias, recurrent syncope, and failure to thrive were leading indications for heart transplant. The most common complications post-heart transplant included recurrent viral infections and pleural and pericardial effusions. Isolated complications included lymphangiectasias, posterior reversible encephalopathy syndrome, and aspergillus pneumonia. Feeding difficulties were common. All patients are alive at the time of this publication. Noonan syndrome is highly associated with hypertrophic cardiomyopathy, and severe cases may necessitate heart transplantation. The post-heart transplant complications seen in our cohort are not unique to the Noonan syndrome population. Survival post-heart transplant is high.
{"title":"Heart Transplantation Outcomes in Pediatric Patients with Noonan Syndrome: An Institutional Case Series.","authors":"Joseph Andreola, Svetlana Shugh, Robert Winchester, Fredrick Fricker, Maryanne Chrisant, Laura D'Addese","doi":"10.1007/s00246-025-03812-w","DOIUrl":"https://doi.org/10.1007/s00246-025-03812-w","url":null,"abstract":"<p><p>Noonan syndrome is an autosomal dominant genetic condition associated with cardiac manifestations that may necessitate heart transplantation. This case series describes the short- and medium-term outcomes in five patients with Noonan syndrome status-post heart transplant followed at our institution. Retrospective, single center chart review of the electronic medical record in post-heart transplant patients with a diagnosis of Noonan syndrome. Five out of 88 heart transplant patients at our institution had genetically confirmed Noonan syndrome with either RAF1 or PTPN11 mutations. All patients were noted to have hypertrophic cardiomyopathy. Severe outflow tract obstruction, in conjunction with comorbidities such as intractable arrhythmias, recurrent syncope, and failure to thrive were leading indications for heart transplant. The most common complications post-heart transplant included recurrent viral infections and pleural and pericardial effusions. Isolated complications included lymphangiectasias, posterior reversible encephalopathy syndrome, and aspergillus pneumonia. Feeding difficulties were common. All patients are alive at the time of this publication. Noonan syndrome is highly associated with hypertrophic cardiomyopathy, and severe cases may necessitate heart transplantation. The post-heart transplant complications seen in our cohort are not unique to the Noonan syndrome population. Survival post-heart transplant is high.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502433","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}
Pub Date : 2025-02-25DOI: 10.1007/s00246-025-03807-7
Deborah U Frank, Cameron T Kasmai, Melissa M Winder, Ron W Reeder, Rebecca Bertrandt, Brooke Debroux, Priya Bhaskar, Stephanie P Schwartz, Amy Lay, Megan Matiasek, Tia T Raymond, Ashima Das, Alyssa N Bautista, Bao Nguyen Puente, AliGray Tollison, Alissa Lyman, Kathy Holmes, David K Bailly
Chylothorax following pediatric cardiac surgery increases morbidity and mortality. The clinical outcomes of patients with chylothorax with prolonged drainage compared to prompt resolution have not been described. This is a retrospective cohort study across eight United States pediatric cardiac intensive care units (ICU). Patients < 18 years old treated for chylothorax within 30 days of cardiac surgery were included, excluding Fontan palliations. Patients with chest tube duration ≥ 14 days were classified as long chylothorax (LC) vs. < 14 days as short chylothorax (SC). Univariable and multivariable logistic regression modeled patient characteristics associated with LC vs. SC. 134 patients had chylothorax, and 51 (38%) were LC. The proportion of LC increased with surgical complexity. LC was diagnosed later and had longer duration of mechanical ventilation, and ICU and hospital lengths of stay. In-hospital mortality was not different between groups. On POD 7, chest tube output (CTO, ml/kg) difference between LC and SC was greatest, with an area under the receiver operating characteristic curve of 0.76 for CTO predicting chylothorax. By multivariable analysis, clinical events associated with LC were sepsis or CLABSI (adjusted odds ratio (aOR) 8.8), postoperative open sternum (aOR 3.3), and CTO > 20 ml/kg on POD 7 (aOR 7.3). High chest tube output on POD 7 may predict LC in children post-cardiac surgery. LC is associated with increased resource utilization and morbidity. Early identification of patients at risk for LC may allow for tailored treatment strategies and improved outcomes.
{"title":"Refractory Chylothorax: Descriptive Analysis and Predictive Model in Children with Postoperative Chylothorax.","authors":"Deborah U Frank, Cameron T Kasmai, Melissa M Winder, Ron W Reeder, Rebecca Bertrandt, Brooke Debroux, Priya Bhaskar, Stephanie P Schwartz, Amy Lay, Megan Matiasek, Tia T Raymond, Ashima Das, Alyssa N Bautista, Bao Nguyen Puente, AliGray Tollison, Alissa Lyman, Kathy Holmes, David K Bailly","doi":"10.1007/s00246-025-03807-7","DOIUrl":"https://doi.org/10.1007/s00246-025-03807-7","url":null,"abstract":"<p><p>Chylothorax following pediatric cardiac surgery increases morbidity and mortality. The clinical outcomes of patients with chylothorax with prolonged drainage compared to prompt resolution have not been described. This is a retrospective cohort study across eight United States pediatric cardiac intensive care units (ICU). Patients < 18 years old treated for chylothorax within 30 days of cardiac surgery were included, excluding Fontan palliations. Patients with chest tube duration ≥ 14 days were classified as long chylothorax (LC) vs. < 14 days as short chylothorax (SC). Univariable and multivariable logistic regression modeled patient characteristics associated with LC vs. SC. 134 patients had chylothorax, and 51 (38%) were LC. The proportion of LC increased with surgical complexity. LC was diagnosed later and had longer duration of mechanical ventilation, and ICU and hospital lengths of stay. In-hospital mortality was not different between groups. On POD 7, chest tube output (CTO, ml/kg) difference between LC and SC was greatest, with an area under the receiver operating characteristic curve of 0.76 for CTO predicting chylothorax. By multivariable analysis, clinical events associated with LC were sepsis or CLABSI (adjusted odds ratio (aOR) 8.8), postoperative open sternum (aOR 3.3), and CTO > 20 ml/kg on POD 7 (aOR 7.3). High chest tube output on POD 7 may predict LC in children post-cardiac surgery. LC is associated with increased resource utilization and morbidity. Early identification of patients at risk for LC may allow for tailored treatment strategies and improved outcomes.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502550","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}