{"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}
Neha Bansal, Nadia Ovchinsky, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Nadine Choueiter
{"title":"Comment on: Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.","authors":"Neha Bansal, Nadia Ovchinsky, Joseph Mahgerefteh, Jacqueline M Lamour, Debora Kogan-Liberman, Nadine Choueiter","doi":"10.1007/s00246-025-03818-4","DOIUrl":"https://doi.org/10.1007/s00246-025-03818-4","url":null,"abstract":"<p><p>The authors recognize the limitations of our study, most of which are due to being a single-center study and the small sample size of our cohort. We tried to keep our population as homogeneous as possible and included patients only with cirrhosis. Imaging modalities like cardiac MRI, 3D echocardiography, and tissue Doppler imaging as well as exercise testing often give us significantly more information about cardiac function but come with their inherent limitations of requiring general anesthesia, limited availability, and age-dependent variability. We agree with both reviewers that our study lacks the longitudinal aspect which would allow us to study the reversibility of findings post-liver transplantation. However, the purpose of our study was to correlate the pre-transplantation echocardiographic findings as a risk stratification method for post-transplantation clinical measures like duration of mechanical ventilation and length of hospital stay. We believe that the results of our study can serve as a basis for further prospective studies in the evaluation of patients prior to liver transplantation as well as demonstrating reversal of findings in long-term post-transplant period.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524045","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":"Early and Midterm Reintervention in Valve-Sparing Tetralogy of Fallot Repair: Role of Residual Lesion Score.","authors":"Javeria Tariq, Asefa Shariq Ansari, Akbar Mistry, Qalab Abbas, Waris Ahmad, Muneer Amanullah, Mahim Malik, Shazia Mohsin","doi":"10.1007/s00246-025-03813-9","DOIUrl":"https://doi.org/10.1007/s00246-025-03813-9","url":null,"abstract":"<p><p>Tetralogy of Fallot (TOF) is a common congenital heart defect with a traditionally high reintervention rate of post-surgical correction. The Residual Lesion Score (RLS) is a tool used to evaluate the quality of surgical outcomes by assessing postoperative echocardiography findings and the need for unplanned reinterventions. The study assesses the association between the Residual Lesion Score (RLS) and early and midterm outcomes, including reintervention rates, in patients undergoing valve-sparing TOF repair at a tertiary care center in a low- and middle-income country (LMIC). We conducted a retrospective analysis of 62 patients who underwent valve-sparing TOF repair between January 2017 and December 2019 at Aga Khan University, Pakistan. The RLS was calculated based on postoperative echocardiograms and unplanned surgical or catheter-based reinterventions. Patients were categorized into RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), and RLS 3 (major residual lesions or reinterventions before discharge). Two (3.2%) patients lacked postoperative echocardiography, were assigned an RLS score of 5, and excluded from further analysis. Of the 60 patients, 13 (21%) had an RLS of 3, indicating major residual lesions, and 29 (48.3%) had an RLS of 2, indicating minor residual lesions. Early reinterventions were required in 11.7% of patients. Patients with RLS 3 had a significantly higher reintervention rate compared to those with RLS 2 (p = 0.003) reintervention and longer hospital stay (p-value = 0.3). Implementing RLS can help optimize postoperative management and patient outcomes by identifying patients at higher risk of early reintervention.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516288","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}
Daisuke Kobayashi, Dominic B Zanaboni, Andrew C Glatz, David Balzer
{"title":"Significant Practice Variation in Oxygen Consumption Values in Congenital Cardiac Catheterization: Cross Sectional Survey in the United States.","authors":"Daisuke Kobayashi, Dominic B Zanaboni, Andrew C Glatz, David Balzer","doi":"10.1007/s00246-025-03817-5","DOIUrl":"https://doi.org/10.1007/s00246-025-03817-5","url":null,"abstract":"<p><p>Oxygen consumption (VO<sub>2</sub>) is an essential component to calculate cardiac output using the Fick principle in the congenital cardiac catheterization laboratory (CCCL). Although VO<sub>2</sub> can be measured, the value of VO<sub>2</sub> is frequently assumed based on predictive equations. The objective of this study was to investigate current practice variation in measurement and estimation of VO<sub>2</sub> in CCCL. This was a cross-sectional survey study conducted in January-February 2024. The survey (n = 114) was sent to one interventional cardiologist for each CCCL program in the United States utilizing REDCap. The responses were summarized in a descriptive fashion. Eighty eight programs completed the survey (response rate 77%). Only 13 (15%) programs measured VO<sub>2</sub>, though 67 programs (76%) thought VO<sub>2</sub> should be measured for all cases (n = 41) and selective cases (n = 26) and 17 programs thought assumed VO<sub>2</sub> was satisfactory. When VO<sub>2</sub> is referred from predictive equations, most programs primarily used LaFarge (n = 64, 73%), followed by Seckeler (n = 23, 26%). For patients aged < 3 years (where LaFarge equation does not provide VO2 value) in 64 programs using LaFarge, 16 used Seckeler and the remaining 48 programs used a wide range of arbitrary VO<sub>2</sub> values. Most CCCL thought VO<sub>2</sub> should be measured but only 13 (15%) programs actually measured VO<sub>2</sub> in the United States. LaFarge remains frequently used as a predictive equation. There is no standardization of clinical practice in VO<sub>2</sub> measurement/estimation among CCCL in the United States.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516290","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}
Joseph Andreola, Svetlana Shugh, Robert Winchester, Fredrick Fricker, Maryanne Chrisant, Laura D'Addese
{"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}
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
{"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}
Nikkan Das, Havisha Pedamallu, Kristen Young, Laura H Rosenthal, Taher Valika, Andrada R Popescu, Allison B Davila, Osama M Eltayeb, Stefani M Samples, Michael R Carr, Angira Patel, Sheetal R Patel
{"title":"Postnatal Outcomes in Prenatally Detected Vascular Rings.","authors":"Nikkan Das, Havisha Pedamallu, Kristen Young, Laura H Rosenthal, Taher Valika, Andrada R Popescu, Allison B Davila, Osama M Eltayeb, Stefani M Samples, Michael R Carr, Angira Patel, Sheetal R Patel","doi":"10.1007/s00246-025-03803-x","DOIUrl":"https://doi.org/10.1007/s00246-025-03803-x","url":null,"abstract":"<p><p>Vascular rings are rare congenital defects that can cause tracheal and/or esophageal compression. Prenatal detection is increasing due to advances in screening and fetal echocardiography. Postnatal outcomes remain variable. We describe our single-center experience of postnatal outcomes in prenatally detected vascular rings and evaluate factors associated with surgery. We performed a retrospective review of all fetal diagnoses of possible vascular ring evaluated between 2016 and 2023. Patients with significant intracardiac abnormalities, without postnatal data, or without confirmed postnatal diagnosis were excluded from postnatal analysis. Outcome variables included symptoms, tracheal/esophageal compression, and surgical repair. The prenatal cohort included 109 patients with 80% right aortic arch (RAA) and 20% double aortic arch (DAA). Of 72 patients in the postnatal cohort, 85% underwent computed tomography angiography (CTA) at median age of 2 months with 0.84 ± 0.34 mSv of radiation. On CTA, 69% had a diverticulum. Of those with RAA, 95% had an aberrant left subclavian. Most (79%) had airway abnormalities on CTA. Fifteen (21%) developed symptoms at median age of 5 months. Fifty-eight percent of patients underwent surgery at median age of 10 months, of which 33% were due to symptoms. On univariate analysis, DAA was associated with airway abnormalities on CTA and surgical repair. At 30-month follow up, 99% of patients remained asymptomatic. Prenatal diagnosis of vascular rings is associated with high rates of airway abnormalities, even in those without symptoms. Surgical repair was pursued in asymptomatic patients with DAA and airway abnormalities in our institution. As DAA is associated with airway abnormalities, these patients may require closer monitoring for future symptom development.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492773","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":"Comment on: Evaluation of Cardiac Function in Children Undergoing Liver Transplantation.","authors":"Arafat Hassan Tariq, Huzaifa Ali","doi":"10.1007/s00246-025-03804-w","DOIUrl":"https://doi.org/10.1007/s00246-025-03804-w","url":null,"abstract":"<p><p>In response to the article \"Evaluation of Cardiac Function in Children Undergoing Liver Transplantation\" by Bansal et al., I admire the authors for their valuable insights in evaluating biventricular heart function in pediatric patients undergoing Liver Transplantation. However, I bring up several key concerns regarding study's methodology. The diversity of the pediatric cirrhosis population is limited by the study's small sample size, which consists of only 35 children patients. Advanced diagnostic techniques including tissue Doppler echocardiography and cardiovascular magnetic resonance (CMR), which are valuable for diagnosis of heart diseases, are not included in the study. The lack of a reference group of patients without cirrhosis makes it more difficult to confirm whether the cardiac dysfunction shown is unique to cirrhosis or indicates other chronic disease. Additionally, the study does not provides information regarding the outcomes following liver transplantation especially reversibility of cardaic abnormalities.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476745","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":"Are Arrhythmias Adequately Incorporated into Studies on Long-Term Outcomes After the Fontan Operation?","authors":"Natalie Pexton, Seshadri Balaji","doi":"10.1007/s00246-025-03789-6","DOIUrl":"https://doi.org/10.1007/s00246-025-03789-6","url":null,"abstract":"<p><p>Arrhythmias are common after the Fontan operation and increase risk of morbidity and mortality. Their impact on late outcomes remains poorly understood. Our objective was to assess whether long-term studies after the Fontan incorporated the impact of arrhythmia characteristics on outcome. We reviewed the literature since 2005 for studies evaluating the impact of multiple risk factors on long-term outcomes and mortality after the Fontan. Studies focusing only on one or two risk factors (including arrhythmias) and those with a mean follow-up < 10 years were excluded. Of the 17 studies analyzed, only six mentioned rhythm at follow-up. Nine lumped all arrhythmias (tachyarrhythmia, bradyarrhythmia, paced rhythm, early and late arrhythmia) into one single category. Only eight of the 17 studies reported the incidence of tachyarrhythmias in detail and only two studies described bradyarrhythmias in detail as sinus or atrioventricular (AV) node dysfunction subgroups. Five studies reported on treatment of tachyarrhythmias including Fontan revision surgeries, catheter ablation, pacemaker placement and or the use of antiarrhythmic drugs. However, only one included information on the impact of treatment on outcomes. Out of 15 studies that conducted a multivariate analysis of Fontan outcomes, arrhythmia was included in the univariate model in 14 and in the multivariate model in 9. Overall, we found most studies on late outcomes after the Fontan do not include rhythm and arrhythmia characteristics in data collection and analysis. Incorporating more granular rhythm information in large databases and in multivariate analyses will help better determine and contextualize the impact of arrhythmias on outcomes after the Fontan.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143472786","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}
Taylor S Howard, Michael A Bruno, Hari Tunuguntla, Edward J Hickey, Santiago O Valdes, Christina Y Miyake, John Seger
{"title":"Leadless Pacemaker Implantation in an Adolescent with Durable LVAD Support.","authors":"Taylor S Howard, Michael A Bruno, Hari Tunuguntla, Edward J Hickey, Santiago O Valdes, Christina Y Miyake, John Seger","doi":"10.1007/s00246-025-03801-z","DOIUrl":"https://doi.org/10.1007/s00246-025-03801-z","url":null,"abstract":"<p><p>Left ventricular assist device (LVAD) utilization is increasing in pediatrics. These patients are often complex and may require treatment of AV block. Leadless pacing has been associated with a lower risk of device-related adverse events, but there are no reports of a leadless pacemaker implant in a pediatric patient with LVAD support. This report describes the use of a leadless pacemaker implantation in a 17-year-old patient with dilated cardiomyopathy and continuous flow LVAD support which facilitated transition to outpatient care.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143472787","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}