Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-04-30DOI: 10.1007/s00246-024-03504-x
Daniel Cheong, Qais Alloah, Joanna S Fishbein, Hari G Rajagopal
{"title":"Comparison and Agreement between Cardiovascular Computed Tomography-Derived Mid-Diastolic and End-Diastolic Ventricular Volume in Patients with Congenital Heart Disease.","authors":"Daniel Cheong, Qais Alloah, Joanna S Fishbein, Hari G Rajagopal","doi":"10.1007/s00246-024-03504-x","DOIUrl":"10.1007/s00246-024-03504-x","url":null,"abstract":"<p><p>Prospective electrocardiogram (ECG)-triggered cardiovascular computed tomography (CCT) is primarily utilized for anatomical information in congenital heart disease (CHD) and has not been utilized for calculation of the end-diastolic volume (EDV); however, the mid-diastolic volume (MDV) may be measured. The objective of this study was to evaluate the feasibility and agreement between ventricular EDV and MDV. 31 retrospectively ECG-gated CCT were analyzed for the study of the 450 consecutive CCT. CCT images were processed using syngo.via with automatic contouring followed by manual adjustment of the endocardial borders of the left ventricles (LV) and right ventricles (RV) at end-diastolic and mid-diastolic phase (measured at 70% of cardiac cycle). The correlation and agreements between EDV and MDV were demonstrated using Spearman rank coefficient and intraclass correlation coefficient (ICC), respectively. Mean age ± SD was 28.8 ± 12.5 years, 19 were male (61.3%) and tetralogy of Fallot (TOF) was the most common diagnosis (58.1%), 35% (11/31) patients with a pacemaker, ICD or other such contraindication for a CMRI, 23% (7/31) with claustrophobia, and 6.5% (2/31) with developmental delay with refusal for sedation did not have a previous CMRI. The mean ± SD indexed LV EDV and LV MDV were 91.1 ± 24.5 and 84.8 ± 22.3 ml/m<sup>2</sup>, respectively. The mean ± SD indexed RV EDV and RV MDV were 136.8 ± 41 and 130.2 ± 41.5 ml/m<sup>2</sup>, respectively. EDV and MDV had a strong positive correlation and good agreement (ICC 0.92 for LV and 0.95 for RV). This agreement was preserved in a subset of patients (21) with dilated RV (indexed RV EDV z-score > 2). Intra-observer reliability (0.97 and 0.98 for LV and RV MDV, respectively) and inter-observer reliability (0.96 and 0.90 for LV and RV MDV, respectively) were excellent. In a select group of patients with CHD, measuring MDV by CCT is feasible and these values have good agreements with EDV. This may be used to derive functional data from prospectively ECG-triggered CCT studies. Further large-scale analysis is needed to determine accuracy and clinical correlation.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"844-852"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861136","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-05-03DOI: 10.1007/s00246-024-03497-7
Jennifer Walter, Douglas L Hill, Arzu Cetin, Aaron DeWitt, Katie Kellom, William Quarshie, Heather Griffis, Justine Shults, Robert Arnold, Jennifer Tjia, Karen Puopolo, Martha A Q Curley, Chris Feudtner
{"title":"A Pediatric Interprofessional Cardiac Intensive Care Unit Intervention: CICU Teams and Loved Ones Communicating (CICU TALC) is Feasible, Acceptable, and Improves Clinician Communication Behaviors in Family Meetings.","authors":"Jennifer Walter, Douglas L Hill, Arzu Cetin, Aaron DeWitt, Katie Kellom, William Quarshie, Heather Griffis, Justine Shults, Robert Arnold, Jennifer Tjia, Karen Puopolo, Martha A Q Curley, Chris Feudtner","doi":"10.1007/s00246-024-03497-7","DOIUrl":"10.1007/s00246-024-03497-7","url":null,"abstract":"<p><p>Parents of children in the pediatric cardiac intensive care unit (CICU) are often unprepared for family meetings (FM). Clinicians often do not follow best practices for communicating with families, adding to distress. An interprofessional team intervention for FM is feasible, acceptable, and positively impacts family preparation and conduct of FM in the CICU. We implemented a family- and team-support intervention for conducting FM and conducted a pretest-posttest study with parents of patients selected for a FM and clinicians. We measured feasibility, fidelity to intervention protocol, and parent acceptability via questionnaire and semi-structured interviews. Clinician behavior in meetings was assessed through semantic content analyses of meeting transcripts tracking elicitation of parental concerns, questions asked of parents, and responses to parental empathic opportunities. Logistic and ordinal logistic regression assessed intervention impact on clinician communication behaviors in meetings comparing pre- and post-intervention data. Sixty parents (95% of approached) were enrolled, with collection of 97% FM and 98% questionnaire data. We accomplished > 85% fidelity to intervention protocol. Most parents (80%) said the preparation worksheet had the right amount of information and felt positive about families receiving this worksheet. Clinicians were more likely to elicit parental concerns (adjusted odds ratio = 3.42; 95%CI [1.13, 11.0]) in post-intervention FM. There were no significant differences in remaining measures. Implementing an interprofessional team intervention to improve family preparation and conduct of FM is locally feasible, acceptable, and changes clinician behaviors. Future research should assess broader impact of training on clinicians, patients, and families.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"785-797"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11531608/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140853792","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-05-21DOI: 10.1007/s00246-024-03522-9
Adriana Innocenzi, Isabela Rangel, Mariana Póvoa-Corrêa, Daniella Braz Parente, Renata Perez, Rosana Souza Rodrigues, Lúcia Tomoko Fukuyama, Julia Machado Barroso, Jaime Araújo Oliveira Neto, Andréa Silvestre de Sousa, Ronir Raggio Luiz, Rosa Célia Pimentel Barbosa, Gabriel Cordeiro Camargo, Renata Moll-Bernardes
{"title":"Cardiac and Liver Fibrosis Assessed by Multiparametric MRI in Patients with Fontan Circulation.","authors":"Adriana Innocenzi, Isabela Rangel, Mariana Póvoa-Corrêa, Daniella Braz Parente, Renata Perez, Rosana Souza Rodrigues, Lúcia Tomoko Fukuyama, Julia Machado Barroso, Jaime Araújo Oliveira Neto, Andréa Silvestre de Sousa, Ronir Raggio Luiz, Rosa Célia Pimentel Barbosa, Gabriel Cordeiro Camargo, Renata Moll-Bernardes","doi":"10.1007/s00246-024-03522-9","DOIUrl":"10.1007/s00246-024-03522-9","url":null,"abstract":"<p><p>The abnormal hemodynamics in Fontan circulation due to persistently increased systemic venous pressure results in hepatic venous congestion and Fontan-associated liver disease. Combined assessment of cardiac and liver fibrosis and cardiac remodeling using multiparametric MRI in this context have not been fully explored. To evaluate cardiac and liver fibrosis and cardiac remodeling using multiparametric MRI in patients who have undergone Fontan procedures. Thirty-eight patients and 23 controls underwent cardiac and liver MRI examinations in a 3.0-T scanner. Mann-Whitney, Fisher exact test, and Spearman's correlation were applied to evaluate myocardial volumes, function, native cardiac and liver T1 mapping, ECVs and liver stiffness. The mean native cardiac T1 value (p = 0.018), cardiac ECV (p < 0.001), liver native T1 (p < 0.001), liver ECV (p < 0.001), and liver stiffness (p < 0.001) were higher in patients than controls. The indexed end-diastolic volume (EDVi) correlated with the myocardial ECV (r = 0.356; p = 0.033), native liver T1 (r = 0.571; p < 0.001), and with liver stiffness (r = 0.391; p = 0.015). In addition, liver stiffness correlated with liver ECV (r = 0.361; p = 0.031) and native liver T1 (r = 0.458; p = 0.004). An association between cardiac remodeling and cardiac and liver fibrosis were found in this population. The usefulness of MRI to follow cardiac and liver involvement in these patients is critical to improve treatment strategies and to prevent the need for combined liver and heart transplantation.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"966-975"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11903587/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141070991","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-06-07DOI: 10.1007/s00246-024-03503-y
Benjamin R Ittleman, Justin T Tretter, Anna S Bader, Sarah Mcollum, Veronika Shabanova, Jeremy M Steele
{"title":"Longitudinal Evaluation of Atrial Function in Patients with Tetralogy of Fallot.","authors":"Benjamin R Ittleman, Justin T Tretter, Anna S Bader, Sarah Mcollum, Veronika Shabanova, Jeremy M Steele","doi":"10.1007/s00246-024-03503-y","DOIUrl":"10.1007/s00246-024-03503-y","url":null,"abstract":"<p><p>Atrial function provides insight into ventricular diastolic function. Invasive assessment of left ventricular (LV) diastolic function correlates with development of sustained ventricular tachyarrhythmias in patients with repaired tetralogy of Fallot (rTOF). Non-invasive assessment of atrial function may prove key towards assessment of diastolic function. We longitudinally evaluated the progression of biatrial function in patients with rTOF, regardless of pulmonary valve replacement (PVR). Patients with rTOF who had multiple CMR were identified. CMR examinations were retrospectively reviewed. Left (LA) and right (RA) atrial size and function were measured in the two and four-chamber views and assessed over time and after PVR. Left and right atrial reservoir, conduit, pump strain and strain rates were determined using tissue tracking. Thirty-six patients with rTOF were identified (64% male), ten (28%) had PVR during the study. Median age of PVR was 16.5 years. No improvement in RA or LA function was observed after PVR. A decline in RA reservoir strain rate (p < 0.05) and RA pump strain (p < 0.05) were observed despite improvements in right ventricular systolic function (p < 0.05). In patients who had multiple CMR without PVR, RA reservoir strain rate (p < 0.05) and pump strain rate (p < 0.05) worsened over time. LA pump strain decreased over time in all patients. There is progressive decline of several RA functional parameters over time. No significant improvement in LA or RA function after PVR was observed. Additional studies are needed to understand how these changes may relate to poor outcomes and potentially better guide timing of PVR.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"835-843"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141288381","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-06-12DOI: 10.1007/s00246-024-03519-4
Brennan V Higgins, Philip T Levy, Molly K Ball, Minso Kim, Shabnam Peyvandi, Martina A Steurer
{"title":"Double Jeopardy: A Distinct Mortality Pattern Among Preterm Infants with Congenital Heart Disease.","authors":"Brennan V Higgins, Philip T Levy, Molly K Ball, Minso Kim, Shabnam Peyvandi, Martina A Steurer","doi":"10.1007/s00246-024-03519-4","DOIUrl":"10.1007/s00246-024-03519-4","url":null,"abstract":"<p><p>Contemporary United States (US) data on the survival of preterm infants with congenital heart disease (CHD) are unavailable despite the over-representation of CHD and improving surgical outcomes in the preterm population. The aim of this study is to use population-based data to compare 1-year survival and early mortality (< 3 days) by gestational age (GA) between preterm infants with and without cyanotic CHD (CCHD) in the US. This national retrospective cohort included all liveborn, preterm infants between 21 and 36 weeks GA with a birth certificate indicating the presence or absence of CCHD (n = 2,654,253) born between 2014 and 2019 in the US. Data were provided by the US Center for Disease Control database linking birth and death certificates. Of liveborn preterm infants, 0.13% (n = 3619) had CCHD. 1-year survival was significantly lower in infants 23-36 weeks with CCHD compared to those without. The greatest survival gap occurred between 28 and 31 weeks (28 weeks adjusted risk difference 37.5%; 95% CI 28.4, 46.5; 31 weeks 37.9%; 30.5, 45.3). Early mortality accounted for more than half of deaths among infants 23-31 weeks with CCHD (23 weeks-68%, CI 46.7, 83.7; 31 weeks-63.9%, 52.9, 73.6). Survival trends demonstrated worsened 1-year survival in infants 35-36 weeks with CCHD over the study period. The pattern of mortality for preterm infants with CCHD is distinct from those without. The significant survival gap in the very preterm population and notably high rate of early death in the infants with CCHD calls for renewed attention to early neonatal intensive care for this dually affected population.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"939-946"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11903637/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141306504","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-05-10DOI: 10.1007/s00246-024-03513-w
Madeline Grossman, Stephen Walker, E Zachary Ramsey
{"title":"Selexipag Dosing Strategies for Pediatric Patients with Pulmonary Arterial Hypertension.","authors":"Madeline Grossman, Stephen Walker, E Zachary Ramsey","doi":"10.1007/s00246-024-03513-w","DOIUrl":"10.1007/s00246-024-03513-w","url":null,"abstract":"<p><p>This retrospective chart review of patients less than 18 years old with pulmonary arterial hypertension (PAH) receiving selexipag was conducted to describe selexipag dosing practices, impact on concomitant PAH therapies, and the safety and efficacy of selexipag. Twenty-seven patients aged 1-17 years started a median dose of oral selexipag 100 µg twice daily. Therapy was increased by a median of 100 µg twice daily every 6 days to a maximally tolerated median dose of 800 µg twice daily. All 24 patients on another prostacyclin derivative were able to discontinue therapy at their maximum tolerated selexipag dose; other concomitant PAH therapies did not change. Changes in echocardiogram data and 6-MWT results were variable. No patients discontinued selexipag; four patients received decreased doses due to flushing (n = 1), drug interactions (n = 2), or increased frequency of nose bleeds (n = 1).</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"902-907"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140898717","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":"Response to \"Health-Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot: Insights from the Congenital Cardiac Research Collaborative\".","authors":"Zainab Muhammad Hanif, Muzna Murtaza, Syeda Zuha Sami, Ariba Nazir, Fnu Venjhraj","doi":"10.1007/s00246-024-03721-4","DOIUrl":"10.1007/s00246-024-03721-4","url":null,"abstract":"<p><p>This piece of writing commends the article \"Health Related Quality of Life After Neonatal Treatment of Symptomatic Tetralogy of Fallot\" for its contributions to pediatric cardiology while noting key limitations. The limited sample size may limit generalizability, and further investigation of comorbidities is required to understand their influence on health-related quality of life (HRQOL). The lack of a control group limits interpretation, and longer follow-up periods are required to capture the changing obstacles experienced by children after therapy. Addressing these challenges will provide better insights into HRQOL for children with symptomatic Tetralogy of Fallot and improve treatment procedures.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"1125-1126"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142709523","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-06-28DOI: 10.1007/s00246-024-03524-7
Danton Mhd, Hadjisoteriou A, Noonan P, Young D, Burns P
{"title":"Cardiopulmonary Exercise Testing in Children and Adolescents with Repaired Tetralogy of Fallot: Mechanisms of Exercise Intolerance and Clinical Implications.","authors":"Danton Mhd, Hadjisoteriou A, Noonan P, Young D, Burns P","doi":"10.1007/s00246-024-03524-7","DOIUrl":"10.1007/s00246-024-03524-7","url":null,"abstract":"<p><p>By comparison with adults, cardiopulmonary exercise testing in children with Tetralogy of Fallot is limited, and its clinical application less clarified. This study provides a comprehensive CPET profile in a child-adolescent population with repaired TOF, explores mechanisms underpinning exercise intolerance and associations with clinical outcome. Seventy-four CPETs were completed in 58 child-adolescents with rTOF (age 13.8 SD 2.4 years). CPET parameters were corrected for age, sex and body size. At follow-up (4.9 years, IQR 3.5-7.9) clinical status and re-intervention was evaluated and CPET indices predicting these outcomes determined. Cohort peak V̇O<sub>2</sub> was within low-normal limits (% pred: 74.1% SD 15.4) with 15 patients (26%) displaying moderately severe reduction in V̇O<sub>2peak</sub> (< 65% pred). Oxygen uptake efficiency slope highly correlated with V̇O<sub>2peak</sub> (r = 0.94, p < 0.001) and was insensitive to exercise intensity. No significant change in CPET occurred in patients who underwent interval testing at 24 SD 14.5 months, although there was a variable response in V̇O<sub>2peak</sub> between individuals. Chronotropic response, lung vital capacity, heart rate-V̇O<sub>2</sub> slope (indicator of stroke volume) predicted oxygen consumption: V̇O<sub>2peak</sub> (R<sup>2</sup> = 50.91%, p < 0.001) and workload (R<sup>2</sup> = 58.39%, p < 0.001). Adverse clinical status was associated with reduced workload (OR 0.97, p = 0.011). V̇<sub>E</sub>/V̇<sub>CO2</sub> slope was steeper in those that died ((%pred:137.8 SD 60.5 vs. 108.4 SD 17.0, p < 0.019). RVOT reintervention post-CPET (24 patients, 43.8%) was associated with an increased gradient of HR-VO<sub>2</sub> slope (OR 1.042, p = 0.004). In child-adolescents with TOF important reductions in cardiopulmonary functioning were apparent in 25% of patients. Exercise intolerance was related to reduced vital capacity, impaired chronotropic response and deficient stroke volume increment.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"985-999"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141469982","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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-06-15DOI: 10.1007/s00246-024-03510-z
Stephanie N Nguyen, Jan M Quaegebeur, Rozelle Corda, Amee Shah, Matan I Setton, Emile A Bacha, Andrew B Goldstone
{"title":"Ventricular Septation of the Double-Inlet Ventricle: Over Three Decades of Follow-Up.","authors":"Stephanie N Nguyen, Jan M Quaegebeur, Rozelle Corda, Amee Shah, Matan I Setton, Emile A Bacha, Andrew B Goldstone","doi":"10.1007/s00246-024-03510-z","DOIUrl":"10.1007/s00246-024-03510-z","url":null,"abstract":"<p><p>There is renewed interest in septation of the double-inlet ventricle as an alternative to Fontan palliation. We examined our septation experience with over 30 years of follow-up. We retrospectively reviewed patients with double-inlet ventricle from 1990 to 2011. Patients with two adequate atrioventricular valves, a volume-overloaded ventricle, and no significant subaortic obstruction were septation candidates. Of 98 double-inlet ventricle patients, 9 (9.2%) underwent attempted septation via a one-stage (n = 2, 22.2%) or two-stage (n = 7, 77.8%) approach. Ages at primary septation were 7.5 and 20.2 months. In the staged group, median age at the first and second stage was 8.3 months [range 4.1-14.7] and 22.4 months [range 11.4-195.7], respectively. There were no operative mortalities. Median follow-up was 18.8 years [range 0.4-32.9] and 30-year transplant-free survival was 77.8% ± 13.9%. Both single stage patients are alive and in sinus rhythm; 1 underwent bilateral outflow tract obstruction repair 27 years later. Of 7 patients planned for two-stage septation, there was 1 interval mortality and 1 deferred the second stage. Five patients underwent the second stage; 1 required early reintervention for a residual neo-septal defect and 1 underwent right atrioventricular valve replacement 28 years later. Three patients required a pacemaker preoperatively (n = 1) or after partial septation (n = 2). At latest follow-up, 7 patients have normal biventricular function and no significant valvulopathy. All remain NYHA functional class I. Select double-inlet ventricles may be septated with excellent long-term outcomes. Reconsideration of this strategy is warranted to avoid the sequelae of Fontan circulation.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"874-883"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141327765","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":"Transcatheter Closure of Perimembranous Ventricular Septal Defect Using KONAR-MF™: A Multicenter Experience.","authors":"Nageswara Rao Koneti, Sushil Azad, Shweta Bakhru, Bhargavi Dhulipudi, Radhakrishnan Sitaraman, Raman Krishna Kumar","doi":"10.1007/s00246-024-03505-w","DOIUrl":"10.1007/s00246-024-03505-w","url":null,"abstract":"<p><p>Transcatheter closure of perimembranous ventricular septal defect (PmVSD) is an established procedure. However, the occurrence of complete heart block limits its scope. The newer KONAR-MF™ occluder has specific design characteristics that may improve the safety of PmVSD closure. The objective of the study was to describe the efficacy and mid-term follow-up of transcatheter closure of PmVSD using KONAR-MF™. The study was conducted prospectively in 3 Indian centers (January 2018-December 2022). PmVSD closure was done by both antegrade and retrograde methods, and patients were followed up at 1, 3, 6, 12 months, and annually after that. 121 out of 123 patients were included with the following characteristics: median age 4.4 (0.18-40) years; weight 15 (2.1-88) kg; mean Qp/Qs ratio 1.87 ± 0.52 and pulmonary artery mean pressure: 22 ± 6.9 mmHg. The procedure was successful in all but 3; the device was removed due to significant residual shunt (n = 2) and new development of aortic regurgitation (AR) (≥ mild) in 1. The median defect size was 5.2 (2.5-12) mm. Device sizes from 6/4 to 14/12 were deployed (median fluoroscopy time 13.3 min; range 3.6-47.8). Shunt occlusion rates were 90%-Immediate, 95%-pre-discharge, and 97%-1 month, with no instances of complete heart block after the procedure and during follow-up. Six had new onset AR (mild: 2, trivial 4), and one had increased tricuspid regurgitation. All patients were well during follow-up (median: 20 months; range: 6-46). The new KONAR-MF™ occluder appears to be a promising and safe alternative for the closure of the PmVSD; further long-term follow is merited.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"853-861"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140870803","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}