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}
Pediatric CardiologyPub Date : 2025-04-01Epub Date: 2024-05-07DOI: 10.1007/s00246-024-03498-6
Justin Godown, Emily H Kim, Melanie D Everitt, Wendy K Chung, Irene D Lytrivi, Sonya Kirmani, Paul F Kantor, Stephanie M Ware, Jean A Ballweg, Ashwin K Lal, Neha Bansal, Jeffrey Towbin, Steven E Lipshultz, Teresa M Lee
{"title":"Genetic Testing Resources and Practice Patterns Among Pediatric Cardiomyopathy Programs.","authors":"Justin Godown, Emily H Kim, Melanie D Everitt, Wendy K Chung, Irene D Lytrivi, Sonya Kirmani, Paul F Kantor, Stephanie M Ware, Jean A Ballweg, Ashwin K Lal, Neha Bansal, Jeffrey Towbin, Steven E Lipshultz, Teresa M Lee","doi":"10.1007/s00246-024-03498-6","DOIUrl":"10.1007/s00246-024-03498-6","url":null,"abstract":"<p><p>The use of genetic testing has enhanced the diagnostic accuracy of heritable genetic cardiomyopathies. However, it remains unclear how genetic information is interpreted and incorporated into clinical practice for children with cardiomyopathy. The primary aim of this study was to understand how clinical practice differs regarding sequence variant classifications amongst pediatric cardiologists who treat children with cardiomyopathy. A secondary aim was to understand the availability of genetic testing and counseling resources across participating pediatric cardiomyopathy programs. An electronic survey was distributed to pediatric heart failure, cardiomyopathy, or heart transplantation physicians between August and September 2022. A total of 106 individual providers from 68 unique centers responded to the survey. Resources for genetic testing and genetic counseling vary among large pediatric cardiomyopathy programs. A minority of centers reported having a geneticist (N = 16, 23.5%) or a genetic counselor (N = 21, 31%) on faculty within the division of pediatric cardiology. A total of 9 centers reported having both (13%). Few centers (N = 13, 19%) have a formal process in place to re-engage patients who were previously discharged from cardiology follow-up if variant reclassification would alter clinical management. Clinical practice patterns were uniform in response to pathogenic or likely pathogenic variants but were more variable for variants of uncertain significance. Efforts to better incorporate genetic expertise and resources into the clinical practice of pediatric cardiomyopathy may help to standardize the interpretation of genetic information and better inform clinical decision-making surrounding heritable cardiomyopathies.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"798-803"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11903623/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140877025","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: 2024-05-16DOI: 10.1007/s00246-024-03509-6
Irene E Regan, Dermot Cox, Sean T Kelleher, Colin J McMahon
{"title":"The Effects of Aspirin dose in Children with Congenital and Acquired Heart Disease. Results from the Paediatric Study of Aspirin Efficacy using Diagnostic and Monitoring Tools (PAED-M).","authors":"Irene E Regan, Dermot Cox, Sean T Kelleher, Colin J McMahon","doi":"10.1007/s00246-024-03509-6","DOIUrl":"10.1007/s00246-024-03509-6","url":null,"abstract":"<p><p>The optimal dose of aspirin required in children with congenital and acquired heart disease is not known. The primary aim of this prospective observational study was to evaluate the effects of aspirin dose on platelet inhibition. The secondary aim was to determine the prevalence and clinical predictors of aspirin non-responsiveness. Measurements were by Thromboelastography with Platelet Mapping (TEGPM) only in children less than 2 years (y) of age with particular emphasis on the parameter known as maximum amplitude with arachidonic acid (MAAA) and using both TEGPM, and light transmission aggregometry (LTA) in children greater than 2 y. We prospectively studied 101 patients with congenital and acquired cardiac disease who were receiving empirical doses of aspirin for a minimum of 4 weeks but no other antiplatelet agents. Patients were stratified according to dose concentration and age. There was a trend toward lower age in patients with no response or semi-response to aspirin. All patients were considered responsive to aspirin in the higher-dose quartile (Q4) with a median dose of 4.72 (4.18-6.05) mg/kg/day suggesting that patients in this age group may require 5 mg/kg/day as an empirical dose. In children > 2 y, there was no significant difference in inhibition found in patients dosed at higher doses in Q3 versus Q4 suggesting that patients in this cohort are responsive with 3 mg/kg/day dose. The current practices may lead to reduced platelet inhibition in some children due to under-dosing or overdosing in others. In conclusion, younger children require higher doses of aspirin. Laboratory assessment is warranted in this population to mitigate against under and overdosing.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"862-873"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140958742","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: 2024-05-16DOI: 10.1007/s00246-024-03518-5
Amanda D McCormick, Kriti Puri, S Yukiko Asaki, Shahnawaz Amdani, Devyani Chowdhury, Julie S Glickstein, Seda Tierney, Patricia Ibeziako, Melissa K Cousino, Christina Ronai
{"title":"Mental Health Care for Children with Heart Disease and Their Families: Practical Approaches and Considerations for the Pediatric and Pediatric Cardiology Clinician.","authors":"Amanda D McCormick, Kriti Puri, S Yukiko Asaki, Shahnawaz Amdani, Devyani Chowdhury, Julie S Glickstein, Seda Tierney, Patricia Ibeziako, Melissa K Cousino, Christina Ronai","doi":"10.1007/s00246-024-03518-5","DOIUrl":"10.1007/s00246-024-03518-5","url":null,"abstract":"<p><p>Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"757-768"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140945350","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: 2024-05-10DOI: 10.1007/s00246-024-03511-y
Jennifer Cohen, Son Q Duong, Naveen Arivazhagan, David M Barris, Surkhay Bebiya, Rosalie Castaldo, Marjorie Gayanilo, Kali Hopkins, Maya Kailas, Grace Kong, Xiye Ma, Molly Marshall, Erin A Paul, Melanie Tan, Jen Lie Yau, Girish N Nadkarni, David Ezon
{"title":"Machine Learning Quantification of Pulmonary Regurgitation Fraction from Echocardiography.","authors":"Jennifer Cohen, Son Q Duong, Naveen Arivazhagan, David M Barris, Surkhay Bebiya, Rosalie Castaldo, Marjorie Gayanilo, Kali Hopkins, Maya Kailas, Grace Kong, Xiye Ma, Molly Marshall, Erin A Paul, Melanie Tan, Jen Lie Yau, Girish N Nadkarni, David Ezon","doi":"10.1007/s00246-024-03511-y","DOIUrl":"10.1007/s00246-024-03511-y","url":null,"abstract":"<p><p>Assessment of pulmonary regurgitation (PR) guides treatment for patients with congenital heart disease. Quantitative assessment of PR fraction (PRF) by echocardiography is limited. Cardiac MRI (cMRI) is the reference-standard for PRF quantification. We created an algorithm to predict cMRI-quantified PRF from echocardiography using machine learning (ML). We retrospectively performed echocardiographic measurements paired to cMRI within 3 months in patients with ≥ mild PR from 2009 to 2022. Model inputs were vena contracta ratio, PR index, PR pressure half-time, main and branch pulmonary artery diastolic flow reversal (BPAFR), and transannular patch repair. A gradient boosted trees ML algorithm was trained using k-fold cross-validation to predict cMRI PRF by phase contrast imaging as a continuous number and at > mild (PRF ≥ 20%) and severe (PRF ≥ 40%) thresholds. Regression performance was evaluated with mean absolute error (MAE), and at clinical thresholds with area-under-the-receiver-operating-characteristic curve (AUROC). Prediction accuracy was compared to historical clinician accuracy. We externally validated prior reported studies for comparison. We included 243 subjects (median age 21 years, 58% repaired tetralogy of Fallot). The regression MAE = 7.0%. For prediction of > mild PR, AUROC = 0.96, but BPAFR alone outperformed the ML model (sensitivity 94%, specificity 97%). The ML model detection of severe PR had AUROC = 0.86, but in the subgroup with BPAFR, performance dropped (AUROC = 0.73). Accuracy between clinicians and the ML model was similar (70% vs. 69%). There was decrement in performance of prior reported algorithms on external validation in our dataset. A novel ML model for echocardiographic quantification of PRF outperforms prior studies and has comparable overall accuracy to clinicians. BPAFR is an excellent marker for > mild PRF, and has moderate capacity to detect severe PR, but more work is required to distinguish moderate from severe PR. Poor external validation of prior works highlights reproducibility challenges.</p>","PeriodicalId":19814,"journal":{"name":"Pediatric Cardiology","volume":" ","pages":"884-894"},"PeriodicalIF":1.5,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140903447","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}