Research & Literature
Explore the leading researchers and institutions driving advances in this area, and dive into the full body of literature that informs this resource.
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Boston Children's Hospital
Boston, United States
Cincinnati Children's Hospital Medical Center
Cincinnati, United States
Children's Hospital of Philadelphia
Philadelphia, United States
University of Michigan
Ann Arbor, United States
Children's National
Washington, United States
University of Toronto
Toronto, Canada
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Sydney, Australia
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Los Angeles, United States
Mayo Clinic
Rochester, United States
References
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Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation.
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The Fontan Procedure for Single-Ventricle Physiology.
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Critical care nurse 2018; (38(1)):e1-e10 doi:10.4037/ccn2018994.
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Acceptability of a parental early warning tool for parents of infants with complex congenital heart disease: a qualitative feasibility study.
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Archives of disease in childhood 2018; (103(9)):880-886 doi:10.1136/archdischild-2017-313227.
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Elevated Pulmonary Artery Pressure, Not Pulmonary Vascular Resistance, is an Independent Predictor of Short-Term Morbidity Following Bidirectional Cavopulmonary Connection.
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Pediatric cardiology 2018; (39(8)):1572-1580 doi:10.1007/s00246-018-1932-6.
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Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment.
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International journal of cardiology 2018; (271()):312-316 doi:10.1016/j.ijcard.2018.05.039.
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Factors Associated With Interstage Mortality Following Neonatal Single Ventricle Palliation.
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The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort.
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European heart journal 2019; (40(13)):1057-1068 doi:10.1093/eurheartj/ehy620.
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Longitudinal Assessment of Outcome From Prenatal Diagnosis Through Fontan Operation for Over 500 Fetuses With Single Ventricle-Type Congenital Heart Disease: The Philadelphia Fetus-to-Fontan Cohort Study.
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Journal of the American Heart Association 2018; (7(19)):e009145 doi:10.1161/JAHA.118.009145.
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Long-lasting benefits of exercise for those living with a Fontan circulation.
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Current opinion in cardiology 2019; (34(1)):79-86 doi:10.1097/HCO.0000000000000582.
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Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation.
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Power of a Learning Network in Congenital Heart Disease.
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The Superior Cavopulmonary Connection: History and Current Perspectives.
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World journal for pediatric & congenital heart surgery 2019; (10(2)):216-222 doi:10.1177/2150135119825560.
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The Bilateral Bidirectional Glenn Operation as a Risk Factor Prior to Fontan Completion in Complex Congenital Heart Disease Patients.
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Fontan-associated liver disease: A review.
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Journal of cardiology 2019; (74(3)):223-232 doi:10.1016/j.jjcc.2019.02.016.
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Management of Congenital Heart Disease: State of the Art-Part II-Cyanotic Heart Defects.
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Children (Basel, Switzerland) 2019; (6(4)) doi:10.3390/children6040054.
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Prediction of heart failure and death in an adult population of Fontan patients.
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Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.
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The Neurodevelopmental Outcomes of Patients With Single Ventricles Across the Lifespan.
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The Annals of thoracic surgery 2019; (108(5)):1565-1572 doi:10.1016/j.athoracsur.2019.05.043.
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Behavior and Quality of Life at 6 Years for Children With Hypoplastic Left Heart Syndrome.
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Pediatrics 2019; (144(5)) doi:10.1542/peds.2019-1010.
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Management of the adult Fontan patient.
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Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents.
Rathgeber SL, Guttman OR, Lee AF, et al.
Journal of the American Heart Association 2020; (9(1)):e012529 doi:10.1161/JAHA.119.012529.
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Fetal cardiac interventions: Where do we stand?
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Archives of cardiovascular diseases 2020; (113(2)):121-128 doi:10.1016/j.acvd.2019.06.007.
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Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry.
Hansen JE, Brown DW, Hanke SP, et al.
Journal of the American Heart Association 2020; (9(10)):e014823 doi:10.1161/JAHA.119.014823.
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Cobra-Head Cuffed Vascular Graft as Right Ventricle-to-Pulmonary Artery Shunt in Norwood Procedure.
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The Annals of thoracic surgery 2021; (112(1)):156-161 doi:10.1016/j.athoracsur.2020.05.050.
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Superior Cavopulmonary Connection: Its Physiology, Limitations, and Anesthetic Implications.
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Relationship of Ventricular Morphology and Atrioventricular Valve Function to Long-Term Outcomes Following Fontan Procedures.
Moon J, Shen L, Likosky DS, et al.
Journal of the American College of Cardiology 2020; (76(4)):419-431 doi:10.1016/j.jacc.2020.05.059.
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Echocardiographic Identification of Pulmonary Artery Flow Reversal: An Indicator of Adverse Outcomes in Single Ventricle Physiology.
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Balancing a single-ventricle circulation: 'physiology to therapy'.
Magoon R, Makhija N, Jangid SK
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Fontan-associated liver disease and hepatocellular carcinoma in adults.
Kogiso T, Tokushige K
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Trends in Discharge Prescription of Digoxin After Norwood Operation: An Analysis of Data from the Pediatric Health Information System (PHIS) Database.
O'Byrne ML, Song L, Huang J, et al.
Pediatric cardiology 2021; (42(4)):793-803 doi:10.1007/s00246-021-02543-y.
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Total vascular resistance increases during volume-unloading in asymptomatic single ventricle patients.
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American heart journal 2021; (236()):69-79 doi:10.1016/j.ahj.2021.02.019.
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Peripheral Pulmonary Artery Stenosis Is Not a Contraindication to a Single-ventricle Pathway.
Al-Khaldi A, Alsahari A, Alotay A, et al.
The Annals of thoracic surgery 2022; (113(5)):1563-1568 doi:10.1016/j.athoracsur.2021.03.059.
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Characteristics of Interstage Death After Discharge from Stage I Palliation.
Ahmed H, Anderson JB, Bates KE, et al.
Pediatric cardiology 2021; (42(6)):1372-1378 doi:10.1007/s00246-021-02621-1.
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Impact of Digoxin Use on Interstage Outcomes of Single Ventricle Heart Disease (From a NPC-QIC Registry Analysis).
Klausner RE, Parra D, Kohl K, et al.
The American journal of cardiology 2021; (154()):99-105 doi:10.1016/j.amjcard.2021.05.048.
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The Influence of Respiration on Blood Flow in the Fontan Circulation: Insights for Imaging-Based Clinical Evaluation of the Total Cavopulmonary Connection.
van der Woude SFS, Rijnberg FM, Hazekamp MG, et al.
Frontiers in cardiovascular medicine 2021; (8()):683849 doi:10.3389/fcvm.2021.683849.
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The Lymphatic System in the Fontan Patient-Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should Know.
RochéRodríguez M, DiNardo JA
Journal of cardiothoracic and vascular anesthesia 2022; (36(8 Pt A)):2669-2678 doi:10.1053/j.jvca.2021.07.049.
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Endurance training may improve exercise capacity, lung function and quality of life in Fontan patients.
Hedlund E, Lundell B
Acta paediatrica (Oslo, Norway : 1992) 2022; (111(1)):17-23 doi:10.1111/apa.16121.
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Impact of Atrial Fibrillation on Fontan Circulation: Fontan Computational Model.
Sughimoto K, Ueda T, Fujiwara T, et al.
The Annals of thoracic surgery 2022; (114(4)):1460-1467 doi:10.1016/j.athoracsur.2021.08.061.
PMID: 34600904 - 54
Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design.
Tran DL, Gibson H, Maiorana AJ, et al.
Frontiers in pediatrics 2021; (9()):799125 doi:10.3389/fped.2021.799125.
PMID: 35071139 - 55
Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications.
Dipchand AI, Honjo O, Alonso-Gonzalez R, et al.
The Canadian journal of cardiology 2022; (38(7)):1072-1085 doi:10.1016/j.cjca.2022.02.019.
PMID: 35240250 - 56
Plastic Bronchitis and Protein-Losing Enteropathy in the Fontan Patient: Evolving Understanding and Emerging Therapies.
Mackie AS, Veldtman GR, Thorup L, et al.
The Canadian journal of cardiology 2022; (38(7)):988-1001 doi:10.1016/j.cjca.2022.03.011.
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Cardiac Imaging in Patients After Fontan Palliation: Which Test and When?
Ciliberti P, Ciancarella P, Bruno P, et al.
Frontiers in pediatrics 2022; (10()):876742 doi:10.3389/fped.2022.876742.
PMID: 35652057 - 58
Impact of Home Monitoring Program and Early Gastrostomy Tube on Interstage Outcomes following Stage 1 Norwood Palliation.
Matthews CR, Hartman D, Farrell AG, et al.
Pediatric cardiology 2023; (44(1)):124-131 doi:10.1007/s00246-022-02947-4.
PMID: 35727331 - 59
Early prediction of failure to progress in single ventricle palliation: A step toward personalizing care for severe congenital heart disease.
Weisert M, Menteer J, Durazo-Arvizu R, et al.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2022; (41(9)):1268-1276 doi:10.1016/j.healun.2022.06.002.
PMID: 35778260 - 60
Hypoplastic Left Heart Syndrome: Definition, Morphology, and Classification.
Jacobs JP
World journal for pediatric & congenital heart surgery 2022; (13(5)):559-564 doi:10.1177/21501351221114770.
PMID: 36053108 - 61
Long-term management of Fontan patients: The importance of a multidisciplinary approach.
Martino D, Rizzardi C, Vigezzi S, et al.
Frontiers in pediatrics 2022; (10()):886208 doi:10.3389/fped.2022.886208.
PMID: 36090574 - 62
Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.
Jalal Z, Gewillig M, Boudjemline Y, et al.
Frontiers in pediatrics 2022; (10()):965989 doi:10.3389/fped.2022.965989.
PMID: 36110107 - 63
Ventricular morphology of single-ventricle hearts has a significant impact on outcomes after Fontan palliation: a meta-analysis.
Ponzoni M, Azzolina D, Vedovelli L, et al.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2022; (62(6)) doi:10.1093/ejcts/ezac535.
PMID: 36367236 - 64
A guide to prenatal counseling regarding neurodevelopment in congenital heart disease.
Lee FT, Sun L, Freud L, Seed M
Prenatal diagnosis 2023; (43(5)):661-673 doi:10.1002/pd.6292.
PMID: 36575573 - 65
Attributable mortality benefit of digoxin treatment in hypoplastic left heart syndrome after the Norwood operation: An instrumental variable-based analysis using data from the Pediatric Health Information Systems Database.
O'Byrne ML, Song L, Huang J, et al.
American heart journal 2023; (263()):35-45 doi:10.1016/j.ahj.2023.05.005.
PMID: 37169122 - 66
Predictors of Human Milk Feeding and Direct Breastfeeding for Infants with Single Ventricle Congenital Heart Disease: Machine Learning Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry.
Elgersma KM, Wolfson J, Fulkerson JA, et al.
The Journal of pediatrics 2023; (261()):113562 doi:10.1016/j.jpeds.2023.113562.
PMID: 37329981 - 67
Predictors of protein losing enteropathy after Fontan completion: An 8-year retrospective study at Sheikh Khalifa Medical City.
AbdelMassih AF, Kiraly L, Badaoui HE, et al.
Global cardiology science & practice 2023; (2023(3)):e202317 doi:10.21542/gcsp.2023.17.
PMID: 37575289 - 68
Impact of home monitoring program on interstage mortality after the Norwood procedure.
Staehler H, Schaeffer T, Wasner J, et al.
Frontiers in cardiovascular medicine 2023; (10()):1239477 doi:10.3389/fcvm.2023.1239477.
PMID: 37900558 - 69
Defining the Spectrum of Hypoplastic Left Heart Syndrome (HLHS).
Jacobs JP, Bleiweis MS, Cohen MS, et al.
World journal for pediatric & congenital heart surgery 2023; (14(6)):741-745 doi:10.1177/21501351231189277.
PMID: 37933696 - 70
Exercise Pathophysiology and Testing in Individuals With a Fontan Circulation.
Mahendran AK, Katz D, Opotowsky AR, Lubert AM
CJC pediatric and congenital heart disease 2023; (2(3)):112-123 doi:10.1016/j.cjcpc.2023.01.001.
PMID: 37969357 - 71
Exercise Capacity and Training Programs in Paediatric Fontan Patients: A Systematic Review.
Herrmann JE, Selamet Tierney ES
CJC pediatric and congenital heart disease 2022; (1(3)):108-118 doi:10.1016/j.cjcpc.2022.04.005.
PMID: 37970491 - 72
Neuroimaging and Neurodevelopmental Outcomes Among Individuals With Complex Congenital Heart Disease: JACC State-of-the-Art Review.
Phillips K, Callaghan B, Rajagopalan V, et al.
Journal of the American College of Cardiology 2023; (82(23)):2225-2245 doi:10.1016/j.jacc.2023.09.824.
PMID: 38030353 - 73
Utility of Balloon Occlusion Testing in Determining Fontan Suitability Among Patients with Elevated Pulmonary Artery Pressure and Additional Antegrade Pulmonary Blood Flow.
Demir IH, Celebi A, Ozdemir DM, et al.
Pediatric cardiology 2024; (45(3)):632-639 doi:10.1007/s00246-023-03380-x.
PMID: 38182891 - 74
Stage 1 and 2 Palliation: Comparing Ductal Stenting and Aorto-Pulmonary Shunts in Single Ventricles with Duct-Dependent Pulmonary Blood Flow.
Ganta S, Haley J, El-Said H, et al.
Pediatric cardiology 2024; (45(3)):471-482 doi:10.1007/s00246-023-03386-5.
PMID: 38265483 - 75
Surgical Considerations in Adult Congenital Heart Disease Heart Failure.
Marshall V WH, McConnell P
Heart failure clinics 2024; (20(2)):199-208 doi:10.1016/j.hfc.2023.12.007.
PMID: 38462324 - 76
Thrombocytopenia is Associated with Higher Fontan Pressure and Increased Morbidity in Patients with Fontan Circulation.
Gould M, Gunsaulus M, Feingold B, et al.
Pediatric cardiology 2025; (46(6)):1537-1543 doi:10.1007/s00246-024-03567-w.
PMID: 38951145 - 77
Advances in Diagnostic and Interventional Catheterization in Adults with Fontan Circulation.
Belahnech Y, Martí Aguasca G, Dos Subirà L
Journal of clinical medicine 2024; (13(16)) doi:10.3390/jcm13164633.
PMID: 39200775 - 78
Practice variation using the hybrid stage I procedure in congenital heart disease: Results from a national survey.
Zanaboni DB, Sower CT, Yu S, et al.
JTCVS open 2024; (21()):248-256 doi:10.1016/j.xjon.2024.07.020.
PMID: 39534324 - 79
Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management.
Tsuchihashi T, Cho Y, Tokuhara D
Frontiers in medicine 2024; (11()):1354857 doi:10.3389/fmed.2024.1354857.
PMID: 39664312 - 80
Serial changes of systemic ventricular function and atrioventricular valve function in patients with failing Fontan.
Matsubara M, Dahmen V, Gaebert P, et al.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2025; (67(2)) doi:10.1093/ejcts/ezaf005.
PMID: 39820488 - 81
Review of rhythm disturbances in patient after fontan completion: epidemiology, management, and surveillance.
Wall K, Hebson C, D'Souza R, Balaji S
Frontiers in pediatrics 2025; (13()):1506690 doi:10.3389/fped.2025.1506690.
PMID: 40013112 - 82
Fontan-Associated Liver Disease.
Hilscher MB, Johnson JN
Seminars in liver disease 2025; (45(1)):114-128 doi:10.1055/a-2556-4897.
PMID: 40081822 - 83
Ultrasound-MRI Agreement in Individuals Undergoing Surveillance of Fontan-associated Liver Disease.
Wang H, Rubadeux D, Trout AT, et al.
Journal of computer assisted tomography 2025; (49(5)):705-713 doi:10.1097/RCT.0000000000001751.
PMID: 40165034 - 84
Prevalence, Risk Factors, and Prognosis for Fontan-Associated Liver Disease: A Systematic Review and Exploratory Meta-Analysis.
Cao JY, Wales KM, d'Udekem Y, et al.
JACC. Advances 2025; (4(5)):101694 doi:10.1016/j.jacadv.2025.101694.
PMID: 40286360 - 85
Four-Dimensional Flow in Fontan Patients: Advanced Haemodynamic Assessment.
Gabbert DD, Uebing AS, Voges I
Journal of clinical medicine 2025; (14(11)) doi:10.3390/jcm14113801.
PMID: 40507563 - 86
A Retrospective Study on the Imaging Spectrum of Functional Single Ventricle and Its Associations.
Niva B, Rajeshkannan R
The Indian journal of radiology & imaging 2025; (35(3)):431-441 doi:10.1055/s-0044-1801762.
PMID: 40529966 - 87
Risk Factors for Reintervention Following Treatment for Aortic Coarctation With or Without Aortic Arch Hypoplasia in Sweden: From Isolated Coarctation to Complex Bi- and Univentricular Congenital Heart Disease.
Hlebowicz J, Floberg Cardenas W, Jeremiasen I, et al.
Journal of the American Heart Association 2025; (14(20)):e040253 doi:10.1161/JAHA.124.040253.
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Optimizing outcomes for patients undergoing superior cavopulmonary connection procedure: what we know.
Sabati A, Alaeddine M, Quinones-Carrasquillo S, et al.
Future cardiology 2025; (21(12)):1177-1185 doi:10.1080/14796678.2025.2598218.
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Outcome of Fontan Patients After Reaching Adolescence: The Impact of Hypoplastic Left Heart Syndrome.
Bambul Heck P, Schüttler A, Hager A, et al.
Journal of clinical medicine 2025; (14(23)) doi:10.3390/jcm14238611.
PMID: 41375913 - 90
Live(r) with a Fontan circulation: A European survey and a proposed expert consensus on liver surveillance in collaboration with the EuroFontan group.
Montanaro C, Tamborrino PP, Verma S, et al.
International journal of cardiology 2026; (447()):134118 doi:10.1016/j.ijcard.2025.134118.
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Addressing Survivorship Bias in Neurocognitive Outcomes After Early Complex Cardiac Surgery Using Clustering and Propensity Scores.
Hajihosseini M, Amiri S, Robertson C, et al.
CJC pediatric and congenital heart disease 2025; (4(6)):315-329 doi:10.1016/j.cjcpc.2025.05.005.
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Prognostic biomarkers in Fontan associated liver disease.
Anouti A, Aqul A, Bartlett JA, et al.
Translational pediatrics 2026; (15(2)):54 doi:10.21037/tp-2025-1-861.
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