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1. Full Text Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: A randomized, double-blind, placebo-controlled, crossover trial
by Goldberg, David J and French, Benjamin and McBride, Michael G and Marino, Bradley S and Mirarchi, Nicole and Hanna, Brian D and Wernovsky, Gil and Paridon, Stephen M and Rychik, Jack
Circulation, ISSN 0009-7322, 03/2011, Volume 123, Issue 11, pp. 1185 - 1193
Background-Children and young adults with single-ventricle physiology have abnormal exercise capacity after the Fontan operation. A medication capable of... 
physiology | exercise | Fontan procedure | trials | CAPACITY | CARDIAC & CARDIOVASCULAR SYSTEMS | TRICUSPID ATRESIA | CIRCULATION | PULMONARY ARTERIAL-HYPERTENSION | CARDIORESPIRATORY RESPONSE | HEMODYNAMIC-RESPONSE | PRESSURE | THERAPY | CITRATE | PERIPHERAL VASCULAR DISEASE | Sulfones - therapeutic use | Double-Blind Method | Administration, Oral | Heart Defects, Congenital - surgery | Humans | Sulfones - adverse effects | Male | Piperazines - therapeutic use | Piperazines - adverse effects | Cross-Over Studies | Sildenafil Citrate | Young Adult | Fontan Procedure | Exercise | Oxygen Consumption - drug effects | Adolescent | Adult | Female | Heart Defects, Congenital - physiopathology | Phosphodiesterase 5 Inhibitors - therapeutic use | Purines - adverse effects | Purines - therapeutic use | Child | Natriuretic Peptide, Brain - blood | Heart | Usage | Demographic aspects | Surgery | Patient outcomes | Dosage and administration | Ventricular function | Sildenafil | Research | Exercise tests | Index Medicus | Abridged Index Medicus
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2. Full Text 40-Year Follow-Up after the Fontan Operation Long-Term Outcomes of 1,052 Patients
by Pundi, Kavitha N and Johnson, Jonathan N and Dearani, Joseph A and Pundi, Krishna N and Li, Zhuo and Hinck, Cynthia A and Dahl, Sonja H and Cannon, Bryan C and O'Leary, Patrick W and Driscoll, David J and Cetta, Frank
Journal of the American College of Cardiology, ISSN 0735-1097, 10/2015, Volume 66, Issue 15, pp. 1700 - 1710
Background There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation. Objectives This study sought to... 
protein-losing enteropathy | Fontan procedure | survival | MORTALITY | FAILED FONTAN | CARDIAC & CARDIOVASCULAR SYSTEMS | LIVER-TRANSPLANTATION | HEART-TRANSPLANTATION | CONVERSION | PREDICTORS | EXPERIENCE | CHILDREN | Reoperation | Survival Rate - trends | Heart Defects, Congenital - surgery | Humans | Middle Aged | Risk Factors | Child, Preschool | Infant | Male | Treatment Outcome | Postoperative Complications - epidemiology | Fontan Procedure - methods | Forecasting | Incidence | Minnesota - epidemiology | Young Adult | Adolescent | Adult | Female | Surveys and Questionnaires | Aged | Retrospective Studies | Child | Index Medicus | Abridged Index Medicus
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3. Full Text Thrombotic and Embolic Complications Associated With Atrial Arrhythmia After Fontan Operation: Role of Prophylactic Therapy
by Egbe, Alexander C and Connolly, Heidi M and McLeod, Christopher J and Ammash, Naser M and Niaz, Talha and Yogeswaran, Vidhushei and Poterucha, Joseph T and Qureshi, Muhammad Y and Driscoll, David J
Journal of the American College of Cardiology, ISSN 0735-1097, 09/2016, Volume 68, Issue 12, pp. 1312 - 1319
Background There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation.... 
atriopulmonary | thromboembolism | antiplatelet | anticoagulation | ADULT PATIENTS | CARDIAC & CARDIOVASCULAR SYSTEMS | METAANALYSIS | CIRCULATION | FLUTTER | RISK | ENDOTHELIAL FUNCTION | ANTICOAGULATION THERAPY | COAGULATION-FACTOR ABNORMALITIES | INTRACARDIAC THROMBUS | THROMBOEMBOLIC COMPLICATIONS | Postoperative Complications - etiology | Embolism - prevention & control | Risk Assessment | Humans | Postoperative Complications - prevention & control | Anticoagulants - therapeutic use | Male | Thrombosis - epidemiology | Postoperative Complications - epidemiology | Fontan Procedure | Thrombosis - etiology | Adult | Female | Arrhythmias, Cardiac - complications | Retrospective Studies | Embolism - etiology | Thrombosis - prevention & control | Embolism - epidemiology | Electrocardiography | Cardiac arrhythmia | Medical imaging | Nuclear magnetic resonance--NMR | Index Medicus | Abridged Index Medicus
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4. Full Text Bosentan Improves Exercise Capacity in Adolescents and Adults After Fontan Operation: The TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) Study
by Hebert, Anders and Mikkelsen, Ulla R and Thilen, Ulf and Idorn, Lars and Jensen, Annette S and Nagy, Edit and Hanseus, Katarina and Sørensen, Keld E and Søndergaard, Lars and Kardiologi and Paediatrics (Lund) and Cardiology and Lund University and Lunds universitet and Pediatrik, Lund
Circulation, ISSN 0009-7322, 12/2014, Volume 130, Issue 23, pp. 2021 - 2030
BACKGROUND—The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced... 
Heart defects | Bosentan | Fontan procedure | Congenital | Exercise test | Vasodilation | VENTRICLE PHYSIOLOGY | CARDIAC & CARDIOVASCULAR SYSTEMS | CIRCULATION | vasodilation | INTOLERANCE | exercise test | CHILDREN | bosentan | CONGENITAL HEART-DISEASE | heart defects, congenital | PERIPHERAL VASCULAR DISEASE | HYPERTENSION | Double-Blind Method | Endothelin Receptor Antagonists - administration & dosage | Humans | Child, Preschool | Infant | Male | Treatment Outcome | Fontan Procedure - adverse effects | Exercise Tolerance - drug effects | Young Adult | Oxygen Consumption - drug effects | Adolescent | Statistics, Nonparametric | Placebos | Sulfonamides - adverse effects | Adult | Endothelin Receptor Antagonists - adverse effects | Female | Receptor, Endothelin A - blood | Hemodynamics | Postoperative Complications - drug therapy | Child | Sulfonamides - administration & dosage | Index Medicus | Abridged Index Medicus | Kardiologi | Clinical Medicine | Cardiac and Cardiovascular Systems | Medical and Health Sciences | Klinisk medicin | Medicin och hälsovetenskap
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5. Full Text Non‐invasive measurement of cardiac output using AESCULON® mini after Fontan operation
by Yoshitake, Shuichi and Miyamoto, Takashi and Tanaka, Yuki and Naito, Yuji
Pediatrics International, ISSN 1328-8067, 02/2017, Volume 59, Issue 2, pp. 141 - 144
BackgroundElectrical velocimetry correlates well with established methods of measuring cardiac output (CO) such as thermodilution and echocardiography. In this... 
pediatric cardiac surgery | single ventricle | Fontan operation | electrical velocimetry | SURGERY | VENTRICULAR MORPHOLOGY | THERMODILUTION | MONITOR | PEDIATRICS | CHILDREN | Heart Defects, Congenital - surgery | Humans | Cardiac Output | Child, Preschool | Infant | Fontan Procedure | Heart Ventricles - abnormalities | Heart Ventricles - physiopathology | Cardiography, Impedance - instrumentation | Heart Ventricles - surgery | Monitoring, Physiologic - instrumentation | Heart Defects, Congenital - physiopathology | Retrospective Studies | Postoperative Care - instrumentation | Measurement | Heart | Heart beat | Analysis | Children | Cardiology | Health aspects | Electrocardiography | Pediatrics | Heart surgery | Monitoring systems | Stroke | Echocardiography | Parameters | Medical records | Patients | Stroke volume | Heart rate | Demographics | Measurement methods | Hemodynamics | Ventricle | Cardiac output | Velocity measurement | Velocimetry | Ejection | Index Medicus
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6. Full Text Fontan operation in the current era: A 15-year single institution experience
by Hirsch, Jennifer C and Goldberg, Caren and Bove, Edward L and Salehian, Sepand and Lee, Timothy and Ohye, Richard G and Devaney, Eric J
Annals of Surgery, ISSN 0003-4932, 09/2008, Volume 248, Issue 3, pp. 402 - 408
Objective: Evaluate current risk factors for mortality and morbidity in patients undergoing the Fontan procedure at a single institution in the current era.... 
ARRHYTHMIAS | SURGERY | IMPACT | EXTRACARDIAC CONDUIT | ATRESIA | LEFT-HEART SYNDROME | TOTAL CAVOPULMONARY CONNECTION | PROTEIN-LOSING ENTEROPATHY | INTRAATRIAL LATERAL TUNNEL | STAGED RECONSTRUCTION | Cross-Sectional Studies | Heart Defects, Congenital - surgery | Humans | Risk Factors | Hypoplastic Left Heart Syndrome - surgery | Child, Preschool | Infant | Treatment Outcome | Fontan Procedure - adverse effects | Morbidity | Fontan Procedure - mortality | Fontan Procedure - statistics & numerical data | Survival Analysis | Retrospective Studies | Index Medicus | Abridged Index Medicus
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7. Full Text Hepatocellular Carcinoma After Fontan Operation: Multicenter Case Series
by Egbe, Alexander C and Poterucha, Joseph T and Warnes, Carole A and Connolly, Heidi M and Baskar, Shankar and Ginde, Salil and Clift, Paul and Kogon, Brian and Book, Wendy M and Walker, Niki and Wagenaar, Lodewijk and Moe, Tabitha and Oechslin, Erwin and Kay, W Aaron and Norris, Mark and Gordon-Walker, Timothy and Dillman, Jonathan R and Trout, Andrew and Anwar, Nadeem and Hoskoppal, Arvind and Veldtman, Gruschen R
Circulation, ISSN 0009-7322, 08/2018, Volume 138, Issue 7, pp. 746 - 748
Hepatocellular | Carcinoma | early detection of cancer | Fontan procedures | carcinoma, hepatocellular | mortality | fibrosis | CARDIAC & CARDIOVASCULAR SYSTEMS | carcinoma | PERIPHERAL VASCULAR DISEASE | hepatocellular
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8. Full Text Early and Late Results of the Modified Fontan Operation for Heterotaxy Syndrome. 30 Years of Experience in 142 Patients
by Bartz, Peter J and Driscoll, David J and Dearani, Joseph A and Puga, Francisco J and Danielson, Gordon K and O'Leary, Patrick W and Earing, Michael G and Warnes, Carole A and Hodge, David O and Cetta, Frank
Journal of the American College of Cardiology, ISSN 0735-1097, 12/2006, Volume 48, Issue 11, pp. 2301 - 2305
Objectives: We sought to evaluate the early and late results of the modified Fontan operation for patients with heterotaxy syndrome, assess variables... 
VENTRICLE | CARDIAC & CARDIOVASCULAR SYSTEMS | OUTCOMES | ATRIAL ISOMERISM | POLYSPLENIA | Prognosis | Follow-Up Studies | Heart Defects, Congenital - surgery | Humans | Child, Preschool | Male | Situs Inversus - mortality | Time Factors | Adult | Female | Surveys and Questionnaires | Child | Situs Inversus - complications | Treatment Outcome | Arrhythmias, Cardiac - etiology | Thromboembolism - etiology | Spleen - abnormalities | Fontan Procedure - methods | Syndrome | Protein-Losing Enteropathies - etiology | Situs Inversus - physiopathology | Heart Defects, Congenital - complications | Adolescent | Survival Analysis | Heart Defects, Congenital - mortality | Heart Defects, Congenital - physiopathology | Health Status | Confidence intervals | Variables | Hospitals | Pulmonary arteries | Mortality | Veins & arteries | Index Medicus | Abridged Index Medicus
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9. Full Text Case Series: Heart Transplantation After Fontan Operation—Single-Center Experience
by Lin, S.-N and Huang, S.-C and Chen, Y.-S and Chih, N.-H and Wang, C.-H and Chou, N.-K and Yu, H.-Y and Wu, I.-H and Shun, C.-T and Wang, S.-S
Transplantation Proceedings, ISSN 0041-1345, 2016, Volume 48, Issue 3, pp. 959 - 964
Abstract Background Fontan failure (FF) occurs rarely. In patients with Fontan failure, heart transplantation is believed to be the most effective therapy. We... 
Surgery | SURGERY | CIRCULATION | ADULTS | IMMUNOLOGY | CHILDREN | TRANSPLANTATION | FAILED FONTAN | FAILING FONTAN | ORTHOTOPIC CARDIAC TRANSPLANTATION | DISEASE | VENTRICULAR ASSIST DEVICE | PATIENT | OUTCOMES | Reoperation | Survival Rate - trends | Follow-Up Studies | Heart Defects, Congenital - surgery | Humans | Heart Transplantation | Male | Treatment Outcome | Fontan Procedure - adverse effects | Hospital Mortality - trends | Time Factors | Adolescent | Female | Heart Defects, Congenital - mortality | Retrospective Studies | Child | Heart | Medical colleges | Transplantation | Mortality | Therapeutics | Homeopathy | Materia medica and therapeutics | Index Medicus
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10. Full Text 18 years of the Fontan operation at a single institution: Results from 771 consecutive patients
by Rogers, Lindsay S and Glatz, Andrew C and Ravishankar, Chitra and Spray, Thomas L and Nicolson, Susan C and Rychik, Jack and Rush, Christina Hayden and Gaynor, J. William and Goldberg, David J
Journal of the American College of Cardiology, ISSN 0735-1097, 09/2012, Volume 60, Issue 11, pp. 1018 - 1025
Objectives: The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients. Background: Since the initial description by... 
cardiopulmonary bypass | Fontan procedure | single ventricle | congenital heart defects | MORTALITY | CARDIAC & CARDIOVASCULAR SYSTEMS | TRICUSPID ATRESIA | RISK-FACTORS | EXTRACARDIAC CONDUIT | CAVOPULMONARY CONNECTION | YOUNG INFANTS | CURRENT ERA | IMPACT | EXPERIENCE | OUTCOMES | Heart Defects, Congenital - surgery | Humans | Risk Factors | Child, Preschool | Infant | Male | Treatment Outcome | Postoperative Complications - epidemiology | Fontan Procedure - adverse effects | Fontan Procedure - mortality | Young Adult | Fontan Procedure - statistics & numerical data | Adolescent | Survival Analysis | Adult | Female | Retrospective Studies | Child | Index Medicus | Abridged Index Medicus
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11. Full Text Clinical outcome of patients 20 years after Fontan operation - effect of fenestration on late morbidity
by Ono, Masamichi and Boethig, Dietmar and Goerler, Heidi and Lange, Melanie and Westhoff-Bleck, Mechthild and Breymann, Thomas
European Journal of Cardio-Thoracic Surgery, ISSN 1010-7940, 12/2006, Volume 30, Issue 6, pp. 923 - 929
Objective: The Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy, but late... 
Fenestration | Tachyarrhythmia | Fontan procedure | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | TRICUSPID ATRESIA | CIRCULATION | tachyarrhythmia | RISK | CLOSURE | PULMONARY ARTERIOVENOUS-MALFORMATIONS | CAVOPULMONARY ANASTOMOSIS | FAILURE | CONNECTION | SHUNT | RESPIRATORY SYSTEM | fenestration | Blood Pressure | Reoperation | Prognosis | Heart Defects, Congenital - surgery | Humans | Child, Preschool | Catheter Ablation | Infant | Treatment Outcome | Arrhythmias, Cardiac - etiology | Thromboembolism - etiology | Fontan Procedure - methods | Oxygen - blood | Epidemiologic Methods | Treatment Failure | Adolescent | Adult | Child | Heart | Complications and side effects | Medical colleges | Tachycardia | Patient outcomes | Transplantation | Morbidity | Index Medicus
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12. Full Text Hemodynamic Determinants of Mortality after Fontan Operation
by Ohuchi, Hideo and Miyazaki, Aya and Negishi, Jun and Hayama, Yosuke and Nakai, Michikazu and Nishimura, Kunihiro and Ichikawa, Hajime and Shiraishi, Isao and Yamada, Osamu
American Heart Journal, ISSN 0002-8703, 2017, Volume 189, pp. 9 - 18
Background Elevated central venous pressure (CVP), low cardiac output and mild hypoxia are common early and late after Fontan operation. However, the... 
Cardiovascular | CARDIAC & CARDIOVASCULAR SYSTEMS | CIRCULATION | PREDICTORS | CONGENITAL HEART-DISEASE | LIVER | ADULTS | EXERCISE CAPACITY | VARIABLES | DYSFUNCTION | FAILURE | PALLIATION | Japan - epidemiology | Survival Rate - trends | Age Factors | Follow-Up Studies | Risk Assessment | Heart Defects, Congenital - surgery | Humans | Risk Factors | Child, Preschool | Infant | Male | Hemodynamics - physiology | Young Adult | Fontan Procedure | Adolescent | Female | Heart Defects, Congenital - mortality | Heart Defects, Congenital - physiopathology | Retrospective Studies | Child | Health aspects | Mortality | Venous pressure | Cardiology | Heart | Oxygen | Congenital diseases | Pulmonary arteries | Saturation | Cardiovascular disease | Gene expression | Oxygen content | Patients | Blood | Exercise | Rodents | Hypoxia | Determinants | Intubation | Hemodynamics | Ventricle | Cardiac output | Mercury | Heart diseases | Ejection | Index Medicus | Abridged Index Medicus
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13. Full Text An Early Glenn Operation May be Associated with the Later Occurrence of Protein-Losing Enteropathy in Fontan Patients
: Association of Early Glenn and Failing Fontan
by Unseld, Bettina and Stiller, Brigitte and Borth-Bruhns, Thomas and du Bois, Florian and Kroll, Johannes and Grohmann, Jochen and Fleck, Thilo
Pediatric Cardiology, ISSN 0172-0643, 8/2017, Volume 38, Issue 6, pp. 1155 - 1161
Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to... 
Protein-losing enteropathy | Medicine & Public Health | Plastic bronchitis | Cardiac Surgery | Congenital heart disease | Vascular Surgery | Children | Cardiology | Fontan operation | Heart Defects, Congenital - surgery | Humans | Risk Factors | Hypoplastic Left Heart Syndrome - surgery | Child, Preschool | Infant | Male | Bronchitis - etiology | Fontan Procedure - adverse effects | Fontan Procedure - rehabilitation | Protein-Losing Enteropathies - etiology | Heart Defects, Congenital - rehabilitation | Time Factors | Vascular Surgical Procedures - adverse effects | Vascular Surgical Procedures - rehabilitation | Female | Surveys and Questionnaires | Retrospective Studies | Child | Hypoplastic Left Heart Syndrome - rehabilitation | Heart | Prognosis | Surgery | Patient outcomes | Bronchitis | Research | Risk factors | Index Medicus
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14. Full Text Liver Disease in Patients After the Fontan Operation
by Pundi, Krishna, BS and Pundi, Kavitha N., MD and Kamath, Patrick S., MD and Cetta, Frank, MD and Li, Zhuo, BS and Poterucha, Joseph T., MD and Driscoll, David J., MD and Johnson, Jonathan N., MD
American Journal of Cardiology, The, ISSN 0002-9149, 2016, Volume 117, Issue 3, pp. 456 - 460
We reviewed records of all patients with an initial Fontan operation or revision from 1973 to 2012 at our institution (n = 1,138); 195 patients had... 
Cardiovascular | MORTALITY | FIBROSIS | CARDIAC & CARDIOVASCULAR SYSTEMS | TRICUSPID ATRESIA | LONG-TERM SURVIVAL | PREDICTORS | PATHOLOGY | CARDIAC CIRRHOSIS | HEART-TRANSPLANT | United States - epidemiology | Liver - pathology | Follow-Up Studies | Heart Defects, Congenital - surgery | Humans | Middle Aged | Child, Preschool | Infant | Male | Fontan Procedure - adverse effects | Incidence | Liver Cirrhosis - epidemiology | Young Adult | Time Factors | Adult | Female | Retrospective Studies | Child | Postoperative Complications | Liver Cirrhosis - etiology | Liver Cirrhosis - diagnosis | Risk Factors | Forecasting | Magnetic Resonance Imaging | Biopsy | Adolescent | Heart failure | Liver cirrhosis | Congenital heart disease | Heart | Cardiac arrhythmia | Medical imaging | Nuclear magnetic resonance--NMR | Liver diseases | Pulmonary arteries | Mortality | Variables | Survival analysis | Ultrasonic imaging | Gastroenterology | Index Medicus | Abridged Index Medicus
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15. Full Text Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation
by John, Anitha S., MD, PhD and Johnson, Jennifer A., MD and Khan, Munziba, MPH and Driscoll, David J., MD and Warnes, Carole A., MD and Cetta, Frank, MD
JACC (Journal of the American College of Cardiology), ISSN 0735-1097, 2014, Volume 64, Issue 1, pp. 54 - 62
Abstract Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.... 
Cardiovascular | Internal Medicine | protein-losing enteropathy | Fontan procedure | ORAL BUDESONIDE | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | MANAGEMENT | SILDENAFIL | CIRCULATION | PULMONARY ARTERIAL-HYPERTENSION | FENESTRATION | SPIRONOLACTONE | CONGENITAL HEART-DISEASE | proteinlosing enteropathy | Protein-Losing Enteropathies - surgery | Survival Rate - trends | Humans | Middle Aged | Child, Preschool | Infant | Male | Treatment Outcome | Fontan Procedure - mortality | Young Adult | Adolescent | Fontan Procedure - trends | Adult | Female | Retrospective Studies | Child | Protein-Losing Enteropathies - mortality | Cohort Studies | Medical colleges | Mortality | Proteins | Nuclear magnetic resonance--NMR | Intubation | Surgery | Age | Index Medicus | Abridged Index Medicus
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16. Full Text The Optimal Timing of Stage-2-Palliation After the Norwood Operation
by Meza, James M and Hickey, Edward and McCrindle, Brian and Blackstone, Eugene and Anderson, Brett and Overman, David and Kirklin, James K and Karamlou, Tara and Caldarone, Christopher and Kim, Richard and DeCampli, William and Jacobs, Jeffrey P and Jacobs, Marshall and Guleserian, Kristine and Jaquiss, Robert and Congenital Heart Surg Soc Timing and Congenital Heart Surgeons’ Society Timing of S2P Working Group
The Annals of Thoracic Surgery, ISSN 0003-4975, 01/2018, Volume 105, Issue 1, pp. 193 - 199
The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal... 
MORTALITY | SURGERY | CARDIAC & CARDIOVASCULAR SYSTEMS | VENTRICLE RECONSTRUCTION TRIAL | RISK-FACTORS | SINGLE-VENTRICLE | LENGTH-OF-STAY | CHILDREN | RESPIRATORY SYSTEM | LEFT-HEART SYNDROME | CRITICAL AORTIC-STENOSIS | BIDIRECTIONAL CAVOPULMONARY ANASTOMOSIS | OUTCOMES | Fontan Procedure | Time Factors | Humans | Risk Factors | Survival Analysis | Infant | Palliative Care | Index Medicus | Abridged Index Medicus | Norwood operation | modeling | hypoplastic left heart syndrome | survival analysis | Glenn | Statistics | CHD | Fontan | risk analysis
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