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  • Open Access


    Associations between Bodyweight and Clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

    Emma Payne1,2, Thomas Wilson2,3, Marjan Haghighi1,4, Zoe McCallum2,3, Yves d’Udekem5, Julian Ayer1,4,*

    Congenital Heart Disease, Vol.17, No.6, pp. 617-639, 2022, DOI:10.32604/chd.2022.024775

    Abstract Background: Patients born with a single ventricle circulation commonly experience growth failure in early life, which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectory and clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, a systematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patients with clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluated by Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional studies. Results: Of 527 studies… More >

  • Open Access


    A 20-Year Follow-up after the Fontan Operation in a Population with Hypoplastic Left Heart Syndrome

    Eleni P. Asimacopoulos*, Steven J. Staffa, Peter C. Laussen, Kirsten C. Odegard

    Congenital Heart Disease, Vol.17, No.5, pp. 579-590, 2022, DOI:10.32604/chd.2022.020334

    Abstract Background: Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population. We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome (HLHS) from Stage I through Fontan completion. In this report, we examine their clinical status, anticoagulation and incidence of thromboembolic events up to 20 years post Fontan. Methods: A retrospective chart review was conducted for twenty (20) surviving patients, from 1998 through December 2020. Patients who underwent orthotopic heart transplantation (OTx) were followed until their transplant. Patients who were found in the original study… More >

  • Open Access


    A Single Institution’s Experience with Later Extracardiac Fontan Procedure Off Bypass

    Carter Biewen1,*, Hitendra Patel2, Olaf Reinhartz3, Ginny Gildengorin4, Natalie Cvijanovich5

    Congenital Heart Disease, Vol.15, No.4, pp. 229-238, 2020, DOI:10.32604/CHD.2020.011671

    Abstract Purpose: To summarize Fontan procedure data from our institution, UCSF Benioff Children’s Hospital Oakland, in order to better understand outcomes of our surgical and post-operative care. Basic Procedures: This is a retrospective cross-sectional chart review summarizing characteristics and outcomes of patients who underwent the Fontan procedure at our institution between 2005 and 2016. Main Findings: Sixty-five Fontan procedures were performed at our institution between 2005 and 2016, all of which were extracardiac Fontan procedures performed by the same surgical team. At the time of Fontan procedure, the mean patient age was 56.4 ± 18.7 months and mean weight… More >

  • Open Access


    Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound

    Rachel Wong1, Mohammed Al‐Omary2, David Baker2, Neil Spratt1,2, Andrew Boyle1,2, Natasha Baker Cert1, Peter Howe1, Nicholas Collins2

    Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763

    Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
    Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐… More >

  • Open Access


    Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis

    Zhiyong Lin, Hanwei Ge, Jiyang Xue, Guowei Wu, Jie Du, Xingti Hu, Qifeng Zhao

    Congenital Heart Disease, Vol.12, No.6, pp. 711-720, 2017, DOI:10.1111/chd.12503

    Abstract Objective: This study aims to assess and compare the early and long-term effects of extracardiac conduit (EC) and lateral tunnel (LT) in patients with a functional single ventricle through metaanalysis.
    Design: A systematic search was performed in PubMed, Embase, Cochrane Library, CNKI, VIP, CBM, and WanFang databases for papers that were published until August 1, 2016. Cochrane systematic review method was used for paper screening and information retrieve, and RevMan 5.3 software was applied for the meta-analysis.
    Results: Data for 10 studies with a total of 3814 patients were retrieved. The advantages of EC comparing to LT include:… More >

  • Open Access


    Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies

    Tarek Alsaied1, Jouke P. Bokma2, Mark E. Engel3, Joey M. Kuijpers2, Samuel P. Hanke1, Liesl Zuhlke4, Bin Zhang5, Gruschen R. Veldtman6

    Congenital Heart Disease, Vol.12, No.4, pp. 393-398, 2017, DOI:10.1111/chd.12468

    Abstract Background: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.
    Methods: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality… More >

  • Open Access


    Surveillance for liver complications after the Fontan procedure

    Moira B. Hilscher1, Jonathan N. Johnson2,3, Frank Cetta2,3, David J. Driscoll2,3, John J. Poterucha1, William Sanchez1, Heidi M. Connolly3, Patrick S. Kamath1

    Congenital Heart Disease, Vol.12, No.2, pp. 124-132, 2017, DOI:10.1111/chd.12446

    Abstract The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population. More >

  • Open Access


    Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot

    Keri M. Shafer1,2, Alexander R. Opotowsky1,2, Jonathan Rhodes1

    Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

    Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
    Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
    Results: Of the 42 cases, 27 had… More >

  • Open Access


    Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected

    Mi Kyoung Song1, Gi Beom Kim1, Eun Jung Bae1, Young Ah Lee1, Hyun-Young Kim2, Seung-Kee Min3, Jung Hee Kim4, Jae-Kyung Won5

    Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625

    Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
    Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s Hospital.
    Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence… More >

  • Open Access


    Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation

    Laura S. Kverneland1,2, Peter Kramer2, Stanislav Ovroutski2

    Congenital Heart Disease, Vol.13, No.2, pp. 181-193, 2018, DOI:10.1111/chd.12570

    Abstract Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients’ characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published… More >

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