Carter Biewen^1,*, 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 was 16.6… More >

Karina V. Bunting^1,2,*, Francesco Formisano³, Jennifer Green¹, Richard P. Steeds^1,2, Lucy Hudsmith¹, Paul Clift^1,2

Congenital Heart Disease, Vol.15, No.2, pp. 89-100, 2020, DOI:10.32604/CHD.2020.011376

Abstract Objective: To determine the accuracy of assessing univentricular function in adult Fontan patients using three-dimensional (3D) volumetric echocardiography. Design: A prospective observational study in an adult Fontan patient cohort. Setting: University Hospitals Birmingham, NHS Foundation Trust. Patients: 26 patients were enrolled in the study all aged over 18 years, possessing the Fontan anatomy, with no contraindications to Cardiac Magnetic Resonance (CMR) imaging and in sinus rhythm. Intervention: All patients underwent transthoracic echocardiography using a Philips EPIQ 7 and X5-1 transducer. End diastolic volume (EDV), end systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were obtained using two dimensional… More >

Jose A. Silva‐Sepulveda¹, Yudy Fonseca², Irine Vodkin³, Gabrielle Vaughn¹, Robert Newbury⁴, Vera Vavinskaya⁵, Jerry Dwek⁶, James C. Perry¹, Preeti Reshamwala⁷, Cynthia Baehling⁸, James Lyon⁹, Christopher Davis¹, Jesse W. Lee¹, Hannah El‐Sabrout¹⁰, Doaa Shahbah¹¹, Laith Alshawabkeh¹², John W Moore¹, Howaida El‐Said¹

Congenital Heart Disease, Vol.14, No.4, pp. 600-608, 2019, DOI:10.1111/chd.12770

Abstract Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.
Methods: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
Results: Median age 17.8 years, median time since… More >

Michael V. Di Maria¹, Cindy Barrett¹, Carey Rafferty¹, Kelly Wolfe¹, Sarah L. Kelly¹, Deborah R. Liptzin², Dania Brigham³, Adel Younoszai¹

Congenital Heart Disease, Vol.14, No.4, pp. 590-599, 2019, DOI:10.1111/chd.12769

Abstract Background: Children with single ventricle (SV) heart disease who undergo Fontan operation are at risk for developing multiorgan dysfunction. Although survival has improved, significant comorbidities involving multiple organ systems may develop, requiring evaluation and management by many subspecialists. Using data from an internal survey, we documented high care variability for our Fontan population. We then developed a multidisciplinary clinic, designed and implemented a clinical care pathway to decrease variability of patient assessment.
Methods: After creating a multidisciplinary team and a clinical care pathway, we initiated a multidisciplinary clinic (MDC) where patients could see multiple subspecialists during a single encounter. We… More >

Jenny Alenius Dahlqvist¹, Jan Sunnegårdh², Katarina Hanséus³, Eva Strömvall Larsson², Anders Nygren², Magnus Dalén^4,5, Håkan Berggren⁶, Jens Johansson Ramgren⁷, Urban Wiklund⁸, Annika Rydberg¹

Congenital Heart Disease, Vol.14, No.4, pp. 582-589, 2019, DOI:10.1111/chd.12766

Abstract Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indica‐ tions in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.
Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).
Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection… More >

Rachel Wong¹, Mohammed Al‐Omary², David Baker², Neil Spratt^1,2, Andrew Boyle^1,2, 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‐ companying cognitive assessment.
… More >

Donald J. Hagler^1,2, William R. Miranda², Brielle J. Haggerty¹, Jason H. Anderson¹, Jonathan N. Johnson¹, Frank Cetta^1,2, Sameh M. Said^1,3, Nathaniel W. Taggart¹

Congenital Heart Disease, Vol.14, No.4, pp. 571-581, 2019, DOI:10.1111/chd.12757

Abstract Background: Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percuta‐ neous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.
Methods: Retrospective review of all Fontan patients who had cardiac catheteriza‐ tion from January 2002 to October 2018 was performed. Hemodynamic and angio‐ graphic data… More >

Gruschen R. Veldtman¹, Alexander R. Opotowsky², Samuel G. Wittekind¹, Jack Rychik³, Daniel J. Penny⁴, Mark Fogel³, Bradley S. Marino⁵, Marc Gewillig⁶

Congenital Heart Disease, Vol.12, No.6, pp. 699-710, 2017, DOI:10.1111/chd.12526

Abstract Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide… More >

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: lower 30… More >

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 using meta-analysis techniques.
… More >