Tim Takken¹, Alyanne Evertse¹, Fleur de Waard¹, Mandy Spoorenburg¹, Martijn Kuijpers¹, Christian Schroer^2,3, Erik H. Hulzebos¹

Congenital Heart Disease, Vol.14, No.6, pp. 1005-1012, 2019, DOI:10.1111/chd.12850

Abstract Background: Traveling to high altitude has become more popular. High‐altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns regarding safety of altitude exposure for patients with a Fontan circulation. Therefore, investigating hemodynamic and pulmonary responses of acute high‐altitude exposure (±2500 m) at rest and during maximal exercise in patients with Fontan circulation can provide clarity in this dispute and may contribute to improvement of clinical counseling.
Methods: Twenty‐one Fontan patients with 21 age‐matched… More >

Simone Goa Diab¹, Kristin Godang², Lil‐Sofie Ording Müller³, Runar Almaas⁴, Charlotte de Lange³, Leif Brunvand¹, Kari Margrethe Hansen¹, Anne Grethe Myhre⁵, Gaute Døhlen¹, Erik Thaulow^1,6, Jens Bollerslev^2,6, Thomas Möller¹

Congenital Heart Disease, Vol.14, No.6, pp. 996-1004, 2019, DOI:10.1111/chd.12848

Abstract Objective: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.
Methods: A cross‐sectional sample of 64 patients was examined with dual‐energy X‐ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4‐9 years old (n = 22), B: 10‐15 years old (n = 21), and C:… More >

Paolo D’Ambrosio^1,2, Derek Tran^1,2, Charlotte E. Verrall^3,4, Chantal Attard⁵, Maria Fiatarone Singh^6,7, Julian Ayer^3,4,8, Yves d’Udekem^5,9,10, Stephen Twigg^2,11, David S. Celermajer^1,2,12, Rachael Cordina^1,2,5

Congenital Heart Disease, Vol.14, No.6, pp. 987-995, 2019, DOI:10.1111/chd.12836

Abstract Objective and Patients: This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.
Design: Participants underwent bone mineral density measurement using dual‐energy X‐ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.
Results: In our cohort (n = 28), 29% had osteopenic‐range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L,… More >

Alexander Lemmer¹, Lisa VanWagner^1,2, Zaira Gasanova³, Steve Helmke^4,5, Gregory T. Everson^4,5, Daniel Ganger¹

Congenital Heart Disease, Vol.14, No.6, pp. 978-986, 2019, DOI:10.1111/chd.12831

Abstract Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances… More >

Uri Pollak^1,2,3,4,*, Tatyana Ruderman^5,*, Sharon Borik‐Chiger^5,6, David Mishaly^5,7, Alain Serraf^5,7, Amir Vardi^5,8

Congenital Heart Disease, Vol.14, No.6, pp. 968-977, 2019, DOI:10.1111/chd.12825

Abstract Objective: The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.
Design: Single center retrospective cohort study.
Setting: Pediatric Cardiac Intensive Care Unit at a tertiary medical center.
Patients: Ninety‐nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.
Interventions: None.
Measurements and Main Results: Out of the four types of blood products, transfusion of platelets was found… More >

Ryan D. Byrne¹, Angela J. Weingarten², Daniel E. Clark², Shi Huang³, Roman E. Perri⁴, Andrew E. Scanga⁴, Jonathan N. Menachem², Larry W. Markham⁵, Benjamin P. Frischhertz²

Congenital Heart Disease, Vol.14, No.5, pp. 765-771, 2019, DOI:10.1111/chd.12820

Abstract Setting: Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and MELD‐XI.
Objective: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD‐XI scores.
Design: Single‐center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function… More >

Anudeep K. Dodeja¹, Courtney Thomas¹, Curt J. Daniels^1,2, Naomi Kertesz^1,2, Anna Kamp^1,2

Congenital Heart Disease, Vol.14, No.5, pp. 745-751, 2019, DOI:10.1111/chd.12815

Abstract Background: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhyth‐ mias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQ^TM, have been useful in long‐term monitoring for arrhythmias in adults with cryptogenic stroke.
Objective: We propose the Reveal LINQ^TM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients.
Methods: This is a single center retrospective review of Reveal LINQ^TM use in ACHD patients from 2014‐2017. Medical records were reviewed to determine cardiac diag‐… More >

Erin K. Davis¹, Salil Ginde¹, Jessica Stelter², Peter Frommelt¹, Garick D. Hill³

Congenital Heart Disease, Vol.14, No.5, pp. 720-725, 2019, DOI:10.1111/chd.12814

Abstract Background/Hypothesis/Objectives: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure.
Design/Methods: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and… More >

Danielle Kirelik^1,2, Mark Fisher², Michael DiMaria², Danielle E. Soranno³, Katja M. Gist²

Congenital Heart Disease, Vol.14, No.5, pp. 760-764, 2019, DOI:10.1111/chd.12776

Abstract Background: There are several limitations when using creatinine to estimate glomerular filtration rate, especially in children with chronic medical conditions who are at high risk of kidney dysfunction. Cystatin C has been the recent focus of research as a replacement biomarker for creatinine. Our objective was to compare the 2 biomarkers in pediatric single‐ventricle heart disease patients who have undergone the Fontan operation. We hypothesized that there would be poor correlation and agreement between the 2 estimates of renal function.
Methods: This was a single center retrospective chart review of 20 patients who had previously undergone Fontan operation. Demographic and… More >

Thomas G. Wilson^1,2, Yves d’Udekem^1,2,3, David S. Winlaw^4,5, Rachael L. Cordina^5,6, Julian Ayer^4,5, Thomas L. Gentles⁷, Robert G. Weintraub^1,2,8, Leeanne E. Grigg⁹, Michael Cheung^1,8, Timothy M. Cain¹⁰, Padma Rao¹⁰, Charlotte Verrall⁴, Karin Du Plessis¹, Kathryn Rice⁷, Ajay J. Iyengar^1,3

Congenital Heart Disease, Vol.14, No.3, pp. 454-463, 2019, DOI:10.1111/chd.12746

Abstract Background: Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross‐sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine‐based methods in estimating glo‐ merular filtration rate (GFR). Methods: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo ^99mTc‐DTPA clear‐ ance was performed. Various serum creatinine‐based equations were used to calcu‐ late estimated GFR (eGFR). Results: Mean mGFR was 108 ± 28 mL/min/1.73 m² in children and 92 ± 20 mL/ min/1.73 m²… More >