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 >

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 >