Djoeke Wolff¹, Henricus B. M. van de Wiel², Mirthe E. de Muinck Keizer¹, Joost P. van Melle³, Petronella G. Pieper³, Rolf M. F. Berger¹, Tjark Ebels⁴, Willebrord C. M. Weijmar Schultz⁵

Congenital Heart Disease, Vol.13, No.2, pp. 319-326, 2018, DOI:10.1111/chd.12576

Abstract Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.
Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.
Results: Fontan patients scored significantly lower on general health than their healthy peers (t (19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable to normal… More >

Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

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 >

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 >

Anthony Merlocco

Congenital Heart Disease, Vol.14, No.1, pp. 100-104, 2019, DOI:10.1111/chd.12719

Abstract The ductus arteriosus (DA) has been studied since Galen. Initially after birth in neo‐ nates with obstruction to pulmonary blood flow, DA patency is integral to ensure out‐ put and oxygenation. While DA stenting dates back 25 years, there is emerging interest in better understanding how and when to utilize this strategy as an alternative to surgi‐ cal shunt placement or ongoing prostaglandin administration. Understanding the nor‐ mal fetal circulation and the perturbations that affect flow and oxygenation is integral to comprehending how normal DA anatomy and morphology may change and how this may influence technical and clinical considerations. In… More >

Dana M. Boucek¹, Athar M. Qureshi², Bryan H. Goldstein ³, Christopher J. Petit⁴, Andrew C. Glatz^1,5

Congenital Heart Disease, Vol.14, No.1, pp. 105-109, 2019, DOI:10.1111/chd.12707

Abstract Background: Infants with ductal-dependent pulmonary blood flow (PBF) often undergo a palliative procedure to provide a stable source of PBF prior to definitive palliation or repair. In the current era, a surgical shunt or ductal stent is used to provide PBF. We aimed to review the current literature comparing ductal stents to surgical shunts.
Methods and Results: Four small, single-center studies and two larger multicenter studies were identified comparing ductal stent to surgical shunt. Combined, these studies showed ductal stent resulted in similar or improved pulmonary artery growth, fewer complications, shorter length of stay, less diuretic use, and improved survival… More >

Sitong Zhou¹, Jiandi Wan^1,*

Molecular & Cellular Biomechanics, Vol.16, Suppl.2, pp. 70-70, 2019, DOI:10.32604/mcb.2019.06978

Abstract Piezo proteins (Piezo1 and Piezo2) are recently identified mechanically activated cation channels in eukaryotic cells and associated with physiological responses to touch, pressure, and stretch. In particular, human RBCs express Piezo1 on their membranes, and mutations of Piezo1 have been linked to hereditary xerocytosis. To date, however, physiological functions of Piezo1 on normal RBCs remain poorly understood. Here, we show that Piezo1 regulates mechanotransductive release of ATP from human RBCs by controlling the shear-induced Ca2+ influx [1]. We find that, in human RBCs treated with Piezo1 inhibitors or having mutant Piezo1 channels, the amounts of shear-induced ATP release and Ca2+… More >

Ken-ichi Tsubota^1,2,*, Yuya Kodama¹, Hiroyoshi Aoki², Yutaka Yamagata²

Molecular & Cellular Biomechanics, Vol.16, Suppl.2, pp. 38-39, 2019, DOI:10.32604/mcb.2019.07285

Abstract We measured a blood flow in a polydimethysiloxane micro channel to reflect the complex geometry of a microvascular network. A flow rate was compared between two working fluids: water and blood. The measured flow rate reflected the bifurcation effects on the apparent viscosity determined by hematocrit, as well as the effects of the surrounding flow channels as bypasses. More >