Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients. We retrospectively measured EFV manually on cardiac magnetic resonance imaging in Fontan patients, ≥15 years, and 1:1 age, sex, and body mass index-matched patients with rTOF. EFV was indexed to body surface area. A random subset of studies was re-measured to assess intra- and interobserver reliability. Fontan patients (n = 63, median age 21.6 years, 51% male, mean body mass index 24.2 ± 5.6 kg/m) had a larger indexed EFV compared with matched rTOF patients (75.3 ± 29.2 ml/m vs 60.0 ± 19.9 ml/m, p = 0.001). In Fontan patients, indexed EFV was inversely correlated with ventricular ejection fraction (r = -0.26, p = 0.04) and cardiac index (r = -0.33, p = 0.01). Intra- and interobserver reliabilities of the indexed EFV measurements in both groups were excellent (intraclass correlation coefficient ranges from 0.93 to 0.97). In conclusion, indexed EFV is higher in Fontan patients compared with patients with rTOF and is associated with lower ventricular ejection fraction and cardiac index. Increased EFV could play a role in the failing Fontan circulation, but longitudinal studies are necessary to establish any causative role.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.amjcard.2017.12.026 | DOI Listing |
Int J Cardiovasc Imaging
December 2024
Department of Cardiology, Fukuoka Children's Hospital, 5-1-1, Kashiiteriha, Higashi-Ku, Fukuoka City, Fukuoka, 813-0017, Japan.
Fontan-associated liver disease (FALD) may be caused by chronic liver congestion due to high central venous pressure (CVP). Recently, the usefulness of liver native T1 mapping in magnetic resonance imaging (MRI) in adulthood has been reported. To evaluate the usefulness of native liver T1 mapping in children with congenital heart disease (CHD), we investigated the utility of native liver T1 relaxation time (LT1) in pediatric Fontan patients in comparison to other CHDs.
View Article and Find Full Text PDFASAIO J
December 2024
Department of Pediatrics, Children's Hospital of Colorado, Aurora, Colorado.
As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
March 2024
Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Introduction: Each year the number of combined heart-liver transplants (HLT) increases, with two distinct patient populations proceeding down this pathway. The first are patients with congenital heart disease (CHD), most commonly single ventricle patients palliated with Fontan. The second group are those with long standing congestive hepatopathy, amyloidosis, hemochromatosis, or alcohol induced myopathies and liver disease.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
December 2024
Paediatric Diabetes and Endocrine Service, Starship Child Health, Te Toka Tumai Auckland Te Whatu Ora, Auckland, New Zealand.
Objective: Short stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.
Methods: MRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex-matched normal published controls.
Int J Cardiol Congenit Heart Dis
June 2024
University of Sydney, Sydney, Australia.
Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.
Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!