Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The ...purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.
A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described.
Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1–50) months.
HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.
•HCC is asymptomatic at the time of diagnosis in 50% of Fontan patients.•26% of Fontan patients with HCC have a normal alpha-fetoprotein.•1-year survival after HCC diagnosis in patients with a Fontan circulation is 50%.•Meticulous liver surveillance is important in patients with a Fontan circulation.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation ...(AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation. Multiple imaging modalities including recent advances in 3-dimensional echocardiography and cross-sectional imaging have been used to further understand AVVR. Surgery to address AVVR must be tailored to the underlying mechanism and the timing of surgical repair should be chosen carefully. In this review, we discuss the etiologies, treatment options, surgical timing, and outcomes of valve repair or replacement for AVVR in patients with single ventricle congenital heart disease, with a focus on those with a Fontan circulation as AVVR is associated with increased risk for Fontan failure and mortality. In-depth understanding of the current literature will help guide clinicians in their approach and management of AVVR in this population.
Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this ...study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation. Thirty-nine Fontan patients with echocardiographic and invasive hemodynamic studies done within 48 h were included and were compared to 40 age-matched healthy controls. Atrial and ventricular strain measurements were measured offline using 2-dimensional speckle-tracking. Mean age was 10.2 ± 6.7 years and 24 (62%) were male. Atrial strain measures were lower in Fontan patients compared to healthy controls. There was no significant association between atrial strain measurements and Fontan systemic ventricular filling pressures (SVFP) as indicated by pulmonary artery occlusion pressures, direct left atrial pressure or systemic ventricular end-diastolic pressure. Global atrial strain was not correlated with segmental atrial strain in the pulmonary venous atrium. Global atrial reservoir strain was positively correlated with pulmonary vascular resistance (r = 0.508, p = 0.045). Global atrial conduit strain was positively correlated with E/A ratio of the AV valve inflow (r = 0.555, p = 0.002). Atrial and ventricular strain measurements were not significantly correlated. In patients with a Fontan, global atrial function is significantly depressed, and is uncoupled from segmental left lateral atrial function. Global as well as segmental atrial mechanics are not significantly associated with SVFPs in Fontan patients. Instead, global atrial reservoir function appears to parallel pulmonary vascular resistance.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
The aim of the study was to evaluate the impact of the latest coronary computed tomography angiography (CCTA) techniques allowing a radiation- and contrast-sparing protocol on image quality in ...unselected patients referred for exclusion of suspected coronary artery disease (CAD).
This prospective study was approved by the local ethics committee, and all patients provided written informed consent. Between March and June 2015, 89 consecutive patients (61% male; mean age 55 ± 11 years) referred for exclusion of CAD by 256-slice CCTA using prospective electrocardiogram triggering were included. Tube voltage (80-120 kVp), tube current (180-310 mA) as well contrast agent volume (25-45 mL) and flow rate (3.5-5 mL/s) were adapted to body mass index. Signal intensity was measured by placing a region of interest in the aortic root, the left main artery, and the proximal right coronary artery. Image noise was measured in the aortic root. Two independent blinded readers semi-quantitatively assessed the image quality regarding motion, noise, and contrast on a 4-point scale.
Median contrast agent volume and median effective radiation dose were 35 mL (interquartile range, 30-40 mL) and 0.5 mSv (interquartile range, 0.4-0.6 mSv), respectively. Mean attenuation in the aortic root was 412 ± 89 Hounsfield units. Diagnostic image quality was obtained in 1050 of 1067 (98.4%) coronary segments and, on an intention-to-diagnosis basis, in 85 of 89 (95.5%) patients. Below a cut-off heart rate of 67 beats/min, only 1 of 974 (0.1%) coronary segments was nondiagnostic.
A radiation- and contrast-sparing protocol for CCTA on a latest generation 256-slice computed tomography scanner yields diagnostic image quality in patients referred for CAD exclusion in daily clinical routine.
Coronary computed tomography angiography (CCTA) allows three-dimensional visualisation of the origin, course and ending of the coronary vessels with high spatial resolution, yielding an accurate ...depiction of coronary artery anomalies (CAAs). This study sought to determine the prevalence, incidence and characteristics of CAAs detected with CCTA in a single centre in Switzerland.
CAAs were retrospectively identified in 5 634 consecutive patients referred for CCTA between March 2007 and July 2015. Single coronary arteries, Bland-White-Garland syndrome, anomalous coronary arteries originating from the opposite site of the sinus of Valsalva (ACAOS) with an interarterial course and coronary artery fistulas were classified as potentially malignant CAAs.
We identified 145 patients with CAAs, resulting in an overall prevalence of 2.6% and cumulative incidence of 2.1% in all patients referred for CCTA in the observation period. Forty-nine (33.8%) patients showed malignant CAAs including 1 (0.7%) patient with Bland-White-Garland syndrome, 7 (4.8%) with single coronary arteries, 36 (24.8%) with ACAOS and an interarterial course, and 5 (3.5%) with coronary artery fistulas. The remaining 96 (66.2%) patients were classified as having benign variants.
The prevalence of CAA detected by CCTA is not negligible. Because of its noninvasive nature, relatively low cost and low radiation exposure, a further increase in the utilisation of CCTA may be expected, which may consequently be paralleled by an increasing absolute number of incidentally detected CAAs. Hence, awareness of the main issues and possible management strategies regarding CAAs is of importance for every treating physician.
Patients with chronic obstructive pulmonary disease (COPD) suffer from significantly more cardiovascular comorbidity and mortality than would be anticipated from conventional risk factors. The aim of ...this study was to determine whether COPD patients have a higher coronary artery calcium score (CACS) and epicardial fat burden, compared to control subjects, and their association with cardiovascular events.
From a registry of 1906 patients 81 patients with clinically diagnosed COPD were one-to-one matched to 81 non-COPD control subjects with a smoking history, according to their age, sex, and the number of classic cardiovascular risk factors (arterial hypertension, diabetes mellitus, dyslipidemia, family history of premature coronary artery disease). CACS, epicardial fat, and subsequent major adverse cardiovascular events (MACE) during follow-up were compared between groups.
Patients with COPD (Global Initiative for Chronic Obstructive Lung Disease-classification I: 5%, II: 23%, III: 16% and IV: 56%) showed no difference in CACS (median difference 68 Agatston Units 95% confidence interval -176.5 to 192.5, p=0.899) or epicardial fat volume (mean difference -0.5 cm3 95% confidence interval -20.9 to 21.9, p=0.961) compared with controls. After a median follow-up of 42.6 months a higher incidence of MACE was observed in COPD patients (RR=2.80, p=0.016) compared with controls. Cox proportional hazard regression identified cardiac ischemias and CACS as independent predictors for MACE.
COPD patients experienced a higher MACE incidence compared to controls despite no baseline differences in coronary calcification and epicardial fat burden. Other mechanisms such as undersupply of medication seem to account for an excess cardiovascular comorbidity in COPD patients.
Full text
Available for:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Abstract
Aims
Iterative reconstruction (IR) algorithms allow for a significant reduction in radiation dose of coronary computed tomography angiography (CCTA). We performed a head-to-head comparison ...of adaptive statistical IR (ASiR) and model-based IR (MBIR) algorithms to assess their impact on quantitative image parameters and diagnostic accuracy for submillisievert CCTA.
Methods and results
CCTA datasets of 91 patients were reconstructed using filtered back projection (FBP), increasing contributions of ASiR (20, 40, 60, 80, and 100%), and MBIR. Signal and noise were measured in the aortic root to calculate signal-to-noise ratio (SNR). In a subgroup of 36 patients, diagnostic accuracy of ASiR 40%, ASiR 100%, and MBIR for diagnosis of coronary artery disease (CAD) was compared with invasive coronary angiography. Median radiation dose was 0.21 mSv for CCTA. While increasing levels of ASiR gradually reduced image noise compared with FBP (up to − 48%, P < 0.001), MBIR provided largest noise reduction (−79% compared with FBP) outperforming ASiR (−59% compared with ASiR 100%; P < 0.001). Increased noise and lower SNR with ASiR 40% and ASiR 100% resulted in substantially lower diagnostic accuracy to detect CAD as diagnosed by invasive coronary angiography compared with MBIR: sensitivity and specificity were 100 and 37%, 100 and 57%, and 100 and 74% for ASiR 40%, ASiR 100%, and MBIR, respectively.
Conclusion
MBIR offers substantial noise reduction with increased SNR, paving the way for implementation of submillisievert CCTA protocols in clinical routine. In contrast, inferior noise reduction by ASiR negatively affects diagnostic accuracy of submillisievert CCTA for CAD detection.
Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both ...liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.
Background Hepatic steatosis, caused by nonalcoholic fatty liver disease, is a leading cause of chronic liver disease. The interplay between hepatic steatosis and the development of liver disease ...following the Fontan procedure is not well understood. This study examined the prevalence and associations of hepatic steatosis in patients with a Fontan circulation. Methods and Results This was a single-center retrospective study of 95 patients with a Fontan circulation with liver magnetic resonance imaging performed between 2012 and 2019. The average age at magnetic resonance imaging was 21.5±8.5 years. The percent liver fat signal was determined using magnetic resonance chemical shift-encoded proton density fat fraction imaging. Hepatic steatosis was defined as liver fat ≥5% and was present in 10.5% of the cohort. The presence of hepatic steatosis was associated with higher body mass index (29±4 versus 24±6 kg/m
,
=0.006), a higher frequency of obesity (50% versus 12%,
=0.015), lower high-density lipoprotein cholesterol (35±9 versus 43±14 mg/dL,
=0.050), and greater subcutaneous fat thickness (2.6±0.7 versus 1.8±1.0 cm,
=0.043). There was no association between hepatic steatosis and cardiovascular imaging or hemodynamic variables from cardiac catheterization. Conclusions Risk factors for hepatic steatosis in patients with Fontan circulation include obesity and dyslipidemia, similar to what is seen in the general population. Fontan hemodynamics were not associated with hepatic steatosis.
Abstract Background Coronary computed tomography angiography (CTA) is increasingly being used for evaluation of coronary artery disease (CAD). As a result of the widely reported potential of ...carcinogenic risk from x-ray based examinations, many strategies have been developed for dose reduction with CTA. Objectives The purpose of this study was to assess the diagnostic accuracy of CTA acquired with a submillisievert fraction of effective radiation dose reconstructed with a model-based iterative reconstruction (MBIR) using invasive coronary angiography (ICA) as a standard of reference. Methods In 36 patients (body mass index range 17 to 39 kg/m2 ) undergoing ICA for CAD evaluation, a CTA was acquired using very low tube voltage (80 to 100 kV) and current (150 to 210 mA) and was reconstructed with MBIR. CAD (defined as ≥50% luminal narrowing) was assessed on CTA and on ICA. Results CTA resulted in an estimated radiation dose exposure of 0.29 ± 0.12 mSv (range 0.16 to 0.53 mSv), yielding 96.9% (436 of 450) interpretable segments. On an intention-to-diagnose basis, no segment was excluded, and vessels with at least 1 nonevaluable segment and no further finding were classified as false positive. This resulted in a sensitivity, specificity, positive, and negative predictive value and accuracy of 100%, 74%, 77%, 100%, and 86% per patient and 85%, 86%, 56%, 96%, and 85% per vessel, respectively. Conclusions The use of MBIR reconstruction allows accurate noninvasive diagnosis of CAD with CTA at a submillisievert fraction of effective radiation dose comparable with a chest x-ray in 2 views.
Full text
Available for:
GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
PDF
