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Several genes involved in the familial appearance of thoracic aortic aneurysms and dissections ( FTAAD) have been characterized recently, one of which is SMAD3. Mutations of SMAD3 cause a new syndromic form of aortic aneurysms and dissections associated with skeletal abnormalities. We discovered a small interstitial deletion of chromosome 15, leading to disruption of SMAD3, in a boy with mild mental retardation, behavioral problems and revealed features of the aneurysms-osteoarthritis syndrome ( AOS). Several family members carried the same deletion and showed features including aortic aneurysms and a dissection. This finding demonstrates that haploinsufficiency of SMAD3 leads to development of both thoracic aortic aneurysms and dissections, and the skeletal abnormalities that form part of the aneurysms-osteoarthritis syndrome. Interestingly, the identification of this familial deletion is an example of an unanticipated result of a genomic microarray and led to the discovery of important but unrelated serious aortic disease in the proband and family members. [ABSTRACT FROM AUTHOR]

To investigate in type-1 diabetes mellitus (DM1) patients the role of hypertension and of DM1 itself on aortic stiffness by using magnetic resonance imaging (MRI). Consecutive patients from the diabetes and hypertension outpatient clinic and healthy volunteers were included in our study. Subjects were divided into four groups: 32 healthy volunteers (mean age: 54.5 ± 6.8 years), 20 DM1 patients (mean age: 48.3 ± 5.9 years), 31 hypertensive patients (mean age: 59.9 ± 7.2 years) and 28 patients with both DM1 and hypertension (mean age: 50.1 ± 6.2 years). Aortic stiffness was measured by means of pulse wave velocity (PWV) using velocity-encoded MRI. Analysis of variance (ANOVA), uni- and multivariable regression models and the Bonferroni-test for multiple testing, were used for statistical analyses. Mean aortic PWV was 5.7 ± 1.2 m/s in healthy volunteers, 5.9 ± 1.2 m/s in DM1 patients without hypertension, 7.3 ± 1.2 m/s in hypertensive patients and 7.3 ± 1.3 m/s in patients with both DM1 and hypertension. Compared to healthy control subjects, aortic PWV was significantly higher in patients with hypertension (P < 0.001) and in patients with both DM1 and hypertension (P < 0.001), whereas aortic PWV was not increased in patients having DM1 alone. Furthermore, aortic PWV was significantly higher in DM1 patients with hypertension than in patients with DM1 alone (P = 0.002). These findings remained after adjustment for confounding factors. Hypertension has a predominant contributive effect on aortic stiffness in DM1 patients whereas the direct diabetic effect on aortic stiffness is small. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the diagnostic performance of 320-slice computed tomography coronary angiography (CTA) in the evaluation of patients with prior coronary artery bypass grafting (CABG). Invasive coronary angiography (ICA) served as the standard of reference, using a quantitative approach.Methods: CTA studies were performed using CT equipment with 320 detector-rows, each 0.5 mm wide, and a gantry rotation time of 0.35 s. All grafts, recipient and nongrafted vessels were deemed interpretable or uninterpretable. The presence of significant (≥50%) stenosis and occlusion were determined on vessel and patient basis. Results were compared to ICA using quantitative coronary angiography.Results: A total of 40 patients (28 men, 76 ± 15 years), with 89 grafts, were included in the study. On a graft analysis, the sensitivity, specificity, positive and negative predictive values in the evaluation of significant stenosis were 96%, 92%, 83% and 98% respectively. The diagnostic accuracy for the assessment of recipient and nongrafted vessels was 89% and 80%, respectively. The diagnostic accuracy for the assessment of graft, recipient and nongrafted vessel occlusion was 96%, 92% and 100%, respectively.Conclusions: 320-slice CTA allows accurate non-invasive assessment of significant graft, recipient vessel and nongrafted vessel stenosis in patients with prior CABG. [ABSTRACT FROM AUTHOR]

Background: Right ventricular (RV) function is predictive of outcome in patients with acute pulmonary embolism (PE). We assessed the possible incremental value of ventricular function with ECG-synchronized cardiac CT scanning over pulmonary CT scan angiography (CTA) for predicting short-term outcome in patients with suspected acute PE.Methods: The local ethics committee approved the study, and informed consent was obtained. In addition to standard CTA, 430 consecutive patients (193 men, 237 women; age, 55 ± 17 years) with suspected acute PE underwent ECG-synchronized CT scanning to assess ventricular function. RV/left ventricular (LV) function ratio and pulmonary obstruction index were obtained from non-ECG-synchronized CTA. Ventricular function was used to predict adverse events (< 6 weeks). Receiver operating characteristic analysis was performed to determine differences between ECG-synchronized CT scan and CTA in predicting outcome.Results: In 113 patients with PE, RV and LV ejection fraction (EF) and RV/LV diameter and volume ratios were associated with adverse outcome (P < .05), whereas vascular obstruction index was not. RVEF had the largest area under the receiver operating characteristic curve (0.75; 95% CI, 0.62-0.88) for predicting adverse outcome but had no significant incremental value over the RV/LV function ratio (0.72; 95% CI, 0.57-0.86; P = .25). All parameters revealed high negative predictive values (94%-98%) but low positive predictive values (13%-18%). For disease-specific outcome, areas under the curve were 0.80 (95% CI, 0.69-0.91) for RVEF vs 0.68 (95% CI, 0.48-0.88) for axial RV/LV ratio; the difference was not significant (P = .07). RVEF and RV/LV ratio proved better predictors for outcome than pulmonary obstruction index (both P < .001).Conclusions: RVEF was the best predictor for clinical outcome in patients with acute PE. However, incremental value of RVEF over axial RV/LV ratio was not found. [ABSTRACT FROM AUTHOR]

Objective: To evaluate, with the use of magnetic resonance imaging (MRI), whether aortic pulse wave velocity (PWV) is associated with cardiac left ventricular (LV) function and mass as well as with cerebral small vessel disease in patients with type 1 diabetes mellitus (DM).Materials and Methods: We included 86 consecutive type 1 DM patients (49 male, mean age 46.9 +/- 11.7 years) in a prospective, cross-sectional study. Exclusion criteria included aortic/heart disease and general MRI contra-indications. MRI of the aorta, heart and brain was performed for assessment of aortic PWV, as a marker of aortic stiffness, systolic LV function and mass, as well as for the presence of cerebral white matter hyperintensities (WMHs), microbleeds and lacunar infarcts. Multivariate linear or logistic regression was performed to analyse the association between aortic PWV and outcome parameters, with covariates defined as age, gender, mean arterial pressure, heart rate, BMI, smoking, DM duration and hypertension.Results: Mean aortic PWV was 7.1 +/- 2.5 m/s. Aortic PWV was independently associated with LV ejection fraction (ss = -0.406, P = 0.006), LV stroke volume (ss = -0.407, P = 0.001), LV cardiac output (ss = -0.458, P = 0.001), and with cerebral WMHs (P < 0.05). There were no independent associations between aortic stiffness and LV mass, cerebral microbleeds or lacunar infarcts.Conclusion: Aortic stiffness is independently associated with systolic LV function and cerebral WMHs in patients with type 1 DM. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess the performance of 0.5 versus 3.0 mm slice reconstructions in depicting coronary calcium with special attention to patients having zero calcium scores at 3.0 mm reconstructions by using computed tomography (CT). Imaging was performed by volumetric 320-detector row CT. Scans of 100 patients with a negative and 100 patients with a positive Agatston score at 3.0 mm reconstructions were consecutively selected. Non-overlapping volume sets with 3.0 and 0.5 mm slice thickness were reconstructed from the same raw data and Agatston and volume scores were obtained. The Wilcoxon signed ranks test was used to determine statistical differences between 3.0 and 0.5 mm calcium scores. Agatston and volume scores obtained at 0.5 mm were significantly higher than at 3.0 mm reconstructions (mean Agatston score: 266 +/- 495 vs. 231 +/- 461. Mean volume score: 223 +/- 399 vs. 206 +/- 385, both P < 0.01). In 21% of patients with zero 3.0 mm Agatston scores, a positive Agatston and/or volume score was found at 0.5 mm reconstructions. With volumetric 320-detector row CT, prospective ECG-triggered calcium scoring at 0.5 mm compared to 3.0 mm reconstructions leads to an increase in Agatston and volume scores and small amounts of coronary calcium are earlier depicted. This may be of special interest in patients with zero calcium scores with traditional 3.0 mm measures, where 0.5 mm reconstructions may help in superior depicting or ruling out coronary artery disease. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To assess pulmonary flow dynamics and right ventricular (RV) function in patients without significant anatomical narrowing of the pulmonary arteries late after the arterial switch operation (ASO) by using magnetic resonance imaging (MRI). METHODS: 17 patients (mean (SD), 16.5 (3.6) years after ASO) and 17 matched healthy subjects were included. MRI was used to assess flow across the pulmonary trunk, RV systolic and diastolic function, and RV mass. RESULTS: Increased peak flow velocity (>1.5 m/s) was found across the pulmonary trunk in 14 of 17 patients. Increased RV mass was found in ASO patients: 14.9 (3.4) vs 10.0 (2.6) g/m2 in normal subjects (p<0.01). Delayed RV relaxation was found after ASO: mean tricuspid valve E/A peak flow velocity ratio = 1.60 (0.96) vs 1.92 (0.61) in normal subjects (p = 0.03), and E-deceleration gradients = -1.69 (0.73) vs -2.66 (0.96) (p<0.01). After ASO, RV mass correlated with pulmonary trunk peak flow velocity (r = 0.49, p<0.01) and tricuspid valve E-deceleration gradients (r = 0.35, p = 0.04). RV systolic function was well preserved in patients (ejection fraction = 53 (7)% vs 52 (8)% in normal subjects, p = 0.72). CONCLUSIONS: Increased peak flow velocity in the pulmonary trunk was often observed late after ASO, even in the absence of significant pulmonary artery stenosis. Haemodynamic consequences were RV hypertrophy and RV relaxation abnormalities as early markers of disease, while systolic RV function was well preserved. [ABSTRACT FROM AUTHOR]

AIM: To evaluate recovery of cardiac function after acute pulmonary embolism (PE). MATERIALS AND METHODS: Routine breath-held computed tomography (CT)-pulmonary angiography was performed in patients with suspected PE to confirm or exclude the diagnosis of PE at initial presentation. Electrocardiogram (ECG)-triggered cardiac CT was performed to assess biventricular function. After 6 months, cardiac magnetic resonance imaging (MRI) was performed. In total, 15 consecutive patients with PE and 10 without were studied. A significant change in ventricular volume was defined as a >15% change in end-diastolic or -systolic volumes (EDV, ESV), and significant ventricular function improvement as a >5% increase in ejection fraction (EF) as based on reported cut-off values. RESULTS: Right and left ventricular (RV and LV) EDV and ESV changed non-significantly (<1.3%) in the patients without PE, indicating good comparability of those values measured by CT and MRI. PE patients with baseline normal RV function (RVEF >=47%) revealed a >5% improvement in the RVEF (+5.4±3.1%) due to a decrease in the RVESV. Patients with baseline abnormal RV function showed a >5% improvement in the RVEF (+14±15%) due to decreases in both the RVESV and RVEDV. Furthermore, the LVEDV increased in this latter patient group. CONCLUSIONS: The present study demonstrated an improvement in RV function in the majority of patients with PE, independent of baseline RV function. The degree of RV and LV recovery was dependent on the severity of baseline RV dysfunction. [ABSTRACT FROM AUTHOR]

A considerable number of patients with an acute coronary syndrome (ACS) who present with a 0 or low calcium score (CS) still demonstrate coronary artery disease (CAD) and significant stenosis. The aim of the present study was to evaluate the relation between the CS and the degree and character of atherosclerosis in patients with suspected ACS versus patients with stable CAD obtained by computed tomography angiography and virtual histology intravascular ultrasound (VH IVUS). Overall 112 patients were studied, 53 with ACS and 59 with stable CAD. Calcium scoring and computed tomography angiography were performed and followed by VH IVUS. On computed tomography angiography each segment was evaluated for plaque and classified as noncalcified, mixed, or calcified. Vulnerable plaque characteristics on VH IVUS were defined by percent necrotic core and presence of thin-cap fibroatheroma. If the CS was 0, patients with ACS had a higher mean number of plaques (5.0 ± 2.0 vs 2.0 ± 1.9, p <0.05) and noncalcified plaques (4.6 ± 3.5 vs 1.3 ± 1.9, p <0.05) on computed tomography angiography than those with stable CAD. If the CS was 0, VH IVUS demonstrated that patients with ACS had a larger amount of necrotic core area (0.58 ± 0.73 vs 0.22 ± 0.43 mm(2), p <0.05) and a higher mean number of thin-cap fibroatheromas (0.6 ± 0.7 vs 0.1 ± 0.3, p <0.05) than patients with stable CAD. In conclusion, even in the presence of a 0 CS, patients with ACS have increased plaque burden and increased vulnerability compared to patients with stable CAD. Therefore, absence of coronary calcification does not exclude the presence of clinically relevant and potentially vulnerable atherosclerotic plaque burden in patients with ACS. [ABSTRACT FROM AUTHOR]

Background: Cardiac emboli are important causes of (recurrent) ischaemic stroke. Aorta atherosclerosis might also be associated with an increased risk of stroke recurrence. This study aimed to evaluate the yield and clinical implications of CT-angiography (CTA) of the heart and aorta in the diagnostic workup of transient ischaemic attack (TIA) or ischaemic stroke., Methods: CTA of the heart and aortic arch was performed in TIA/ischaemic stroke patients, in addition to routine diagnostic workup. Occurrence of cardioembolic (CE) risk sources and complex aortic plaques were assessed. Implications of cardiac CTA for therapeutic management were evaluated RESULTS: Sixty-seven patients were included (TIA n = 33, ischaemic stroke n = 34) with a mean age of 68 years (range 51-89) and median NIHSS of 0 (interquartile range 0-2). CE risk sources were detected in 29 (43%) patients. An intracardiac thrombus was present in 2 patients (3%; TIA 0%; ischaemic stroke 6%). Medium/low-risk CE sources included mitral annular calcification (9%), aortic valve calcification (18%) and patent foramen ovale (18%). Complex aortic plaque was identified in 16 patients (24%). In two patients with an intracardiac thrombus, therapeutic management changed from antiplatelet to oral anticoagulation., Conclusions: CTA of the heart and aorta has a high yield for detection of embolic risk sources in TIA/ischaemic stroke, with clinical consequences for 6% of ischaemic stroke patients. Implementation of CTA of the heart and aorta in the acute stroke setting seems valuable, but cost-effectiveness of this approach remains to be determined., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

A 43-year-old man complaining of abdominal angina for several months showed a large suprarenal aneurysm of the abdominal aorta with extensive circumferential wall thrombosis, complete occlusion of the right renal artery and a critically stenosed left renal artery on CT angiography. He suffered from severe hypertension and renal failure. A percutaneous transluminal angioplasty (PTA) was planned. After the PTA procedure, which was complicated by the development of left renal artery occlusion, successful rescue revascularization surgery was performed. Since we were hesitant to start anticoagulant treatment because of a high bleeding risk, magnetic resonance direct thrombus imaging was performed to assess the age of the extensive arterial thrombosis. The aortic thrombus showed a low signal intensity, which is indicative of chronic rather than acute thrombosis. Therefore, oral anticoagulant treatment was not started. The patient recovered without major complications., Learning Points: Accurate diagnosis and treatment of aortic intraluminal thrombosis are of the utmost importance to prevent serious complications such as (peripheral) arterial embolic occlusion with resultant ischemia.Current imaging modalities do not allow for accurate distinction between acute and chronic thrombosis in the abdominal aorta. Hence, differentiating between stable and unstable thrombosis is challenging.The non-invasive magnetic resonance direct thrombus imaging technique may be a valuable additional imaging test to establish a definitive diagnosis and treatment plan in patients with abdominal aortic thrombosis., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2019.)

Purpose: Gas exchange in systemic sclerosis (SSc) is known to be affected by fibrotic changes in the pulmonary parenchyma. However, SSc patients without detectable fibrosis can still have impaired gas transfer. We aim to investigate whether pulmonary vascular changes could partly explain a reduction in gas transfer of SSc patients without fibrosis., Materials and Methods: We selected 77 patients whose visual computed tomography (CT) scoring showed no fibrosis. Pulmonary vessels were detected automatically in CT images, and their local radii were calculated. The frequency of occurrence for each radius was calculated, and, from this radius histogram, 2 imaging biomarkers (α and β) were extracted, wherein α reflects the relative contribution of small vessels compared with large vessels, and β represents the vessel tree capacity. Correlations between imaging biomarkers and gas transfer [single-breath diffusion capacity for carbon monoxide corrected for hemoglobin concentration (DLCOc) %predicted] were evaluated with Spearman correlation. Multivariable stepwise linear regression was performed with DLCOc %predicted as the dependent variable and age, BMI, sPAP, FEV1 %predicted, TLC %predicted, FVC %predicted, α, β, voxel size, and CT-derived lung volume as independent variables., Results: Both α and β were significantly correlated with gas transfer (R=-0.29, P-value=0.011 and R=0.32, P-value=0.004, respectively). The multivariable stepwise linear regression analysis selected sPAP [coefficient=-0.78; 95% confidence interval (CI)=-1.07, -0.49; P-value<0.001], β (coefficient=8.6; 95% CI=4.07, 13.1; P-value<0.001), and FEV1% predicted (coefficient=0.3; 95% CI=0.12, 0.48; P-value=0.001) as significant independent predictors of DLCOc %predicted (R=0.71, P-value<0.001)., Conclusions: In SSc patients without detectable pulmonary fibrosis, impaired gas exchange is associated with alterations in pulmonary vascular morphology.

Purpose: Vascular remodeling is a significant pathological feature of various pulmonary diseases, which may be assessed by quantitative computed tomography (CT) imaging. The purpose of this study was therefore to develop and validate an automatic method for quantifying pulmonary vascular morphology in CT images., Methods: The proposed method consists of pulmonary vessel extraction and quantification. For extracting pulmonary vessels, a graph-cuts-based method is proposed which considers appearance (CT intensity) and shape (vesselness from a Hessian-based filter) features, and incorporates distance to the airways into the cost function to prevent false detection of airway walls. For quantifying the extracted pulmonary vessels, a radius histogram is generated by counting the occurrence of vessel radii, calculated from a distance transform-based method. Subsequently, two biomarkers, slope α and intercept β, are calculated by linear regression on the radius histogram. A public data set from the VESSEL12 challenge was used to independently evaluate the vessel extraction. The quantitative analysis method was validated using images of a three-dimensional (3D) printed vessel phantom, scanned by a clinical CT scanner and a micro-CT scanner (to obtain a gold standard). To confirm the association between imaging biomarkers and pulmonary function, 77 scleroderma patients were investigated with the proposed method., Results: In the independent evaluation with the public data set, our vessel segmentation method obtained an area under the receiver operating characteristic (ROC) curve of 0.976. The median radius difference between clinical and micro-CT scans of a 3D printed vessel phantom was 0.062 ± 0.020 mm, with interquartile range of 0.199 ± 0.050 mm. In the studied patient group, a significant correlation between diffusion capacity for carbon monoxide and the biomarkers, α (R = -0.27, P = 0.018) and β (R = 0.321, P = 0.004), was obtained., Conclusion: In conclusion, the proposed method was validated independently using a public data set resulting in an area under the ROC curve of 0.976 and using a 3D printed vessel phantom data set, showing a vessel sizing error of 0.062 mm (0.16 in-plane pixel units). The correlation between imaging biomarkers and diffusion capacity in a clinical data set confirmed an association between lung structure and function. This quantification of pulmonary vascular morphology may be helpful in understanding the pathophysiology of pulmonary vascular diseases., (© 2019 The Authors. Medical Physics published by Wiley Periodicals, Inc. on behalf of American Association of Physicists in Medicine.)

Fontan failure remains a significant problem, especially in patients with an atriopulmonary Fontan. Fontan baffle volume change during the cardiac cycle (Fontan baffle stroke volume) may affect outcomes in Fontan circulation. Assuming that increased Fontan baffle stroke volume is associated with increased energy loss in the baffle, we hypothesized that higher baffle stroke volume is associated with worse exercise capacity and increased incidence of Fontan failure. Patients from 6 centers with an atriopulmonary or lateral tunnel Fontan operation were included if they had a cardiac magnetic resonance (CMR) study and an adequate cardiopulmonary exercise test. Fontan baffle stroke volume was defined as the difference between maximum and minimum Fontan baffle volumes. Fontan failure was defined as death, listing for transplantation, heart failure symptoms requiring medications, or peak VO 2 below 16 ml/kg/min. The study group consisted of 107 patients (median age 19 years, interquartile range, 14 to 29 years). Most patients (84%) had lateral tunnel procedure. During a median follow-up period of 6.8 [interquartile range: 3.2 to 8.8] years after the CMR, 25 (23%) patients had Fontan failure (7 deaths, 3 listed for transplantation, and 15 with heart failure symptoms). Predictors of Fontan failure on multivariable analysis were ventricular tachycardia, protein losing enteropathy, and additionally in atriopulmonary Fontan only, larger Fontan baffle stroke volume. Predictors of lower peak VO 2 on multivariable analysis were older age at CMR and additionally in atriopulmonary Fontan only, larger Fontan baffle stroke volume. In conclusion, larger Fontan baffle stroke volume was independently associated with lower peak VO 2 and Fontan failure in atriopulmonary Fontan., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: In patients with the Fontan circulation, systemic venous return flows passively towards the lungs. Because of the absence of the subpulmonary ventricle, favourable blood flow patterns with minimal energy loss are clinically relevant. The region where the inferior vena cava, the hepatic veins and the extracardiac conduit join (IVC-conduit junction) is a potential source of increased energy loss. The aim of this study was to evaluate the relationship between geometry and blood flow patterns in the IVC-conduit junction with associated kinetic energy and energy loss using 4-dimensional flow magnetic resonance imaging (MRI)., Methods: Fourteen extracardiac conduit-Fontan patients underwent 4-dimensional flow MRI. The IVC-conduit junctions were ranked into 3 groups for 3 categories: the geometry, the flow complexity and the conduit mean velocity. The relative increase in the mean velocity from the IVC to the conduit (representing IVC-conduit mismatch) was determined. The peak kinetic energy and mean kinetic energy and energy loss were determined and normalized for volume., Results: In 4 of 14 patients, adverse geometries led to helical flow patterns and/or acute changes in flow direction. For each category, the most adverse IVC-conduit junctions were associated with an approximate 2.3-3.2-fold and 2.0-2.9-fold increase in kinetic energy and energy loss, respectively. The IVC-conduit mismatch is strongly correlated with the mean kinetic energy and energy loss (r  = 0.80, P  =  0.001 and ρ = 0.83, P <  0.001, respectively) and with body surface area in patients with 16- mm conduits (r  =  0.88, P  =  0.010)., Conclusions: The IVC-conduit junction is a potential source of increased energy loss. Junctions with increased energy loss showed: (i) a distorted geometry leading to adverse blood flow patterns and/or (ii) the IVC-conduit mismatch. Sixteen-millimetre conduits appear to be inadequate for older patients., (© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Aims: The aim of this study was to assess the impact of adding stress computed tomography (CT) myocardial perfusion (CTP) to coronary CT angiography (CTA) on downstream referral for invasive coronary angiography (ICA), revascularization, and outcome in patients presenting with new-onset chest pain., Methods and Results: Three hundred and eighty-four patients were referred for cardiac CT. Patients with lesions ≥50% stenosis underwent subsequently stress CTP. Perfusion scans were considered abnormal if a defect was observed in ≥ 1 segment. Downstream performance of ICA, revascularization, and the occurrence of major cardiovascular events (death, non-fatal myocardial infarction, and unstable angina requiring urgent revascularization) were assessed within 12 months. In total, 119 patients showed ≥50% stenosis on coronary CTA; stress CTP was normal in 61 patients, abnormal in 38 patients and was not performed in 20 patients. After normal stress CTP, 19 (31%) patients underwent ICA and 9 (15%) underwent revascularization. After abnormal stress CTP, 36 (95%) patients underwent ICA and 29 (76%) revascularizations were performed. Multivariable analyses showed a five-fold reduction in likelihood of proceeding to ICA when a normal stress CTP was added to a coronary CTA showing obstructive CAD. Major cardiovascular event rates at 12 months for patients with obstructive CAD and normal stress CTP (N = 61) were low: 1 myocardial infarction, 1 urgent revascularization, and 1 non-cardiac death., Conclusion: The performance of stress CTP in patients with obstructive CAD at coronary CTA in the same setting is feasible and reduces the referral rate for ICA and revascularization. Secondly, the occurrence of major cardiovascular events at 12 months follow-up in patients with normal stress CTP is low., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Background: Coronary-computed tomography angiography (CTA) has limited accuracy to predict myocardial ischemia. Besides luminal area stenosis, other coronary plaque morphology and composition parameters may help to assess ischemia. With the integration of coronary CTA and adenosine stress CT myocardial perfusion (CTP), reliable information regarding coronary anatomy and function can be derived in one procedure. This analysis aimed to investigate the association between coronary stenosis severity, plaque composition and morphology and the presence of ischemia measured with adenosine stress myocardial CTP., Methods and Results: 84 patients (age, 62 ± 10 years; 48% men) who underwent sequential coronary CTA and adenosine stress myocardial CT perfusion were analyzed. Automated quantification was performed in all coronary lesions (quantitative CTA). Downstream myocardial ischemia was assessed by visual analysis of the rest and stress CTP images and defined as a summed difference score of ≥1. One or more coronary plaques were present in 146 coronary arteries of which 31 (21%) were ischemia-related. Of the lesions with a stenosis percentage <50%, 50%-70%, and >70%, respectively, 9% (6/67), 18% (9/51), and 57% (16/28) demonstrated downstream ischemia. Furthermore, mean plaque burden, plaque volume, lesion length, maximal plaque thickness, and dense calcium volume were significantly higher in ischemia-related lesions, but only stenosis severity (%) (OR 1.06; 95% CI 1.02-1.10; P = .006) and lesion length (mm) (OR 1.26; 95% CI 1.02-1.55; P = .029) were independent correlates., Conclusions: Increasing stenosis percentage by quantitative CTA is positively correlated to myocardial ischemia measured with adenosine stress myocardial CTP. However, stenosis percentage remains a moderate determinant. Lumen area stenosis and lesion length were independently associated with ischemia, adjusted for coronary plaque volume, mean plaque burden, maximal lesion thickness, and dense calcium volume.

Aims: The interpretation of adenosine stress myocardial computed tomography perfusion (CTP) is often hampered by image artefacts caused by cardiac motion, beam hardening, and cone beam. The aim of the present analysis was to assess the influence of the heart-rate response during adenosine infusion, patient characteristics, and medication use on the interpretability of stress myocardial CTP examinations., Methods and Results: Interpretability of stress myocardial CTP examinations was evaluated in 120 patients who underwent sequentially coronary CTA and adenosine stress myocardial CTP (320-row CT scanner, temporal resolution 175 ms) and scored as follows: excellent = absence of any artefact (n = 27, 22%); good = presence of artefacts that do not interfere with the study interpretability (n = 56, 47%); fair = artefacts that do interfere with interpretability (n = 35, 29%); poor = uninterpretable study due to artefacts (n = 2, 2%). 'Fair' and 'poor' were merged into 'reduced' for comparisons. Increasing heart rate during stress myocardial CTP acquisition was related to worse interpretability (excellent: 61.7 ± 13.4 bpm vs. good: 69.8 ± 13.5 bpm vs. reduced: 78.1 ± 17.0 bpm, P < 0.001). Thirteen (11%) of all examinations were considered non-diagnostic. In patients with a heart rate exceeding 85 bpm, 76% of the studies were 'reduced' interpretable. In multivariate analysis, no use of beta blocker (baseline or additional use prior to coronary CTA) (OR: 0.2, P = 0.012), increasing heart rate during coronary CTA (OR: 1.09, P = 0.032), younger age (OR: 0.92, P = 0.021), and the use of calcium antagonist (OR: 6.48, P = 0.017) were independently associated with a heart rate ≥85 bpm during stress myocardial CTP., Conclusion: Higher heart rate during the acquisition of stress myocardial CTP was related to worse interpretability. Furthermore, increasing heart rate during and no beta blocker use prior to the previously performed coronary CTA, younger age, and the use of calcium antagonist were independently associated with a heart rate ≥85 bpm during stress myocardial CTP acquisition., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

Purpose: This study aims to investigate if the time saving biplane method can be an alternative to short-axis measurements for left atrium (LA) and left ventricle (LV) in cardiac magnetic resonance of patients with extensive LV remodeling., Materials and Methods: In 45 patients with coronary artery disease and systolic LV dysfunction [ejection fraction (EF)<50%], LA and LV end-diastolic volume, end-systolic volume, stroke volume, and EF were measured by the biplane area length and by the short-axis volume method., Results: No statistical differences between the biplane area length and short-axis volume were found for the LA end-systolic or the LV end-diastolic volumes. However, the biplane area length method overestimated LA stroke volume by 7.1±12.3 ml, LA EF by 6.7±8.8% (both P<.001), LV stroke volume by 6.2±12.9 ml (P=.002), and LV EF by 2.6±4.6% (P<.001). The differences in LV measurements were more evident in patients with severely distorted LV. For LA volume parameters, overall consistency between biplane area length and short-axis approach was good [all intraclass correlation coefficient (ICC)≥0.74] and for LV volume parameters, overall consistency between both approaches was excellent (all ICC≥0.93)., Conclusion: In patients with LV systolic dysfunction, the short-axis volume is superior to the biplane method and should be the first choice, when accurate determination of LA and LV EF is the main clinical indication for cardiac magnetic resonance., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Obesity has been related to structural brain abnormalities. Increasing evidence suggests that accumulation of fat in the liver is an important determinant of cardiometabolic complications of obesity. The purpose of this study was to investigate the association between CT-assessed liver-to-spleen attenuation ratio as a measure of liver steatosis and MRI-assessed brain tissue integrity in middle-aged to elderly persons., Subjects and Methods: CT and MRI were performed on 213 participants (114 women, 99 men; mean age, 65 ± 7 years). Brain tissue integrity was assessed by magnetization transfer imaging. Linear regression analysis was adjusted for age, sex, vascular risk factors, and total body fat estimated from bioelectrical impedance analysis., Results: Seventy-nine participants had normal weight (body mass index [BMI], 18.5-24.9), and 134 were overweight (BMI ≥ 25). Significant interaction was found between liver-to-spleen ratio and BMI (p = 0.001). In the overweight group, liver fat was associated with reduced brain tissue integrity in both gray matter (standardized β = 0.22; 95% CI, 0.07-0.36) and white matter (standardized β = 0.31; 95% CI, 0.15-0.45). These associations were not found in the normal weight group (gray matter standardized β = -0.08; 95% CI, -0.33 to 0.16; white matter standardized β = -0.09; 95% CI, -0.36-0.14)., Conclusion: The results indicate that liver fat assessed with CT relates to MRI markers of incipient brain injury in middle-aged to elderly overweight persons.

Unlabelled: Essentials Patients with unprovoked pulmonary embolism (PE) are at increased risk of arterial thromboembolism. Coronary and thoracic aorta calcium were evaluated in patients with and without (unprovoked) PE. No association was found between (unprovoked) PE and coronary or aortic calcification. Assessment of both calcium scores on computed tomography pulmonary angiography was highly reproducible., Summary: Objective To evaluate the potential association between (unprovoked) pulmonary embolism (PE) and the presence and extent of coronary artery calcium (CAC) and thoracic aorta calcium (TAC). Methods CAC and TAC derived from computed tomography pulmonary angiography of 100 patients with PE were compared to that of 100 patients in whom PE was ruled out. Results Intraobserver and interobserver agreements for both TAC and CAC were excellent (intraclass correlation > 0.95 for both). In patients with PE vs. patients without PE, no significant differences were found in the presence of CAC or TAC (CAC 64% vs. 67%, odds ratio [OR] 1.0, 95% confidence interval [CI] 0.67-1.6; TAC 46% vs. 59%, OR 1.2, 95% CI 0.80-2.1). Mean CAC and TAC scores were significantly lower in patients with PE than in patients without PE (CAC 3.4 vs. 4.9, absolute difference 1.5, 95% CI 0.2-2.8; TAC 1.1 vs. 1.8, absolute difference 0.9, 95% CI 0.2-1.2). No significant differences were found in the presence of CAC or TAC or in mean CAC and TAC scores between patients with unprovoked PE vs. patients with provoked PE, or between patients with unprovoked PE vs. no PE. Conclusion No significant differences were found between the extent of CAC and TAC in patients with unprovoked PE compared to those with provoked PE or without PE. The observed difference in the extend of CAC and TAC between patients with and without PE was dependent on prevalent cardiovascular risk factors., (© 2016 International Society on Thrombosis and Haemostasis.)

Objectives: To determine the outcomes, including number of medical interventions and initiation of immunosuppressive treatment of a standardised, comprehensive, diagnostic care pathway for patients with systemic sclerosis (SSc). Patient characteristics associated with need for medical interventions and with need for immunosuppressive treatment were determined., Methods: Data were routinely gathered in connection with a 2-day care pathway combining multidisciplinary care and complete diagnostic work-up of organ involvement in SSc. The number of patients in whom the pathway resulted in medical interventions, and/or initiation of immunosuppressives was recorded. Patient characteristics and diagnostic tests results were compared between patients with and without medical interventions, and patients with and without initiation of immunosuppressives by means of multivariable logistic regression analyses., Results: During a period of 44 months, 226 patients with SSc were referred to the care pathway. They included 186 (82%) women with mean age of 54 (SD 14.5) years, and median disease duration of 4 years (range 1-11); 73 (32%) of them had diffuse cutaneous SSc. Medical interventions were initiated in 191 (85%) patients, including initiation of immunosuppressive treatment in n=49 (22%). Presence of telangiectasias and higher erythrocyte sedimentation rate were associated with any medical intervention. Of commonly available variables, lower age, higher skin score and absence of anticentromere antibody were associated with initiation of immunosuppressives., Conclusions: A standardised comprehensive 2-day care pathway for patients with SSc resulted in additional diagnostic or therapeutic interventions in 85% of the patients, regardless of SSc subtype and disease duration. In 22% of the patients, immunosuppressive treatment was initiated.

Aspirin use for primary prevention in patients at high risk with diabetes mellitus (DM) is often recommended under the assumption that most patients with DM have coronary artery disease (CAD). However, not all patients may have CAD. The present study evaluated, in 425 patients at high risk with DM (without chest pain syndrome or a history of cardiac disease), the prevalence of CAD on coronary computed tomography angiography (CTA). Moreover, the association between the presence and number of traditional cardiovascular (CV) risk factors and CAD (on coronary CTA) was evaluated. The median coronary artery calcium score was 29 (interquartile range 0 to 298). On coronary CTA, 116 patients (27%) had no CAD (defined as <30% stenosis). Of the 309 patients (73%) with any CAD (≥30% stenosis), 35% had obstructive CAD (≥50% stenosis). The number of traditional CV risk factors was not associated with the presence of any CAD (≥30% stenosis; p = 0.18) or obstructive CAD (≥50% stenosis; p = 0.13). Hypertension was the only traditional CV risk factor associated with a higher frequency of any CAD (≥30% stenosis; odds ratio = 2.21, 95% CI 1.43 to 3.41, p <0.001) and obstructive CAD (≥50% stenosis; odds ratio 2.03, 95% CI 1.33 to 3.11, p = 0.001). In conclusion, in patients at high risk with DM without chest pain syndrome, any CAD was ruled out by coronary CTA in 27%, whereas 65% of the patients did not have obstructive CAD. The number of CV risk factors was not associated with the presence of CAD. Hypertension was the only traditional CV risk factor that was associated with a higher frequency of CAD. These observations support potential use of coronary CTA to tailor aspirin therapy in patients at high risk with DM., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: Right ventricular (RV) dysfunction is of great prognostic value in patients with SSc. The aim of the present study was to assess in these patients the relationship between pulmonary fibrosis and elevated pulmonary pressure (PHT) with RV function., Methods: A total of 102 SSc patients who underwent thoracic CT and transthoracic echocardiography were included. Speckle tracking-derived RV free wall strain was used to assess RV function., Results: A total of 51 (50%) SSc patients did not have pulmonary fibrosis or PHT, 32 (31%) patients had pulmonary fibrosis but no PHT and the remaining 19 (19%) patients had both pulmonary fibrosis and PHT. Patients with both pulmonary fibrosis and PHT had the most impaired RV free wall strain [-16.8% (s.d. 3.1)] compared with patients with pulmonary fibrosis and no PHT [-21.5% (s.d. 3.6)] and patients with no pulmonary fibrosis and no PHT [-24.0% (s.d. 4.4)]. All three SSc groups showed impaired RV free wall strain compared with controls [-28.0% (s.d. 4.2)]. Importantly, multivariate regression analysis demonstrated that pulmonary fibrosis and left ventricular ejection fraction were independently associated with impaired RV free wall strain in SSc patients., Conclusion: SSc patients show impaired RV function compared with controls. Both pulmonary fibrosis and PHT are independently associated with RV dysfunction., (© The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Aims: Diabetic patients with coronary artery disease (CAD) are often free of chest pain syndrome. A useful modality for non-invasive assessment of CAD is coronary computed tomography angiography (CTA). However, the prognostic value of CAD on coronary CTA in diabetic patients without chest pain syndrome is relatively unknown. Therefore, the aim was to investigate the long-term prognostic value of coronary CTA in a large population diabetic patients without chest pain syndrome., Methods: Between 2005 and 2013, 525 diabetic patients without chest pain syndrome were prospectively included to undergo coronary artery calcium (CAC)-scoring followed by coronary CTA. During follow-up, the composite endpoint of all-cause mortality, non-fatal myocardial infarction (MI), and late revascularization (>90 days) was registered., Results: In total, CAC-scoring was performed in 410 patients and coronary CTA in 444 patients (431 interpretable). After median follow-up of 5.0 (IQR 2.7-6.5) years, the composite endpoint occurred in 65 (14%) patients. Coronary CTA demonstrated a high prevalence of CAD (85%), mostly non-obstructive CAD (51%). Furthermore, patients with a normal CTA had an excellent prognosis (event-rate 3%). An incremental increase in event-rate was observed with increasing CAC-risk category or coronary stenosis severity. Finally, obstructive (50-70%) or severe CAD (>70%) was independently predictive of events (HR 11.10 [2.52;48.79] (P = .001), HR 15.16 [3.01;76.36] (P = .001)). Obstructive (50-70%) or severe CAD (>70%) provided increased value over baseline risk factors., Conclusion: Coronary CTA provided prognostic value in diabetic patients without chest pain syndrome. Most importantly, the prognosis of patients with a normal CTA was excellent.

This case report shows the full spectrum evolution of type B intramural hematoma under conservative treatment, with initial progression into a true aortic dissection, followed by extremely rare near-complete healing of the dissection at followup.

Objectives: Four-dimensional (3 spatial directions and time) velocity-encoded flow magnetic resonance imaging with quantitative particle tracing analysis allows assessment of left ventricular (LV) blood flow organization. Corrected atrioventricular septal defect (AVSD) patients have an abnormal left atrioventricular valve shape. We aimed to analyze flow organization in corrected AVSD patients and healthy controls., Methods: A total of 32 patients (age, 25 ± 14 years), 21 after partial AVSD correction and 11 after complete/intermediate AVSD correction, and 30 healthy volunteers (26 ± 12 years) underwent whole-heart four-dimensional velocity-encoded flow magnetic resonance imaging. Particle tracing in the 16-segment LV cavity model was used to quantitatively evaluate blood flow organization discriminating multiple components., Results: Patients showed a smaller percentage of direct flow compared with controls (30% ± 9% vs 44% ± 11%; P < 0.001). In patients, more inflow was observed in the basal inferior segment (22% ± 11% vs controls, 17% ± 5%; P = 0.005), with less direct but more retained inflow (ie, part of inflow that is not ejected from LV in subsequent systole). In patients, more inflow reached the midventricular level (68% ± 13% vs controls, 58% ± 9%; P < 0.001), most notably as retained inflow in the lateral segments. Subsequently, in patients, more (mostly retained) inflow reached the apex (23% ± 13% vs 14% ± 7%; P < 0.001), which correlated with early peak filling velocity (r = 0.637, P < 0.001). Patients with a corrected complete or intermediate AVSD presented with less direct flow (24% ± 8% vs 33% ± 8%; P = 0.003) and more apical inflow (30% ± 14% vs 18% ± 12%; P = 0.014) compared with a corrected partial AVSD., Conclusion: Multicomponent particle tracing combined with 16-segment analysis quantitatively demonstrated altered LV flow organization after AVSD correction, with less direct and more retained inflow in apical and lateral LV cavity segments, which may contribute to decreased cardiac pumping efficiency.

Acute chest pain (ACP) is one of the most common presenting symptoms at the emergency department. The differential diagnosis is vast. To exclude life-threatening causes, radiologists encounter an increasing amount of thoracic computed tomography (CT) examinations including CT angiography of the heart and great vessels. The dual- and triple-rule CT examinations are currently implemented in clinical practice. We retrospectively identified chest CT examinations in the setting of acute chest pain in our hospitals and collected a variety of common and uncommon cases. In this pictorial essay, we present the most educative cases from patients who presented with acute chest pain in the emergency department of our hospitals and for whom a thoracic CT was ordered. When aortic emergencies, acute coronary syndrome, and pulmonary embolism are excluded, these cases may help the radiologist to suggest alternative diagnoses in the diagnostic challenge of acute chest pain. Teaching Points • The number of chest CT examinations for ACP is increasing.• Chest CT examinations may help suggesting alternative diagnosis in ACP.• Radiologists should be aware of the differential diagnosis of ACP.

Objectives: During normal left ventricular (LV) filling, a vortex ring structure is formed distal to the left atrioventricular valve (LAVV). Vortex structures contribute to efficient flow organization. We aimed to investigate whether LAVV abnormality in patients with a corrected atrioventricular septal defect (AVSD) has an impact on vortex ring formation., Methods: Whole-heart 4D flow MRI was performed in 32 patients (age: 26 ± 12 years), and 30 healthy subjects (age: 25 ± 14 years). Vortex ring cores were detected at peak early (E-peak) and peak late filling (A-peak). When present, the 3-dimensional position and orientation of the vortex ring was defined, and the circularity index was calculated. Through-plane flow over the LAVV, and the vortex formation time (VFT), were quantified to analyze the relationship of vortex flow with the inflow jet., Results: Absence of a vortex ring during E-peak (healthy subjects 0%, vs patients 19%; P = .015), and A-peak (healthy subjects 10% vs patients 44%; P = .008) was more frequent in patients. In 4 patients, this was accompanied by a high VFT (5.1-7.8 vs 2.4 ± 0.6 in healthy subjects), and in another 2 patients with abnormal valve anatomy. In patients compared with controls, the vortex cores had a more-anterior and apical position, closer to the ventricular wall, with a more-elliptical shape and oblique orientation. The shape of the vortex core closely resembled the valve shape, and its orientation was related to the LV inflow direction., Conclusions: This study quantitatively shows the influence of abnormal LAVV and LV inflow on 3D vortex ring formation during LV inflow in patients with corrected AVSD, compared with healthy subjects., (Copyright © 2015. Published by Elsevier Inc.)

Background/objective: Total cavopulmonary connection (TCPC) has been the preferred treatment for patients with univentricular hearts. Current TCPC-techniques are the intra-atrial lateral tunnel (ILT) and the extracardiac conduit (ECC). We aimed to determine ventricular function during rest and stress, and to compare results for both techniques and for left (LV) versus right ventricular (RV) dominance., Methods: 99 patients, aged 12.5 ± 4.0 years underwent echocardiography and magnetic resonance imaging (MRI), and 69 patients underwent stress MRI., Results: Echocardiography showed impaired systolic and diastolic function. MRI parameters were comparable between ILT and ECC at rest. During dobutamine there was a decrease in end-diastolic volume (EDVi) (91 ± 21 vs. 80 ± 20 ml/m(2) p<0.001). Ejection fraction (EF) and cardiac index (CI) during dobutamine were lower for ILT patients (59 ± 11 (ILT) vs. 64 ± 7% (ECC), p=0.027 and 4.2 ± 1.0 (ILT) vs. 4.9 ± 1.0L/min/m(2) (ECC), p=0.006), whereas other parameters were comparable. TEI-index was higher in ILT-patients (0.72 ± 0.27 (ILT) vs. 0.56 ± 0.22 (ECC), p=0.002). Diastolic function was frequently impaired in patients with a dominant RV (67% (RV) vs. 39% (LV), p=0.011). Patients with dominant LV's had smaller end-systolic volume (ESVi) (40 ± 13 (LV) vs. 47 ± 16 (RV) ml/m(2), p=0.030) and higher EF (55 ± 8 (LV) vs. 49 ± 9 ml/m(2) (RV), p=0.001) and contractility (2.3 ± 0.8 (LV) vs. 1.9 ± 0.7 mmHg/ml/m(2) (RV), p=0.050) during rest and higher EF during dobutamine (63 ± 8 (LV) vs. 58 ± 10 ml/m(2) (RV), p=0.043)., Conclusion: Ventricular function is relatively well preserved in modern-day Fontan patients. With dobutamine stress there is a decrease in EDVi. ECC patients have higher CI and EF during stress. Patients with a dominant RV have lower systolic, including impaired contractility, and diastolic function., (Copyright © 2015. Published by Elsevier Ireland Ltd.)

Background: With the routine use of advanced multi-slice CT scanners, pulmonary embolism (PE) is increasingly detected as an incidental finding among cancer patients. Although this generally leads to therapeutic interventions, the accuracy of diagnosing PE on routinely performed contrast enhanced CT scans is unknown., Methods: Consecutive cancer patients diagnosed with incidental PE were eligible for inclusion. Their CT images were reassessed in a blinded fashion by two thoracic radiologists. To ensure blindness, a total of 19 cancer staging CT images without PE were included. The inter-observer reliability for the presence of PE was calculated with use of Kappa statistics., Results: A total of 62 incidental PE patients (mean age 64years, 60% male) were included. All patients received anticoagulant treatment upon diagnosis. Level of agreement between the two expert readers was high: they disagreed on the presence of PE in only two patients (3.2%), resulting in a Kappa statistic of 0.93. After final consensus reading, it was concluded that the CT images of all 62 patients initially diagnosed with incidental PE were indeed positive for PE., Conclusions: This study indicates that an incidental PE diagnosis is reliable and highly reproducible, despite the suboptimal reading conditions of a non-dedicated scan protocol., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Aims: The study aims (i) to evaluate changes in myocardial ischaemia on single-photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI) after 2 years in a cohort of high-risk patients with diabetes without cardiac symptoms or known coronary artery disease (CAD) and (ii) to assess the value of baseline computed tomography coronary angiography (CTA)-derived coronary atherosclerosis parameters to predict changes in myocardial ischaemia., Methods and Results: The population consisted of 100 high-risk patients with diabetes without cardiac symptoms referred for cardiovascular risk stratification. All patients underwent coronary artery calcium (CAC) scoring, CTA, and SPECT MPI. After 2 years of follow-up, SPECT MPI was repeated to evaluate potential progression of ischaemia.In total, 20% of patients presented with ischaemia at baseline. Of these 20 patients, 7 (35%) still had ischaemia at follow-up, whereas 13 (65%) showed resolution and 4 (20%) showed progression of ischaemia at follow-up. Of the 80 patients without ischaemia at baseline, 65 (81%) had a normal MPI at follow-up and 15 patients (19%) presented with new ischaemia. There were no significant differences in the CAC score or the extent, severity, and composition of CAD on CTA between patients with and without ischaemia at baseline. Similarly, no differences could be demonstrated between patients with and without ischaemia at follow-up or between patients with and without progression of ischaemia., Conclusion: The rate of progression of ischaemia in high-risk patients with diabetes without cardiac symptoms is limited. Few patients presented with new ischaemia, whereas some patients showed resolution of ischaemia. Atherosclerosis parameters on CTA were not predictive of new-onset ischaemia or progression of ischaemia., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Pulmonary embolism (PE) is a potentially life threatening condition requiring adequate diagnosis and treatment. Computed tomography pulmonary angiography (CTPA) is excellent for including and excluding PE, therefore CT is the first-choice diagnostic imaging technique in patients suspected of having acute PE. Due to its wide availability and low invasiveness, CTPA tends to be overused. Correct implementation of clinical decision rules in diagnostic workup for PE improves adequate use of CT. Also, CT adds prognostic value by evaluating right ventricular (RV) function. CT-assessed RV dysfunction and to lesser extent central emboli location predicts PE-related mortality in normotensive and hypotensive patients, while PE embolic obstruction index has limited prognostic value. Simple RV/left ventricular (LV) diameter ratio measures >1.0 already predict risk for adverse outcome, whereas ratios <1.0 can safely exclude adverse outcome. Consequently, assessing the RV/LV diameter ratio may help identify patients who are potential candidates for treatment at home instead of treatment in the hospital. A minority of patients develop chronic thromboembolic pulmonary hypertension (CTEPH) following acute PE, which is a life-threatening condition that can be diagnosed by CT. In proximal CTEPH, involving the more central pulmonary arteries, thrombectomy usually results in good outcome in terms of both functional status and long-term survival rate. CT is becoming the imaging method of choice for diagnosing CTEPH as it can identify patients who may benefit from thrombectomy. New CT developments such as distensibility measurements and dual-energy or subtraction techniques may further refine diagnosis and prognosis for improved patient care.

Purpose: familial longevity is marked by an exceptionally healthy metabolic profile and low prevalence of cardiometabolic disease observed already at middle age. We aim to investigate whether regional body fat distribution, which has previously shown to be associated with cardiometabolic risk, is different in offspring of long-lived siblings compared with controls., Methods: our institutional review board approved the study, and all participants (n = 344, average age in years 65.6) gave written informed consent. Offspring (n = 175) of non-agenarian siblings were included. Their partners (n = 169) were enrolled as controls. For abdominal visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) measurements, a single-slice 8.0 mm computed tomography (CT) acquisition was planned at the level of the 5th lumbar vertebra. In addition, participants underwent prospectively electrocardiography-triggered unenhanced volumetric CT of the heart. Abdominal VAT and SAT areas (cm(2)) and epicardial adipose tissue (EAT) volumes (ml) were acquired by semi-automated segmentation techniques. Linear regression analysis was performed adjusting for cardiovascular risk factors., Results: total abdominal fat areas were smaller in male offspring compared with controls (353.0 versus 382.9 cm(2), P = 0.022). The association between low abdominal VAT areas in male offspring (149.7 versus 167.0 cm(2) in controls, P = 0.043) attenuated after additional adjustment for diabetes (P = 0.078). Differences were not observed for females. EAT volumes were similar between offspring of long-lived siblings and controls., Conclusion: males who have genetically determined prospect to become long-lived have less abdominal fat and in particular less abdominal VAT compared with controls., (© The Author 2015. Published by Oxford University Press on behalf of the British Geriatrics Society. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

The systematic assessment of residual thromboembolic obstruction after treatment for acute pulmonary embolism (PE) has been understudied. This assessment is of potential clinical importance, should clinically suspected recurrent PE occur, or as tool for risk stratification of cardiopulmonary complications or recurrent venous thromboembolism (VTE). This study aimed to assess the rate of PE resolution and its implications for clinical outcome. In this prospective, multi-center cohort study, 157 patients with acute PE diagnosed by CT pulmonary angiography (CTPA) underwent follow-up CTPA-imaging after six months of anticoagulant treatment. Two expert thoracic radiologists independently assessed the presence of residual thromboembolic obstruction. The degree of obstruction at baseline and follow-up was calculated using the Qanadli obstruction index. All patients were followed-up for 2.5 years. At baseline, the median obstruction index was 27.5 %. After six months of treatment, complete PE resolution had occurred in 84.1 % of the patients (95 % confidence interval (CI): 77.4-89.4 %). The median obstruction index of the 25 patients with residual thrombotic obstruction was 5.0 %. During follow-up, 16 (10.2 %) patients experienced recurrent VTE. The presence of residual thromboembolic obstruction was not associated with recurrent VTE (adjusted hazard ratio: 0.92; 95 % CI: 0.2-4.1).This study indicates that the incidence of residual thrombotic obstruction following treatment for PE is considerably lower than currently anticipated. These findings, combined with the absence of a correlation between residual thrombotic obstruction and recurrent VTE, do not support the routine use of follow-up CTPA-imaging in patients treated for acute PE.

Purpose: To evaluate trans-left atrioventricular valve (LAVV) blood flow and optimize left ventricular inflow quantification in healthy controls and patients after atrioventricular septal defect (AVSD) correction., Materials and Methods: Twenty-five patients after AVSD correction and 25 controls underwent 4DFlow MRI. Using streamline visualization in four- and two-chamber views, inflow direction at early and late filling was defined at the annulus level and at the peak inflow velocity (PIV) level. Trans-LAVV flow volume and velocity were assessed from a static 2D-multiplanar-reformat (MPR), a 4D-MPR tracking LAVV annulus and a 4D-MPR tracking the PIV-level, angulated perpendicular to the inflow., Results: In patients, on average 9° more laterally directed inflow was found at the PIV-level compared to controls. In controls, 4DFlow velocity mapping with LAVV annulus tracking resulted in lower absolute error with aortic flow (3 (1-8) mL) than with static 2D-MPR (7 (4-16) mL, P = 0.001). In patients, 4D-MPR tracking the PIV-level, resulted in lower absolute error with aortic flow (2 (1-4) mL) than with 4D-MPR LAVV annulus tracking (6 (2-10) mL, P = 0.003)., Conclusion: Streamline visualization of 4DFlow MRI data revealed dynamic trans-LAVV inflow and more lateral flow after AVSD correction. Streamline visualization improved trans-LAVV flow quantification as the positioning and angulation of the measurement plane was optimized, allowing an accurate assessment of left ventricular inflow., (© 2014 Wiley Periodicals, Inc.)

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.

Introduction: Interstitial lung disease occurs frequently in patients with systemic sclerosis (SSc). Quantitative computed tomography (CT) densitometry using the percentile density method may provide a sensitive assessment of lung structure for monitoring parenchymal damage. Therefore, we aimed to evaluate the optimal percentile density score in SSc by quantitative CT densitometry, against pulmonary function., Material and Methods: We investigated 41 SSc patients by chest CT scan, spirometry and gas transfer tests. Lung volumes and the nth percentile density (between 1 and 99%) of the entire lungs were calculated from CT histograms. The nth percentile density is defined as the threshold value of densities expressed in Hounsfield units. A prerequisite for an optimal percentage was its correlation with baseline DLCO %predicted. Two patients showed distinct changes in lung function 2 years after baseline. We obtained CT scans from these patients and performed progression analysis., Results: Regression analysis for the relation between DLCO %predicted and the nth percentile density was optimal at 85% (Perc85). There was significant agreement between Perc85 and DLCO %predicted (R=-0.49, P=0.001) and FVC %predicted (R=-0.64, P<0.001). Two patients showed a marked change in Perc85 over a 2 year period, but the localization of change differed clearly., Conclusions: We identified Perc85 as optimal lung density parameter, which correlated significantly with DLCO and FVC, confirming a lung parenchymal structure-function relation in SSc. This provides support for future studies to determine whether structural changes do precede lung function decline., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: Obesity has been associated with microstructural brain tissue damage. Different fat compartments demonstrate different metabolic and endocrine behaviors. The aim was to investigate the individual associations between abdominal visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) and microstructural integrity in the brain., Methods: This study comprised 243 subjects aged 65.4 ± 6.7 years. The associations between abdominal VAT and SAT, assessed by CT, and magnetization transfer imaging markers of brain microstructure for gray and white matter were analyzed and adjusted for confounding factors., Results: VAT was associated with normalized MTR peak height in gray (β -0.216) and white matter (β -0.240) (both P < 0.01) after adjustment for confounding factors. After adjustment for sex, age, and descent, SAT was associated with normalized MTR peak height in gray and white matter, but not after additional correction for BMI, hypertension, current smoking, statin use, and type 2 diabetes (respectively, β -0.055 and β 0.035, both P > 0.05). Stepwise linear regression analysis showed that only VAT was associated with normalized MTR peak height in gray and white matter (both P < 0.001)., Conclusions: Our data indicate that increased abdominal VAT rather than SAT is associated with microstructural brain tissue damage in elderly individuals., (© 2015 The Obesity Society.)

Background: To characterize and directly quantify regurgitant jets of left atrioventricular valve (LAVV) in patients with corrected atrioventricular septal defect (AVSD) by four-dimensional (4D)Flow Cardiovascular Magnetic Resonance (CMR), streamline visualization and retrospective valve tracking., Methods: Medical ethical committee approval and informed consent from all patients or their parents were obtained. In 32 corrected AVSD patients (age 26 ± 12 years), echocardiography and whole-heart 4DFlow CMR were performed. Using streamline visualization on 2- and 4-chamber views, the angle between regurgitation and annulus was followed throughout systole. On through-plane velocity-encoded images reformatted perpendicular to the regurgitation jet the cross-sectional jet circularity index was assessed and regurgitant volume and fraction were calculated. Correlation and agreement between different techniques was performed with Pearson's r and Spearman's rho correlation and Bland-Altman analysis., Results: In 8 patients, multiple regurgitant jets over the LAVV were identified. Median variation in regurgitant jet angle within patients was 36°(IQR 18-64°) on the 2-chamber and 30°(IQR 20-40°) on the 4-chamber. Regurgitant jets had a circularity index of 0.61 ± 0.16. Quantification of the regurgitation volume was feasible with 4DFlow CMR with excellent correlation between LAVV effective forward flow and aortic flow (r = 0.97, p < 0.001) for internal validation and moderate correlation with planimetry derived regurgitant volume (r = 0.65, p < 0.001) and echocardiographic grading (rho = 0.51, p = 0.003)., Conclusions: 4DFlow CMR with streamline visualization revealed multiple, dynamic and eccentric regurgitant jets with non-circular cross-sectional shape in patients after AVSD correction. 4DFlow with retrospective valve tracking allows direct and accurate quantification of the regurgitation of these complex jets.

Coronary computed tomography angiography (CTA) has important prognostic value. Additionally, quantitative CTA (QCT) provides a more detailed accurate assessment of coronary artery disease (CAD) on CTA. Potentially, a risk score incorporating all quantitative stenosis parameters allows accurate risk stratification. Therefore, the purpose of this study was to determine if an automatic quantitative assessment of CAD using QCT combined into a CTA risk score allows risk stratification of patients. In 300 patients, QCT was performed to automatically detect and quantify all lesions in the coronary tree. Using QCT, a novel CTA risk score was calculated based on plaque extent, severity, composition, and location on a segment basis. During follow-up, the composite end point of all-cause mortality, revascularization, and nonfatal infarction was recorded. In total, 10% of patients experienced an event during a median follow-up of 2.14 years. The CTA risk score was significantly higher in patients with an event (12.5 [interquartile range 8.6 to 16.4] vs 1.7 [interquartile range 0 to 8.4], p <0.001). In 127 patients with obstructive CAD (≥50% stenosis), 27 events were recorded, all in patients with a high CTA risk score. In conclusion, the present study demonstrated that a fully automatic QCT analysis of CAD is feasible and can be applied for risk stratification of patients with suspected CAD. Furthermore, a novel CTA risk score incorporating location, severity, and composition of coronary lesion was developed. This score may improve risk stratification but needs to be confirmed in larger studies., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: Familial longevity is marked by enhanced peripheral but not hepatic insulin sensitivity. The liver has a critical role in the pathogenesis of hepatic insulin resistance. Therefore we hypothesized that the extent of liver steatosis would be similar between offspring of long-lived siblings and control subjects. To test our hypothesis, we investigated the extent of liver steatosis in non-diabetic offspring of long-lived siblings and age-matched controls by measuring liver enzymes in plasma and liver fat by computed tomography (CT)., Research Design and Methods: We measured nonfasting alanine transaminase (ALT), aspartate aminotransferase (AST), and Υ-glutamyl transferase (GGT) in 1625 subjects (736 men, mean age 59.1 years) from the Leiden Longevity Study, comprising offspring of long-lived siblings and partners thereof. In a random subgroup, fasting serum samples (n = 230) were evaluated and CT was performed (n = 268) for assessment of liver-spleen (L/S) ratio and the prevalence of moderate-to-severe non-alcoholic fatty liver disease (NAFLD). Linear mixed model analysis was performed adjusting for age, gender, body mass index, smoking, use of alcohol and hepatotoxic medication, and correlation of sibling relationship., Results: Offspring of long-lived siblings had higher nonfasting ALT levels as compared to control subjects (24.3 mmol/L versus 23.2 mmol/L, p = 0.03), while AST and GGT levels were similar between the two groups. All fasting liver enzyme levels were similar between the two groups. CT L/S ratio and prevalence of moderate-to-severe NAFLD was similar between groups (1.12 vs 1.14, p = 0.25 and 8% versus 8%, p = 0.91, respectively)., Conclusions: Except for nonfasting levels of ALT, which were slightly higher in the offspring of long-lived siblings compared to controls, no differences were found between groups in the extent of liver steatosis, as assessed with liver biochemical tests and CT. Thus, our data indicate that the extent of liver steatosis is similar between offspring of long-lived siblings and control subjects.

Introduction: It has been reported that the time between symptom onset and objective diagnosis of pulmonary embolism (PE) does not affect patients' prognosis with regard to re-thrombosis and mortality risk. However, this observation is contra-intuitive and poorly understood. We further elaborated on this paradox by evaluating thrombus load and right ventricular function in patients with and without diagnostic delay., Materials and Methods: We performed a post hoc analysis of a previously published observational prospective outcome study in 113 consecutive PE patients. Qanadli-score and RV/LV ratio were scored in all patients, as was the duration from symptom onset to clinical presentation and diagnosis. Diagnostic delay was defined as a period of more than 7 days between symptom onset and clinical presentation. Further endpoints were mortality and hospital readmission in a 6-week follow-up period., Results: Twenty patients with and 93 patients without delay were studied, who had comparable baseline characteristics and co-morbidities. In linear analyses, Qanadli-score (R² of 0.021; P = 0.130) and RV/LV ratio (R² < 0.001; P = 0.991) were not associated with diagnostic delay. Likewise, longer delay was not predictive of 6-week mortality (odds ratio, 0.65; 95% CI, 0.08-5.57) or hospital readmission (odds ratio, 0.75; 95% CI, 0.15-3.65)., Conclusion: In our patient cohort, diagnostic delay was not associated with higher thrombus load or right ventricular dysfunction. This provides a possible explanation for the lack of prognostic relevance of diagnostic delay., (© 2013 International Society on Thrombosis and Haemostasis.)

Offspring of long-lived parents have a low prevalence of cardiovascular disease in middle age. The purposes of this study were to investigate calcium scores in offspring as compared to controls and to determine the influence of cardiovascular risk factors. CT coronary artery calcium score was measured in offspring of long-lived families (n = 244, 125 males) and their partners (n = 223, 96 males) who served as controls. Calcium scores were analyzed separately for sexes. Subjects were grouped by very low calcium score ≤10 and scores above 10. Nonparametric Mann-Whitney test, chi-squared tests, and logistic regression analyses were performed to determine the association between calcium scores, familial longevity, and cardiovascular risk factors. More offspring of long-lived parents had lower calcium scores than controls. In men, 34 % of offspring had score ≤10 versus 21 % of controls (odds ratio (OR) and 95 % confidence interval (CI) 2.0, 1.08-3.7, p = 0.028). In women, 70 % of offspring had score ≤10 versus 54 % of controls (OR 1.9, 95 % CI 1.13-3.4, p = 0.019). Differences remained significant after correction for age (men, p = 0.043 and women, p = 0.003) and further correction for major risk factors in women, indicating genetic influence for lower calcium scores. In men, the association was found to be influenced by cardiovascular risk factors. Men and women with a familial propensity to become long-lived have lower coronary artery calcium scores than controls. Low scores may indicate a younger biologic arterial age associated with a low risk for incident cardiovascular disease.