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Abstract Visual scoring of interstitial lung disease in systemic sclerosis (SSc-ILD) from CT scans is laborious, subjective and time-consuming. This study aims to develop a deep learning framework to automate SSc-ILD scoring. The automated framework is a cascade of two neural networks. The first network selects the craniocaudal positions of the five scoring levels. Subsequently, for each level, the second network estimates the ratio of three patterns to the total lung area: the total extent of disease (TOT), ground glass (GG) and reticulation (RET). To overcome the score imbalance in the second network, we propose a method to augment the training dataset with synthetic data. To explain the network’s output, a heat map method is introduced to highlight the candidate interstitial lung disease regions. The explainability of heat maps was evaluated by two human experts and a quantitative method that uses the heat map to produce the score. The results show that our framework achieved a $$\kappa$$ κ of 0.66, 0.58, and 0.65, for the TOT, GG and RET scoring, respectively. Both experts agreed with the heat maps in 91%, 90% and 80% of cases, respectively. Therefore, it is feasible to develop a framework for automated SSc-ILD scoring, which performs competitively with human experts and provides high-quality explanations using heat maps. Confirming the model’s generalizability is needed in future studies.

Pulmonary function tests (PFTs) play an important role in screening and following-up pulmonary involvement in systemic sclerosis (SSc). However, some patients are not able to perform PFTs due to contraindications. In addition, it is unclear how lung function is affected by changes in lung structure in SSc. Therefore, this study aims to explore the potential of automatically estimating PFT results from chest CT scans of SSc patients and how different regions influence the estimation of PFTs. Deep regression networks were developed with transfer learning to estimate PFTs from 316 SSc patients. Segmented lungs and vessels were used to mask the CT images to train the network with different inputs: from entire CT scan, lungs-only to vessels-only. The network trained on entire CT scans with transfer learning achieved an ICC of 0.71, 0.76, 0.80, and 0.81 for the estimation of DLCO, FEV1, FVC and TLC, respectively. The performance of the networks gradually decreased when trained on data from lungs-only and vessels-only. Regression attention maps showed that regions close to large vessels were highlighted more than other regions, and occasionally regions outside the lungs were highlighted. These experiments show that apart from the lungs and large vessels, other regions contribute to PFT estimation. In addition, adding manually designed biomarkers increased the correlation (R) from 0.75, 0.74, 0.82, and 0.83 to 0.81, 0.83, 0.88, and 0.90, respectively. This suggests that that manually designed imaging biomarkers can still contribute to explaining the relation between lung function and structure.

Abstract The shape and distribution of vascular lesions in pulmonary embolism (PE) and chronic thromboembolic pulmonary hypertension (CTEPH) are different. We investigated whether automated quantification of pulmonary vascular morphology and densitometry in arteries and veins imaged by computed tomographic pulmonary angiography (CTPA) could distinguish PE from CTEPH. We analyzed CTPA images from a cohort of 16 PE patients, 6 CTEPH patients, and 15 controls. Pulmonary vessels were extracted with a graph‐cut method, and separated into arteries and veins using deep‐learning classification. Vascular morphology was quantified by the slope (α) and intercept (β) of the vessel radii distribution. To quantify lung perfusion defects, the median pulmonary vascular density was calculated. By combining these measurements with densities measured in parenchymal areas, pulmonary trunk, and descending aorta, a static perfusion curve was constructed. All separate quantifications were compared between the three groups. No vascular morphology differences were detected in contrast to vascular density values. The median vascular density (interquartile range) was −567 (113), −452 (95), and −470 (323) HU, for the control, PE, and CTEPH group. The static perfusion curves showed different patterns between groups, with a statistically significant difference in aorta‐pulmonary trunk gradient between the PE and CTEPH groups (p = 0.008). In this proof of concept study, not vasculature morphology but densities differentiated between patients of three groups. Further technical improvements are needed to allow for accurate differentiation between PE and CTEPH, which in this study was only possible statistically by measuring the density gradient between aorta and pulmonary trunk.

Abstract Aim This study explores the relationship between in vivo 4D flow cardiovascular magnetic resonance (CMR) derived blood flow energetics in the total cavopulmonary connection (TCPC), exercise capacity and CMR-derived liver fibrosis/congestion. Background The Fontan circulation, in which both caval veins are directly connected with the pulmonary arteries (i.e. the TCPC) is the palliative approach for single ventricle patients. Blood flow efficiency in the TCPC has been associated with exercise capacity and liver fibrosis using computational fluid dynamic modelling. 4D flow CMR allows for assessment of in vivo blood flow energetics, including kinetic energy (KE) and viscous energy loss rate (EL). Methods Fontan patients were prospectively evaluated between 2018 and 2021 using a comprehensive cardiovascular and liver CMR protocol, including 4D flow imaging of the TCPC. Peak oxygen consumption (VO2) was determined using cardiopulmonary exercise testing (CPET). Iron-corrected whole liver T1 (cT1) mapping was performed as a marker of liver fibrosis/congestion. KE and EL in the TCPC were computed from 4D flow CMR and normalized for inflow. Furthermore, blood flow energetics were compared between standardized segments of the TCPC. Results Sixty-two Fontan patients were included (53% male, 17.3 ± 5.1 years). Maximal effort CPET was obtained in 50 patients (peak VO2 27.1 ± 6.2 ml/kg/min, 56 ± 12% of predicted). Both KE and EL in the entire TCPC (n = 28) were significantly correlated with cT1 (r = 0.50, p = 0.006 and r = 0.39, p = 0.04, respectively), peak VO2 (r = − 0.61, p = 0.003 and r = − 0.54, p = 0.009, respectively) and % predicted peak VO2 (r = − 0.44, p = 0.04 and r = − 0.46, p = 0.03, respectively). Segmental analysis indicated that the most adverse flow energetics were found in the Fontan tunnel and left pulmonary artery. Conclusions Adverse 4D flow CMR derived KE and EL in the TCPC correlate with decreased exercise capacity and increased levels of liver fibrosis/congestion. 4D flow CMR is promising as a non-invasive screening tool for identification of patients with adverse TCPC flow efficiency.

Abstract Long scan times prohibit a widespread clinical applicability of 4D flow MRI in Fontan patients. As pulsatility in the Fontan pathway is minimal during the cardiac cycle, acquiring non-ECG gated 3D flow MRI may result in a reduction of scan time while accurately obtaining time-averaged clinical parameters in comparison with 2D and 4D flow MRI. Thirty-two Fontan patients prospectively underwent 2D (reference), 3D and 4D flow MRI of the Fontan pathway. Multiple clinical parameters were assessed from time-averaged flow rates, including the right-to-left pulmonary flow distribution (main endpoint) and systemic-to-pulmonary collateral flow (SPCF). A ten-fold reduction in scan time was achieved [4D flow 15.9 min (SD 2.7 min) and 3D flow 1.6 min (SD 7.8 s), p

Abstract Background We hypothesize that dobutamine-induced stress impacts intracardiac hemodynamic parameters and that this may be linked to decreased exercise capacity in Fontan patients. Therefore, the purpose of this study was to assess the effect of pharmacologic stress on intraventricular kinetic energy (KE), viscous energy loss (EL) and vorticity from four-dimensional (4D) Flow cardiovascular magnetic resonance (CMR) imaging in Fontan patients and to study the association between stress response and exercise capacity. Methods Ten Fontan patients underwent whole-heart 4D flow CMR before and during 7.5 μg/kg/min dobutamine infusion and cardiopulmonary exercise testing (CPET) on the same day. Average ventricular KE, EL and vorticity were computed over systole, diastole and the total cardiac cycle (vorticity_volavg cycle, KEavg cycle, ELavg cycle). The relation to maximum oxygen uptake (VO2 max) from CPET was tested by Pearson’s correlation or Spearman’s rank correlation in case of non-normality of the data. Results Dobutamine stress caused a significant 88 ± 52% increase in KE (KEavg cycle: 1.8 ± 0.5 vs 3.3 ± 0.9 mJ, P

Background Computed tomography pulmonary angiography (CTPA) is the imaging modality of choice for the diagnosis of acute pulmonary embolism (PE). With computed tomography pulmonary perfusion (CTPP) additional information on lung perfusion can be assessed, but its value in PE risk stratification is unknown. We aimed to evaluate the correlation between CTPP-assessed perfusion defect score (PDS) and clinical presentation and its predictive value for adverse short-term outcome of acute PE. Patients and Methods This was an exploratory, observational study in 100 hemodynamically stable patients with CTPA-confirmed acute PE in whom CTPP was performed as part of routine clinical practice. We calculated the difference between the mean PDS in patients with versus without chest pain, dyspnea, and hemoptysis and 7-day adverse outcome. Multivariable logistic regression analysis and likelihood-ratio test were used to assess the added predictive value of PDS to CTPA parameters of right ventricle dysfunction and total thrombus load, for intensive care unit admission, reperfusion therapy and PE-related death. Results We found no correlation between PDS and clinical symptoms. PDS was correlated to reperfusion therapy (n = 4 with 16% higher PDS, 95% confidence interval [CI]: 3.5–28%) and PE-related mortality (n = 2 with 22% higher PDS, 95% CI: 4.9–38). Moreover, PDS had an added predictive value to CTPA assessment for PE-related mortality (from Chi-square 14 to 19, p = 0.02). Conclusion CTPP-assessed PDS was not correlated to clinical presentation of acute PE. However, PDS was correlated to reperfusion therapy and PE-related mortality and had an added predictive value to CTPA-reading for PE-related mortality; this added value needs to be demonstrated in larger studies.

The prevalence of end-stage kidney disease (ESKD) is rapidly increasing and mostly occurring in patients aged 65 years or older. The main cause of death in these patients is cardiovascular disease (CVD). Novel markers of vascular integrity may thus be of clinical value for identifying patients at high risk for CVD. Here we associated the levels of selected circulating angiogenic miRNAs, angiopoietin-2 (Ang-2) and asymmetric dimethylarginine (ADMA) with cardiovascular structure and function (as determined by cardiovascular MRI) in 67 older patients reaching ESKD that were included from ‘The Cognitive decline in Older Patients with End stage renal disease’ (COPE) prospective, multicentered cohort study. We first determined the association between the vascular injury markers and specific heart conditions and observed that ESKD patients with coronary heart disease have significantly higher levels of circulating ADMA and miR-27a. Moreover, circulating levels of miR-27a were higher in patients with atrial fibrillation. In addition, the circulating levels of the vascular injury markers were associated with measures of cardiovascular structure and function obtained from cardiovascular MRI: pulse wave velocity (PWV), ejection fraction (EF) and cardiac index (CI). We found Ang-2 and miR-27a to be strongly correlated to the PWV, while Ang-2 also associated with ejection fraction. Finally, we observed that in contrast to miR-27a, Ang-2 was not associated with a vascular cause of the primary kidney disease, suggesting Ang-2 may be an ESKD-specific marker of vascular injury. Taken together, among older patients with ESKD, aberrant levels of vascular injury markers (miR-27a, Ang-2 and ADMA) associated with impaired cardiovascular function. These markers may serve to identify individuals at higher risk of CVD, as well as give insight into the underlying (vascular) pathophysiology.

Abstract Background The YEARS algorithm was designed to simplify the diagnostic process of suspected pulmonary embolism (PE) and to reduce the number of required computed tomography pulmonary angiography (CTPA) scans. Chest X-ray (CXR) is often used as initial imaging test in patients suspected for PE. Aim To determine if CXR results differ between patients with confirmed PE and with PE ruled out, and to investigate whether CXR provides incremental diagnostic value to the YEARS criteria that is used for selecting patients with CTPA indication. Methods This post-hoc analysis concerned 1,473 consecutive patients with suspected PE who were managed according to YEARS and were subjected to CXR as part of routine care. The prevalence and likelihood ratios of seven main CXR findings for a final diagnosis of PE were calculated. Results A total of 214 patients were diagnosed with PE at baseline (15%). Abnormal CXR occurred more often in patients with confirmed PE (36%, 77/214) than in patients without PE (26%; 327/1,259), for an odds ratio of 1.60 (95% confidence interval: 1.18–2.18). Only the unexpected finding of a (rib)fracture or pneumothorax, present in as few as six patients (0.4%), significantly lowered the post-test probability of PE to an extent that CTPA could have been avoided. Conclusion The incremental diagnostic value of CXR to the YEARS algorithm to rule out PE was limited. CXR was more frequently abnormal in patients with PE than in those in whom PE was ruled out. These data do not support to perform CXR routinely in all patients with suspected PE, prior to CTPA imaging.

Abstract Introduction Magnetic resonance direct thrombus imaging (MRDTI) is a promising technique to improve the diagnostic management of patients with a suspected ipsilateral recurrent deep vein thrombosis (DVT) by direct visualization of a thrombus. Another magnetic resonance imaging (MRI) technique, T1-weighted turbo spin-echo spectral attenuated inversion recovery (TSE-SPAIR), has the potential to image a thrombus directly with a high spatial resolution as well. The main aim of this pilot study was to investigate if adding the TSE-SPAIR sequence to an MRDTI sequence performed in patients with suspected recurrent DVT may increase the diagnostic confidence of expert MRDTI readers. Methods Fifteen patients with suspected acute recurrent DVT were included in this study. The TSE-SPAIR sequence was scanned directly after the MRDTI scan but not used to guide clinical decision making, and both scans were adjudicated post hoc two times separately by three independent expert MRDTI readers. Diagnostic confidence was scored on a 4-point Likert scale: (1) poor (definite diagnosis impossible), (2) fair (evaluation of major findings possible), (3) good (definite diagnosis possible), and (4) excellent (exact diagnosis possible). Results The diagnostic confidence of expert readers increased when adding the TSE-SPAIR sequence on top of the MRDTI sequence from “good” (median, 3.0; interquartile range [IQR], 2.66–3.0) to “excellent” (median, 3.67; IQR 3.33–3.67; p = 0.001). Evaluation of the scans in the reversed order 5 months after initial reading showed similar results. Diagnostic accuracy for proximal DVT of both scan techniques was good. Conclusion The extra TSE-SPAIR sequence may help increase diagnostic confidence of radiologists in cases of uncertain diagnosis in patients with suspected ipsilateral recurrent DVT.

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

We present 3 patients with 4 causes of mediastinal fluid collection after congenital cardiac surgery in this extended case report. Volumetric computed tomography played an essential role in diagnosing causes and extent, relevant to subsequent management. Recent advances in volumetric computed tomography allow fast and accurate imaging of cardiovascular and extravascular structures in children with acceptable radiation dose, providing a powerful imaging tool for the evaluation of complications after congenital cardiac surgery.

Objective To assess the atrial and ventricular diastolic function response to dobutamine stress in Fontan patients, and to relate these measurements to exercise capacity and events during the follow-up.Methods We performed a secondary analysis of a cross-sectional multicentre study of Fontan patients with intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) modification. Subjects underwent cardiac MRI during rest and low-dose dobutamine stress, and cardiopulmonary exercise testing. Atrial and diastolic ventricular function parameters were derived from volume-time curves.Medical records were abstracted for a composite end-point of death, listing for transplant, arrhythmia and reintervention. Spearman’s r correlation tests and Cox proportional hazards models were used to assess the relation between the dobutamine response for atrial and diastolic ventricular function and outcomes, including exercise capacity.Results We included 57 patients (26 ECC; 31 ILT) aged 12.8 (IQR (10.3–15.5)) years. During dobutamine stress atrial cyclic volume change increased (3.0 (0.4–5.9) mL/m2, p

BACKGROUND:The pathophysiology of chronic thromboembolic pulmonary hypertension (CTEPH) is not fully understood. Poor-quality anticoagulation may contribute to a higher risk of CTEPH after acute pulmonary embolism (PE), partly explaining the transition from acute PE to CTEPH. We assessed the association between the time in therapeutic range (TTR) of vitamin-K antagonist (VKA) treatment and incidence of CTEPH after a PE diagnosis. METHODS:Case-control study in which the time spent in, under and above therapeutic range was calculated in 44 PE patients who were subsequently diagnosed with CTEPH (cases). Controls comprised 150 consecutive PE patients in whom echocardiograms two years later did not show pulmonary hypertension. All patients were treated with VKA for at least 6 months after the PE diagnosis. Time in (TTR), under and above range were calculated. Mean differences between cases and controls were estimated by linear regression. RESULTS:Mean TTR during the initial 6-month treatment period was 72% in cases versus 78% in controls (mean difference -6%, 95%CI -12 to -0.1), mainly explained by more time above the therapeutic range in the cases. Mean difference of time under range was 0% (95%CI -6 to 7) and 2% (95CI% -3 to 7) during the first 3 and 6 months, respectively. In a multivariable model, adjusted odds ratios (ORs) for CTEPH were around unity considering different thresholds for 'poor anticoagulation', i.e. TTR

[This corrects the article DOI: 10.1371/journal.pone.0232354.].

Right ventricular (RV) dysfunction caused by acute pulmonary embolism (PE) is associated with poor short- and long-term prognosis. RV dilatation as a proxy for RV dysfunction can be assessed by calculating the right-to-left ventricle diameter (RV/LV) ratio on standard computed tomography pulmonary angiography (CTPA) images. It is unknown whether dedicated training is required to accurately and reproducibly measure RV/LV ratio therefore we aimed to assess these parameters in residents in internal medicine without experience in CTPA reading.CTPA images of 100 patients with PE were assessed by three residents after single instruction, and one experienced thoracic radiologist. Maximum diameters were evaluated in the axial view by measuring the distance between the ventricular endocardium and the interventricular septum, perpendicular to the long axis of the heart. RV dilatation was defined as a ratio of ≥1.0. Interobserver accuracy and reproducibility was determined using Kappa statistics, Bland-Altman analysis and Spearman's rank correlation.The kappa statistic for the presence of RV dilatation of the residents compared to the experienced radiologist ranged from 0.83-0.94. The average interobserver difference in calculated RV/LV ratio's (±SD) between the three residents was: -0.01 (SD0.11), 0.07 (SD0.14) and 0.06 (SD0.18) with an overall mean RV/LV diameter ratio of 1.04. In line with this, Spearman's rank correlation coefficients were 0.92, 0.88 and 0.85 respectively indicating very good correlation (p

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.