European Association of Cardiovascular Imaging (EACVI) survey on Cardiovascular Multimodality Imaging in Acute Myocarditis.

AIMS: To assess the current role of cardiac imaging in the diagnosis, management, and follow-up of patients with acute myocarditis (AM) through an European Association of Cardiovascular Imaging survey. METHODS AND RESULTS: A total of 412 volunteers from 74 countries responded to the survey. Most participants worked in tertiary centres(56%). All participants had access to echocardiography, while 79% and 75% had access to cardiac computed tomography (CCTA) and cardiac magnetic resonance (CMR), respectively. Less than half(47%) had access to myocardial biopsy and only 5% used this test routinely. CMR was performed within 7 days of presentation in 73% of cases. Non-ischemic late gadolinium enhancement (LGE,88%) and high-signal intensity in T2-weighted images(74%) were the most used diagnostic criteria for AM. CCTA was preferred to coronary angiography by 47% of participants to exclude coronary artery disease. Systematic prescription of beta-blockers and ACEi was reported by 38% and 32% of participants. Around a quarter of participants declared considering LGE burden as a reason to treat. Most participants (90%) reported performing a follow-up echocardiogram, while 63% scheduled a follow-up CMR. The main reason for treatment discontinuation was improvement of left ventricular ejection fraction(89%), followed by LGE regression(60%). In two-thirds of participants the decision to resume high-intensity sport was influenced by residual LGE. CONCLUSION: This survey confirms the high utilization of cardiac imaging in AM, but reveals major differences in how cardiac imaging is used and how the condition is managed between centres, underlining the need for recommendation statements in this topic.

Virtual calcium removal in calcified coronary arteries with photon-counting detector CT-first in-vivo experience.

Purpose: To evaluate the feasibility and accuracy of quantification of calcified coronary stenoses using virtual non-calcium (VNCa) images in coronary CT angiography (CCTA) with photon-counting detector (PCD) CT compared with quantitative coronary angiography (QCA). Materials and methods: This retrospective, institutional-review board approved study included consecutive patients with calcified coronary artery plaques undergoing CCTA with PCD-CT and invasive coronary angiography between July and December 2022. Virtual monoenergetic images (VMI) and VNCa images were reconstructed. Diameter stenoses were quantified on VMI and VNCa images by two readers. 3D-QCA served as the standard of reference. Measurements were compared using Bland-Altman analyses, Wilcoxon tests, and intraclass correlation coefficients (ICC). Results: Thirty patients [mean age, 64 years ± 8 (standard deviation); 26 men] with 81 coronary stenoses from calcified plaques were included. Ten of the 81 stenoses (12%) had to be excluded because of erroneous plaque subtraction on VNCa images. Median diameter stenosis determined on 3D-QCA was 22% (interquartile range, 11%-35%; total range, 4%-88%). As compared with 3D-QCA, VMI overestimated diameter stenoses (mean differences -10%, p < .001, ICC: .87 and -7%, p < .001, ICC: .84 for reader 1 and 2, respectively), whereas VNCa images showed similar diameter stenoses (mean differences 0%, p = .68, ICC: .94 and 1%, p = .07, ICC: .93 for reader 1 and 2, respectively). Conclusion: First experience in mainly minimal to moderate stenoses suggests that virtual calcium removal in CCTA with PCD-CT, when feasible, has the potential to improve the quantification of calcified stenoses.

Impact of late gadolinium enhancement image acquisition resolution on neural network based automatic scar segmentation.

BACKGROUND: Automatic myocardial scar segmentation from late gadolinium enhancement (LGE) images using neural networks promises an alternative to time-consuming and observer-dependent semi-automatic approaches. However, alterations in data acquisition, reconstruction as well as post-processing may compromise network performance. The objective of the present work was to systematically assess network performance degradation due to a mismatch of point-spread function between training and testing data. METHODS: Thirty-six high-resolution (0.7×0.7×2.0 mm3) LGE k-space datasets were acquired post-mortem in porcine models of myocardial infarction. The in-plane point-spread function and hence in-plane resolution Δx was retrospectively degraded using k-space lowpass filtering, while field-of-view and matrix size were kept constant. Manual segmentation of the left ventricle (LV) and healthy remote myocardium was performed to quantify location and area (% of myocardium) of scar by thresholding (≥ SD5 above remote). Three standard U-Nets were trained on training resolutions Δxtrain = 0.7, 1.2 and 1.7 mm to predict endo- and epicardial borders of LV myocardium and scar. The scar prediction of the three networks for varying test resolutions (Δxtest = 0.7 to 1.7 mm) was compared against the reference SD5 thresholding at 0.7 mm. Finally, a fourth network trained on a combination of resolutions (Δxtrain = 0.7 to 1.7 mm) was tested. RESULTS: The prediction of relative scar areas showed the highest precision when the resolution of the test data was identical to or close to the resolution used during training. The median fractional scar errors and precisions (IQR) from networks trained and tested on the same resolution were 0.0 percentage points (p.p.) (1.24 - 1.45), and - 0.5 - 0.0 p.p. (2.00 - 3.25) for networks trained and tested on the most differing resolutions, respectively. Deploying the network trained on multiple resolutions resulted in reduced resolution dependency with median scar errors and IQRs of 0.0 p.p. (1.24 - 1.69) for all investigated test resolutions. CONCLUSION: A mismatch of the imaging point-spread function between training and test data can lead to degradation of scar segmentation when using current U-Net architectures as demonstrated on LGE porcine myocardial infarction data. Training networks on multi-resolution data can alleviate the resolution dependency.

EACVI survey on the evaluation of mitral regurgitation.

AIMS: To evaluate the diagnosis and imaging of patients with mitral regurgitation and the management in routine clinical practice across Europe, the EACVI Scientific Initiatives Committee performed a survey across European centres. In particular, the routine use of echocardiography, advanced imaging modalities, heart valve clinics and heart valve teams was explored. METHODS AND RESULTS: A total of 61 responders, mainly tertiary centres or university hospitals, from 26 different countries responded to the survey, which consisted of 22 questions. For most questions related to echocardiography and advanced imaging, the answers were relatively homogeneous and demonstrated good adherence to current recommendations. In particular, the centres used a multiparametric echocardiographic approach, and selected the effective regurgitant orifice and vena contracta width as their preferred assessments. Two-dimensional (2D) measurements are still the most widely used parameters to assess left ventricular structure, however the majority use three-dimensional (3D) trans-oesophageal echocardiography (TOE) to evaluate valve morphology in severe MR. The majority of centres reported the onsite availability and clinical use of ergometric stress echocardiography, cardiac computed tomography (CCT) and cardiac magnetic resonance imaging (CMR). Heart valve clinics and heart valve teams were also widely prevalent. CONCLUSION: Consistent with current guidelines echocardiography (TTE and TOE) remains the first line and central imaging modality for the assessment of mitral regurgitation although the complementary use of 3D TOE, CT and CMR appears to be growing. Heart valve clinics and heart valve teams are now widely prevalent.

Effect of temporal resolution on calcium scoring: insights from photon-counting detector CT.

To intra-individually investigate the variation of coronary artery calcium (CAC), aortic valve calcium (AVC), and mitral annular calcium (MAC) scores and the presence of blur artifacts as a function of temporal resolution in patients undergoing non-contrast cardiac CT on a dual-source photon counting detector (PCD) CT. This retrospective, IRB-approved study included 70 patients (30 women, 40 men, mean age 78 ± 9 years) who underwent ECG-gated cardiac non-contrast CT with PCD-CT (gantry rotation time 0.25 s) prior to transcatheter aortic valve replacement. Each scan was reconstructed at a temporal resolution of 66 ms using the dual-source information and at 125 ms using the single-source information. Average heart rate and heart rate variability were calculated from the recorded ECG. CAC, AVC, and MAC were quantified according to the Agatston method on images with both temporal resolutions. Two readers assessed blur artifacts using a 4-point visual grading scale. The influence of average heart rate and heart rate variability on calcium quantification and blur artifacts of the respective structures were analyzed by linear regression analysis. Mean heart rate and heart rate variability during data acquisition were 76 ± 17 beats per minute (bpm) and 4 ± 6 bpm, respectively. CAC scores were smaller on 66 ms (median, 511; interquartile range, 220-978) than on 125 ms reconstructions (538; 203-1050, p < 0.001). Median AVC scores [2809 (2009-3952) versus 3177 (2158-4273)] and median MAC scores [226 (0-1284) versus 251 (0-1574)] were also significantly smaller on 66ms than on 125ms reconstructions (p < 0.001). Reclassification of CAC and AVC risk categories occurred in 4% and 11% of cases, respectively, whereby the risk category was always overestimated on 125ms reconstructions. Image blur artifacts were significantly less on 66ms as opposed to 125 ms reconstructions (p < 0.001). Intra-individual analyses indicate that temporal resolution significantly impacts on calcium scoring with cardiac CT, with CAC, MAC, and AVC being overestimated at lower temporal resolution because of increased motion artifacts eventually leading to an overestimation of patient risk.

Spontaneous Dissection of a Septal Coronary Artery Mimicking Myocarditis.

Acute chest pain and dyspnea often raise coronary disease suspicion. When echocardiography and cardiac computed tomography findings appear normal, alternative diagnoses should be explored. We present a case initially suggestive of myocarditis but later revealed as coronary dissection by cardiac magnetic resonance. This case emphasizes the role of advanced imaging in atypical cardiac presentations.

A Novel Reconstruction Technique to Reduce Stair-Step Artifacts in Sequential Mode Coronary CT Angiography.

PURPOSE: Prospective electrocardiography-triggering is one of the most commonly used cardiac computed tomography (CT) scan modes but can be susceptible to stair-step artifacts in the transition areas of an acquisition over multiple cardiac cycles. We evaluated a novel reconstruction algorithm to reduce the occurrence and severity of such artifacts in sequential coronary CT angiography. MATERIALS AND METHODS: In this institutional review board-approved, retrospective study, 50 consecutive patients (16 females; mean age, 58.9 ± 15.2) were included who underwent coronary CT angiography on a dual-source photon-counting detector CT in the sequential ultra-high-resolution mode with a detector collimation of 120 × 0.2 mm. Each scan was reconstructed without (hereafter called standard reconstruction) and with the novel ZeeFree reconstruction algorithm, which aims to minimize stair-step artifacts. The presence and extent of stair-step artifacts were rated by 2 independent, blinded readers on a 4-point discrete visual scale. The relationship between the occurrences of artifacts was correlated with the average and variability of heart rate and with patient characteristics. RESULTS: A total of 504 coronary segments were included into the analyses. In standard reconstructions, reader 1 reported stair-step artifacts in 40/504 (7.9%) segments, from which 12/504 led to nondiagnostic image quality (2.4% of all segments). Reader 2 reported 56/504 (11.1%) stair-step artifacts, from which 11/504 lead to nondiagnostic image quality (2.2% of all segments). With the ZeeFree algorithm, 9/12 (75%) and 8/11 (73%) of the nondiagnostic segments improved to a diagnostic quality for readers 1 and 2, respectively. The ZeeFree reconstruction algorithm significantly reduced the frequency and extent of stair-step artifacts compared with standard reconstructions for both readers (P < 0.001, each). Heart rate variability and body mass index were significantly related to the occurrence of stair-step artifacts (P < 0.05). CONCLUSIONS: Our study demonstrates the feasibility and effectiveness of a novel reconstruction algorithm leading to a significant reduction of stair-step artifacts and, hence, a reduction of coronary segments with a nondiagnostic image quality in sequential ultra-high-resolution coronary photon-counting detector CT angiography.

Dose escalation for stereotactic arrhythmia radioablation of recurrent ventricular tachyarrhythmia - a phase II clinical trial.

BACKGROUND: Stereotactic arrhythmia radioablation (STAR) is delivered with a planning target volume (PTV) prescription dose of 25 Gy, mostly to the surrounding 75-85% isodose line. This means that the average and maximum dose received by the target is less than 35 Gy, which is the minimum threshold required to create a homogenous transmural fibrosis. Similar to catheter ablation, the primary objective of STAR should be transmural fibrosis to prevent heterogenous intracardiac conduction velocities and the occurrence of sustained ventricular arrhythmias (sVA) caused by reentry. We hypothesize that the current dose prescription used in STAR is inadequate for the long-term prevention of sVA and that a significant increase in dose is necessary to induce transmural scar formation. OBJECTIVE: A single arm, multi-center, phase II, dose escalation prospective clinical trial employing the i3 + 3 design is being conducted to examine the safety of a radiation dose-escalation strategy aimed at inducing transmural scar formation. The ultimate objective of this trial is to decrease the likelihood of sVA recurrence in patients at risk. METHODS: Patients with ischemic or non-ischemic cardiomyopathy and recurrent sVA, with an ICD and history of ≥ 1 catheter ablation for sVA will be included. This is a prospective, multicenter, one-arm, dose-escalation trial utilizing the i3 + 3 design, a modified 3 + 3 specifically created to overcome limitations in traditional dose-finding studies. A total of 15 patients will be recruited. The trial aims to escalate the ITV dose from 27.0 Gy to an ITV prescription dose-equivalent level of maximum 35.1 Gy by keeping the PTV prescription dose constant at 25 Gy while increasing the dose to the target (i.e. the VT substrate without PTV margin) by step-wise reduction of the prescribing isodose line (85% down to 65%). The primary outcome of this trial is safety measured by registered radiation associated adverse events (AE) up to 90 days after study intervention including radiation associated serious adverse events graded as at least 4 or 5 according to CTCAE v5, radiation pneumonitis or pericarditis requiring hospitalization and decrease in LVEF ≥ 10% as assessed by echocardiography or cardiac MRI at 90 days after STAR. The sample size was determined assuming an acceptable primary outcome event rate of 20%. Secondary outcomes include sVA burden at 6 months after STAR, time to first sVA recurrence, reduction in appropriate ICD therapies, the need for escalation of antiarrhythmic drugs, non-radiation associated safety and patient reported outcome measures such as SF-36 and EQ5D. DISCUSSION: DEFT-STAR is an innovative prospective phase II trial that aims to evaluate the optimal radiation dose for STAR in patients with therapy-refractory sVA. The trial has obtained IRB approval and focuses on determining the safe and effective radiation dose to be employed in the STAR procedure. TRIAL REGISTRATION: NCT05594368.

Dipeptidyl peptidase 3 plasma levels predict cardiogenic shock and mortality in acute coronary syndromes.

BACKGROUND AND AIMS: Dipeptidyl peptidase 3 (DPP3) is a protease involved in the degradation of angiotensin II which disturbs peripheral blood pressure regulation and compromises left ventricular function. This study examined the relationship of circulating DPP3 (cDPP3) with cardiogenic shock (CS) and mortality in patients presenting with acute coronary syndromes (ACS). METHODS: Plasma cDPP3 levels were assessed at baseline and 12-24 h after presentation in patients with ACS prospectively enrolled into the multi-centre SPUM-ACS study (n = 4787). RESULTS: Circulating DPP3 levels were associated with in-hospital CS when accounting for established risk factors including the ORBI risk score [per log-2 increase, hazard ratio (HR) 1.38, 95% confidence interval (CI) 1.05-1.82, P = .021]. High cDPP3 was an independent predictor of mortality at 30 days (HR 1.87, 95% CI 1.36-2.58, P < .001) and at one year (HR 1.61, 95% CI 1.28-2.02, P < .001) after adjustment for established risk factors and the GRACE 2.0 score. Compared to values within the normal range, persistently elevated cDPP3 levels at 12-24 h were associated with 13.4-fold increased 30-day mortality risk (HR 13.42, 95% CI 4.86-37.09, P < .001) and 5.8-fold increased 1-year mortality risk (HR 5.79, 95% CI 2.70-12.42, P < .001). Results were consistent across various patient subgroups. CONCLUSIONS: This study identifies cDPP3 as a novel marker of CS and increased mortality in patients with ACS. Circulating DPP3 offers prognostic information beyond established risk factors and improves early risk assessment.

Cardiac Virtual Noncontrast Images for Calcium Quantification with Photon-counting Detector CT.

Purpose: To assess the accuracy of aortic valve calcium (AVC), mitral annular calcium (MAC), and coronary artery calcium (CAC) quantification and risk stratification using virtual noncontrast (VNC) images from late enhancement photon-counting detector CT as compared with true noncontrast images. Materials and Methods: This retrospective, institutional review board-approved study evaluated patients undergoing photon-counting detector CT between January and September 2022. VNC images were reconstructed from late enhancement cardiac scans at 60, 70, 80, and 90 keV using quantum iterative reconstruction (QIR) strengths of 2-4. AVC, MAC, and CAC were quantified on VNC images and compared with quantification of AVC, MAC, and CAC on true noncontrast images using Bland-Altman analyses, regression models, intraclass correlation coefficients (ICC), and Wilcoxon tests. Agreement between severe aortic stenosis likelihood categories and CAC risk categories determined from VNC and true noncontrast images was assessed by weighted κ analysis. Results: Ninety patients were included (mean age, 80 years ± 8 [SD]; 49 male patients). Scores were similar on true noncontrast images and VNC images at 80 keV for AVC and MAC, regardless of QIR strengths, and VNC images at 70 keV with QIR 4 for CAC (all P > .05). The best results were achieved using VNC images at 80 keV with QIR 4 for AVC (mean difference, 3; ICC = 0.992; r = 0.98) and MAC (mean difference, 6; ICC = 0.998; r = 0.99), and VNC images at 70 keV with QIR 4 for CAC (mean difference, 28; ICC = 0.996; r = 0.99). Agreement between calcification categories was excellent on VNC images at 80 keV for AVC (κ = 0.974) and on VNC images at 70 keV for CAC (κ = 0.967). Conclusion: VNC images from cardiac photon-counting detector CT enables patient risk stratification and accurate quantification of AVC, MAC, and CAC.Keywords: Coronary Arteries, Aortic Valve, Mitral Valve, Aortic Stenosis, Calcifications, Photon-counting Detector CT Supplemental material is available for this article © RSNA, 2023.

Current clinical use of speckle-tracking strain imaging: insights from a worldwide survey from the European Association of Cardiovascular Imaging (EACVI).

AIMS: Speckle-tracking echocardiography (STE) strain imaging has been a major advancement in myocardial function quantification. We aimed to explore current worldwide clinical application of STE. METHODS AND RESULTS: Access, feasibility, access, and clinical implementation of STE were investigated with a worldwide open-access online survey of the European Association of Cardiovascular Imaging. Participants (429 respondents and 77 countries) from tertiary centres (46%), private clinics, or public hospitals (54%) using different vendors for data acquisition and analysis were represented. Despite almost universal access (98%) to STE, only 39% of the participants performed and reported STE results frequently (>50%). Incomplete training and time constraints were the main reasons for not using STE more regularly. STE was mainly used to assess the LV (99%) and less frequently the right ventricular (57%) and the left atrial (46%) function. Cardiotoxicity (88%) and cardiac amyloidosis (87%) were the most frequent reasons for the clinical use of LV STE. Left atrial STE was used most frequently for the diagnosis of diastolic dysfunction and right ventricular STE for the assessment of right ventricle (RV) function in pulmonary hypertension (51%). Frequency of STE use, adherence to optimal techniques, and clinical appropriateness of STE differed according to training experience and across vendors. Key suggestions outlined by respondents to increase the clinical use of STE included improved reproducibility (48%) and standardization of strain values across vendors (42%). CONCLUSION: Although STE is now readily available, it is underutilized in the majority of centres. Structured training, improved reproducibility, and inter-vendor standardization may increase its uptake.

Clinical quantitative coronary artery stenosis and coronary atherosclerosis imaging: a Consensus Statement from the Quantitative Cardiovascular Imaging Study Group.

The detection and characterization of coronary artery stenosis and atherosclerosis using imaging tools are key for clinical decision-making in patients with known or suspected coronary artery disease. In this regard, imaging-based quantification can be improved by choosing the most appropriate imaging modality for diagnosis, treatment and procedural planning. In this Consensus Statement, we provide clinical consensus recommendations on the optimal use of different imaging techniques in various patient populations and describe the advances in imaging technology. Clinical consensus recommendations on the appropriateness of each imaging technique for direct coronary artery visualization were derived through a three-step, real-time Delphi process that took place before, during and after the Second International Quantitative Cardiovascular Imaging Meeting in September 2022. According to the Delphi survey answers, CT is the method of choice to rule out obstructive stenosis in patients with an intermediate pre-test probability of coronary artery disease and enables quantitative assessment of coronary plaque with respect to dimensions, composition, location and related risk of future cardiovascular events, whereas MRI facilitates the visualization of coronary plaque and can be used in experienced centres as a radiation-free, second-line option for non-invasive coronary angiography. PET has the greatest potential for quantifying inflammation in coronary plaque but SPECT currently has a limited role in clinical coronary artery stenosis and atherosclerosis imaging. Invasive coronary angiography is the reference standard for stenosis assessment but cannot characterize coronary plaques. Finally, intravascular ultrasonography and optical coherence tomography are the most important invasive imaging modalities for the identification of plaques at high risk of rupture. The recommendations made in this Consensus Statement will help clinicians to choose the most appropriate imaging modality on the basis of the specific clinical scenario, individual patient characteristics and the availability of each imaging modality.

Long-term impact of myocardial inflammation on quantitative myocardial perfusion-a descriptive PET/MR myocarditis study.

PURPOSE: Whether myocardial inflammation causes long-term sequelae potentially affecting myocardial blood flow (MBF) is unknown. We aimed to assess the effect of myocardial inflammation on quantitative MBF parameters, as assessed by 13N-ammonia positron emission tomography myocardial perfusion imaging (PET-MPI) late after myocarditis. METHODS: Fifty patients with a history of myocarditis underwent cardiac magnetic resonance (CMR) imaging at diagnosis and PET/MR imaging at follow-up at least 6 months later. Segmental MBF, myocardial flow reserve (MFR), and 13N-ammonia washout were obtained from PET, and segments with reduced 13N-ammonia retention, resembling scar, were recorded. Based on CMR, segments were classified as remote (n = 469), healed (inflammation at baseline but no late gadolinium enhancement [LGE] at follow-up, n = 118), and scarred (LGE at follow-up, n = 72). Additionally, apparently healed segments but with scar at PET were classified as PET discordant (n = 18). RESULTS: Compared to remote segments, healed segments showed higher stress MBF (2.71 mL*min-1*g-1 [IQR 2.18-3.08] vs. 2.20 mL*min-1*g-1 [1.75-2.68], p < 0.0001), MFR (3.78 [2.83-4.79] vs. 3.36 [2.60-4.03], p < 0.0001), and washout (rest 0.24/min [0.18-0.31] and stress 0.53/min [0.40-0.67] vs. 0.22/min [0.16-0.27] and 0.46/min [0.32-0.63], p = 0.010 and p = 0.021, respectively). While PET discordant segments did not differ from healed segments regarding MBF and MFR, washout was higher by ~ 30% (p < 0.014). Finally, 10 (20%) patients were diagnosed by PET-MPI as presenting with a myocardial scar but without a corresponding LGE. CONCLUSION: In patients with a history of myocarditis, quantitative measurements of myocardial perfusion as obtained from PET-MPI remain altered in areas initially affected by inflammation. CMR = cardiac magnetic resonance; PET = positron emission tomography; LGE = late gadolinium enhancement.

Long-Term Monitoring of Cardiac Involvement under Migalastat Treatment Using Magnetic Resonance Tomography in Fabry Disease.

Background: Fabry cardiomyopathy is characterized by left ventricular hypertrophy, myocardial fibrosis, arrhythmia, and premature death. Treatment with migalastat, an oral pharmacological chaperone, was associated with a stabilization of cardiac biomarkers and a reduction in left ventricular mass index, as measured by echocardiography. A recent study, using cardiac magnetic resonance (CMR) as the gold standard, found a stable course of myocardial involvement after 18 months of treatment with migalastat. Our study aimed to provide long-term CMR data for the treatment with migalastat. Methods: A total of 11 females and four males with pathogenic amenable GLA mutations were treated with migalastat and underwent 1.5T CMR imaging for routine treatment effect monitoring. The main outcome was a long-term myocardial structural change, reflected by CMR. Results: After migalastat treatment initiation, left ventricular mass index, end diastolic volume, interventricular septal thickness, posterior wall thickness, estimated glomerular filtration rate, and plasma lyso-Gb3 remained stable during the median follow-up time of 34 months (min.: 25; max.: 47). The T1 relaxation times, reflecting glycosphingolipid accumulation and subsequent processes up to fibrosis, fluctuated over the time without a clear trend. No new onset of late gadolinium enhancement (LGE) areas, reflecting local fibrosis or scar formation of the myocardium, could be detected. However, patients with initially present LGE showed an increase in LGE as a percentage of left ventricular mass. The median α-galactosidase A enzymatic activity increased from 37.3% (IQR 5.88-89.3) to 105% (IQR 37.2-177) of the lower limit of the respective reference level (p = 0.005). Conclusion: Our study confirms an overall stable course of LVMi in patients with FD, treated with migalastat. However, individual patients may experience disease progression, especially those who present with fibrosis of the myocardium already at the time of therapy initiation. Thus, a regular treatment re-evaluation including CMR is needed to provide the optimal management for each patient.

The Importance of Temporal Resolution for Ultra-High-Resolution Coronary Angiography: Evidence From Photon-Counting Detector CT.

PURPOSE: The aim of this study was to assess the effect of temporal resolution on subjective and objective image quality of coronary computed tomography angiography (CCTA) in the ultra-high-resolution (UHR) mode with dual-source photon-counting detector (PCD) CT. MATERIALS AND METHODS: This retrospective, institutional review board-approved study evaluated 30 patients (9 women; mean age, 80 ± 10 years) undergoing UHR CCTA with a clinical dual-source PCD-CT scanner. Images were acquired with a tube voltage of 120 kV and using a collimation of 120 × 0.2 mm. Gantry rotation time was 0.25 seconds. Each scan was reconstructed using both single-source and dual-source data resulting in an image temporal resolution of 125 milliseconds and 66 milliseconds, respectively. The average heart rate and the heart rate variability were recorded. Images were reconstructed with a slice thickness of 0.2 mm, quantum iterative reconstruction strength level 4, and using the Bv64 and Bv72 kernel for patients without and with coronary stents, respectively. For subjective image quality, 2 experienced readers rated motion artifacts and vessel delineation, or in-stent lumen visualization using 5-point discrete visual scales. For objective image quality, signal-to-noise ratio, contrast-to-noise ratio, stent blooming artifacts, and vessel and stent sharpness were quantified. RESULTS: Fifteen patients had coronary stents, and 15 patients had no coronary stents. The mean heart rate and heart rate variability during data acquisition were 72 ± 10 beats per minute and 5 ± 6 beats per minute, respectively. Subjective image quality in the right coronary artery, left anterior descending, and circumflex artery was significantly superior in 66 milliseconds reconstructions compared with 125 milliseconds reconstructions for both readers (all P 's < 0.01; interreader agreement, Krippendorff α = 0.84-1.00). Subjective image quality deteriorated significantly at higher heart rates for 125 milliseconds (ρ = 0.21, P < 0.05) but not for 66 milliseconds reconstructions (ρ = 0.11, P = 0.22). No association was found between heart rate variability and image quality for both 125 milliseconds (ρ = 0.09, P = 0.33) and 66 milliseconds reconstructions (ρ = 0.13, P = 0.17), respectively. Signal-to-noise ratio and contrast-to-noise ratio were similar between 66 milliseconds and 125 milliseconds reconstructions (both P 's > 0.05), respectively. Stent blooming artifacts were significantly lower on 66 milliseconds than on 125 milliseconds reconstructions (46.7% ± 10% vs 52.9% ± 8.9%, P < 0.001). Higher sharpness was found in 66 milliseconds than in 125 milliseconds reconstructions both in native coronary arteries (left anterior descending artery: 1031 ± 265 ∆HU/mm vs 819 ± 253 ∆HU/mm, P < 0.01; right coronary artery: 884 ± 352 ∆HU/mm vs 654 ± 377 ∆HU/mm, P < 0.001) and stents (5318 ± 3874 ∆HU/mm vs 4267 ± 3521 ∆HU/mm, P < 0.001). CONCLUSIONS: Coronary angiography with PCD-CT in the UHR mode profits considerably from a high temporal resolution, resulting in less motion artifacts, superior vessel delineation and in-stent lumen visualization, less stent blooming artifacts, and superior vessel and stent sharpness.

Clinical and CMR characteristics associated with cardiac events in patients with Fabry disease.

BACKGROUND: The assessment of late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) by cardiac magnetic resonance (CMR) as diagnostic and prognostic maker in Fabry disease is advancing. We aimed to investigate the impact of clinical characteristics and CMR findings on cardiac outcome in patients with FD. METHODS: In this study 55 patients with genetically confirmed FD and available CMR imaging were included. The primary endpoint was defined as a composite of cardiac events including cardiac death, new occurrence of atrial fibrillation, heart failure, ventricular tachycardia and bradycardia requiring device insertion. RESULTS: During a median follow-up of 4.9 years (IQR 3.7-5.9), 9 patients (16.3%) reached the primary cardiac end point. The global amount of LGE was associated with an increased risk for primary endpoint in the univariate analysis (HR 1.4 per 10% increase in LGE, p = 0.002). However maximal wall thickness (MWT) was the sole independent predictor of the primary endpoint in a stepwise logistic regression model (HR 9.8 per mm increase in MWT, p < 0.0001). Kaplan-Meier analysis revealed significant difference in event free survival rate between patients with and without LVH (Long-rank p = 0.006) and in patients with and without LGE (Long-rank p < 0.001). Patients without LVH and LGE were free of adverse cardiac events. CONCLUSION: LVH and LGE detected by CMR were associated with adverse cardiac events in FD. In particular maximal wall thickness can be useful in cardiac risk stratification of FD patients.