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Background: Cardiac involvement in idiopathic inflammatory myopathy (IIM or “myositis”) is associated with an approximate 4% mortality, but standardised screening strategies are lacking. Objective: We explored a multimodality screening on potentially reversible cardiac involvement –i.e. active (peri)myocarditis –in newly diagnosed IIM. Methods: We included adult IIM patients from 2017 to 2020. At time of diagnosis, patients underwent cardiac evaluation including laboratory biomarkers, electrocardiography, echocardiography, and cardiac magnetic resonance imaging (CMR). Based on 2019 consensus criteria for myocarditis, an adjudication committee made diagnoses of definite, probable, possible or no (peri)myocarditis. We explored diagnostic values of sequentially added diagnostic modalities by Constructing Classification and Regression Tree (CART) analysis in patients with definite/probable versus no (peri)myocarditis. Results: We included 34 IIM patients, in whom diagnoses of definite (six, 18%), probable (two, 6%), possible (11, 32%), or no (peri)myocarditis (15, 44%) were adjudicated. CART-analysis showed high-sensitivity cardiac troponin T (cut-off value 2.9 xULN for females and > 1.8 xULN for males) ruled in (peri)myocarditis with a specificity of 100%. Applying high-sensitivity cardiac troponins with these cut-off values in a diagnostic algorithm without and with a CMR to the total population of 34 patients demonstrated a diagnostic accuracy for a clear diagnosis of probable/definite or no (peri)myocarditis of 59% and 68%, respectively. Conclusions: A diagnostic algorithm for detection of (peri)myocarditis in adult IIM may consist of sequential testing with high-sensitivity cardiac troponins and CMR.

Background and ObjectivesGuidelines recommend echocardiography to screen for structural sources of cardioembolism in patients with ischemic stroke. Cardiac CT is a promising alternative as a first-line screening method. We aimed to determine whether cardiac CT, acquired during the initial stroke imaging protocol, has a higher yield for detecting high-risk cardioaortic sources of embolism than transthoracic echocardiography (TTE).MethodsWe performed a prospective, single-center, observational cohort study and included consecutive adult patients with acute ischemic stroke who underwent ECG-gated cardiac CT during the initial stroke imaging protocol. Patients also underwent the routine stroke workup, including TTE. The main outcome was the proportion of patients with a predefined high-risk cardioaortic source of embolism on cardiac CT vs TTE in patients undergoing both investigations.ResultsBetween May 2018 and November 2020, 774 patients with a suspected ischemic stroke underwent hyperacute cardiac CT. We excluded 228 patients with a diagnosis other than ischemic stroke and 94 because they did not provide informed consent. Therefore, 452 patients (59.3% male, median age 72) were included. The median additional scan time of cardiac CT was 6 (interquartile range 5–7) minutes with poor scan quality in only 3%. In total, 350 of the 452 patients (77.4%) underwent TTE, 99 of whom were performed in an outpatient setting. Reasons for not undergoing TTE were death (33, 7.3%) and TTE being too burdensome to perform in the outpatient setting (69, 15.3%). A high-risk cardioaortic source of embolism was detected in 40 of the 350 patients (11.4%) on CT, compared with 17 of the 350 (4.9%) on TTE (odds ratio 5.60, 95% CI 2.28–16.33). Cardiac thrombus was the most frequent finding (7.1% vs 0.6%). The diagnostic yield of cardiac CT in the full study population was 55 of the 452 (12.2%). Among the 175 patients with cryptogenic stroke after the routine workup, cardiac CT identified a cause of the stroke in 11 (6.3%).DiscussionCardiac CT acquired in the acute phase of ischemic stroke is technically feasible and has a superior diagnostic yield compared with TTE for the detection of high-risk sources of embolism. Cardiac CT may be considered as an alternative to TTE to screen for cardioembolism.

Background Pseudo-spiral Cartesian sampling with compressed sensing reconstruction has facilitated highly accelerated 4D flow magnetic resonance imaging (MRI) in various cardiovascular structures. However, unlike echo planar imaging (EPI)-accelerated 4D flow MRI, it has not been validated in whole-heart applications. Hypothesis Pseudo-spiral 4D flow MRI (PROUD [PROspective Undersampling in multiple Dimensions]) is comparable to EPI in robustness of valvular flow measurements and remains comparable as the undersampling factor is increased and scan time reduced. Study Type Prospective. Population Twelve healthy subjects and eight patients with valvular regurgitation. Field Strength/Sequence 3.0 T; PROUD and EPI 4D flow sequences, 2D flow and balanced steady-state free precession sequences. Assessment Valvular blood flow was quantified using valve tracking. PROUD- and EPI-based measurements of aortic (AV) and pulmonary (PV) flow volumes and left and right ventricular stroke volumes were tested for agreement with 2D MRI-based measurements. PROUD reconstructions with undersampling factors (R) of 9, 14, 28, and 56 were tested for intervalve consistency (per valve, compared to the other valves) and preservation of peak velocities and E/A ratios. Statistical Tests We used repeated measures ANOVA, Bland-Altman, Wilcoxon signed rank, and intraclass correlation coefficients. P < 0.05 was considered statistically significant. Results PROUD and EPI intervalve consistencies were not significantly different both in healthy subjects (valve-averaged mean difference [limits of agreement width]: 3.2 +/- 0.8 [8.7 +/- 1.1] mL/beat for PROUD, 5.5 +/- 2.9 [13.7 +/- 2.3] mL/beat for EPI, P = 0.07) and in patients with valvular regurgitation (2.3 +/- 1.2 [15.3 +/- 5.9] mL/beat for PROUD, 0.6 +/- 0.6 [19.3 +/- 2.9] mL/beat for EPI, P = 0.47). Agreement between EPI and PROUD was higher than between 4D flow (EPI or PROUD) and 2D MRI for forward flow, stroke volumes, and regurgitant volumes. Up to R = 28 in healthy subjects and R = 14 in patients with valvular regurgitation, PROUD intervalve consistency remained comparable to that of EPI. Peak velocities and E/A ratios were preserved up to R = 9. Conclusion PROUD is comparable to EPI in terms of intervalve consistency and may be used with higher undersampling factors to shorten scan times further. Level of Evidence 1 Technical Efficacy Stage 2

IntroductionThe cardiovascular benefits of physical exercise are well-known. However, vigorous exercise has also been associated with adverse cardiac effects. To improve our understanding of cardiovascular adaptation to exercise versus maladaptation and pathology, the limits of adaptation should be firmly established using state-of-the-art diagnostic modalities. We therefore initiated the Evaluation of Lifetime participation in Intensive Top-level sports and Exercise (ELITE) cohort to investigate the longitudinal (beneficial and pathological) cardiovascular effects of intensive elite sports and exercise.Methods and analysisELITE is a prospective, multicentre, longitudinal cohort study. Elite athletes, from the age of sixteen, are recruited in The Netherlands. The primary objective is to determine the association between elite sports and exercise-induced cardiac remodelling, cardiac pathology, and health benefits over time. Secondary objectives include determining and identifying genetic profiles of elite athletes, and how these are associated with cardiac indices. ELITE will collect data from consultations, electrocardiography, echocardiography and cardiac magnetic resonance imaging, and training- and injury data. ELITE will also collect blood for biobanking and cardiogenetics. Follow-up will take place at intervals of two to five years, and after the elite athletes’ professional careers have ended. In addition, a subcohort of ELITE has been established to investigate cardiac sequelae following infections associated with myocardial involvement, including SARS-CoV-2. ELITE is a prospective observational study; therefore, analyses will be primarily explorative.Ethics and disseminationThis study has been approved by the Medical Ethics Review Board of the Amsterdam University Medical Centers (NL71682.018.19). The results of the study will be disseminated by publication in peer-reviewed journals (Netherlands Trial Register number: NL9328).

Background: Truncating variants in desmoplakin ( DSP tv) are an important cause of arrhythmogenic cardiomyopathy; however the genetic architecture and genotype-specific risk factors are incompletely understood. We evaluated phenotype, risk factors for ventricular arrhythmias, and underlying genetics of DSP tv cardiomyopathy. Methods: Individuals with DSP tv and any cardiac phenotype, and their gene-positive family members were included from multiple international centers. Clinical data and family history information were collected. Event-free survival from ventricular arrhythmia was assessed. Variant location was compared between cases and controls, and literature review of reported DSP tv performed. Results: There were 98 probands and 72 family members (mean age at diagnosis 43±8 years, 59% women) with a DSP tv, of which 146 were considered clinically affected. Ventricular arrhythmia (sudden cardiac arrest, sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator therapy) occurred in 56 (33%) individuals. DSP tv location and proband status were independent risk factors for ventricular arrhythmia. Further, gene region was important with variants in cases (cohort n=98; Clinvar n=167) more likely to occur in the regions resulting in nonsense mediated decay of both major DSP isoforms, compared with n=124 genome aggregation database control variants (148 [83.6%] versus 29 [16.4%]; P Conclusions: In the largest series of individuals with DSP tv, we demonstrate that variant location is a novel risk factor for ventricular arrhythmia, can inform variant interpretation, and provide critical insights to allow for precision-based clinical management.

AimsData on the warranty period of coronary computed tomography angiography (CTA) and combined coronary CTA/positron emission tomography (PET) are scarce. The present study aimed to determine the event-free (warranty) period after coronary CTA and the potential additional value of PET.Method and resultsPatients with suspected but not previously diagnosed coronary artery disease (CAD) who underwent coronary CTA and/or [15O]H2O PET were categorized based upon coronary CTA as no CAD, non-obstructive CAD, or obstructive CAD. A hyperaemic myocardial blood flow (MBF) ≤ 2.3 mL/min/g was considered abnormal. The warranty period was defined as the time for which the cumulative event rate of death and non-fatal myocardial infarction (MI) was below 5%. Of 2575 included patients (mean age 61.4 ± 9.9 years, 41% male), 1319 (51.2%) underwent coronary CTA only and 1237 (48.0%) underwent combined coronary CTA/PET. During a median follow-up of 7.0 years 163 deaths and 68 MIs occurred. The warranty period for patients with no CAD on coronary CTA was ≥10 years, whereas patients with non-obstructive CAD had a 5-year warranty period. Patients with obstructive CAD and normal hyperaemic MBF had a 2-year longer warranty period compared to patients with obstructive CAD and abnormal MBF (3 years vs. 1 year).ConclusionAs standalone imaging, the warranty period for normal coronary CTA is ≥10 years, whereas patients with non-obstructive CAD have a warranty period of 5 years. Normal PET yielded a 2-year longer warranty period in patients with obstructive CAD.

Truncating variants in desmoplakin (Individuals withThere were 98 probands and 72 family members (mean age at diagnosis 43±8 years, 59% women) with aIn the largest series of individuals with

Objectives Computed tomography angiography (CTA) is performed routinely in the work-up for transcatheter aortic valve implantation (TAVI), and could potentially replace invasive coronary angiography (ICA) to rule out left main (LM) and proximal coronary stenosis. The objectives were to assess the diagnostic yield and accuracy of pre-TAVI CTA to detect LM and proximal coronary stenosis of ≥ 50% and ≥ 70% diameter stenosis (DS). Methods The DEPICT CTA database consists of individual patient data from four studies with a retrospective design that analyzed the diagnostic accuracy of pre-TAVI CTA to detect coronary stenosis, as compared with ICA. Pooled data were used to assess diagnostic accuracy to detect coronary stenosis in the left main and the three proximal coronary segments on a per-patient and a per-segment level. We included 1060 patients (mean age: 81.5 years, 42.7% male). Results On ICA, the prevalence of proximal stenosis was 29.0% (≥ 50% DS) and 15.7% (≥ 70% DS). Pre-TAVI CTA ruled out ≥ 50% DS in 51.6% of patients with a sensitivity of 96.4%, specificity of 71.2%, PPV of 57.7%, and NPV of 98.0%. For ≥ 70% DS, pre-TAVI CTA ruled out stenosis in 70.0% of patients with a sensitivity of 96.7%, specificity of 87.5%, PPV of 66.9%, and NPV of 99.0%. Conclusion CTA provides high diagnostic accuracy to rule out LM and proximal coronary stenosis in patients undergoing work-up for TAVI. Clinical application of CTA as a gatekeeper for ICA would reduce the need for ICA in 52% or 70% of patients, using a threshold of ≥ 50% or ≥ 70% DS, respectively. Key Points • Clinical application of CTA as a gatekeeper for ICA would reduce the need for ICA in 52% or 70% of TAVI patients, using a threshold of ≥ 50% or ≥ 70% diameter stenosis. • The diagnostic accuracy of CTA to exclude proximal coronary stenosis in these patients is high, with a sensitivity of 96.4% and NPV of 98.0% for a threshold of ≥ 50%, and a sensitivity of 96.7% and NPV of 99.0% for a threshold of ≥ 70% diameter stenosis. • Atrial fibrillation and heart rate did not significantly affect sensitivity and NPV. However, a heart rate of < 70 b/min during CTA was associated with a significantly improved specificity and PPV.

Multimodality imaging is recommended in patients in shock after seemingly uneventful pericardiocentesis. The aim of this study was to heighten awareness that LIMA injury can lead to a life‐threatening hemothorax in postoperative cardiac surgery patients.

Background: Respiratory gating is generally recommended in 4D flow MRI of the heart to avoid blurring and motion artifacts. Recently, a novel automated contact-less camera-based respiratory motion sensor has been introduced. Purpose: To compare camera-based respiratory gating (CAM) with liver-lung-navigator-based gating (NAV) and no gating (NO) for whole-heart 4D flow MRI. Study Type: Retrospective. Subjects: Thirty two patients with a spectrum of cardiovascular diseases. Field Strength/Sequence: A 3T, 3D-cine spoiled-gradient-echo-T1-weighted-sequence with flow-encoding in three spatial directions. Assessment: Respiratory phases were derived and compared against each other by cross-correlation. Three radiologists/cardiologist scored images reconstructed with camera-based, navigator-based, and no respiratory gating with a 4-point Likert scale (qualitative analysis). Quantitative image quality analysis, in form of signal-to-noise ratio (SNR) and liver-lung-edge (LLE) for sharpness and quantitative flow analysis of the valves were performed semi-automatically. Statistical Tests: One-way repeated measured analysis of variance (ANOVA) with Wilks's lambda testing and follow-up pairwise comparisons. Significance level of P ≤ 0.05. Krippendorff's-alpha-test for inter-rater reliability. Results: The respiratory signal analysis revealed that CAM and NAV phases were highly correlated (C = 0.93 ± 0.09, P < 0.01). Image scoring showed poor inter-rater reliability and no significant differences were observed (P ≥ 0.16). The image quality comparison showed that NAV and CAM were superior to NO with higher SNR (P = 0.02) and smaller LLE (P < 0.01). The quantitative flow analysis showed significant differences between the three respiratory-gated reconstructions in the tricuspid and pulmonary valves (P ≤ 0.05), but not in the mitral and aortic valves (P > 0.05). Pairwise comparisons showed that reconstructions without respiratory gating were different in flow measurements to either CAM or NAV or both, but no differences were found between CAM and NAV reconstructions. Data Conclusion: Camera-based respiratory gating performed as well as conventional liver-lung-navigator-based respiratory gating. Quantitative image quality analysis showed that both techniques were equivalent and superior to no-gating-reconstructions. Quantitative flow analysis revealed local flow differences (tricuspid/pulmonary valves) in images of no-gating-reconstructions, but no differences were found between images reconstructed with camera-based and navigator-based respiratory gating. Level of Evidence: 3. Technical Efficacy: Stage 2.

Rationale About one-third of ischaemic strokes are caused by cardioembolism, and a substantial proportion of cryptogenic strokes likely also originate from the heart or aortic arch. Early determination of aetiology is important to optimise management. Computed Tomography-angiography of the heart is emerging as an alternative to echocardiography to detect cardio-aortic sources of embolism in stroke patients, but its diagnostic yield in acute ischaemic stroke has not been thoroughly assessed. Hypothesis: We hypothesise that electrocardiography-gated computed tomography-angiography of the heart and aortic arch, acquired in the acute phase in patients with ischaemic stroke, has a higher diagnostic yield than transthoracic echocardiography as a first-line screening method for detection of cardio-aortic sources of embolism. Methods and design Mind the Heart is a single-centre prospective observational cohort study. We will include consecutive adult patients with acute ischaemic stroke who are potentially eligible for reperfusion therapy. Patients undergo non-electrocardiography-gated computed tomography-angiography of the aortic arch, cervical and intracranial arteries, directly followed by prospective sequential electrocardiography-gated cardiac computed tomography-angiography. Routine work-up for cardioembolism including 12-leads electrocardiography, Holter electrocardiography and transthoracic echocardiography is performed as soon as possible. The primary endpoint is the proportion of patients with a predefined high-risk cardio-aortic source of embolism on computed tomography-angiography versus transthoracic echocardiography in patients who underwent both investigations. Based on an expected 5% additional yield of computed tomography-angiography, a sample size of 450 patients is required. Conclusions The Mind the Heart study will generate a reliable estimate of the diagnostic yield of echocardiography-gated cardio-aortic computed tomography-angiography performed in the acute phase of ischaemic stroke.

Automatic cardiac chamber and left ventricular (LV) myocardium segmentation over the cardiac cycle significantly extends the utilization of contrast-enhanced cardiac CT, potentially enabling in-depth assessment of cardiac function. Therefore, we evaluate an automatic method for cardiac chamber and LV myocardium segmentation in 4D cardiac CT. In this study, 4D contrast-enhanced cardiac CT scans of 1509 patients selected for transcatheter aortic valve implantation with 21,605 3D images, were divided into development (N = 12) and test set (N = 1497). 3D convolutional neural networks were trained with end-systolic (ES) and end-diastolic (ED) images. Dice similarity coefficient (DSC) and average symmetric surface distance (ASSD) were computed for 3D segmentations at ES and ED in the development set via cross-validation, and for 2D segmentations in four cardiac phases for 81 test set patients. Segmentation quality in the full test set of 1497 patients was assessed visually on a three-point scale per structure based on estimated overlap with the ground truth. Automatic segmentation resulted in a mean DSC of 0.89 ± 0.10 and ASSD of 1.43 ± 1.45 mm in 12 patients in 3D, and a DSC of 0.89 ± 0.08 and ASSD of 1.86 ± 1.20 mm in 81 patients in 2D. The qualitative evaluation in the whole test set of 1497 patients showed that automatic segmentations were assigned grade 1 (clinically useful) in 98.5%, 92.2%, 83.1%, 96.3%, and 91.6% of cases for LV cavity and myocardium, right ventricle, left atrium, and right atrium. Our automatic method using convolutional neural networks performed clinically useful segmentation across the cardiac cycle in a large set of 4D cardiac CT images, potentially enabling in-depth assessment of cardiac function.

BACKGROUND: Baffle complications, ie, leakage or stenosis, after an atrial switch operation (AtrSO) for transposition of the great arteries (TGA) are difficult to detect with the use of routine transthoracic echocardiography (TTE). We examined baffle interventions and the prevalence of baffle complications.METHODS: This dual-centre study followed TGA-AtrSO patients for the occurrence of baffle interventions. In addition, in 2017-2019, prevalence of baffle complications was determined in patients undergoing routine contrast-enhanced (CE) TTE including various hemodynamic conditions and computed tomography (CT). Baffle leaks were defined as right-to-left shunting on CE-TTE and baffle stenosis as a systemic venous baffle diameter of < 10 mm on CT.RESULTS: In total, 67 TGA-AtrSO patients were followed to a median age of 38 (interquartile range 34-42) years, for a median of 9 (6-13) years. Baffle interventions were documented in 24 patients (36%). Cumulative risk of baffle interventions was 25% after 15 years of follow-up. Prevalence of baffle complications was determined in 29/67 patients. In total, 4 (14%) had patent baffles, 11 (38%) had leakage only, 5 (17%) had stenosis only, and 9 (31%) had both, while 24/29 (84%) were asymptomatic. Although baffle leaks were not associated with clinical characteristics, peak work rate during exercise TTE was lower in patients with vs without stenosis (89 ± 24 W vs 123 ± 21 W; P < 0.001).CONCLUSIONS: Baffle complications are common in TGA-AtrSO. The cumulative risk of baffle interventions was 25% after 15 years of follow-up. CE-TTE uncovered asymptomatic baffle leakage in the majority of patients, especially with examination during exercise. CT revealed baffle stenosis in almost half of the patients, which was associated with decreased exercise tolerance. Awareness of these findings may alter clinical follow-up.

Background: Cardiac thrombi are a major risk factor for ischemic stroke, but are rarely diagnosed in the acute phase. We examined characteristics and functional outcome of patients with ischemic stroke and a concomitant cardiac thrombus detected on cardiac CT performed in the acute phase. Patients and Methods: We used data from “Mind the Heart,” a prospective cohort study in which consecutive adult patients with acute ischemic stroke underwent prospective ECG-gated cardiac CT during their acute stroke imaging protocol. We compared characteristics, functional outcome (modified Rankin scale) and stroke recurrence rate at 90 days of patients with a cardiac thrombus on CT (defined as filling defect Results: Among 452 included patients, cardiac CT detected 41 thrombi in 38 (8%) patients. Thrombi were most often located in the left atrial appendage (31/38 [82%]). Patients with a cardiac thrombus more frequently had intracranial occlusions in multiple vascular territories (5% vs 0.5%, p = 0.04) and a higher baseline NIHSS score (17 [IQR 6–22] vs 5 [IQR 2–3], p Discussion and Conclusion: Cardiac CT detected a cardiac thrombus in one in every 12 patients with acute ischemic stroke, and these patients had more severe deficits, multivessel occlusions, and a worse functional outcome.

Background Helices and vortices in thoracic aortic blood flow measured with 4D flow cardiovascular magnetic resonance (CMR) have been associated with aortic dilation and aneurysms. Current approaches are semi-quantitative or when fully quantitative based on 2D plane placement. In this study, we present a fully quantitative and three-dimensional approach to map and quantify abnormal velocity and wall shear stress (WSS) at peak systole in patients with a bicuspid aortic valve (BAV) of which 52% had a repaired coarctation. Methods 4D flow CMR was performed in 48 patients with BAV and in 25 healthy subjects at a spatiotemporal resolution of 2.5 × 2.5 × 2.5mm3/ ~ 42 ms and TE/TR/FA of 2.1 ms/3.4 ms/8° with k-t Principal Component Analysis factor R = 8. A 3D average of velocity and WSS direction was created for the normal subjects. Comparing BAV patient data with the 3D average map and selecting voxels deviating between 60° and 120° and > 120° yielded 3D maps and volume (in cm3) and surface (in cm2) quantification of abnormally directed velocity and WSS, respectively. Linear regression with Bonferroni corrected significance of P Results The velocity volumes > 120° correlated moderately with the vorticity scores (R ~ 0.50, P 120° did not correlate with quantitative vorticity. For abnormal velocity and WSS deviating between 60° and 120°, moderate correlations were found with aortic diameters (R = 0.50–0.70). For abnormal velocity and WSS deviating > 120°, additional moderate correlations were found with age and with peak velocity (stenosis severity) and a weak correlation with gender. Ensemble maps showed that more than 60% of the patients had abnormally directed velocity and WSS. Additionally, abnormally directed velocity and WSS was higher in the proximal descending aorta in the patients with repaired coarctation than in the patients where coarctation was never present. Conclusion The possibility to reveal directional abnormalities of velocity and WSS in 3D provides a new tool for hemodynamic characterization in BAV disease.

ObjectivesQuantification of pericardial/myocardial involvement and risks of sudden cardiac arrest/sudden cardiac death (SCA/SCD) after SARS-CoV-2 infection in athletes who return to sports.DesignSystematic review on post-SARS-CoV-2 infection pericardial/myocardial manifestations in athletes.Data sourcesCombinations of key terms in Medline, Embase and Scopus (through 2 June 2021).Eligibility criteria for selecting studiesInclusion: athletes, with cardiovascular magnetic resonance (CMR) or echocardiography after recovery from SARS-CoV-2 infection, including arrhythmia outcomes. Exclusion: study population ≥1 individual comorbidity and mean age 64 years. Quality assessment was performed using Joanna Briggs Institute Critical Appraisal tools checklists.ResultsIn total, 12 manuscripts (1650 papers reviewed) comprising 3131 athletes (2198 college/student athletes, 879 professional athletes and 54 elite athletes) were included. The prevalence of myocarditis on echocardiography and/or CMR was 0%–15%, pericardial effusion 0%–58% and late gadolinium enhancement (LGE) 0%–46%. Weighted means of diagnosed myocarditis were 2.1% in college/student athletes and 0% in elite athletes. The prevalence of LGE was markedly lower in studies with high-quality assessment scores (3%–4%) versus low scores (38%–42%). A single study reported reversibility of myocardial involvement in 40.7%. No important arrhythmias were reported. Ten studies (n=4171) reporting postrecovery troponin T/I found no clear relationship with cardiac abnormalities.Summary/conclusionAthletes have an overall low risk of SARS-CoV-2 pericardial/myocardial involvement, arrhythmias and SCA/SCD. Rates of pericardial/myocardial abnormalities in athletes are highly variable and dependent on study quality. Troponin screenings seem unreliable to identify athletes at risk for myocardial involvement. Prospective athlete studies, with pre-SARS-CoV-2 imaging (CMR), including structured follow-up and arrhythmia monitoring, are urgently needed.

BackgroundTruncating variants in desmoplakin (DSPtv) are an important cause of arrhythmogenic cardiomyopathy (ACM), however the genetic architecture and genotype-specific risk factors are incompletely understood. We evaluated phenotype, risk factors for ventricular arrhythmias, and underlying genetics ofDSPtv cardiomyopathy.MethodsIndividuals withDSPtv and any cardiac phenotype, and their gene-positive family members were included from multiple international centers. Clinical data and family history information were collected. Event-free survival from ventricular arrhythmia was assessed. Variant location was compared between cases and controls, and literature review of reportedDSPtv performed.ResultsThere were 98 probands and 72 family members (mean age at diagnosis 43 ± 18 years, 59% female) with aDSPtv, of which 146 were considered clinically affected. Ventricular arrhythmia (sudden cardiac arrest, sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator therapy) occurred in 56 (33%) individuals.DSPtv location and proband status were independent risk factors for ventricular arrhythmia, while prior risk factors showed no association. Further, gene region was important with variants in cases (cohort n=98, Clinvar n=168) more likely to occur in the regions resulting in nonsense mediated decay of both majorDSPisoforms, compared to n=124 gnomAD control variants (148 [83.6%] versus 29 [16.4%], pConclusionsIn the largest series of individuals withDSPtv, we demonstrate variant location is a novel risk factor for ventricular arrhythmia, can inform variant interpretation, and provide critical insights to allow precision-based clinical management.

Introduction Data on the incidence of acute aortic dissection in the code stroke population are scarce. We report estimated incidence, clinical manifestations, treatment and outcomes of patients with an acute aortic dissection in a code stroke cohort from a comprehensive stroke centre. Patients and methods We used data from a single-centre prospective registry of consecutive adult patients who presented to the emergency department between 2015 and 2018 with neurological deficits suggestive of an acute stroke (‘code stroke’). All patients routinely underwent non-contrast computed tomography of the brain and computed tomography-angiography of the aortic arch, cervical and intracranial arteries. Results Of 2874 code stroke patients, 1563 (54.4%) had acute ischaemia (ischaemic stroke or transient ischaemic attack). Fifteen patients (0.5% of code stroke patients and 0.8% of patients with acute ischaemia) had an acute aortic dissection (all Stanford classification type A). Discerning clinical manifestations were decreased consciousness in 11/15 (73%), pain in 8/15 (53%) and low systolic blood pressure (mean 106 mmHg, SD30). Acute aortic dissection was an incidental finding during computed tomography-angiography in 4/15 (27%). Two out of 15 patients (13%) received intravenous thrombolysis, 9/15 (60%) underwent aortic surgery and 10/15 (67%) died. Of those who survived, 3/5 (60%) had a good functional outcome (modified Rankin Scale 0–2). Discussion and Conclusion In our comprehensive stroke centre, about 1/200 code stroke patients and 1/125 patients with acute ischaemia had an acute aortic dissection. Multicentre studies are necessary to acquire a more reliable estimate of the incidence of acute aortic dissection in the code stroke population. Given the ramifications of missing this diagnosis, imaging of the entire aortic arch is important in these patients.

Purpose: To evaluate whether the biomechanical marker known as rupture risk equivalent diameter (RRED) was superior to the actual abdominal aortic aneurysm (AAA) diameter in estimating future rupture risk in patients who had undergone pre-rupture computed tomography (CT) angiography.Materials and Methods: A retrospective study was conducted in 13 patients with ruptured AAAs who had undergone CT angiography before and after rupture between 2001 and 2015. The median time between the 2 scans was 731 days. Biomechanical and geometrical markers such as maximal AAA diameter, peak wall stress (PWS), and RRED were calculated with AAA-dedicated software. The main analyses determined whether RRED was higher than the actual diameter and the threshold diameter for elective surgery (55 mm for men, 50 mm for women) in AAAs before and after rupture. Differences between diameter and biomechanical markers before and after rupture were tested with appropriate statistical tests.Results: RRED before and after rupture was smaller than the actual diameter in 7 of 13 cases. Post-rupture RRED was estimated to be smaller than the threshold diameter for elective repair in 4 cases, again suggesting a low rupture risk. The median PWS before and after rupture was 181.7 kPa (interquartile range [IQR], 152.1-244.2 kPa) and 274.1 kPa (IQR, 172.2-377.2 kPa), respectively.Conclusions: RRED was smaller than the actual diameter in more than half of pre-rupture AAAs, suggesting a lower rupture risk than estimated with the actual diameter. The results suggest that the currently available biomechanical imaging markers might not be ready for use in clinical practice.

OBJECTIVES This study determined: 1) the interobserver agreement; 2) valvular flow variation; and 3) which variables independently predicted the variation of valvular flow quantification from 4-dimensional (4D) flow cardiac magnetic resonance (CMR) with automated retrospective valve tracking at multiple sites. BACKGROUND Automated retrospective valve tracking in 4D flow CMR allows consistent assessment of valvular flow through all intracardiac valves. However, due to the variance of CMR scanners and protocols, it remains uncertain if the published consistency holds for other clinical centers. METHODS Seven sites each retrospectively or prospectively selected 20 subjects who underwent whole heart 4D flow CMR (64 patients and 76 healthy volunteers; aged 32 years [range 24 to 48 years], 47% men, from 2014 to 2020), which was acquired with locally used CMR scanners (scanners from 3 vendors; 2 1.5-T and 5 3-T scanners) and protocols. Automated retrospective valve tracking was locally performed at each site to quantify the valvular flow and repeated by 1 central site. Interobserver agreement was evaluated with intraclass correlation coefficients (ICCs). Net forward volume (NFV) consistency among the valves was evaluated by calculating the intervalvular variation. Multiple regression analysis was performed to assess the predicting effect of local CMR scanners and protocols on the intervalvular inconsistency. RESULTS The interobserver analysis demonstrated strong-to-excellent agreement for NFV (ICC: 0.85 to 0.96) and moderate-to-excellent agreement for regurgitation fraction (ICC: 0.53 to 0.97) for all sites and valves. In addition, all observers established a low intervalvular variation (#10.5%) in their analysis. The availability of 2 cine images per valve for valve tracking compared with 1 cine image predicted a decreasing variation in NFV among the 4 valves (beta =-1.3; p = 0.01). CONCLUSIONS Independently of locally used CMR scanners and protocols, valvular flow quantification can be performed consistently with automated retrospective valve tracking in 4D flow CMR. (J Am Coll Cardiol Img 2021;14:1354-66) (c) 2021 by the American College of Cardiology Foundation.

To assess the need for additional invasive coronary angiography (CAG) after initial computed tomography coronary angiography (CTCA) in patients awaiting non-coronary cardiac surgery and in patients with cardiomyopathy, heart failure or ventricular arrhythmias, and to determine differences between patients that were referred to initial CTCA or direct CAG, consecutive patients were included between August 2017 and January 2020 and categorized as those referred to initial CTCA (conform protocol), and to direct CAG (non-conform protocol). Out of a total of 415 patients, 78.8% (327 patients, mean age: 57.9 years, 67.3% male) were referred to initial CTCA, of whom 260 patients (79.5%) had no obstructive lesions (&lt, 50% DS). A total of 55 patients (16.8%) underwent additional CAG after initial CTCA, which showed coronary lesions of &gt, 50% DS in 21 patients (6.3% of 327). Eighty-eight patients (mean age: 66.0 years, 59.1% male) were directly referred to CAG (non-conform protocol). These patients were older and had more cardiovascular risk factors compared to patients that underwent initial CTCA (conform protocol), and coronary lesions of &gt, 50% DS were detected in 16 patients (17.2%). Revascularization procedures were infrequently performed in both groups: initial CTCA (3.0%), direct CAG (3.4%). The use of CTCA as a gatekeeper CAG in the diagnostic work-up of non-coronary cardiac surgery, cardiomyopathy, heart failure and ventricular arrhythmias is feasible, and only 17% of these patients required additional CAG after initial CTCA. Therefore, CTCA should be considered as the initial imaging modality to rule out CAD in these patients.

Contains fulltext : 239064.pdf (Publisher’s version ) (Open Access) BACKGROUND: Proton magnetic resonance spectroscopy ((1) H-MRS) of the human heart is deemed to be a quantitative method to investigate myocardial metabolite content, but thorough validations of in vivo measurements against invasive techniques are lacking. PURPOSE: To determine measurement precision and accuracy for quantifications of myocardial total creatine and triglyceride content with localized (1) H-MRS. STUDY TYPE: Test-retest repeatability and measurement validation study. SUBJECTS: Sixteen volunteers and 22 patients scheduled for open-heart aortic valve replacement or septal myectomy. FIELD STRENGTH/SEQUENCE: Prospectively ECG-triggered respiratory-gated free-breathing single-voxel point-resolved spectroscopy (PRESS) sequence at 3 T. ASSESSMENT: Myocardial total creatine and triglyceride content were quantified relative to the total water content by fitting the (1) H-MR spectra. Precision was assessed with measurement repeatability. Accuracy was assessed by validating in vivo (1) H-MRS measurements against biochemical assays in myocardial tissue from the same subjects. STATISTICAL TESTS: Intrasession and intersession repeatability was assessed using Bland-Altman analyses. Agreement between (1) H-MRS measurements and biochemical assay was tested with regression analyses. RESULTS: The intersession repeatability coefficient for myocardial total creatine content was 41.8% with a mean value of 0.083% ± 0.020% of the total water signal, and 36.7% for myocardial triglyceride content with a mean value of 0.35% ± 0.13% of the total water signal. Ex vivo myocardial total creatine concentrations in tissue samples correlated with the in vivo myocardial total creatine content measured with (1) H-MRS: n = 22, r = 0.44; P

Background and aims Both plasma low-density lipoprotein (LDL) cholesterol levels and risk for premature cardiovascular disease are extremely elevated in patients with homozygous familial hypercholesterolemia (HoFH), despite the use of multiple cholesterol lowering treatments. Given its inborn nature, atherosclerotic plaques are commonly observed in young HoFH patients. Whether intensive lipid lowering strategies result in plaque regression in adolescent patients is unknown. Methods Two HoFH patients with null/null LDLR variants, who participated in the R1500-CL-1629 randomized clinical trial (NCT03399786) evaluating the LDL cholesterol lowering effect of evinacumab (a human antibody directed against ANGPTL3; 15 mg/kg intravenously once monthly), were included in this study. Patients underwent coronary computed tomography angiography (CCTA) before randomization and after 6 months of treatment. Results Both patient A (aged 12) and B (aged 16) were treated with a statin, ezetimibe and weekly apheresis. Evinacumab decreased mean pre-apheresis LDL cholesterol levels from 5.51 ± 0.75 and 5.07 ± 1.45 mmol/l to 2.48 ± 0.31 and 2.20 ± 0.13 mmol/l and post-apheresis LDL levels from 1.45 ± 0.26 and 1.37 ± 39 mmol/l to 0.80 ± 0.16 and 0.78 ± 0.13 mmol/l in patient A and B, respectively. Total plaque volumes were reduced by 76% and 85% after 6 months of evinacumab treatment in patient A and B, respectively. Conclusions We describe two severely affected young HoFH patients in whom profound plaque reduction was observed with CCTA after intensive lipid lowering therapy with statins, ezetimibe, LDL apheresis, and evinacumab. This shows that atherosclerotic plaques possess the ability to regress at young age, even in HoFH patients.

Additional file 1: Table S1. Quantification of LNH and vorticity with differences between regions and subjects, and correlations with qualitative scorings and abnormally directed velocity.

Purpose: The first objective of this study was to evaluate the efficacy of a patient-tailored contrast delivery protocol for coronary computed tomography angiography (CTCA), in terms of diagnostic coronary attenuation and total iodine load (TIL), by adjusting the iodine delivery rate (IDR) via dilution for body weight and tube voltage (kV), as compared with a protocol with a fixed bolus of contrast in a clinical setting. The secondary objective was to assess the association between the test-bolus data and luminal attenuation in CTCA. Materials and Methods: Patients who underwent CTCA with fixed IDR contrast delivery (cohort 1) or with IDR adjusted for body weight and kV settings (70 to 120 kV) (cohort 2) were included, and compared for intravascular luminal attenuation and TIL. The association between intravascular luminal attenuation and test-bolus scan data was investigated with linear regression. Results: In cohort 1 (176 patients), the mean luminal attenuation differed markedly between kV categories, whereas in cohort 2 (154 patients), there were no marked differences. The mean TIL reduced significantly (20.1±1.2 g in cohort 1, 17.7±3.0 g in cohort 2, P

Progressive ascending aortic dilatation has been observed after mechanical aortic valve replacement (mAVR), possibly due to altered blood flow and wall shear stress (WSS) patterns induced by their bileaflet design. We examined the effect of mAVR on WSS in the ascending aorta using time-resolved 4D flow MRI. Fifteen patients with mechanical aortic valve prostheses, 10 patients with bicuspid aortic valve disease and 10 healthy individuals underwent thoracic 4D flow MRI. Peak systolic hemodynamic parameters (velocity and WSS) and vessel diameters were assessed in the ascending aorta. In addition, three-dimensional per-voxel analysis was used to compare velocity and WSS between patient groups and healthy controls. Peak aortic diameters were significantly higher in mAVR and BAV patients compared to healthy controls (p = 0.011). Mean aortic diameters were comparable between mAVR and BAV patients. No differences in 4D flow MRI-derived mean blood flow velocity and peak WSS were found between the three groups. Compared to healthy controls, mean WSS was significantly lower in mAVR patients (p = 0.031). Per-voxel analysis revealed no increased WSS in the ascending aortic wall and significantly lower velocity and WSS values in mAVR patients compared to healthy controls. In contrast, regions of significantly increased outer lumen velocities and WSS in BAV patients compared to healthy controls were found. This study shows that there is no increased ascending aortic WSS after mAVR. Our results suggest that, in contrast to BAV patients, there is no indication for intensified follow-up of the ascending aorta after mAVR.

Aims: The p.Arg14del founder mutation in the gene encoding phospholamban (PLN) is associated with an increased risk of malignant ventricular arrhythmia (VA) and heart failure. It has been shown to lead to calcium overload, cardiomyocyte damage, and eventually to myocardial fibrosis. This study sought to investigate ventricular function, the extent and localization of myocardial fibrosis and the associations with ECG features and VA in PLN p.Arg14del mutation carriers. Methods and results: Cardiovascular magnetic resonance (CMR) data of 150 mutation carriers were analysed retrospectively. Left ventricular (LV) and right ventricular (RV) volumes, mass, and ejection fraction were measured. The extent of late gadolinium enhancement (LGE) was expressed as a percentage of myocardial mass. All standard ECG parameters were measured. Occurrence of VA was analysed on ambulatory 24-h and/or exercise electrocardiography, if available. Mean age was 40 ± 15 years, 42% males, and 7% were index patients while 93% were pre-symptomatic carriers identified after family cascade screening. Mean LV ejection fraction (LVEF) and RV ejection fraction were 58 ± 9% and 55 ± 9%, respectively. LV-LGE was present in 91% of mutation carriers with reduced LVEF (

Background: High-risk patients with mitral regurgitation (MR) may be treated by a percutaneous mitral valve repair with the MitraClip, but identification of patients who may benefit remains difficult. We aimed to determine whether myocardial fibrosis predicts outcome in MR patients undergoing MitraClip implantation and is beneficial in clinical decision making. Methods: Preprocedural to the MitraClip implantation, myocardial fibrosis was analyzed with cardiovascular magnetic resonance (CMR) through late gadolinium enhancement. The CMR data were core-lab adjudicated measured before the MitraClip implantation. Adverse outcome was defined as New York Heart Association (NYHA) class III or IV after 1 month or death within 1 month after the MitraClip implantation. Results: In total, 23 patients underwent preprocedural CMR, mean age 80 ± 9 years, 45% male, 64% atrial fibrillation and 73% NYHA class III or IV at baseline. Myocardial fibrosis was present in 55% of the patients with degenerative MR and in 64% of the patients with functional MR. An adverse outcome occurred in 69% of the patients with myocardial fibrosis and in 11% of the patients without myocardial fibrosis (P = 0.01). Conclusions: Our hypothesis-generating study showed that the presence of myocardial fibrosis predicts adverse outcome in patients undergoing MitraClip implantation. After confirmation with larger sample size, identification of myocardial fibrosis might contribute to assess prognosis and to clinical decision making.

Vascular complications (VCs) after transfemoral transcatheter aortic valve implantation (TAVI) have always been reported to occur frequently. Studies addressing VCs have been conducted with older-generation prostheses. We aimed to evaluate the incidence, predictors, and impact of VCs after transfemoral TAVI with the balloon-expandable SAPIEN 3. We report a single-center retrospective analysis of 400 consecutive patients of a prospectively acquired cohort. All patients underwent transfemoral TAVI with SAPIEN 3 between January 2014 and December 2016. VC was defined according to the Valve Academic Research Consortium. In this cohort 83 patients had VCs (20.8%), 5.8% major and 15.0% minor. Sheath-to-iliofemoral artery ratio was the only predictor of major VCs (odds ratio 7.51, 95% confidence interval 1.61 to 34.95, p = 0.010). The area under the receiver-operator characteristic curve for sheath-to-iliofemoral artery ratio was 0.63 (poor accuracy). Thirty-day mortality rates were 17.4%, 1.7%, and 0.6% for major, minor, and no VCs, respectively (log-rank p ≤0.001). After adjustment, only major VCs were associated with 30-day mortality (adjusted hazard ratio 48.31, 95% confidence interval 7.80 to 299.24). Mortality from 30 days until 1 year did not differ between patients with and without VCs (log-rank p = 0.61). In conclusion we report that VCs remain an issue of transfemoral TAVI with the SAPIEN 3, and their prediction continues to be difficult, albeit the low-incidence, major VCs were associated with higher 30-day mortality. However, after these first 30 days, they were not of influence on survival anymore.

BACKGROUND Use of 4-dimensional flow magnetic resonance imaging (4D-flow MRI) derived wall shear stress (WSS) heat maps enables identification of regions in the ascending aorta with increased WSS. These regions are subject to dysregulation of the extracellular matrix and elastic fiber degeneration, which is associated with aortic dilatation and dissection. PURPOSE To evaluate the effect of the presence of aortic valve stenosis and the aortic diameter on the peak WSS and surface area of increased WSS in the ascending aorta. STUDY TYPE Prospective. SUBJECTS In all, 48 bicuspid aortic valve (BAV) patients (38.1 ± 12.4 years) and 25 age- and gender-matched healthy individuals. FIELD STRENGTH/SEQUENCE Time-resolved 3D phase contrast MRI with three-directional velocity encoding at 3.0T. ASSESSMENT Peak systolic velocity, WSS, and aortic diameters were assessed in the ascending aorta and 3D heat maps were used to identify regions with elevated WSS. STATISTICAL TESTS Comparisons between groups were performed by t-tests. Correlations were investigated by univariate and multivariate regression analysis. RESULTS Elevated WSS was present in 15 ± 11% (range; 1-35%) of the surface area of the ascending aorta of BAV patients with aortic valve stenosis (AS) (n = 10) and in 6 ± 8% (range; 0-31%) of the ascending aorta of BAV patients without AS (P = 0.005). The mid-ascending aortic diameter negatively correlated with the peak ascending aortic WSS (R = -0.413, P = 0.004) and the surface area of elevated WSS (R = -0.419, P = 0.003). Multivariate linear regression analysis yielded that the height of peak WSS and the amount of elevated WSS depended individually on the presence of aortic valve stenosis and the diameter of the ascending aorta. DATA CONCLUSION The extent of increased WSS in the ascending aorta of BAV patients depends on the presence of aortic valve stenosis and aortic dilatation and is most pronounced in the presence of AS and a nondilated ascending aorta. LEVEL OF EVIDENCE 2 Technical Efficacy: Stage 2 J. MAGN. RESON. IMAGING 2018;48:522-530.

Objectives Sports related aneurysmal degeneration and thrombosis of the Posterior Circumflex Humeral Artery (PCHA) has been known to cause symptoms of digital ischemia (DI) in elite volleyball players. Studies have reported symptoms of DI in as much as 28% of the elite indoor volleyball players. The purpose of this study was to determine the diagnostic value of the S houlder P CHA Pathology and digital I schemia – Q uestionnaire (SPI-Q) for detection of sports related PCHA disease using ultrasound data as the standard of reference. Methods The SPI-Q was completed by elite indoor volleyball players from the highest and single highest Dutch volleyball division and by elite beach volleyball players participating in the 2014 Grand Slam Beach Tournament The Hague (GSBTH). Ultrasound assessment of the dominant shoulder was performed on-site using the SPI-US protocol. The SPI-Q sensitivity, specificity, positive – and negative predictive value and positive – and negative likelihood ratios, and the diagnostic odds ratio were calculated for detection of sports related PCHA disease, using ultrasound as the standard of reference. Results Two hundred twenty-four elite male indoor volleyball players from the Dutch division were included in this study and 62 elite male and female beach volleyball players participating in the GSBTH: a total of 278 players. Thirty-five percent of the players reported symptoms of DI. The prevalence of PCHA disease was 6.1%. For the SPI-Q we found a sensitivity of 18% (95% CI 4–43), specificity of 64% (95% CI 58–70), positive predictive value of 3% (95% CI 0.7–8.9) and negative predictive value of 92% (95% CI 87–96), positive likelihood ratio of 0.50 (95% CI 0.18–1.40), negative likelihood ratio of 1.28 (95% CI 1.01–1.62) and a diagnostic odds ratio of 0.39 (95% CI 0.11–1.38). Conclusion The diagnostic value of the SPI-Q to detect PCHA disease in elite volleyball players is poor, which makes it unsuitable as a diagnostic instrument for sports related PCHA disease specifically. However, it can be used to assess all-cause symptoms of DI and raise awareness within athletes and sports physicians, which is important for preventing ischemic complications.