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Post-myocardial infarction ventricular tachycardia (VT) is due to re-entry through surviving conductive myocardial corridors across infarcted tissue. However, not all conductive corridors participate in re-entry.This study sought to test the hypothesis that critical VT corridors are more likely to traverse near lipomatous metaplasia (LM) and that current loss is reduced during impulse propagation through such corridors.Among 30 patients in the Prospective 2-center INFINITY (Intra-Myocardial Fat Deposition and Ventricular Tachycardia in Cardiomyopathy) study, potential VT-viable corridors within myocardial scar or LM were computed from late gadolinium enhancement cardiac magnetic resonance images. Because late gadolinium enhancement highlights both scar and LM, LM was distinguished from scar by using computed tomography. The SD of the current along each corridor was measured.Scar exhibited lower impedance than LM (median Z-score -0.22 [IQR: -0.84 to 0.35] vs -0.07 [IQR: -0.67 to 0.54]; P lt; 0.001). Among all 381 corridors, 84 were proven to participate in VT re-entry circuits, 83 (99%) of which traversed or were adjacent to LM. In comparison, only 13 (4%) non-VT corridors were adjacent to LM. Critical corridors adjacent to LM displayed lower SD of current compared with noncritical corridors through scar but distant from LM (2.0 [IQR: 1.0 to 3.4] μA vs 8.4 [IQR: 5.5 to 12.8] μA; P lt; 0.001).Corridors critical to VT circuitry traverse infarcted tissue through or near LM. This association is likely mediated by increased regional resistance and reduced current loss as impulses traverse corridors adjacent to LM.

Each year, the North American Society of Cardiovascular Imaging (NASCI) selects the Young Investigator Awards, which are amongst the top abstracts presented at the NASCI annual meeting. All residents, postdoctoral students, medical students and fellows are eligible for these awards.The International Journal of Cardiovascular Imaging proudly publishes the following abstracts from the fnalists that were presented in full at the Annual Meeting in Cleveland, Ohio on September 10-13, 2022.The 2023 Meeting will be held on September 9-12 in Scottsdale, Arizona. Learn more at https://www.nasci.org/future-meetings .For information about NASCI, and to register to attend the annual meeting, go to www.nasci.org .

Right ventricular function has prognostic significance in patients with pulmonary hypertension. We evaluated whether cardiac magnetic resonance-derived strain and strain rate parameters could reliably reflect right ventricular systolic and diastolic function in precapillary pulmonary hypertension. End-systolic elastance and the time constant of right ventricular relaxation tau, both derived from invasive high-fidelity micromanometer catheter measurements, were used as gold standards for assessing systolic and diastolic right ventricular function, respectively. Nineteen consecutive precapillary pulmonary hypertension patients underwent cardiac magnetic resonance and right heart catheterization prospectively. Cardiac magnetic resonance data were compared with those of 19 control subjects. In pulmonary hypertension patients, associations between strain- and strain rate-related parameters and invasive hemodynamic parameters were evaluated. Longitudinal peak systolic strain, strain rate, and early diastolic strain rate were lower in PAH patients than in controls; peak atrial-diastolic strain rate was higher in pulmonary hypertension patients. Similarly, circumferential peak systolic strain rate was lower and peak atrial-diastolic strain rate was higher in pulmonary hypertension. In pulmonary hypertension, no correlations existed between cardiac magnetic resonance-derived and hemodynamically derived measures of systolic right ventricular function. Regarding diastolic parameters, tau was significantly correlated with peak longitudinal atrial-diastolic strain rate ( r = −0.61), deceleration time ( r = 0.75), longitudinal systolic to diastolic time ratio ( r = 0.59), early diastolic strain rate ( r = −0.5), circumferential peak atrial-diastolic strain rate ( r = −0.52), and deceleration time ( r = 0.62). Strain analysis of the right ventricular diastolic phase is a reliable non-invasive method for detecting right ventricular diastolic dysfunction in PAH.

Background: The arrhythmogenic right ventricular cardiomyopathy (ARVC) risk calculator stratifies risk for incident sustained ventricular arrhythmias (VA) at the time of ARVC diagnosis. However, included risk factors change over time, and how well the ARVC risk calculator performs at follow-up is unknown. Methods: This was a retrospective analysis of patients with definite ARVC and without prior sustained VA. Risk factors for VA including age, nonsustained ventricular tachycardia, premature ventricular complex burden, T-wave inversions on electrocardiogram, cardiac syncope, right ventricular function, therapeutic medication use, and exercise intensity were assessed at the time of 2010 Task Force Criteria based ARVC diagnosis and upon repeat evaluations. Changes in these risk factors were analyzed over 5-year follow-up. The 5-year risk of VA was predicted longitudinally using (1) the baseline ARVC risk calculator prediction, (2) the ARVC risk prediction calculated using updated risk factors, and (3) time-varying Cox regression. Discrimination and calibration were assessed in comparison to observed VA event rates. Results: Four hundred eight patients with ARVC experiencing 132 primary VA events were included. Matched comparison of risk factors at baseline versus at 5 years of follow-up revealed decreased burdens of premature ventricular complexes (−1200/day) and nonsustained ventricular tachycardia (−14%). Presence of significant right ventricular dysfunction and number of T-wave inversions on electrocardiogram were unchanged. Observed risk for VA decreased by 13% by 5 years follow-up. The baseline ARVC risk calculator’s ability to predict 5-year VA risk worsened during follow-up (C statistics, 0.83 at diagnosis versus 0.68 at 5 years). Both the updated ARVC risk calculator (C statistics of 0.77) and time-varying Cox regression model (C statistics, 0.77) had strong discrimination. The updated ARVC risk calculator overestimated 5-year VA risk by an average of +6%. ConclusionS: Risk factors for VA in ARVC are dynamic, and overall risk for incident sustained VA decreases during follow-up. Up-to-date risk factor assessment improves VA risk stratification.

AimsPost-infarct myocardium contains viable corridors traversing scar or lipomatous metaplasia (LM). Ventricular tachycardia (VT) circuitry has been separately reported to associate with corridors that traverse LM and with repolarization heterogeneity. We examined the association of corridor activation recovery interval (ARI) and ARI dispersion with surrounding tissue type.Methods and resultsThe cohort included 33 post-infarct patients from the prospective Intra-Myocardial Fat Deposition and Ventricular Tachycardia in Cardiomyopathy (INFINITY) study. We co-registered scar and corridors from late gadolinium enhanced magnetic resonance, and LM from computed tomography with intracardiac electrogram locations. Activation recovery interval was calculated during sinus or ventricular pacing, as the time interval from the minimum derivative within the QRS to the maximum derivative within the T-wave on unipolar electrograms. Regional ARI dispersion was defined as the standard deviation (SD) of ARI per AHA segment (ARISD). Lipomatous metaplasia exhibited higher ARI than scar [325 (interquartile range 270–392) vs. 313 (255–374), P < 0.001]. Corridors critical to VT re-entry were more likely to traverse through or near LM and displayed prolonged ARI compared with non-critical corridors [355 (319–397) vs. 302 (279–333) ms, P < 0.001]. ARISD was more closely associated with LM than with scar (likelihood ratio χ2 50 vs. 12, and 4.2-unit vs. 0.9-unit increase in 0.01*Log(ARISD) per 1 cm2 increase per AHA segment). Additionally, LM and scar exhibited interaction (P < 0.001) in their association with ARISD.ConclusionLipomatous metaplasia is closely associated with prolonged local action potential duration of corridors and ARI dispersion, which may facilitate the propensity of VT circuit re-entry.

Pseudoaneurysms arising from the saphenous venous graft (SVGs) are an uncommon complication of the coronary artery bypass graft (CABG). Although angiography is the gold standard, computed tomography angiography (CTA) of the coronary arteries is the most efficient modality to diagnose these cases and their associated complications. Among numerous possible complications of SVG pseudoaneurysms, local mass effect on the cardiac chambers, adjacent pulmonary vasculature or coronary arteries/bypass grafts have been described. Cinematic rendering, an advanced post-processing technique can be valuable for problem solving in complex cases. In this pictorial essay, we report 3 CT cases of SVG pseudoaneurysms that demonstrate a range of CT imaging appearances of SVG pseudoaneurysms and related complications. Additionally, we will emphasize the applications of cinematic rendering in visualization of complex post-surgical cardiovascular pathology. This manuscript is designed as a pictorial essay of 3 complex cases given the relative uncommon nature of this pathology and the availability of cinematic rendering as an advanced post-processing modality. We demonstrated 3 imaging examples of utilization of cinematic rendering (CR) to demonstrate complex anatomical details of the mass-effect and compression related complications associated with SVG pseudoaneurysms. A side-by-side comparison with conventional volume rendering (VR) is also performed in 1 case for perspective and to show improved comparative visual appeal of the CR given better depth perception and more natural appearing illumination. The technical differences of the 2 techniques are also briefly discussed.

AimsDesmoplakin (DSP) cardiomyopathy is an increasingly recognized form of arrhythmogenic cardiomyopathy. With a genotype-specific approach, we characterized the diagnosis, natural history, and risk for ventricular arrhythmia and heart failure in DSP cardiomyopathy.Methods and resultsWe followed 91 individuals [45 probands, 34% male, median age 27.5 years (interquartile interval 20.0–43.9)] with pathogenic or likely pathogenic DSP variants for a median of 4.3 years. Regarding the ventricular involvement, left predominance was most common (n = 22, 28%) followed by bi-ventricular in 12 (15%) and right predominance in 5 (6%). Myocardial injury (chest pain, elevated troponin, normal coronary angiogram) occurred in 20 (22%) individuals. Incidence rates of sustained ventricular arrhythmia and heart failure (ventricular dysfunction ± symptoms) were 5.9 [95% confidence interval (CI): 3.9–9.1] and 6.7 (95% CI: 4.5–9.8) per 100 person-years, respectively. In univariate regression, myocardial injury was associated with sustained ventricular arrhythmia [hazard ratio (HR) 2.53, 95% CI: 1.05–6.11] and heart failure (HR 7.53, 95% CI: 3.10–18.26). After adjustment, left ventricular ejection fraction ConclusionDSP cardiomyopathy affects both ventricles and carries high risk for ventricular arrhythmia and heart failure. Myocardial injury is associated with worse disease outcomes. Both diagnosis and risk stratification of DSP cardiomyopathy need refinement.

This study aimed to investigate the prevalence of hepatic steatosis in racially diverse adults presenting to the emergency department at a tertiary healthcare system in the United States using attenuation values on unenhanced computed tomography (CT) as the reference standard. The utility of known risk factors for predicting the presence of hepatic steatosis was assessed. Additionally, reporting of hepatic steatosis in original radiology reports was evaluated. For 381 consecutive adults (193 women and 188 men; mean age 55.2 ± 17.2 years), hepatic (left medial, left lateral, right anterior, and right posterior) and splenic (upper, middle, and lower) CT attenuation values (Hounsfield units) were obtained by drawing a 1 cm

Nonischemic cardiomyopathies encompass a broad spectrum of myocardial disorders with mechanical or electrical dysfunction without evidence of ischemia. There are five broad variants of nonischemic cardiomyopathies; hypertrophic cardiomyopathy (Variant 1), restrictive or infiltrative cardiomyopathy (Variant 2), dilated or unclassified cardiomyopathy (Variant 3), arrhythmogenic cardiomyopathy (Variant 4), and inflammatory cardiomyopathy (Variant 5). For variants 1, 3, and 4, resting transthoracic echocardiography, MRI heart function and morphology without and with contrast, and MRI heart function and morphology without contrast are the usually appropriate imaging modalities. For variants 2 and 5, resting transthoracic echocardiography and MRI heart function and morphology without and with contrast are the usually appropriate imaging modalities. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Background Quantitative cardiovascular magnetic resonance T1-mapping is increasingly used for myocardial tissue characterization. However, the lack of standardization limits direct comparability between centers and wider roll-out for clinical use or trials. Purpose To develop a quality assurance (QA) program assuring standardized T1 measurements for clinical use. Methods MR phantoms manufactured in 2013 were distributed, including ShMOLLI T1-mapping and reference T1 and T2 protocols. We first studied the T1 and T2 dependency on temperature and phantom aging using phantom datasets from a single site over 4 years. Based on this, we developed a multiparametric QA model, which was then applied to 78 scans from 28 other multi-national sites. Results T1 temperature sensitivity followed a second-order polynomial to baseline T1 values (R2 > 0.996). Some phantoms showed aging effects, where T1 drifted up to 49% over 40 months. The correlation model based on reference T1 and T2, developed on 1004 dedicated phantom scans, predicted ShMOLLI-T1 with high consistency (coefficient of variation 1.54%), and was robust to temperature variations and phantom aging. Using the 95% confidence interval of the correlation model residuals as the tolerance range, we analyzed 390 ShMOLLI T1-maps and confirmed accurate sequence deployment in 90%(70/78) of QA scans across 28 multiple centers, and categorized the rest with specific remedial actions. Conclusions The proposed phantom QA for T1-mapping can assure correct method implementation and protocol adherence, and is robust to temperature variation and phantom aging. This QA program circumvents the need of frequent phantom replacements, and can be readily deployed in multicenter trials., Highlights • CMR T1 correlated with reference T1 and T2; this derives the QA model for T1-map. • The proposed QA model is robust to temperature variations and phantom aging. • This QA method requires no frequent phantom replacements. • The T1-map QA program can be readily deployed in multicenter trials.

High-resolution scar characterization using late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR) is useful for guiding ventricular arrhythmia (VA) treatment. However, imaging study quality may be degraded by breath-holding difficulties, arrhythmias, and implantable cardioverter-defibrillators (ICDs). We evaluated the effect of image quality on left ventricle (LV) base to apex scar interpretation in pre-VA ablation LGE-CMR. 43 consecutive patients referred for VA ablation underwent gradient-recalled-echo LGE-CMR. In ICD patients (n = 24), wide-bandwidth inversion-recovery suppressed ICD artifacts. In non-ICD patients, single-shot steady-state free-precession LGE-CMR could also be performed to reduce respiratory motion/arrhythmia artifacts. Study quality was assessed for adequate/limited scar interpretation due to cardiac/respiratory motion artifacts, ICD-related artifacts, and image contrast. 28% of non-ICD patients had studies where image quality limited scar interpretation in at least one image compared to 71% of ICD patient studies (p = 0.012). A median of five image slices had limited quality per ICD patient study, compared to 0 images per non-ICD patient study. Poorer quality in ICD patients was largely due to motion-related artifacts (54% ICD vs 6% non-ICD studies, p = 0.001) as well as ICD-related image artifacts (25% of studies). In VA ablation patients with ICDs, conventional CMR protocols frequently have image slices with limited scar interpretation, which can limit whole-heart scar assessment. Motion artifacts contribute to suboptimal image quality, particularly in ICD patients. Improved methods for motion and ICD artifact suppression may better delineate high-resolution LGE scar features of interest for guiding VA ablation.

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is diagnosed by a complex set of clinical tests as per 2010 Task Force Criteria (TFC). Avoiding misdiagnosis is crucial to prevent sudden cardiac death as well as unnecessary implantable cardioverter-defibrillator implantations. This study aims to validate the overall performance of the TFC in a real-world cohort of patients referred for ARVC evaluation. Methods and results We included patients consecutively referred to our centres for ARVC evaluation. Patients were diagnosed by consensus of three independent clinical experts. Using this as a reference standard, diagnostic performance was measured for each individual criterion as well as the overall TFC classification. Of 407 evaluated patients (age 38 ± 17 years, 51% male), the expert panel diagnosed 66 (16%) with ARVC. The clinically observed TFC was false negative in 7/66 (11%) patients and false positive in 10/69 (14%) patients. Idiopathic outflow tract ventricular tachycardia was the most common alternative diagnosis. While the TFC performed well overall (sensitivity and specificity 92%), signal-averaged electrocardiogram (SAECG, P = 0.43), and several family history criteria (P ≥ 0.17) failed to discriminate. Eliminating these criteria reduced false positives without increasing false negatives (net reclassification improvement 4.3%, P = 0.019). Furthermore, all ARVC patients met at least one electrocardiogram (ECG) or arrhythmia criterion (sensitivity 100%). Conclusion The TFC perform well but are complex and can lead to misdiagnosis. Simplification by eliminating SAECG and several family history criteria improves diagnostic accuracy. Arrhythmogenic right ventricular cardiomyopathy can be ruled out using ECG and arrhythmia criteria alone, hence these tests may serve as a first-line screening strategy among at-risk individuals.

Chest pain is a frequent cause for emergency department visits and inpatient evaluation, with particular concern for acute coronary syndrome as an etiology, since cardiovascular disease is the leading cause of death in the United States. Although history-based, electrocardiographic, and laboratory evaluations have shown promise in identifying coronary artery disease, early accurate diagnosis is paramount and there is an important role for imaging examinations to determine the presence and extent of anatomic coronary abnormality and ischemic physiology, to guide management with regard to optimal medical therapy or revascularization, and ultimately to thereby improve patient outcomes. A summary of the various methods for initial imaging evaluation of suspected acute coronary syndrome is outlined in this document. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Background It is unknown whether sex-specific differences in mortality observed in HCM are due to older age of women at presentation, or whether women have greater degree of LV myopathy than men. Methods We retrospectively compared clinical/imaging characteristics and outcomes between women and men in our overall cohort composed of 728 HCM patients, and in an age-matched subgroup comprised of 400 age-matched patients. We examined sex-specific differences in LV myopathy, and dissected the influence of age and sex on outcomes. LV myopathy was assessed by measuring LV mass, LVEF, global peak longitudinal systolic strain (LV-GLS), diastolic function (E/A, E/e′), late gadolinium enhancement (LV-LGE) and myocardial blood flow (MBF) at rest/stress. The primary endpoint was a composite outcome, comprising heart failure (HF), atrial fibrillation (AFib), ventricular tachycardia/fibrillation (VT/VF) and death; individual outcomes were defined as the secondary endpoint. Results Women in the overall cohort were older by 6 years. Women were more symptomatic and more likely to have obstructive HCM. Women had smaller LV cavity size, stroke volume and LV mass, higher indexed maximum wall thickness (IMWT), more hyperdynamic LVEF and higher/similar LV-GLS. Women had similar LV-LGE and E/A, but higher E/e′ and rest/stress MBF. Female sex was independently associated with the composite outcome in the overall cohort, and with HF in the overall cohort and age-matched subgroup after adjusting for obstructive HCM, LA diameter, LV-GLS. Conclusions Our results suggest that sex-specific differences in LV geometry, hyper-contractility and diastolic function, not greater degree of LV myopathy, contribute to a higher, age-independent risk of diastolic HF in women with HCM.

Objective To examine differences in image quality of the coronary arteries when performing high-pitch non-electrocardiography (ECG)-gated scans on the second-generation (2G) and third-generation (3G) dual-source CT scanners. Methods We retrospectively examined patients with high-pitch non-ECG-gated CT angiography (CTA) of the chest or chest/abdomen/pelvis. Outpatient scans from 59 patients in the 3G high-pitch group and 53 patients in the 2G high-pitch group were included. Two blinded cardiac imagers independently scored the coronary image quality using a 4-point Likert scale (from completely diagnostic to completely non-diagnostic) and evaluated the presence of coronary artery disease. Results Diagnostic image quality of the coronaries in high-pitch CTA exams using 3G scanner was improved compared to 2G scanner, both on a per-vessel basis (45–94% versus 23–86%) and on a per-study basis (34% versus 14%). The 3G group showed a statistically significant improvement in image quality when evaluating the left main coronary, right coronary, and overall coronary arteries. Coronary artery disease was detected in 65% of high-pitch scans. Radiation doses in terms of CTDIvol and DLP were lower for 3G high-pitch scans (7.5 mGy and 491 mGy∗cm) compared to 2G high-pitch scans (14.8 mGy and 911 mGy∗cm). Conclusions We found a higher proportion of diagnostic image quality of the coronary arteries in high-pitch non-ECG-gated CTA exams using a 3G dual-source CT scanner, compared to 2G. In scans with diagnostic image quality, underlying coronary artery disease may be confidently diagnosed and should prompt considerations for further management in the appropriate clinical setting.

Dyspnea is the symptom of perceived breathing discomfort and is commonly encountered in a variety of clinical settings. Cardiac etiologies of dyspnea are an important consideration; among these, valvular heart disease (Variant 1), arrhythmia (Variant 2), and pericardial disease (Variant 3) are reviewed in this document. Imaging plays an important role in the clinical assessment of these suspected abnormalities, with usually appropriate procedures including resting transthoracic echocardiography in all three variants, radiography for Variants 1 and 3, MRI heart function and morphology in Variants 2 and 3, and CT heart function and morphology with intravenous contrast for Variant 3. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, heritable myocardial disease associated with the development of ventricular arrhythmias, heart failure, and sudden cardiac death in early adulthood. Multimodality imaging is a central component in the diagnosis and evaluation of ARVC. Diagnostic criteria established by an international task force in 2010 include noninvasive parameters from echocardiography and cardiac magnetic resonance imaging. These criteria identify right ventricular structural abnormalities, chamber and outflow tract dilation, and reduced right ventricular function as features of ARVC. Echocardiography is a widely available and cost-effective technique, and it is often selected for initial evaluation. Beyond fulfillment of diagnostic criteria, features such as abnormal tricuspid annular plane excursion, increased right ventricular basal diameter, and abnormal strain patterns have been described. 3-dimensional echocardiography may also expand opportunities for structural and functional assessment of ARVC. Cardiac magnetic resonance has the ability to assess morphological and functional cardiac features of ARVC and is also a core modality in evaluation, however, tissue characterization of the right ventricle is limited by spatial resolution and low specificity for detection of pathological changes. Nonetheless, the ability of cardiac magnetic resonance to identify left ventricular involvement, offer high negative predictive value, and provide a reproducible structural evaluation of the right ventricle enhance the ability and scope of the modality. In this review, the prognostic significance of multimodality imaging is outlined, including the supplemental value of multidetector computed tomography and nuclear imaging. Strengths and weaknesses of imaging techniques, as well as future direction of multimodality assessment, are also described.

Hypertrophic cardiomyopathy (HCM) is associated with risk of sudden cardiac death (SCD) due to ventricular arrhythmias (VAs) arising from the proliferation of fibrosis in the heart. Current clinical risk stratification criteria inadequately identify at-risk patients in need of primary prevention of VA. Here, we use mechanistic computational modeling of the heart to analyze how HCM-specific remodeling promotes arrhythmogenesis and to develop a personalized strategy to forecast risk of VAs in these patients. We combine contrast-enhanced cardiac magnetic resonance imaging and T1 mapping data to construct digital replicas of HCM patient hearts that represent the patient-specific distribution of focal and diffuse fibrosis and evaluate the substrate propensity to VA. Our analysis indicates that the presence of diffuse fibrosis, which is rarely assessed in these patients, increases arrhythmogenic propensity. In forecasting future VA events in HCM patients, the imaging-based computational heart approach achieved 84.6%, 76.9%, and 80.1% sensitivity, specificity, and accuracy, respectively, and significantly outperformed current clinical risk predictors. This novel VA risk assessment may have the potential to prevent SCD and help deploy primary prevention appropriately in HCM patients.

Hypertrophic cardiomyopathy (HCM) causes sudden cardiac death (SCD) due to ventricular arrhythmias (VA) manifesting from myocardial fibrosis proliferation. Current clinical risk stratification criteria inadequately identify at-risk patients in need of primary prevention of VA. Here, we use mechanistic computational modeling of the heart to analyze how HCM-specific remodeling of the heart promotes arrhythmogenesis and to develop a personalized strategy to forecast risk of VAs in these patients. We combine contrast-enhanced cardiac magnetic-resonance (CMR) imaging and T1 mapping data to construct digital replicas of HCM patient hearts that represent the patient-specific distribution of focal and diffuse fibrosis and evaluate the substrate propensity to VA. Our analysis indicates that the presence of diffuse fibrosis, which is rarely assessed in these patients, increases arrhythmogenic propensity. In forecasting future VA events in HCM patients, the imaging-based computational heart approach achieved 84.6%, 76.9%, and 80.1% sensitivity, specificity, and accuracy, respectively, and significantly outperformed current clinical risk predictors. This novel VA risk assessment may have the potential to prevent SCD and help deploy primary prevention appropriately in HCM patients.

Hypertrophic cardiomyopathy (HCM) patients have a high incidence of atrial fibrillation (AF) and increased stroke risk, even with low CHAHCM patients with documented paroxysmal/persistent/permanent AF (n = 191) were considered AF cases, and the remaining patients in sinus rhythm (n = 640) were tagged as No-AF. We evaluated 93 clinical variables; the most informative variables useful for distinguishing AF from No-AF cases were selected based on the 2-sampleWe identified 18 highly informative variables that are positively (n = 11) and negatively (n = 7) correlated with AF in HCM. Next, patient records were represented via these 18 variables. Data imbalance resulting from the relatively low number of AF cases was addressed via a combination of oversampling and undersampling strategies. We trained and tested multiple classifiers under this sampling approach, showing effective classification. Specifically, an ensemble of logistic regression and naïve Bayes classifiers, trained based on the 18 variables and corrected for data imbalance, proved most effective for separating AF from No-AF cases (sensitivity = 0.74, specificity = 0.70, C-index = 0.80).Our model (HCM-AF-Risk Model) is the first machine learning-based method for identification of AF cases in HCM. This model demonstrates good performance, addresses data imbalance, and suggests that AF is associated with a more severe cardiac HCM phenotype.Les patients atteints d’une cardiomyopathie hypertrophique (CMH) présentent une forte incidence de fibrillation auriculaire (FA) et un risque accru d’accident vasculaire cérébral (AVC), malgré des scores CHANous avons considéré les patients atteints d’une CMH qui ont une FA paroxystique/persistante/permanente documentée (n = 191) comme des cas de FA, et avons étiqueté les autres patients en rythme sinusal (n = 640) comme des cas sans FA. Nous avons évalué 93 variables cliniques; nous avons sélectionné les variables les plus informatives qui sont utiles pour distinguer les cas de FA des cas sans FA en fonction du test t pour deux échantillons et du critère de gain d’information.Nous avons relevé 18 variables hautement informatives qui ont une corrélation positive (n = 11) et une corrélation négative (n = 7) avec la FA en présence d’une CMH. Ensuite, nous avons représenté les dossiers des patients au moyen de ces 18 variables. Nous avons remédié au déséquilibre des données, qui résulte du nombre relativement faible de cas de FA, grâce à une combinaison de stratégies de suréchantillonnage et de sous-échantillonnage. Nous avons formé et testé de nombreux classificateurs selon cette approche d’échantillonnage, qui montre une classification efficace. Particulièrement, un ensemble de régression logistique et de classificateurs bayésiens naïfs formés en fonction des 18 variables et corrigés en fonction du déséquilibre des données s’est révélé le plus efficace pour séparer les cas de FA des cas sans FA (sensibilité = 0,74, spécificité = 0,70, indice C = 0,80).Notre modèle (modèle de risque de CMH-FA) est la première méthode d’apprentissage automatique qui sert à déterminer les cas de FA en présence de CMH. Ce modèle permet de démontrer une bonne performance, de remédier au déséquilibre des données, et de croire que la FA est associée à un phénotype grave de CMH.

Cardiac magnetic resonance (CMR) and cardiac CT (CCT) have evolved into powerful diagnostic tools in the evaluation of patients with cardiovascular diseases. However, the use of these imaging techniques poses potential safety concerns for patients with implanted cardiac devices. These concerns result from the potential for electromagnetic interaction between the device and the CMR field or CCT x-ray radiation, which could lead to device heating, malfunction, or dislocation. Additionally, the presence of cardiac devices may induce significant image artifacts due to local magnetic field inhomogeneities (CMR) or photon starvation/beam hardening (CCT). In this review summarizes the safety issues regarding imaging in patients with cardiac devices. Optimization strategies to mitigate image artifacts and to improve imaging efficacy are discussed.

AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P

OBJECTIVES This study hypothesized that paroxysmal atrial fibrillation (PAF) reflects the presence of a more severe cardiac hypertrophic cardiomyopathy (HCM) phenotype.BACKGROUND HCM is characterized by myocyte hypertrophy, fibrosis, and a high prevalence of PAF. It is currently unresolved whether atrial fibrillation (AF) is a marker or a mediator of adverse outcomes in HCM.METHODS This study retrospectively examined 45 HCM patients who underwent cardiovascular magnetic resonance (CMR) imaging in sinus rhythm. The function of all 4 cardiac chambers was assessed, as well as late gadolinium enhancement (LGE) in the left atrium (LA) and left ventricle (LV), as indicators of fibrosis. A fat-saturated, 3-dimensional inversion recovery-prepared, fast-spoiled, gradient-recalled echo sequence, and the image intensity ratio method were used to measure LA-LGE; LGE in the LV was quantified using a semi-automated threshold technique.RESULTS HCM patients (n = 45) were divided into 2 groups (PAF, no AF) based on history of PAF. All HCM patients had LGE in the LA posterior wall. The PAF group (n = 18) had higher LA volume, a lower LA ejection fraction, a lower global peak longitudinal LA strain (PLAS), and a higher amount of LA-LGE compared with the no AF group (n = 27). A modest inverse association was noted between the LA ejection fraction, PLAS, and LA-LGE; a positive association was present between LV-LGE and LA-LGE. The PAF group had lower ejection fractions in the LV, right atrium, and right ventricle compared with those in the no AF group.CONCLUSIONS PAF is associated with a greater degree of structural LA remodeling and global myopathy, which suggests a more severe cardiac HCM phenotype. (c) 2019 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

BackgroundLeft ventricular (LV) fibrofatty infiltration in arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) has been reported, however, detailed cardiovascular magnetic resonance (CMR) characteristics and association with outcomes are uncertain. We aim to describe LV findings on CMR in ARVD/C patients and their relationship with arrhythmic outcomes.MethodsCMR of 73 subjects with ARVD/C according to the 2010 Task Force Criteria (TFC) were analyzed for LV involvement, defined as ≥ 1 of the following features: LV wall motion abnormality, LV late gadolinium enhancement (LGE), LV fat infiltration, or LV ejection fraction (LVEF) ResultsOf the 73 subjects, 50.7% had CMR evidence for LV involvement. Proband status and advanced RV dysfunction were independently associated with LV abnormalities. The most common pattern of LV involvement was focal fatty infiltration in the sub-epicardium of the apicolateral LV with a “bite-like” pattern. LGE in the LV was found in the same distribution and most often had a linear appearance. LV involvement was more common with non-PKP2genetic mutation variants, regardless of proband status. Only RV structural disease on CMR (HR 3.47, 95% CI 1.13–10.70) and prior arrhythmia (HR 2.85, 95% CI 1.33–6.10) were independently associated with arrhythmic events.ConclusionAmong patients with 2010 TFC for ARVD/C, CMR evidence for LV abnormalities are seen in half of patients and typically manifest as fibrofatty infiltration in the subepicardium of the apicolateral wall and are not associated with arrhythmic outcomes.

BACKGROUND –: Right ventricular (RV) contractile reserve shows promise as an indicator of occult RV dysfunction in pulmonary vascular disease. We investigated which measure of RV contractile reserve during exercise best predicts occult RV dysfunction and clinical outcomes. METHODS –: We prospectively studied RV contractile reserve in 35 human subjects referred for right heart catheterization for known or suspected pulmonary hypertension. All underwent cardiac magnetic resonance imaging, echocardiography, and supine invasive cardiopulmonary exercise testing with concomitant RV pressure-volume catheterization. Event-free survival was prospectively adjudicated from time of right heart catheterization for a 4-year follow-up period. RESULTS –: RV contractile reserve during exercise, as measured by a positive change in end-systolic elastance (Ees) during exertion, was associated with elevation in pulmonary pressures but preservation of RV volumes. Lack of RV reserve, on the other hand, was tightly coupled with acute RV dilation during exertion (R(2)=0.76, P

Supplemental Digital Content is available in the text., Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. Methods: We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. Results: A total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77–10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69–0.80) and calibration slope of 0.95 (95% CI, 0.94–0.98) indicating minimal over-optimism. Conclusions: LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.