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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.

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.

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.

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.

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.

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.

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/).

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

Clinical risk stratification for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HC) employs rules derived from American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines or the HCM Risk-SCD model (C-index ∼0.69), which utilize a few clinical variables. We assessed whether data-driven machine learning methods that consider a wider range of variables can effectively identify HC patients with ventricular arrhythmias (VAr) that lead to SCD. We scanned the electronic health records of 711 HC patients for sustained ventricular tachycardia or ventricular fibrillation. Patients with ventricular tachycardia or ventricular fibrillation (n = 61) were tagged as VAr cases and the remaining (n = 650) as non-VAr. The 2-sample ttest and information gain criterion were used to identify the most informative clinical variables that distinguish VAr from non-VAr; patient records were reduced to include only these variables. Data imbalance stemming from low number of VAr cases was addressed by applying a combination of over- and undersampling strategies. We trained and tested multiple classifiers under this sampling approach, showing effective classification. We evaluated 93 clinical variables, of which 22 proved predictive of VAr. The ensemble of logistic regression and naive Bayes classifiers, trained based on these 22 variables and corrected for data imbalance, was most effective in separating VAr from non-VAr cases (sensitivity = 0.73, specificity = 0.76, C-index = 0.83). Our method (HCM-VAr-Risk Model) identified 12 new predictors of VAr, in addition to 10 established SCD predictors. In conclusion, this is the first application of machine learning for identifying HC patients with VAr, using clinical attributes. Our model demonstrates good performance (C-index) compared with currently employed SCD prediction algorithms, while addressing imbalance inherent in clinical data.

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.

Background: Infiltrating adipose tissue (inFAT) is a newly recognized proarrhythmic substrate for postinfarct ventricular tachycardias (VT) identifiable on contrast-enhanced computed tomography. This study presents novel digital-heart technology that incorporates inFAT from contrast-enhanced computed tomography to noninvasively predict VT ablation targets and assesses the capability of the technology by comparing its predictions with VT ablation procedure data from patients with ischemic cardiomyopathy. Methods: Digital-heart models reflecting patient-specific inFAT distributions were reconstructed from contrast-enhanced computed tomography. The digital-heart identification of fat-based ablation targeting (DIFAT) technology evaluated the rapid-pacing–induced VTs in each personalized inFAT-based substrate. DIFAT targets that render the inFAT substrate noninducible to VT, including VTs that arise postablation, were determined. DIFAT predictions were compared with corresponding clinical ablations to assess the capabilities of the technology. Results: DIFAT was developed and applied retrospectively to 29 ischemic cardiomyopathy patients with contrast-enhanced computed tomography. DIFAT ablation volumes were significantly less than the estimated clinical ablation volumes (1.87±0.35 versus 7.05±0.88 cm 3 , P Conclusions: DIFAT is a novel digital-heart technology for individualized VT ablation guidance designed to eliminate VT inducibility following initial ablation. DIFAT predictions colocalized well with clinical ablation locations but provided significantly smaller lesions. DIFAT also predicted VTs targeted in redo procedures years later. As DIFAT uses widely accessible computed tomography, its integration into clinical workflows may augment therapeutic precision and reduce redo procedures.

Preoperative assessment with computed tomography (CT) is critical before transcatheter interventions for structural heart disease. CT provides information for device selection, device sizing, and vascular access approach. The interpreting radiologist must have knowledge of appropriate CT protocols, how and where to obtain the important measurements, and know additional imaging characteristics that are important to describe for optimal support of the interventionalist. CT is the modality of choice for pre-operative evaluation in patients undergoing transcatheter aortic valve replacement and left atrial appendage occlusion, and is also useful before transcatheter mitral valve replacement, which is an ongoing area of research.

Advances in cardiac magnetic resonance (CMR) techniques and image acquisition have made it an excellent tool in the assessment of atrial myopathy. Remolding of the left atrium is the mainstay of atrial fibrillation (AF) development and its progression. CMR can detect phasic atrial volumes, atrial function, and atrial fibrosis using cine, and contrast-enhanced or non-contrast-enhanced images. These abilities make CMR a versatile and extraordinary tool in management of patients with AF including for risk stratification, ablation prognostication and planning, and assessment of stroke risk. We review the latest advancements in utility of CMR in management of patients with AF.

Abnormalities on cardiac magnetic resonance imaging (CMR) and positron emission tomography (PET) predict ventricular arrhythmias (VA) in patients with cardiac sarcoidosis (CS). Little is known whether concurrent abnormalities on CMR and PET increases the risk of developing VA. Our aim was to compare the additive utility of CMR and PET in predicting VA in patients with CS. We included all patients treated at our institution from 2000 to 2018 who (1) had probable or definite CS and (2) had undergone both CMR and PET. The primary endpoint was VA at follow up, which was defined as sustained ventricular tachycardia, sudden cardiac death, or any appropriate device tachytherapy. Fifty patients were included, 88% of whom had a left ventricular ejection fraction >35%. During a mean follow-up 4.1 years, 7/50 (14%) patients had VA. The negative predictive value of LGE for VA was 100% and the negative predictive value of FDG for VA was 79%. Among groups, VA occurred in 4/21 (19%) subjects in the LGE+/FDG+ group, 3/14 (21%) in the LGE+/FDG- group, and 0/15 (0%) in the FDG+/LGE- group. There were no LGE-/FDG- patients. In conclusion, CMR may be the preferred initial clinical risk stratification tool in patients with CS. FDG uptake without LGE on initial imaging may not add additional prognostic information regarding VA risk.

BACKGROUND: The risks associated with MRI in individuals who have implanted cardiac devices are thought to arise from the interaction between the implanted device and static, gradient, and radiofrequency magnetic fields. PURPOSE: To determine the relationship between the peak whole-body averaged specific absorption rate (SAR) and change in magnetic field per unit time (dB/dt), maximum specific energy dose, imaging region, and implanted cardiac device characteristics and their function in patients undergoing MRI. MATERIALS AND METHODS: This prospective observational cohort study was conducted from October 16, 2003, to January 22, 2015 (https://ClinicalTrials.gov, NCT01130896). Any individual with an implanted cardiac device who was referred for MRI was included. Clinical MRI protocols without SAR restriction were used. Exclusion criteria were newly implanted leads, abandoned or epicardial leads, and dependence on a pacemaker with an implantable cardioverter defibrillator without asynchronous pacing capability. For each MRI pulse sequence, the calculated whole-body values for SAR, dB/dt, and scan duration were collected. Atrial and ventricular sensing, lead impedance, and capture threshold were evaluated before and immediately after (within 10 minutes) completion of each MRI examination. Generalized estimating equations with Gaussian family, identity link, and an exchangeable working correlation matrix were used for statistical analysis. RESULTS: A total of 2028 MRI examinations were performed in 1464 study participants with 2755 device leads (mean age, 67 years ± 15 [standard deviation]; 930 men [64%]). There was no evidence of an association between radiofrequency energy deposition, dB/dt, or scan duration and changes in device parameters. Thoracic MRI was associated with decreased battery voltage immediately after MRI (β = −0.008 V, P < .001). Additionally, right ventricular (RV) lead length was associated with decreased RV sensing (β = −0.012 mV, P = .05) and reduced RV capture threshold (β = −0.002 V, P < .01) immediately after MRI. CONCLUSION: There was no evidence of an association between MRI parameters that characterize patient exposure to radiofrequency energy and changes in device and lead parameters immediately after MRI. Nevertheless, device interrogation before and after MRI remains mandatory due to the potential for device reset and changes in lead or generator parameters. © RSNA, 2020 See also the editorial by Shellock in this issue.

Vasodilator-induced transient left ventricular cavity dilation (LVCD) by positron emission tomography (PET) is associated with microvascular dysfunction in hypertrophic cardiomyopathy (HCM). Here we assessed whether HCM patients who develop LVCD by PET during vasodilator stress also develop LV cavity dilation by echocardiography (ECHO-LVCD) following exercise stress. A retrospective analysis of cardiac function and myocardial blood flow (MBF) was conducted in 108 HCM patients who underwent perfusion-PET and exercise-ECHO as part of their clinical evaluation. We performed a head-to-head comparison of LV volumes and ejection fraction (LVEF) at rest and stress (during vasodilator stress, post-exercise), in 108 HCM patients. A ratio > 1.13 of stress to rest LV volumes was used to define PET-LVCD, and a ratio > 1.17 of stress to rest LVESV was used to define ECHO-LVCD. Patients were divided into 2 groups based on the presence/absence of PET-LVCD. MBF and myocardial flow reserve were quantified by PET, and global longitudinal strain (GLS) was assessed by ECHO at rest/stress in the two groups. PET-LVCD was observed in 51% (n = 55) of HCM patients, but only one patient had evidence of ECHO-LVCD (ratio = 1.36)—this patient also had evidence of PET-LVCD (ratio = 1.20). The PET-LVCD group had lower PET-LVEF during vasodilator stress, but ECHO-LVEF increased in both groups post-exercise. The PET-LVCD group demonstrated higher LV mass, worse GLS at rest/stress, and lower myocardial flow reserve. Incidence of ischemic ST-T changes was higher in the PET-LVCD group during vasodilator stress (42 vs 17%), but similar (30%) in the two groups during exercise. PET-LVCD reflects greater degree of myopathy and microvascular dysfunction in HCM. Differences in the cardiac effects of exercise and vasodilators and timing of stress-image acquisition could underlie discordance in ischemic EKG changes and LVCD by ECHO and PET, in HCM.

Chronic chest pain (CCP) of a cardiac etiology is a common clinical problem. The diagnosis and classification of the case of chest pain has rapidly evolved providing the clinician with multiple cardiac imaging strategies. Though scintigraphy and rest echocardiography remain as appropriate imaging tools in the diagnostic evaluation, new technology is available. Current evidence supports the use of alternative imaging tests such as coronary computed tomography angiography (CCTA), cardiac MRI (CMRI), or Rb-82 PET/CT. Since multiple imaging modalities are available to the clinician, the most appropriate noninvasive imaging strategy will be based upon the patient's clinical presentation and clinical status. 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.

While there is no single diagnostic test for heart failure (HF), imaging plays a supportive role beginning with confirmation of HF, especially by detecting ventricular dysfunction ( Variant 1 ). Ejection fraction (EF) is important in HF classification, and imaging plays a subsequent role in differentiation between HF with reduced EF (HFrEF) versus preserved EF (HFpEF) ( Variant 2 ). Once HFrEF is identified, distinction between ischemic and nonischemic etiologies with imaging support ( Variant 3 ) facilitates further planning. Imaging approaches which are usually appropriate include: both resting transthoracic echocardiography (TTE) and chest radiography for Variant 1 ; resting TTE and/or MRI (including functional, without absolute need for contrast) for Variant 2 ; and for Variant 3 , a. Coronary CTA or coronary arteriography (if high pretest probability/symptoms for ischemic disease) for coronary assessment; b. Rest/vasodilator stress SPECT/CT, PET/CT, or MRI for myocardial perfusion assessment; c. Rest/exercise or inotropic stress TTE for myocardial contraction assessment; or d. MRI (including morphologic with contrast) for myocardial characterization. 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 Ablation-induced left atrial (LA) edema may result in procedural failure due to reversible pulmonary vein isolation. Conventional T2-weighted magnetic resonance edema imaging is limited by low spatial resolution. Objective The purpose of this pilot study was to optimize and validate a 3-dimensional (3D) sampling perfection with application-optimized contrasts using different flip-angle evolution (SPACE) sequence for quantification of T2 signal in the LA, and to apply it in recently ablated patients, comparing myocardial edema on T2-SPACE to tissue damage on late gadolinium enhancement (LGE) imaging. Methods Phantom studies were performed to identify 3D-SPACE parameters for optimal contrast between normal and edematous myocardium. Fourteen AF patients were imaged with both 3D-SPACE and dark-blood turbo-spin echo (DB-TSE) to compare image quality and signal intensity between the 2 techniques. Eight patients underwent pre- and postablation 3D-SPACE and 3D-LGE imaging. Ablation points were co-registered with corresponding myocardial sectors, and ablation-induced changes in T2 and LGE signal intensities were measured. Results Signal-to-noise ratio and contrast-to-noise ratio were higher on SPACE vs DB-TSE (65.5 ± 33.9 vs 35.7 ± 17.9; P = .01; and 59.4 ± 33.0 vs 32.9 ± 17.7; P = .04, respectively). T2-signal correlated well on 3D-SPACE and DB-TSE, such that each unit increase in TSE intensity correlated with a 0.69-unit increase in SPACE intensity (95% confidence interval 0.56–0.82; P Conclusion T2-SPACE can be used to map the extent of acute postablation edema in the thin LA myocardium, with improved resolution and lower artifact compared to traditional DB-TSE.

The P-wave terminal force in lead V1 (PTFV1) on the 12-lead electrocardiogram (ECG) quantifies left atrial (LA) structural and electrophysiologic abnormalities. We aimed to evaluate the association between PTFV1 and cerebrovascular accident (CVA) as well as LA structure and function in patients with atrial fibrillation (AF). We conducted a cross-sectional study of 229 patients with AF (60 ± 10years, 72% men) with (n = 21) and without (n = 208) a history of CVA, who underwent preablation ECG and cardiac magnetic resonance in sinus rhythm. PTFV1 was defined as the duration (in milliseconds) of the downward deflection of the P wave in lead V1 multiplied by the absolute value of its amplitude (in microvolts) on ECG. PTFV1 is associated with LA minimum volume (Vmin) and left ventricular ejection fraction but not associated with the extent of LA fibrosis quantified by cardiac magnetic resonance late gadolinium enhancement. In addition, PTFV1 is associated with CVA independent of the CHA2DS2-VASc score and LA Vmin (odds ratio 1.23; 95% confidence interval 1.08 to 1.40; p = 0.002). Furthermore, PTFV1 has an incremental value over the CHA2DS2-VASc score as a marker of CVA (p

Background Exercise intolerance is the most common symptom in hypertrophic cardiomyopathy (HCM). We examined whether inability to augment myocardial mechanics during exercise would influence functional performance and clinical outcomes in HCM. Methods Ninety-five HCM patients (32 nonobstructive, 32 labile-obstructive, 31 obstructive) and 26 controls of similar age and gender distribution were recruited prospectively. They underwent rest and treadmill stress strain echocardiography, and 61 of them underwent magnetic resonance imaging. Mechanical reserve (MRES) was defined as percent change in systolic strain rate (SR) immediately postexercise. Results Global strain and SR were significantly lower in HCM patients at rest (strain: nonobstructive, −15.6 ± 3.0; labile-obstructive, −15.9 ± 3.0; obstructive, −13.8 ± 2.9; control, −17.7% ± 2.1%, P −1 , P = .002); and immediately postexercise (strain: nonobstructive, −15.6 ± 3.0; labile-obstructive, −17.6 ± 3.6; obstructive, −15.6 ± 3.6; control, −19.2 ± 3.1%; P = .001; SR: nonobstructive, −1.41 ± 0.37; labile-obstructive, −1.64 ± 0.38; obstructive, −1.32 ± 0.29; control, −1.82 ± 0.29 s −1 , P P = .001, respectively). Postexercise SR and MRES were associated with exercise capacity ( r = 0.47 and 0.42, P r = 0.74, P = .003). Postexercise SR was correlated with extent of late gadolinium enhancement ( r = 0.34, P = .03). By Cox regression, exercise SR and MRES predicted ventricular tachycardia/ventricular fibrillation (VT/VF) even after adjustment for age, gender, family history of sudden cardiac death, septum ≥ 3 cm and abnormal blood pressure response ( P = .04 and P = .046, respectively). Conclusions Nonobstructive and obstructive patients have reduced MRES compared with labile-obstructive and controls. Postexercise SR correlates with LGE and exercise capacity. Exercise SR and MRES predict VT/VF.

Bolus timing is critical to optimal magnetic resonance angiography (MRA) acquisitions but can be challenging in some patients. Our purpose was to evaluate whether contrast-enhanced time-resolved magnetic resonance angiography (TR-MRA), a dynamic multiphase sequence that does not rely on bolus timing, is a viable alternative method to conventional 3D fast-long angle shot contrast-enhanced magnetic resonance angiography (CE-MRA). Coronal subtracted conventional CE-MRA images in 50 consecutive patients presenting for pre-atrial fibrillation ablation pulmonary venous (PV) mapping were compared with 50 TR-MRA images performed in 50 subsequent patients. The TR-MRA protocol was modified to optimize spatial resolution with slightly reduced temporal resolution (6.1 s scan time). Three experienced readers evaluated each scan’s image quality and relative left atrial (LA) opacification based on a 4-point scale and diagnostic PV visualization in a binary fashion. Additionally, LA signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and PV dimensions were measured for both techniques. TR-MRA had significantly higher overall image quality (3.10 ± 0.69 vs. 2.42 ± 0.69, p

Kawasaki's disease (KD) is a vasculitis that predominantly affects children and can lead to the development of coronary artery aneurysms. These aneurysms can subsequently thrombose and occlude, which may lead to chest pain and other signs and symptoms of acute coronary syndrome in young patients. Coronary CT angiography, including 3D visualization techniques, is a common modality used in the follow-up of KD patients. In this series of three patients, we present the typical coronary artery imaging findings that can appear in these patients, with an emphasis on the use of the novel 3D technique of cinematic rendering (CR). CR utilizes a different lighting model than other 3D methods and is able to produce highly-detailed, photorealistic images. The potential advantages of CR images in understanding the complex mediastinal vascular anatomy and the relationships of coronary artery aneurysms to other anatomic structures are emphasized.

Journal of Cardiovascular Magnetic Resonance, 20, ISSN:1097-6647, ISSN:1532-429X

The aim of this study was to evaluate the effect of upfront combination therapy with ambrisentan and tadalafil on left ventricular (LV) and right ventricular (RV) function in patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH). LV and RV peak longitudinal and circumferential strain and strain rate (SR), which consisted of peak systolic SR (SRs), peak early diastolic SR (SRe), and peak atrial-diastolic SR (SRa) were analyzed using cardiac magnetic resonance imaging (CMRI) data from the recently published ATPAHSS-O trial (ambrisentan and tadalafil upfront combination therapy in SSc-PAH). Twenty-one patients completed the study protocol. Measures of RV systolic function (RV free wall [RVFW] peak longitudinal strain [pLS], RVFW peak longitudinal SRs [pLSRs]) and RV diastolic function (RVFW peak longitudinal SRa [pLSRa], RVFW peak circumferential SRe) were improved after treatment. LV systolic function (LV peak global longitudinal strain [pGLS]) and diastolic function (LV peak global longitudinal SRe [pGLSRe]) were also significantly improved at follow-up. Increased 6-min walk distance was significantly correlated with RVFW pLS and pLSRs, while the decrease in N-terminal pro-brain natriuretic peptide was correlated with LV pGLS. Increased cardiac index was associated with improved LV pGLSRe, and reduction in mean right atrial pressure was correlated with improved RVFW pLS and pLSRa. Combination therapy was associated with a significant improvement in both RV and LV function as assessed by CMR-derived strain and SR. Importantly, the improvement in RV and LV strain and SR correlated with improvements in known prognostic markers of PAH. (Approved by clinicaltrials.gov [NCT01042158] before patient recruitment.).

Hypertrophic cardiomyopathy (HCM) is frequently associated with exercise-related sudden death. One possible contributor to arrhythmias is pathologic hypertrophy, which can lead to QTc prolongation. Since beta-adrenergic stimulation increases inward ionic currents and can increase spatial repolarization heterogeneity, we hypothesized that exercise would prolong QTc and predispose to arrhythmias in HCM patients. We tested this hypothesis by measuring QTc at rest and peak exercise, and examining ECGs for arrhythmias during exercise/recovery in 163 patients with a clinical diagnosis of HCM. Since hypertrophy and fibrosis are risk factors for arrhythmias in HCM, left ventricular (LV) mass and cardiac fibrosis were quantified using cardiac magnetic resonance imaging. Exercise led to an increase in QTc interval (436 +/- 37 to 482 +/- 45, p < 0.001), QRS duration (100 +/- 9 to 109 +/- 12, p < 0.001) and ST-T abnormalities. The proportion of patients with QTc prolongation (>470 ms) increased from 28% at rest, to 58% at peak exercise. An inverse correlation was detected between the approximated T1 relaxation time (using the Look Locker sequence) and rest-QTc (r = -0.68, p < 0.001); this association was increased during exercise (r = -0.81, p < 0.001). Late gadolinium enhancement (LGE) and LV mass were weakly associated with exercise-QTc. A weak inverse correlation was also observed between approximated T1 relaxation time and rest/exercise-QRS duration. A trend for association with arrhythmia (non-sustained ventricular tachycardia on Holler) was observed for rest-QTc >470 ms (OR = 3.0;p = 0.06). We conclude that exercise leads to QTc prolongation in a subset of HCM patients. The association between the approximated T1 relaxation time and QTc interval may reflect concomitant electrical and structural remodeling in HCM.

Objective We sought to derive and validate a model for identifying suspected ACS patients harboring undiagnosed significant coronary artery disease (CAD). Methods This was a secondary analysis of data from a randomized control trial (RCT). Patients randomized to the CTA arm of an RCT examining a CTA-based strategy for ruling-out acute coronary syndrome (ACS) constitute the derivation cohort, which was randomly divided into a training dataset (2/3, used for model derivation) and a test dataset (1/3, used for internal validation (IV)). ED patients from a different center receiving CTA to evaluate for suspected ACS constitute the external validation (EV) cohort. Primary outcome was CTA-assessed significant CAD (stenosis of ≥50% in a major coronary artery). Results In the derivation cohort, 11.2% (76/679) of subjects had CTA-assessed significant CAD, and in the EV cohort, 8.2% of subjects (87/1056) had CTA-assessed significant CAD. Age was the strongest predictor of significant CAD among the clinical risk factors examined. Predictor variables included in the derived logistic regression model were: age, sex, tobacco use, diabetes, and race. This model exhibited an area under the receiver operating characteristic curve (ROC AUC) of 0.72 (95% CI: 0.61–0.83) based on IV, and 0.76 (95% CI: 0.70, 0.82) based on EV. The derived random forest model based on clinical risk factors yielded improved but not sufficient discrimination of significant CAD (ROC AUC = 0.76 [95% CI: 0.67–0.85] based on IV). Coronary artery calcium score was a more accurate predictor of significant CAD than any combination of clinical risk factors (ROC AUC = 0.85 [95% CI: 0.76–0.94] based on IV; ROC AUC = 0.92 [95% CI: 0.88–0.95] based on EV). Conclusions Clinical risk factors, either individually or in combination, are insufficient for accurately identifying suspected ACS patients harboring undiagnosed significant coronary artery disease.

Background: Hypertrophic cardiomyopathy (HCM) is characterized by myocyte hypertrophy, disarray, fibrosis, and increased risk for ventricular arrhythmias. Increased QT dispersion has been reported in patients with HCM, but the underlying mechanisms have not been completely elucidated. In this study, we examined the relationship between diffuse interstitial fibrosis, replacement fibrosis, QTc dispersion and ventricular arrhythmias in patients with HCM. We hypothesized that fibrosis would slow impulse propagation and increase dispersion of ventricular repolarization, resulting in increased QTc dispersion on surface electrocardiogram (ECG) and ventricular arrhythmias. Methods: ECG and cardiac magnetic resonance (CMR) image analyses were performed retrospectively in 112 patients with a clinical diagnosis of HCM. Replacement fibrosis was assessed by measuring late gadolinium (Gd) enhancement (LGE), using a semi-automated threshold technique. Diffuse interstitial fibrosis was assessed by measuring T1 relaxation times after Gd administration, using the Look-Locker sequence. QTc dispersion was measured digitally in the septal/anterior (V1-V4), inferior (II, III, and aVF), and lateral (I, aVL, V5, and V6) lead groups on surface ECG. Results: All patients had evidence of asymmetric septal hypertrophy. LGE was evident in 70 (63%) patients; the median T1 relaxation time was 411 +/- 38 ms. An inverse correlation was observed between T1 relaxation time and QTc dispersion in leads V1-V4 (p < 0.001). Patients with HCM who developed sustained ventricular tachycardia had slightly higher probability of increased QTc dispersion in leads V1-V4 (odds ratio, 1.011 [1.004-1.0178, p=0.003). We found no correlation between presence and percentage of LGE and QTc dispersion. Conclusion: Diffuse interstitial fibrosis is associated with increased dispersion of ventricular repolarization in leads, reflecting electrical activity in the hypertrophied septum. Interstitial fibrosis combined with ion channel/gap junction remodeling in the septum could lead to inhomogeneity of ventricular refractoriness, resulting in increased QTc dispersion in leads V1-V4.

Correct identification of arrhythmogenic right ventricular cardiomyopathy (ARVC) is pertinent because ventricular arrhythmias can occur early in the disease [(1)][1]. Ventricular arrhythmias in ARVC typically have a re-entrant mechanism caused by diffuse fibrosis. Although late gadolinium

BACKGROUND Patients with cardiac sarcoidosis (CS) may present with arrhythmic events (AE): atrioventricular block (AVB) and/ or ventricular arrhythmias (VA). We sought to: (a) use regional analysis of cardiac magnetic resonance imaging (CMR) to describe anatomic and functional phenotypes of patients with CS and AE; (b) Assess the association of regional CMR abnormalities with the combined endpoint of death, heart transplantation (HT) and AE; and (c) use machine learning (ML) to predict the combined endpoint based on CMR features. METHODS we included 76 patients with CS and CMR. We analyzed cine images to determine regional longitudinal (LS) and radial strain (RS); and late gadolinium enhancement imaging to determine regional scar burden (%scar). RESULTS Patients with AVB (n = 7), compared with those without, had higher %scar in the anterior (21.8 ± 27.4 vs 5.1 ± 8.9; P = 0.0005) and anteroseptal (19.3 ± 24.5 vs 3.5 ± 5.5; P

Background: Abnormalities on cardiac imaging (cardiac magnetic resonance imaging [CMR] or positron emission tomography [PET]), left ventricular ejection fraction (LVEF), and electrophysiology study (EPS) all predict ventricular arrhythmias (VA) in patients with cardiac sarcoidosis (CS). We sought to assess the utility of EPS in patients with CS and abnormal cardiac imaging, focusing on those with LVEF >35%. Methods: We identified all patients treated at our institution from 2000 to 2017 who: 1.) had probable or definite CS; 2.) had either late gadolinium enhancement (LGE) on CMR or abnormal 18-flourodeoxyglucose (FDG) uptake on PET, and 3.) had undergone EPS. The primary endpoint was VA during follow up. Results: Twenty five patients were included, of whom 10 (40%) had positive EPS. During a mean follow-up of 4.8 +/− 3.4 years, 11 (44%) patients had VA. The positive predictive value (PPV) of EPS for VA was 100% and the negative predictive value (NPV) of EPS for VA was 93%. Among 12 patients with LVEF >35% and no prior VA, the PPV of EPS for VA was 100% and the NPV of EPS for VA was 90%. Conclusion: EPS may help with risk stratification in patients with CS and abnormal imaging, especially those without conventional indications for ICD placement. Among patients with LVEF >35% and no history of prior VA, a negative EPS has good positive and negative predictive value for future VA events. Keywords: Cardiac sarcoidosis, Ventricular arrhythmia, Sudden cardiac death, Electrophysiology study, Implantable cardioverter defibrillator

Background— Right ventricular (RV) functional reserve affects functional capacity and prognosis in patients with pulmonary arterial hypertension (PAH). PAH associated with systemic sclerosis (SSc-PAH) has a substantially worse prognosis than idiopathic PAH (IPAH), even though many measures of resting RV function and pulmonary vascular load are similar. We therefore tested the hypothesis that RV functional reserve is depressed in SSc-PAH patients. Methods and Results— RV pressure-volume relations were prospectively measured in IPAH (n=9) and SSc-PAH (n=15) patients at rest and during incremental atrial pacing or supine bicycle ergometry. Systolic and lusitropic function increased at faster heart rates in IPAH patients, but were markedly blunted in SSc-PAH. The recirculation fraction, which indexes intracellular calcium recycling, was also depressed in SSc-PAH (0.32±0.05 versus 0.50±0.05; P =0.039). At matched exercise (25 W), SSc-PAH patients did not augment contractility (end-systolic elastance) whereas IPAH did ( P P =0.004, and +19±4% versus –1±6%, P Conclusions— RV contractile reserve is depressed in SSc-PAH versus IPAH subjects, associated with reduced calcium recycling. During exercise, this results in ventricular-pulmonary vascular uncoupling and acute RV dilation. RV dilation during exercise can predict adverse ventricular-vascular coupling in PAH patients.

Prognostic significance of left atrial (LA) function in patients with atrial fibrillation (AF) is poorly defined.To examine the association of LA function measured with cardiac magnetic resonance (CMR) feature-tracking and AF recurrence following catheter ablation.One hundred and twenty-one AF patients (72% paroxysmal, mean age 59 ± 10 years) were enrolled. Baseline LA function was measured by calculating passive, active, and total emptying fractions (LAEF) and analysis of global longitudinal strain and strain rates. Patients were followed up for recurrence of AF or atrial tachycardia (AT). Hazard ratios for recurrence were calculated using Cox proportional models adjusted for potential clinical confounders, type of AF, left ventricular ejection fraction, AF duration, LA volume, and late gadolinium enhancement (LGE).During a mean follow-up of 18 ± 9 months, 52 patients (43%) experienced recurrent AF/AT. Patients with recurrent AF/AT had higher baseline LA volume index and lower LA passive, and total LAEF (P.05 for all). The baseline peak LA strain and strain rates in all phases of LA function were lower in the AF/AT recurrence group (P.01 for all). In multivariable analysis total LAEF, peak LA strain, and systolic and late diastolic strain rates were associated with recurrence. Both peak LA strain and total LAEF improved prediction of recurrent AT/AF compared to the baseline clinical model, including LA LGE (C statistic 0.82 vs 0.77, P.05 for both total LAEF and peak LA strain).LA reservoir function was independently associated with recurrent AF/AT after PVI and can additionally improve risk stratification in patients undergoing PVI.

The feasibility of remote reading of coronary CT examinations on tablet computers has not been evaluated. The purpose of this study is to evaluate the accuracy of coronary CT angiography reading using an iPad compared to standard 3D workstations. Fifty coronary CT angiography exams, including a spectrum of coronary artery disease and anatomic variants, were reviewed. Coronary CT angiography exams were interpreted by two readers independently on an iPad application (Siemens Webviewer) and a clinical 3D workstation at sessions 2 weeks apart. Studies were scored per vessel for severity of stenosis on a 0-3 scale (0 none, 150 %, 2 ≥50-69 %, 3 ≥70 %). Coronary anomalies were recorded. A consensus read by two experienced cardiac imagers was used as the reference standard. Level of agreement with the reference for iPad and 3D workstations was compared. Multivariate logistic regression was used to analyze the relationship between agreement and display type and to adjust for inter-reader differences. For both readers, there was no significant difference in agreement with the reference standard for per-vessel stenosis scores using either the 3D workstation or the iPad. In a multivariable logistic regression analysis including reader, workstation, and vessel as co-variates, there was no significant association between workstation type or reader and agreement with the reference standard (p 0.05). Both readers identified 100 % of coronary anomalies using each technique. Reading of coronary CT angiography examinations on the iPad had no influence on stenosis assessment compared to the standard clinical workstation.

Background The extent of left atrial (LA) late gadolinium enhancement (LGE), as a surrogate for fibrosis, has been associated with atrial fibrillation (AF) recurrence after catheter ablation. Furthermore, there is ex vivo evidence that islands of fibrosis may anchor fibrillatory rotors. Objective The purpose of this study was to examine the anatomical association of AF rotors with LA and right atrial (RA) LGE on cardiac magnetic resonance. Methods The cohort included 9 patients with persistent AF (mean age 61.1 ± 9.7 years) who underwent LGE cardiac magnetic resonance before AF ablation using the focal impulse and rotor modulation system. The extent of LA and RA LGE was quantified globally and in each of the 7 sectors: LA posterior/inferior wall, anterior wall, roof, left and right pulmonary vein antra, and RA lateral and septal regions. The multivariable association of rotor incidence with global and per sector LGE extent was examined using multivariable Bernoulli logistic regression estimated by generalized estimating equations. Results The mean RA and LA volumes were 113.2 ± 37.31 and 143.03 ± 58.25 mL, respectively. The mean RA and LA LGE burden was 17.2% ± 11.0% and 17.4% ± 14.4%, respectively. A total of 18 LA rotors and 9 RA rotors were identified in all patients. No univariable or multivariable association was observed between global or per sector LGE extent and focal impulse and rotor modulation rotor incidence. Conclusion In this cohort of patients, there was no association between AF rotor incidence and the global or regional extent of RA and LA LGE.

This study sought to evaluate the association between contrast-enhanced multidetector computed tomography (CE-MDCT) attenuation and local epicardial conduction speed (ECS) and electrographic abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular tachycardia (VT).CE-MDCT is a widely available and fast imaging technology with high spatial resolution that is less prone to defibrillator generator-related safety issues and image artifacts. However, the association between hypoattenuation on MDCT and VT substrates in ARVC remains unknown.Patients with ARVC who underwent CE-MDCT followed by endocardial (n = 30) and epicardial (n = 21) electroanatomical mapping (EAM) and VT ablation were prospectively enrolled. Right ventricular (RV) mid-myocardial attenuation was calculated from 3-dimensional MDCT images and registered to EAM. Local ECS was calculated by averaging the ECS between each point and 5 adjacent points with concordant wave front direction.A total of 17,311 epicardial and 5,204 endocardial points were included. In multivariable regression analysis clustered by patient, RV myocardial attenuation was associated with epicardial bipolar voltage amplitude (2.5% decrease in amplitude per 10 HU decrease in attenuation; p 0.001), with endocardial unipolar voltage amplitude (0.9% decrease in amplitude per 10 HU decrease in attenuation; p 0.001), and with ECS (0.4% decrease in ECS per 10 HU decrease in attenuation; p = 0.001).CE-MDCT attenuation distribution is associated with regional ECS and electrographic amplitude in ARVC. Regions with low attenuation likely reflect fibro-fatty involvement in the RV and may serve as important VT substrates in patients with ARVC who are undergoing VT ablation.

PURPOSE: To compare epicardial fat in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with that in healthy subjects. MATERIALS AND METHODS: In this retrospective study, cardiac CT scans in 44 patients with ARVD/C (mean age, 39 years ± 12; 23 men) were compared with those in 45 control group participants between January 2008 and July 2015. Volumes of intrathoracic adipose tissue, mediastinal adipose tissue (MAT), and total epicardial adipose tissue (EAT) were quantified. EAT was subdivided into three regions—right ventricular (RV) EAT, left ventricular (LV) EAT, and peri-atrial EAT (atrial EAT)—and normalized to MAT for all regions. Logistic regression and receiver operating characteristic analysis were performed to evaluate the association between epicardial fat with the diagnosis of ARVD/C. RESULTS: Total EAT volume was higher in patients with ARVD/C than in healthy control group participants (median, 98 mL vs 76 mL, respectively; P = .04). Regionally, LV and RV EAT volumes were higher in patients with ARVD/C than in control group participants, most notably when indexed to MAT (median LV EAT index: 0.49 vs 0.15, respectively; median RV EAT index: 0.91 vs 0.52; P ˂ .0005 for both). The optimal cutoff for diagnosis of ARVD/C was an LV EAT index of 0.24, with a sensitivity and specificity of 91% and 71%, respectively. Atrial EAT volume and total intrathoracic adipose tissue volume were not different between groups. RV diameter showed a positive correlation with total EAT index and LV EAT index (r = 0.21, P = .05 and r = 0.33, P = .002, respectively). CONCLUSION: Higher amounts of right ventricular and left ventricular epicardial fat are found in hearts with arrhythmogenic right ventricular dysplasia/cardiomyopathy, particularly adjacent to the left ventricle, which correlates with disease severity and helps differentiate patients from healthy subjects. © RSNA, 2018 Online supplemental material is available for this article.

Coronary artery calcium is a marker of overall atherosclerotic plaque burden, corresponding to approximately 20% overall atherosclerotic plaque burden. Coronary artery calcium screening, most commonly performed using the Agatston score, has been shown to be a predictor of future cardiovascular risk independent of conventional risk scores such as the Framingham risk score. Coronary artery calcium screening is also recommended on routine nongated, noncontrast chest computed tomography scans using several ordinal and visual scoring systems.

Background: Right ventricular (RV) dysfunction is the leading cause of mortality in pulmonary arterial hypertension (PAH), and cardiac magnetic resonance (CMR) measures of RV volumes and mass are known to predict mortality in PAH. Ventricular mass index (VMI), a ratio of RV mass to left ventricular (LV) mass, predicts the presence of PAH in scleroderma. We hypothesized that VMI would predict mortality in incident PAH. Methods: 65 subjects with incident PAH underwent CMR, and VMI was calculated (RV end-diastolic mass/LV end-diastolic mass). Subjects were followed prospectively until death or censor. Associations between VMI and mortality were assessed with Kaplan-Meier (KM) analysis and Cox proportional hazard (CPH) modeling. Results: Subjects were 57±11 years of age; most were women (91%) with connective tissue disease (CTD) (72%) and NYHA functional class I/II symptoms (64%). There was 52% mortality over a median follow up of 8.3 years. KM analysis showed that VMI>0.43, the median for the cohort, was associated with increased risk of death (HR 2.2, 95% CI 1.07-4.46, p Conclusion: VMI, a CMR metric that reflects the effect of PAH on both RV and LV remodeling, most robustly predicts mortality in PAH. Supported by NIH R01HL114910 and U01HL125175