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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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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

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.

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.

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.

We assessed whether cardiac MRI (CMR) and echocardiography (echo) have significant differences measuring left ventricular (LV) wall thickness (WT) in hypertrophic cardiomyopathy (HCM) as performed in the clinical routine. Retrospectively identified, clinically diagnosed HCM patients with interventricular-septal (IVS) pattern hypertrophy who underwent CMR and echo within the same day were included. Left Ventricular WT was measured by CMR in two planes and compared to both echo and contrast echo (cecho). 72 subjects, mean age 50.7 ± 16.2 years, 68 % males. Interventricular septal WT by echo and CMR planes showed good to excellent correlation. However, measurements of the postero-lateral wall showed poor correlation. Bland-Altman plots showed greater maximal IVS WT by echo compared to CMR measurement [SAX = 1.7 mm (-5.8, 9.3); LVOT = 1.1 mm (-5.6, 7.8)]. Differences were smaller between cecho and CMR [SAX = 0.8 mm (-9.2, 10.8); LVOT = -0.2 mm (-10.0, 9.6)]. Severity of WT by quartiles showed greater differences between echo and SAX CMR WT compared to cecho. Echocardiography typically measures greater WT than CMR, with the largest differences in moderate to severe hypertrophy. Contrast echocardiography more closely approximates CMR measurements of WT. These findings have potential clinical implications for risk stratification of subjects with HCM.

The aims of this study were to: 1) use a novel method of late gadolinium enhancement (LGE) quantification that uses normalized intensity measures to confirm the association between LGE extent and atrial fibrillation (AF) recurrence following ablation; and 2) examine the presence of interaction and effect modification between LGE and AF persistence.Recurrent AF after catheter ablation has been reported to associate with the baseline extent of left atrial LGE on cardiac magnetic resonance. Traditional methods for measurement of intensity lack an objective threshold for quantification and interpatient comparisons of LGE.The cohort included 165 participants (mean age 60.0 ± 10.2 years, 77% men, 57% with persistent AF) who underwent initial AF ablation. The association of baseline LGE extent with AF recurrence was examined using multivariable Cox proportional hazards models. Multiplicative and additive interactions between AF type and LGE extent were examined.During 10.2 ± 5.7 months of follow-up, 63 patients (38.2%) experienced AF recurrence. Baseline LGE extent was independently associated with AF recurrence after adjusting for confounders (hazard ratio: 1.5 per 10% increased LGE; p 0.001). The hazard ratio for AF recurrence progressively increased as a function of LGE. The magnitude of association between LGE35% and AF recurrence was greater among patients with persistent AF (hazard ratio: 6.5 [p = 0.001] vs. 3.6 [p = 0.001]); however, there was no evidence for statistical interaction.Regardless of AF persistence at baseline, participants with LGE ≤35% have favorable outcomes, whereas those with LGE35% have a higher rate of AF recurrence in the first year after ablation. These findings suggest a role for: 1) patient selection for AF ablation using LGE extent; and 2) substrate modification in addition to pulmonary vein isolation in patients with LGE extent exceeding 35% of left atrial myocardium.

Symptomatic left atrial (LA) flutter (LAFL) is common after atrial fibrillation (AF) ablation.The purpose of this study was to examine the association of baseline LA function with incident LAFL after AF ablation.The source cohort included 216 patients with cardiac magnetic resonance (CMR) before initial AF ablation between 2010 and 2013. Patients who underwent cryoballoon or laser ablation, patients with AF during CMR, and those with suboptimal CMR, or missing follow-up data were excluded. Baseline LA volume and function were assessed by feature-tracking CMR analysis.The final cohort included 119 patients (mean age 58.9 ± 11 years; 76.5% men; 70.6% patients with paroxysmal AF). During a median follow-up of 421 days (interquartile range 235-751 days), 22 patients (18.5%) had incident LAFL. Baseline LA volume was similar between the 2 groups. In contrast, baseline reservoir, conduit, and contractile function of the LA were significantly impaired in patients with incident LAFL. Baseline global peak longitudinal atrial strain (PLAS)22.65% predicted incident LAFL with 86% sensitivity and 68% specificity (C statistic 0.76). In a multivariable model adjusting for age, heart failure, and LA volume, PLAS (hazard ratio 0.9 per % increase in PLAS; P = .003) and LA linear lesions (hazard ratio 2.94; P = .020) were independently associated with incident LAFL. The coexistence of PLAS22.65% and linear lesions was associated with 9-fold increased hazard of incident LAFL.Baseline LA function and linear lesions were independently associated with incident LAFL after AF ablation. Linear lesions should be limited to selected cases, especially in patients with impaired LA function.

PURPOSE: To evaluate the relationship between CT findings of diffuse lung disease and post–transcatheter aortic valve replacement (TAVR) outcomes. MATERIALS AND METHODS: Retrospective review of pre-TAVR CT scans obtained during 2012–2017 was conducted. Emphysema, reticulation, and honeycombing were separately scored using a five-point scale and applied to 10 images per examination. The fibrosis score was the sum of reticulation and honeycombing scores. Lung diseases were also assessed as dichotomous variables (zero vs nonzero scores). The two outcomes evaluated were death and the composite of death and readmission. RESULTS: The study included 373 patients with median age of 84 years (age range, 51–98 years; interquartile range, 79–88 years) and median follow-up of 333 days. Fibrosis and emphysema were present in 66 (17.7%) and 95 (25.5%) patients, respectively. Fibrosis as a dichotomous variable was independently associated with the composite of death and readmission (hazard ratio [HR], 1.54; P = .030). In those without known chronic lung disease (CLD) (HR, 3.09; P = .024) and those without airway obstruction, defined by a ratio of forced expiratory volume in 1 second to the forced vital capacity greater than or equal to 70% (HR, 1.67, P = .039), CT evidence of fibrosis was a powerful predictor of adverse events. Neither emphysema score nor emphysema as a dichotomous variable was an independent predictor of outcome. CONCLUSION: The presence of fibrosis on baseline CT scans was an independent predictor of adverse events after TAVR. In particular, fibrosis had improved predictive value in both patients without known CLD and patients without airway obstruction. Supplemental material is available for this article. © RSNA, 2020

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

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that is predominantly known to affect the ventricles. Evidence for atrial involvement remains limited. Therefore, we aimed to characterize atrial involvement in ARVC using functional cardiac magnetic resonance, define the extent of atrial size and function variation attributable to ventricular variables, and identify cardiac magnetic resonance–based predictors of atrial arrhythmias (AA) in ARVC. Methods and Results: We analyzed cine cardiac magnetic resonance images of 66 definite ARVC patients without a history of AA or severe heart failure and 24 healthy controls. Using tissue tracking, we evaluated phasic biatrial volumes, ejection fractions (EFs), peak longitudinal strain, and strain rates (SRs). The primary outcome was the occurrence of AA during 6.8 years [3.0–10.8 years] of follow-up. Compared with controls, ARVC patients had higher biatrial volumes, reduced right atrial (RA) conduit function (passive EF [RAEF passive ] and peak early-diastolic SR), reduced RA and left atrial (LA) reservoir function (peak systolic SR), and reduced RA and LA pump function (peak late-diastolic SR; P min and LAV min ), decreased LA reservoir function (total LAEF and LA peak longitudinal strain), and decreased RA conduit function (passive RAEF and RA early-diastolic SR). Conclusions: Compared with controls, patients with ARVC were found to have enlarged atria with decreased function on functional cardiac magnetic resonance examination. RA and LA parameters predict incident AA after adjusting for clinical and ventricular characteristics which suggests atrial involvement in ARVC.

Background: Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy characterized by fibrofatty replacement of right ventricular myocardium resulting in reentrant ventricular tachycardia (VT). Cardiac magnetic resonance imaging (CMR) can noninvasively measure regional abnormalities using tissue-tracking strain as well as late gadolinium enhancement (LGE). In this study, we examine arrhythmogenic substrate using regional CMR strain, LGE, and electroanatomic mapping (EAM) in arrhythmogenic right ventricular cardiomyopathy patients presenting for VT ablation. Methods and Results: Twenty-one patients underwent right ventricular endocardial EAM, whereas 17 underwent epicardial EAM, to detect dense scar (P P =0.001), and at VT culprit sites (−7.4±3.7 versus −10.1±4.1; P P Conclusions: Regional myocardial strain on cine CMR improves detection of arrhythmogenic VT substrate compared with LGE. This may enhance diagnostic accuracy of CMR in arrhythmogenic right ventricular cardiomyopathy without the need for invasive procedures and facilitate the planning of VT ablation procedures.

Atrial fibrillation (AF)-the most common arrhythmia-significantly increases the risk of stroke and heart failure. Although catheter ablation can restore normal heart rhythms, patients with persistent AF who develop atrial fibrosis often undergo multiple failed ablations, and thus increased procedural risks. Here, we present personalized computational modelling for the reliable predetermination of ablation targets, which are then used to guide the ablation procedure in patients with persistent AF and atrial fibrosis. First, we show that a computational model of the atria of patients identifies fibrotic tissue that, if ablated, will not sustain AF. Then, we report the results of integrating the target ablation sites in a clinical mapping system and testing its feasibility in ten patients with persistent AF. The computational prediction of ablation targets avoids lengthy electrical mapping and could improve the accuracy and efficacy of targeted AF ablation in patients while eliminating the need for repeat procedures.

Cardiac magnetic resonance imaging has been shown to be beneficial for identification of the ventricular tachycardia (VT) substrate before catheter ablation. Contrast-enhanced perfusion multidetector computed tomography (CEP-MDCT) is more generalizable to clinical practice, and wall thickness and regional hypoenhancement on CEP-MDCT can identify potential substrate sites, albeit with decreased specificity.The purpose of this study was to evaluate the association between wall thickness and attenuation on CEP-MDCT with local conduction velocity (CV) and electrogram abnormalities in patients with postinfarct VT.Fourteen patients with postinfarct VT underwent preprocedural CEP-MDCT followed by endocardial electroanatomic mapping (EAM) and ablation. Myocardial attenuation and wall thickness were calculated from 3-dimensional MDCT images using ADAS-VT software (Galgo Medical). EAM was registered with 3-dimensional MDCT images using the CartoMERGE module of CARTO3 software (Biosense Webster). Local CV was calculated by averaging the velocity between each point and 5 adjacent points with concordant wavefront direction.A total of 3689 points were included. In multivariable regression analysis clustered by patient, local CV was positively associated with myocardial attenuation, bipolar voltage, unipolar voltage, and wall thickness. Each 10-HU drop in full-thickness attenuation correlated to 2.6% decrease in CV (P.001) and 5.5% decrease in bipolar voltage amplitude (P.001), after adjusting for wall thickness.Myocardial attenuation distribution on CEP-MDCT is associated with regional CV and electrogram amplitude. Regions with low CV identified with low attenuation on CEP-MDCT may serve as important VT substrates in postinfarct patients.

Increased right ventricular mass and VMI, the ratio of RV mass to LV mass, are predictors of mortality in incident, treatment-naive PAH. These findings challenge the notion that RV hypertrophy is adaptive in pulmonary hypertension.http://ow.ly/qzvt30ntJze

We thank Penela et al for their comments and congratulate them for their achievements and unique work in the field. Starting in 2005, our group, as well as others, described the utility of cardiac magnetic resonance for ventricular tachycardia substrate identification in the setting of structural heart disease,1 …

Radiofrequency ablation is an effective treatment strategy for ischemic and nonischemic cardiomyopathy-related ventricular tachycardia (VT). The role of substrate-guided ablation, performed using electrogram characteristics (low amplitude, fractionated or isolated potentials) as scar surrogates, is expanding because of frequent hemodynamic instability during entrainment mapping of scar-related VT. Late gadolinium-enhancement on cardiac magnetic resonance imaging (LGE-MRI) can accurately characterize the transmural extent, location, and configuration of ventricular scar.1 Integration of LGE-MRI into electroanatomical mapping during VT ablation was shown, in preliminary studies, to be feasible and to provide accurate localization of VT substrate and reentry circuits.2–4 However, studies to date examining the impact of MRI scar integration on procedural outcomes have lacked control groups, precluding any comparisons with standard practice. We performed a study to (1) demonstrate the feasibility in clinical practice of integrating MRI-derived scar for guidance of VT ablation; (2) report on the periprocedural performance of LGE-MRI in identifying the arrhythmogenic substrate; and (3) examine the impact of MRI-guided ablation on procedural length and acute and long-term outcomes. In this prospective multicenter study, we enrolled 24 consecutive patients with ischemic (n=9) and nonischemic cardiomyopathy (n=15), referred for catheter ablation of scar-related monomorphic VT. Patients were assigned, at the discretion of the treating physician (not randomized), to undergo either MRI-derived scar–guided ablation or traditional ablation. Clinical characteristics of patients in both groups were statistically comparable with a higher tendency to use scar integration for patients with prior …

Purpose Right ventricular (RV) function is best represented by the ratio of RV contractility to pulmonary arterial (PA) load (RV-PA coupling). Gold standard measurement of RV-PA coupling, using multi-beat pressure-volume (P-V) loop to obtain the ratio of end-systolic elastance (Ees) to effective arterial elastance (Ea), is costly and invasive. Therefore, we sought to identify clinical surrogates of reduced Ees/Ea and RV-PA uncoupling. Methods We prospectively studied 35 patients suspected to have pulmonary hypertension (PH) with same day echocardiography, cardiac magnetic resonance imaging (CMR), right heart catheterization, and supine bicycle submaximal cardiopulmonary exercise testing. Multi-beat RV P-V loop measurements were also performed. Ees/Ea Results Of 35 patients, 26 had resting PH while 9 did not (20/26 had group I PH). Average age was 57±13 years; 30 were women. Compared with baseline Ees/Ea ≥ 1.0, Ees/Ea Conclusion Readily attainable clinical variables correspond to the gold standard Ees/Ea. We identified a novel parameter, exercise change in ΔESV/ΔESP, a marker of RV reserve, which had the best ability to predict RV-PA uncoupling. Future studies will investigate RV ΔESV/ΔESP and other markers of RV reserve.

BACKGROUND: Analysis of regional wall motion of the right ventricle (RV) is primarily qualitative with large interobserver variation in clinical practice. Thus, the purpose of this study was to use feature tracking to analyze regional wall motion abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: We enrolled 110 subjects (39 overt ARVC [mutation+/phenotype+] (35.5%), 40 preclinical ARVC [mutation+/phenotype-] (36.3%), and 31 control subjects (28.2%)). Cine steady state free precession cardiac MR was performed with temporal resolution ≤40 ms in the horizontal long axis (HLA), axial, and short axis directions. Regional strain was analyzed using feature tracking software and reproducibility was assessed by means of intraclass correlation coefficient. Dunnett's test was used in univariate analysis for comparisons to control subjects; cumulative odds logistic regression was used for minimally and fully adjusted multivariate models. RESULTS: Strain was significantly impaired in overt ARVC compared with control subjects both globally (P < 0.01) and regionally (all segments of HLA view, P < 0.01). In the HLA view, regional reproducibility was excellent within (intraclass correlation coefficient [ICC] = 0.81) and moderate between (ICC = 0.62) observers. Using a threshold of -31% subtricuspid strain in the HLA view, the sensitivity and specificity for overt ARVC were 75.0% and 78.2%, respectively. In multivariable analysis involving all three groups, subtricuspid strain less than -31% (beta = 1.38; P = 0.014) and RV end diastolic volume index (beta = 0.06; P = 0.001) were significant predictors of disease presence. CONCLUSION: RV strain can be reproducibly assessed with MR feature tracking, and regional strain is abnormal in overt ARVC compared with control subjects.

We tested the hypothesis that bidimensional measurements of right ventricular (RV) function obtained by cardiac magnetic resonance imaging (CMR) in patients with pulmonary arterial hypertension (PAH) are faster than volumetric measures and highly reproducible, with comparable ability to predict patient survival. CMR-derived tricuspid annular plane systolic excursion (TAPSE), RV fractional shortening (RVFS), RV fractional area change (RVFAC), standard functional and volumetric measures, and ventricular mass index (VMI) were compared with right heart catheterization data. CMR analysis time was recorded. Receiver operating characteristic curves, Kaplan-Meier, Cox proportional hazard (CPH), and Bland-Altman test were used for analysis. Forty-nine subjects with PAH and 18 control subjects were included. TAPSE, RVFS, RVFAC, RV ejection fraction, and VMI correlated significantly with pulmonary vascular resistance and mean pulmonary artery pressure (all P < 0.05). Patients were followed up for a mean (± standard deviation) of 2.5 ± 1.6 years. Kaplan-Meier curves showed that death was strongly associated with TAPSE

Rationale and Objectives To analyze the statistical biases in the studies used to derive cardiac magnetic resonance–based major and minor criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). Materials and Methods ARVC is a rare disorder of the heart that can lead to sudden death in young adults. Cardiac magnetic resonance imaging (CMR) plays a role in the diagnosis by contributing to the criteria set by experts. The original criteria emphasized qualitative analysis of CMR. The criteria were modified in 2010 to provide quantitative cutoffs. Results We apply the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool for systematic review of diagnostic accuracy to the studies cited in the guidelines written in 1994 and revised in 2010. We use the signaling questions in QUADAS-2 to identify different types of statistical bias. Conclusions The studies have understandable biases that affect the sensitivity and specificity of CMR in the diagnosis of ARVC, as well as the truth of the disease state. There is potential to overdiagnose ARVC particularly in low prevalence populations.