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Numerous studies have demonstrated that endostatin (ES), a potent angiostatic peptide derived from collagen type XVIII alpha 1 chain and encoded by COL18A1, is elevated in pulmonary arterial hypertension (PAH). Importantly, elevated ES has consistently been associated with altered hemodynamics, poor functional status, and adverse outcomes in adult and pediatric PAH. This study used serum samples from patients with Group I PAH and plasma and tissue samples derived from the Sugen/Chronic hypoxic (SuHx) rat pulmonary hypertension (PH) model to define associations between COL18A1/ES and disease development, including hemodynamics, right ventricular (RV) remodeling, and RV dysfunction. Using cardiac magnetic resonance (CMR) imaging and advanced hemodynamic assessments with pressure-volume (PV) loops in patients with PAH to assess RV-pulmonary arterial (PA) coupling, we observed a strong relationship between circulating ES levels and metrics of RV structure and function. Specifically, RV mass and the ventricular mass index (VMI) were positively associated with ES while RV ejection fraction and RV-PA coupling were inversely associated with ES levels. Our animal data demonstrates that the development of PH is associated with increased COL18A1/ES in the heart as well as the lungs. Disease-associated increases in COL18A1 mRNA and protein were most pronounced in the RV compared to the left ventricle (LV) and lung. COL18A1 expression in the RV was strongly associated with disease-associated changes in RV mass, fibrosis, and myocardial capillary density. These findings indicate that COL18A1/ES increase early in disease development in the RV and implicate COL18A1/ES in pathologic RV dysfunction in PAH.
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BACKGROUND: Pulmonary arterial hypertension (PAH) is characterised by poor exercise tolerance. The contribution of right ventricular (RV) diastolic function to the augmentation of cardiac output during exercise is not known. This study leverages pressure-volume (P-V) loop analysis to characterise the impact of RV diastology on poor flow augmentation during exercise in PAH. METHODS: RV P-V loops were measured in 41 PAH patients at rest and during supine bike exercise. Patients were stratified by median change in cardiac index (CI) during exercise into two groups: high and low CI reserve. Indices of diastolic function (end-diastolic elastance (E ed)) and ventricular interdependence (left ventricular transmural pressure (LVTMP)) were compared at matched exercise stages. RESULTS: Compared to patients with high CI reserve, those with low reserve exhibited lower exercise stroke volume (36 versus 49â mL·m-2; p=0.0001), with higher associated exercise afterload (effective arterial elastance (E a) 1.76 versus 0.90â mmHg·mL-1; p<0.0001), RV stiffness (E ed 0.68 versus 0.26â mmHg·mL-1; p=0.003) and right-sided pressures (right atrial pressure 14 versus 8â mmHg; p=0.002). Higher right-sided pressures led to significantly lower LV filling among the low CI reserve subjects (LVTMP -4.6 versus 3.2â mmHg; p=0.0001). Interestingly, low exercise flow reserve correlated significantly with high afterload and RV stiffness, but not with RV contractility nor RV-PA coupling. CONCLUSIONS: Patients with poor exercise CI reserve exhibit poor exercise RV afterload, stiffness and right-sided filling pressures that depress LV filling and stroke work. High afterload and RV stiffness were the best correlates to low flow reserve in PAH. Exercise unmasked significant pathophysiological PAH differences unapparent at rest.
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Gasto Cardíaco , Hipertensión Arterial Pulmonar , Humanos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Hipertensión Arterial Pulmonar/fisiopatología , Disfunción Ventricular Derecha/fisiopatología , Tolerancia al Ejercicio , Función Ventricular Derecha , Prueba de Esfuerzo , Volumen Sistólico , Anciano , Ventrículos Cardíacos/fisiopatología , Ejercicio Físico/fisiología , DiástoleRESUMEN
BACKGROUND: While late gadolinium enhancement (LGE) is proposed as a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC), the potential of LGE to distinguish ARVC from differentials remains unknown. We aimed to assess the diagnostic value of LGE for ARVC diagnosis. METHODS: We included 132 subjects (60% male, 47 ± 11 years) who had undergone cardiac magnetic resonance imaging with LGE assessment for ARVC or ARVC differentials. ARVC was diagnosed as per 2010 Task Force Criteria (n = 55). ARVC differentials consisted of familial/genetic dilated cardiomyopathy (n = 25), myocarditis (n = 13), sarcoidosis (n = 20), and amyloidosis (n = 19). The diagnosis of all differentials was based on the most current standard of reference. The presence of LGE was evaluated using a 7-segment right ventricle (RV) and 17-segment left ventricle (LV) model. Subsequently, we assessed LGE patterns for every patient individually for fulfilling LV- and/or RV-LGE per Padua criteria, independent of their clinical diagnosis (i.e. phenotype). Diagnostic values were analyzed using sensitivity and specificity for any RV-LGE, any LV-LGE, RV-LGE per Padua criteria, and prevalence graphs for LV-LGE per Padua criteria. The optimal integration of LGE for ARVC diagnosis was determined using classification and regression tree analysis. RESULTS: One-third (38%) of ARVC patients had RV-LGE, while half (51%) had LV-LGE. RV-LGE was less frequently observed in ARVC vs non-ARVC patients (38% vs 58%, p = 0.034) leading to a poor discriminatory potential (any RV-LGE: sensitivity 38%, specificity 42%; RV-LGE per Padua criteria: sensitivity 36%, specificity 44%). Compared to ARVC patients, non-ARVC patients more often had LV-LGE (91% vs 51%, p < 0.001) which was also more globally distributed (median 9 [interquartile range (IQR): 3-13] vs 0 [IQR: 0-3] segments, p < 0.001). The absence of anteroseptal and absence of extensive (≥5 segments) mid-myocardial LV-LGE, and absence of moderate (≥2 segments) mid-myocardial LV-LGE predicted ARVC with good diagnostic performance (sensitivity 93%, specificity 78%). CONCLUSION: LGE is often present in ARVC differentials and may lead to false positive diagnoses when used without knowledge of LGE patterns. Moderate RV-LGE without anteroseptal and mid-myocardial LV-LGE is typically observed in ARVC.
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BACKGROUND: Pulmonary hypertension (PH) is a leading cause of death in patients with systemic sclerosis (SSc). An important component of SSc patient management is early detection and treatment of PH. Recently the threshold for the diagnosis of PH has been lowered to a mean pulmonary artery pressure (mPAP) threshold of > 20 mmHg on right heart catheterization (RHC). However, it is unknown if PH-specific therapy is beneficial in SSc patients with mildly elevated pressure (SSc-MEP, mPAP 21-24 mmHg). METHODS: The SEPVADIS trial is a randomized, double-blind, placebo-controlled phase 2 trial of sildenafil in SSc-MEP patients with a target enrollment of 30 patients from two academic sites in the United States. The primary outcome is change in six-minute walk distance after 16 weeks of treatment. Secondary endpoints include change in pulmonary arterial compliance by RHC and right ventricular function by cardiac magnetic resonance imaging at 16 weeks. Echocardiography, serum N-terminal probrain natriuretic peptide, and health-related quality of life is being measured at 16 and 52 weeks. DISCUSSION: The SEPVADIS trial will be the first randomized study of sildenafil in SSc-MEP patients. The results of this trial will be used to inform a phase 3 study to investigate the efficacy of treating patients with mild elevations in mPAP. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT04797286.
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Hipertensión Pulmonar , Calidad de Vida , Esclerodermia Sistémica , Citrato de Sildenafil , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cateterismo Cardíaco , Método Doble Ciego , Ecocardiografía , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Arteria Pulmonar , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/tratamiento farmacológico , Citrato de Sildenafil/uso terapéutico , Resultado del Tratamiento , Vasodilatadores/uso terapéutico , Prueba de Paso , Ensayos Clínicos Controlados Aleatorios como Asunto , Ensayos Clínicos Fase II como AsuntoRESUMEN
Personalized, image-based computational heart modelling is a powerful technology that can be used to improve patient-specific arrhythmia risk stratification and ventricular tachycardia (VT) ablation targeting. However, most state-of-the-art methods still require manual interactions by expert users. The goal of this study is to evaluate the feasibility of an automated, deep learning-based workflow for reconstructing personalized computational electrophysiological heart models to guide patient-specific treatment of VT. Contrast-enhanced computed tomography (CE-CT) images with expert ventricular myocardium segmentations were acquired from 111 patients across five cohorts from three different institutions. A deep convolutional neural network (CNN) for segmenting left ventricular myocardium from CE-CT was developed, trained and evaluated. From both CNN-based and expert segmentations in a subset of patients, personalized electrophysiological heart models were reconstructed and rapid pacing was used to induce VTs. CNN-based and expert segmentations were more concordant in the middle myocardium than in the heart's base or apex. Wavefront propagation during pacing was similar between CNN-based and original heart models. Between most sets of heart models, VT inducibility was the same, the number of induced VTs was strongly correlated, and VT circuits co-localized. Our results demonstrate that personalized computational heart models reconstructed from deep learning-based segmentations even with a small training set size can predict similar VT inducibility and circuit locations as those from expertly-derived heart models. Hence, a user-independent, automated framework for simulating arrhythmias in personalized heart models could feasibly be used in clinical settings to aid VT risk stratification and guide VT ablation therapy. KEY POINTS: Personalized electrophysiological heart modelling can aid in patient-specific ventricular tachycardia (VT) risk stratification and VT ablation targeting. Current state-of-the-art, image-based heart models for VT prediction require expert-dependent, manual interactions that may not be accessible across clinical settings. In this study, we develop an automated, deep learning-based workflow for reconstructing personalized heart models capable of simulating arrhythmias and compare its predictions with that of expert-generated heart models. The number and location of VTs was similar between heart models generated from the deep learning-based workflow and expert-generated heart models. These results demonstrate the feasibility of using an automated computational heart modelling workflow to aid in VT therapeutics and has implications for generalizing personalized computational heart technology to a broad range of clinical centres.
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AIMS: Post-infarct myocardium contains viable corridors traversing scar or lipomatous metaplasia (LM). Ventricular tachycardia (VT) circuitry has been separately reported to associate with corridors that traverse LM and with repolarization heterogeneity. We examined the association of corridor activation recovery interval (ARI) and ARI dispersion with surrounding tissue type. METHODS AND RESULTS: The cohort included 33 post-infarct patients from the prospective Intra-Myocardial Fat Deposition and Ventricular Tachycardia in Cardiomyopathy (INFINITY) study. We co-registered scar and corridors from late gadolinium enhanced magnetic resonance, and LM from computed tomography with intracardiac electrogram locations. Activation recovery interval was calculated during sinus or ventricular pacing, as the time interval from the minimum derivative within the QRS to the maximum derivative within the T-wave on unipolar electrograms. Regional ARI dispersion was defined as the standard deviation (SD) of ARI per AHA segment (ARISD). Lipomatous metaplasia exhibited higher ARI than scar [325 (interquartile range 270-392) vs. 313 (255-374), P < 0.001]. Corridors critical to VT re-entry were more likely to traverse through or near LM and displayed prolonged ARI compared with non-critical corridors [355 (319-397) vs. 302 (279-333) ms, P < 0.001]. ARISD was more closely associated with LM than with scar (likelihood ratio χ2 50 vs. 12, and 4.2-unit vs. 0.9-unit increase in 0.01*Log(ARISD) per 1 cm2 increase per AHA segment). Additionally, LM and scar exhibited interaction (P < 0.001) in their association with ARISD. CONCLUSION: Lipomatous metaplasia is closely associated with prolonged local action potential duration of corridors and ARI dispersion, which may facilitate the propensity of VT circuit re-entry.
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Cardiomiopatías , Infarto del Miocardio , Taquicardia Ventricular , Humanos , Cicatriz/diagnóstico por imagen , Cicatriz/complicaciones , Estudios Prospectivos , Taquicardia Ventricular/etiología , Taquicardia Ventricular/complicaciones , Arritmias Cardíacas/complicaciones , Infarto del Miocardio/complicaciones , Infarto del Miocardio/diagnósticoRESUMEN
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 20.3% reduction of ICD placements with the same proportion of protected patients (P < 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).
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Displasia Ventricular Derecha Arritmogénica , Desfibriladores Implantables , Taquicardia Ventricular , Arritmias Cardíacas/etiología , Arritmias Cardíacas/terapia , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/terapia , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Femenino , Humanos , Lactante , Masculino , Factores de Riesgo , Taquicardia Ventricular/etiología , Taquicardia Ventricular/terapiaRESUMEN
OBJECTIVES: To evaluate the effectiveness of a novel artificial intelligence (AI) algorithm for fully automated measurement of left atrial (LA) volumes and function using cardiac CT in patients with atrial fibrillation. METHODS: We included 79 patients (mean age 63 ± 12 years; 35 with atrial fibrillation (AF) and 44 controls) between 2017 and 2020 in this retrospective study. Images were analyzed by a trained AI algorithm and an expert radiologist. Left atrial volumes were obtained at cardiac end-systole, end-diastole, and pre-atrial contraction, which were then used to obtain LA function indices. Intraclass correlation coefficient (ICC) analysis of the LA volumes and function parameters was performed and receiver operating characteristic (ROC) curve analysis was used to compare the ability to detect AF patients. RESULTS: The AI was significantly faster than manual measurement of LA volumes (4 s vs 10.8 min, respectively). Agreement between the manual and automated methods was good to excellent overall, and there was stronger agreement in AF patients (all ICCs ≥ 0.877; p < 0.001) than controls (all ICCs ≥ 0.799; p < 0.001). The AI comparably estimated LA volumes in AF patients (all within 1.3 mL of the manual measurement), but overestimated volumes by clinically negligible amounts in controls (all by ≤ 4.2 mL). The AI's ability to distinguish AF patients from controls using the LA volume index was similar to the expert's (AUC 0.81 vs 0.82, respectively; p = 0.62). CONCLUSION: The novel AI algorithm efficiently performed fully automated multiphasic CT-based quantification of left atrial volume and function with similar accuracy as compared to manual quantification. Novel CT-based AI algorithm efficiently quantifies left atrial volumes and function with similar accuracy as manual quantification in controls and atrial fibrillation patients. KEY POINTS: ⢠There was good-to-excellent agreement between manual and automated methods for left atrial volume quantification. ⢠The AI comparably estimated LA volumes in AF patients, but overestimated volumes by clinically negligible amounts in controls. ⢠The AI's ability to distinguish AF patients from controls was similar to the manual methods.
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Fibrilación Atrial , Anciano , Inteligencia Artificial , Fibrilación Atrial/diagnóstico por imagen , Atrios Cardíacos/diagnóstico por imagen , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodosRESUMEN
AIMS: Desmoplakin (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 RESULTS: We 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 <35% and right ventricular dysfunction were prognostic for sustained ventricular arrhythmia while proband status and myocardial injury were prognostic for heart failure (all P < 0.05). The sensitivity of the arrhythmogenic right ventricular cardiomyopathy Task Force Criteria in diagnosing left dominant disease was 0.73; 5/22 (23%) of patients with sustained ventricular arrhythmias did not meet these criteria. CONCLUSION: DSP 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.
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Displasia Ventricular Derecha Arritmogénica , Cardiomiopatías , Adulto , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/epidemiología , Displasia Ventricular Derecha Arritmogénica/genética , Cardiomiopatías/diagnóstico , Cardiomiopatías/epidemiología , Desmoplaquinas/genética , Femenino , Humanos , Masculino , Medición de Riesgo , Volumen Sistólico , Función Ventricular IzquierdaRESUMEN
The purpose of this study is to demonstrate the appearance of traumatic aortic injuries with the novel 3-dimensional (3D) computed tomography (CT) visualization technique known as cinematic rendering (CR). CR uses a novel lighting model to create photorealistic images with excellent anatomic detail for improved depiction of the extent of traumatic aortic injuries. Four patients with acute traumatic aortic injury identified on thoracic CT angiography were analyzed by creating standard 3D volume-rendered reconstructions and CR images on an independent 3D workstation. In this series of four patients, we present the typical patterns of aortic injury imaging findings and complications associated with traumatic aortic injury, with an emphasis on the utilization of the novel 3D technique of CR. CR can provide realistic imaging of the thoracic aortic contour with excellent spatial details. This methodology allows for an accurate assessment of aortic injury and optimal preoperative planning in patients with traumatic thoracic aortic injury.
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Interpretación de Imagen Radiográfica Asistida por Computador , Lesiones del Sistema Vascular , Humanos , Interpretación de Imagen Radiográfica Asistida por Computador/métodos , Imagenología Tridimensional/métodos , Tomografía Computarizada por Rayos X/métodos , Angiografía por Tomografía Computarizada/métodosRESUMEN
BACKGROUND: Left 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. METHODS: CMR 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) < 50%. Ventricular volumes and function, regional wall motion abnormalities, and the presence of ventricular fat or fibrosis were recorded. Findings on CMR were correlated with arrhythmic outcomes. RESULTS: Of 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-PKP2 genetic 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. CONCLUSION: Among 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.
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Displasia Ventricular Derecha Arritmogénica , Arritmias Cardíacas/diagnóstico por imagen , Arritmias Cardíacas/epidemiología , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/epidemiología , Displasia Ventricular Derecha Arritmogénica/genética , Medios de Contraste , Gadolinio , Humanos , Valor Predictivo de las Pruebas , PrevalenciaRESUMEN
The purpose of this study is to demonstrate the utility of cinematic rendering (CR) techniques for imaging of patients who have undergone hybrid repair of thoracoabdominal aortic aneurysms that are difficult to assess given anatomic complexity, particularly in the emergency setting. In this pictorial essay, we will explain why CR techniques are uniquely suited to improving anatomic visualization in patients with complex postoperative vascular anatomy. Verification of vessel patency is critical to optimal care of these patients in any setting, particularly in the emergency patient when clinical history may be lacking. Cinematic rendering (CR) is a recently developed CT post-processing technique for creating photorealistic anatomic visualization. Hybrid abdominal aortic aneurysm repairs involve the use of multiple tortuous and overlapping grafts to preserve blood flow to visceral arteries, which are difficult to adequately evaluate using standard orthogonal planes and with traditional VR techniques. Several examples of complex aortic repairs show the utility of CR for improved visualization of these structures. CR improves upon standard 3D volumetric techniques through improved depiction of the spatial relationships of anatomic structures in 3D space, permitting near life-like visualization that allows the imager to simplify the visualization of highly complex anatomy.
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Aneurisma de la Aorta Abdominal , Aneurisma de la Aorta Torácica , Procedimientos Endovasculares , Aneurisma de la Aorta Abdominal/diagnóstico por imagen , Aneurisma de la Aorta Abdominal/cirugía , Angiografía por Tomografía Computarizada , Humanos , Imagenología Tridimensional , Stents , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Procedimientos Quirúrgicos VascularesRESUMEN
BACKGROUND: Patients who have pacemakers or defibrillators are often denied the opportunity to undergo magnetic resonance imaging (MRI) because of safety concerns, unless the devices meet certain criteria specified by the Food and Drug Administration (termed "MRI-conditional" devices). METHODS: We performed a prospective, nonrandomized study to assess the safety of MRI at a magnetic field strength of 1.5 Tesla in 1509 patients who had a pacemaker (58%) or an implantable cardioverter-defibrillator (42%) that was not considered to be MRI-conditional (termed a "legacy" device). Overall, the patients underwent 2103 thoracic and nonthoracic MRI examinations that were deemed to be clinically necessary. The pacing mode was changed to asynchronous mode for pacing-dependent patients and to demand mode for other patients. Tachyarrhythmia functions were disabled. Outcome assessments included adverse events and changes in the variables that indicate lead and generator function and interaction with surrounding tissue (device parameters). RESULTS: No long-term clinically significant adverse events were reported. In nine MRI examinations (0.4%; 95% confidence interval, 0.2 to 0.7), the patient's device reset to a backup mode. The reset was transient in eight of the nine examinations. In one case, a pacemaker with less than 1 month left of battery life reset to ventricular inhibited pacing and could not be reprogrammed; the device was subsequently replaced. The most common notable change in device parameters (>50% change from baseline) immediately after MRI was a decrease in P-wave amplitude, which occurred in 1% of the patients. At long-term follow-up (results of which were available for 63% of the patients), the most common notable changes from baseline were decreases in P-wave amplitude (in 4% of the patients), increases in atrial capture threshold (4%), increases in right ventricular capture threshold (4%), and increases in left ventricular capture threshold (3%). The observed changes in lead parameters were not clinically significant and did not require device revision or reprogramming. CONCLUSIONS: We evaluated the safety of MRI, performed with the use of a prespecified safety protocol, in 1509 patients who had a legacy pacemaker or a legacy implantable cardioverter-defibrillator system. No long-term clinically significant adverse events were reported. (Funded by Johns Hopkins University and the National Institutes of Health; ClinicalTrials.gov number, NCT01130896 .).
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Desfibriladores Implantables , Seguridad de Equipos , Imagen por Resonancia Magnética/efectos adversos , Marcapaso Artificial , Anciano , Suministros de Energía Eléctrica , Falla de Equipo , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios ProspectivosRESUMEN
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 (ß = -0.008 V, P < .001). Additionally, right ventricular (RV) lead length was associated with decreased RV sensing (ß = -0.012 mV, P = .05) and reduced RV capture threshold (ß = -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.
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Desfibriladores Implantables , Imagen por Resonancia Magnética/métodos , Marcapaso Artificial , Seguridad del Paciente , Anciano , Protocolos Clínicos , Seguridad de Equipos , Femenino , Humanos , Masculino , Estudios Prospectivos , Ondas de RadioRESUMEN
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.
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Cardiomiopatía Hipertrófica/complicaciones , Insuficiencia Cardíaca/etiología , Factores Sexuales , Factores de Edad , Fibrilación Atrial/etiología , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Cardiomiopatía Hipertrófica/patología , Cardiomiopatía Hipertrófica/fisiopatología , Estudios de Casos y Controles , Medios de Contraste , Circulación Coronaria , Ecocardiografía , Tolerancia al Ejercicio , Femenino , Gadolinio , Pruebas de Función Cardíaca , Tabiques Cardíacos/cirugía , Humanos , Hipertrofia Ventricular Izquierda/complicaciones , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/patología , Hipertrofia Ventricular Izquierda/fisiopatología , Imagen por Resonancia Magnética , Masculino , Análisis por Apareamiento , Persona de Mediana Edad , Fenotipo , Estudios Retrospectivos , Volumen Sistólico , Taquicardia Ventricular/etiología , Resultado del Tratamiento , Remodelación VentricularRESUMEN
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
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Cardiomiopatía Hipertrófica/diagnóstico por imagen , Ecocardiografía/métodos , Prueba de Esfuerzo/métodos , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Tomografía de Emisión de Positrones/métodos , Vasodilatadores/efectos adversos , Adulto , Anciano , Cardiomiopatía Hipertrófica/epidemiología , Ejercicio Físico , Femenino , Genotipo , Ventrículos Cardíacos , Humanos , Hipertrofia Ventricular Izquierda/complicaciones , Incidencia , Masculino , Microcirculación , Persona de Mediana Edad , Enfermedades Musculares , Isquemia Miocárdica , Sistema de Registros , Estudios RetrospectivosRESUMEN
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.
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Displasia Ventricular Derecha Arritmogénica , Taquicardia Ventricular , Adulto , Arritmias Cardíacas , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Muerte Súbita Cardíaca , Electrocardiografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
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 < 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).
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Arritmias Cardíacas , Displasia Ventricular Derecha Arritmogénica , Modelos Estadísticos , Adulto , Arritmias Cardíacas/epidemiología , Arritmias Cardíacas/etiología , Arritmias Cardíacas/mortalidad , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/epidemiología , Displasia Ventricular Derecha Arritmogénica/mortalidad , Muerte Súbita Cardíaca/epidemiología , Desfibriladores Implantables , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Adulto JovenRESUMEN
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 < .0001) walls. Patients with VA (n = 12), compared with those without, had higher %scar in the basal inferoseptum (20.4 ± 30.8 vs 8.3 ± 13.4; P = .03). During mean follow-up of 4.4 ± 3.3 years, four patients died or underwent HT; eight had VA; and zero developed AVB. Multiple regional abnormalities were associated with the combined endpoint, including scar in the anteroseptal wall (HR 1.06 [1.02-1.09] per 1%scar increase, P = .002). The ML algorithm predicted the combined endpoint with a C-statistic of 0.91. CONCLUSION: Regional CMR abnormalities are associated with AE in patients with CS.
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Bloqueo Atrioventricular/etiología , Cardiomiopatías/diagnóstico por imagen , Muerte Súbita Cardíaca/etiología , Imagen por Resonancia Cinemagnética , Sarcoidosis/diagnóstico por imagen , Taquicardia Ventricular/etiología , Fibrilación Ventricular/etiología , Función Ventricular Izquierda , Adulto , Anciano , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/mortalidad , Bloqueo Atrioventricular/fisiopatología , Cardiomiopatías/complicaciones , Cardiomiopatías/mortalidad , Cardiomiopatías/fisiopatología , Medios de Contraste/administración & dosificación , Progresión de la Enfermedad , Femenino , Fibrosis , Gadolinio DTPA/administración & dosificación , Trasplante de Corazón , Humanos , Aprendizaje Automático , Masculino , Persona de Mediana Edad , Miocardio/patología , Compuestos Organometálicos/administración & dosificación , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Sarcoidosis/complicaciones , Sarcoidosis/mortalidad , Sarcoidosis/fisiopatología , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidad , Taquicardia Ventricular/fisiopatología , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/mortalidad , Fibrilación Ventricular/fisiopatologíaRESUMEN
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 Ë .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.