Case Report: Electroanatomic mapping as an early diagnostic tool in arrhythmogenic cardiomyopathy.

Background: Abnormal substrate on invasive electroanatomic mapping (EAM) correlates with areas of myocardial thinning and fibrofatty replacement in Arrhythmogenic Cardiomyopathy (ACM). However, EAM parameters are absent from all sets of diagnostic criteria for ACM. Case summary: A 41-year-old female with no significant family history was referred for evaluation of frequent premature ventricular complexes (PVCs). Twelve-lead ECG showed diffuse low-voltage QRS complexes. Holter monitor showed 28% burden of PVCs with various morphologies consistent with right ventricular (RV) inflow and outflow tract exits. Transthoracic echocardiogram revealed normal biventricular function and dimension. Cardiac magnetic resonance revealed a mildly increased indexed RV end-diastolic volume with normal RV systolic function and no dyssynchrony, akinesia, dyskinesia, or late gadolinium enhancement. Electrophysiologic study demonstrated 2 predominant PVC morphologies that were targeted with ablation, in addition to extensive abnormality with low-voltage and fractionated electrograms in the peri-tricuspid and right ventricular outflow tract free wall regions with septal sparing, suggestive of RV cardiomyopathy. Subsequent genetic testing revealed two pathogenic variants in the desmoplakin and plakophilin-2 genes, confirming the diagnosis of ACM. Conclusion: Advanced RV electropathy can precede RV structural changes in ACM. Invasive evaluation of the electroanatomic substrate should be considered in select cases even when imaging findings are not diagnostic. Future iterations of ACM guidelines may need to consider EAM substrate as one of the diagnostic criteria. A high index of diagnostic suspicion for ACM should be maintained in patients with multifocal RV ectopy.

Predicting postoperative systolic dysfunction in mitral regurgitation: CT vs. echocardiography.

Introduction: Volume overload from mitral regurgitation can result in left ventricular systolic dysfunction. To prevent this, it is essential to operate before irreversible dysfunction occurs, but the optimal timing of intervention remains unclear. Current echocardiographic guidelines are based on 2D linear measurement thresholds only. We compared volumetric CT-based and 2D echocardiographic indices of LV size and function as predictors of post-operative systolic dysfunction following mitral repair. Methods: We retrospectively identified patients with primary mitral valve regurgitation who underwent repair between 2005 and 2021. Several indices of LV size and function measured on preoperative cardiac CT were compared with 2D echocardiography in predicting post-operative LV systolic dysfunction (LVEFecho <50%). Area under the curve (AUC) was the primary metric of predictive performance. Results: A total of 243 patients were included (mean age 57 ± 12 years; 65 females). The most effective CT-based predictors of post-operative LV systolic dysfunction were ejection fraction [LVEFCT; AUC 0.84 (95% CI: 0.77-0.92)] and LV end systolic volume indexed to body surface area [LVESViCT; AUC 0.88 (0.82-0.95)]. The best echocardiographic predictors were LVEFecho [AUC 0.70 (0.58-0.82)] and LVESDecho [AUC 0.79 (0.70-0.89)]. LVEFCT was a significantly better predictor of post-operative LV systolic dysfunction than LVEFecho (p = 0.02) and LVESViCT was a significantly better predictor than LVESDecho (p = 0.03). Ejection fraction measured by CT demonstrated significantly greater reproducibility than echocardiography. Discussion: CT-based volumetric measurements may be superior to established 2D echocardiographic parameters for predicting LV systolic dysfunction following mitral valve repair. Validation with prospective study is warranted.

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study.

Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

SCMR expert consensus statement for cardiovascular magnetic resonance of patients with a cardiac implantable electronic device.

Cardiovascular magnetic resonance (CMR) is a proven imaging modality for informing diagnosis and prognosis, guiding therapeutic decisions, and risk stratifying surgical intervention. Patients with a cardiac implantable electronic device (CIED) would be expected to derive particular benefit from CMR given high prevalence of cardiomyopathy and arrhythmia. While several guidelines have been published over the last 16 years, it is important to recognize that both the CIED and CMR technologies, as well as our knowledge in MR safety, have evolved rapidly during that period. Given increasing utilization of CIED over the past decades, there is an unmet need to establish a consensus statement that integrates latest evidence concerning MR safety and CIED and CMR technologies. While experienced centers currently perform CMR in CIED patients, broad availability of CMR in this population is lacking, partially due to limited availability of resources for programming devices and appropriate monitoring, but also related to knowledge gaps regarding the risk-benefit ratio of CMR in this growing population. To address the knowledge gaps, this SCMR Expert Consensus Statement integrates consensus guidelines, primary data, and opinions from experts across disparate fields towards the shared goal of informing evidenced-based decision-making regarding the risk-benefit ratio of CMR for patients with CIEDs.

MR Lymphangiography in Lymphatic Disorders: Clinical Applications, Institutional Experience, and Practice Development.

Lymphatic flow and anatomy can be challenging to study, owing to variable lymphatic anatomy in patients with diverse primary or secondary lymphatic pathologic conditions and the fact that lymphatic imaging is rarely performed in healthy individuals. The primary components of the lymphatic system outside the head and neck are the peripheral, retroperitoneal, mesenteric, hepatic, and pulmonary lymphatic systems and the thoracic duct. Multiple techniques have been developed for imaging components of the lymphatic system over the past century, with trade-offs in spatial, temporal, and contrast resolution; invasiveness; exposure to ionizing radiation; and the ability to obtain information on dynamic lymphatic flow. More recently, dynamic contrast-enhanced (DCE) MR lymphangiography (MRL) has emerged as a valuable tool for imaging both lymphatic flow and anatomy in a variety of congenital and acquired primary or secondary lymphatic disorders. The authors provide a brief overview of lymphatic physiology, anatomy, and imaging techniques. Next, an overview of DCE MRL and the development of an MRL practice and workflow in a hybrid interventional MRI suite incorporating cart-based in-room US is provided, with an emphasis on multidisciplinary collaboration. The spectrum of congenital and acquired lymphatic disorders encountered early in an MRL practice is provided, with emphasis on the diversity of imaging findings and how DCE MRL can aid in diagnosis and treatment of these patients. Methods such as DCE MRL for assessing the hepatic and mesenteric lymphatic systems and emerging technologies that may further expand DCE MRL use such as three-dimensional printing are introduced. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

4D Flow cardiovascular magnetic resonance consensus statement: 2023 update.

Hemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 '4D Flow CMR Consensus Statement'. We elaborate on 4D Flow CMR sequence options and imaging considerations. The document aims to assist centers starting out with 4D Flow CMR of the heart and great vessels with advice on acquisition parameters, post-processing workflows and integration into clinical practice. Furthermore, we define minimum quality assurance and validation standards for clinical centers. We also address the challenges faced in quality assurance and validation in the research setting. We also include a checklist for recommended publication standards, specifically for 4D Flow CMR. Finally, we discuss the current limitations and the future of 4D Flow CMR. This updated consensus paper will further facilitate widespread adoption of 4D Flow CMR in the clinical workflow across the globe and aid consistently high-quality publication standards.

Non-Contrast Magnetic Resonance Angiography: Techniques, Principles, and Applications.

Several non-contrast magnetic resonance angiography (MRA) techniques have been developed, providing an attractive alternative to contrast-enhanced MRA and a radiation-free alternative to computed tomography (CT) CT angiography. This review describes the physical principles, limitations, and clinical applications of bright-blood (BB) non-contrast MRA techniques. The principles of BB MRA techniques can be broadly divided into (a) flow-independent MRA, (b) blood-inflow-based MRA, (c) cardiac phase dependent, flow-based MRA, (d) velocity sensitive MRA, and (e) arterial spin-labeling MRA. The review also includes emerging multi-contrast MRA techniques that provide simultaneous BB and black-blood images for combined luminal and vessel wall evaluation.

Pathogenesis, Evaluation, and Management of Pulmonary Vein Stenosis: JACC Review Topic of the Week.

Pulmonary vein stenosis (PVS) can arise from several etiologies, including congenital, acquired, and iatrogenic sources. PVS presents insidiously, leading to significant delays in diagnosis. A high index of suspicion and dedicated noninvasive evaluation are key to diagnosis. Once diagnosed, both noninvasive and invasive evaluation may afford further insights into the relative contribution of PVS to symptoms. Treatment of underlying reversible pathologies coupled with transcatheter balloon angioplasty and stenting for persistent severe stenoses are established approaches. Ongoing refinements in diagnostic modalities, interventional approaches, postintervention monitoring, and medical therapies hold promise to further improve patient outcomes.

Emerging Approaches to Management of Left Ventricular Outflow Obstruction Risk in Transcatheter Mitral Valve Replacement.

An increasing number of patients with mitral valve disease are high risk for surgery and in need of less invasive treatments including transcatheter mitral valve replacement (TMVR). Left ventricular outflow tract (LVOT) obstruction is a predictor of poor outcome after TMVR, and its risk can be accurately predicted using cardiac computed tomography analysis. Novel treatment strategies that have shown efficacy in reducing risk of LVOT obstruction after TMVR include pre-emptive alcohol septal ablation, radiofrequency ablation, and anterior leaflet electrosurgical laceration. This review describes recent advances in the management of LVOT obstruction risk after TMVR, provides a new management algorithm, and explores forthcoming studies that will further advance the field.

Clinical Implementation of an Artificial Intelligence Algorithm for Magnetic Resonance-Derived Measurement of Total Kidney Volume.

OBJECTIVE: To evaluate the performance of an internally developed and previously validated artificial intelligence (AI) algorithm for magnetic resonance (MR)-derived total kidney volume (TKV) in autosomal dominant polycystic kidney disease (ADPKD) when implemented in clinical practice. PATIENTS AND METHODS: The study included adult patients with ADPKD seen by a nephrologist at our institution between November 2019 and January 2021 and undergoing an MR imaging examination as part of standard clinical care. Thirty-three nephrologists ordered MR imaging, requesting AI-based TKV calculation for 170 cases in these 161 unique patients. We tracked implementation and performance of the algorithm over 1 year. A radiologist and a radiology technologist reviewed all cases (N=170) for quality and accuracy. Manual editing of algorithm output occurred at radiology or radiology technologist discretion. Performance was assessed by comparing AI-based and manually edited segmentations via measures of similarity and dissimilarity to ensure expected performance. We analyzed ADPKD severity class assignment of algorithm-derived vs manually edited TKV to assess impact. RESULTS: Clinical implementation was successful. Artificial intelligence algorithm-based segmentation showed high levels of agreement and was noninferior to interobserver variability and other methods for determining TKV. Of manually edited cases (n=84), the AI-algorithm TKV output showed a small mean volume difference of -3.3%. Agreement for disease class between AI-based and manually edited segmentation was high (five cases differed). CONCLUSION: Performance of an AI algorithm in real-life clinical practice can be preserved if there is careful development and validation and if the implementation environment closely matches the development conditions.

High-Pitch Multienergy Coronary CT Angiography in Dual-Source Photon-Counting Detector CT Scanner at Low Iodinated Contrast Dose.

OBJECTIVES: The aim of this study was to evaluate the high-helical pitch, multienergy (ME) scanning mode of a clinical dual-source photon-counting detector (PCD) computed tomography (CT) and the benefit of virtual monoenergetic images (VMIs) for low-contrast-dose coronary CT angiography (CTA). MATERIALS AND METHODS: High-pitch (3.2) ME coronary CTA was performed in PCD-CT in 27 patients using low contrast dose (30 mL of iohexol 350 mg/mL) and in 26 patients at routine contrast dose (60 mL). Low-energy-threshold 120 kV images (also known as T3D images) and 50 kiloelectron volts (50 keV) and 100 kiloelectron volts (100 keV) VMIs were reconstructed using a 1024 × 1024 matrix and 0.6-mm slices. The CT numbers, noise, and contrast-to-noise ratio (CNR) were measured in the ascending aorta (AA), left main coronary artery (LMCA), and distal left anterior descending (LAD) artery. Confidence in grading luminal stenosis with calcific plaque, noncalcific plaque, and stent was evaluated by 2 independent readers on a 0-100 scale (0 the lowest), and a CAD-RADS score was assigned. Image contrast enhancement, sharpness, noise, artifacts, and overall image quality were rated using a 5-point ordinal scale (1 the lowest). RESULTS: The radiation doses (CTDI) in low- and routine-contrast cohorts were 2.5 ± 0.6 mGy and 3.1 ± 1.7 mGy, respectively ( P = 0.12). At all measured locations, the mean CT number was >300 HU in 120 kV (LMCA 382.9 ± 76.2, distal LAD 341.0 ± 53.9, AA 399.5 ± 76.1) and 50 keV images (LMCA 667.5 ± 139.9, distal LAD 578.1 ± 121.5, AA 700.8 ± 142.5) in the low-contrast cohort, with a 96% increase in CT numbers for 50 keV over 120 kV. The CT numbers were significantly higher ( P < 0.0001) in 50 keV than 120 kV and 100 keV VMI. The CNR was also significantly ( P < 0.0001) higher in 50 keV than 120 kV and 100 keV images in all vessels. Confidence in the assessment of luminal stenosis in the presence of calcific plaque was significantly higher ( P = 0.001) with the addition of 100 keV VMI (median score, 100) than using 50 keV alone (median score, 70) and 120 kV (median score, 70) for reader 1, but no significant differences were seen for reader 2 who had same median scores of 100 for all image types. The confidence in the assessment of luminal stenosis within a stent improved with the use of 100 keV images for both readers (reader 1: median scores for 50 + 100 keV = 100, 50 keV = 82.5, 120 kV = 82.5; reader 2: 50 + 100 keV = 100, 50 keV = 90, 120 kV = 90). There were no significant differences in confidence scores for assessment of luminal stenosis from noncalcific plaques for both readers. The reader-averaged qualitative scores for vascular enhancement and overall image quality were significantly higher for 50 keV VMI than for 120 kV images in both low- and routine-contrast dose cohorts. The image sharpness was nonsignificantly higher at 50 keV VMI than 120 kV images, and the artifact score was comparable for 50 keV VMI and 120 kV images. The noise was higher in 50 keV VMI than in 120 kV images. CONCLUSIONS: High-pitch ME PCD-CT mode produced diagnostic quality coronary CTA images at low radiation and iodinated contrast doses. The availability of ME VMIs significantly improved the CNR, overall image quality, and confidence in assessment of luminal stenosis in the presence of calcific plaques and stents, and resulted in change of CAD-RADS categories in 9 patients.

A Left ventricle remodeling in patients with bicuspid aortic valve.

PURPOSE: We assessed the impact of bicuspid aortic valve (BAV), aortic stenosis (AS), and regurgitation (AR) on the metrics of left ventricular (LV) remodeling, as measured by electrocardiogram (ECG), transthoracic echocardiography (TTE), and cardiac magnetic resonance (CMR). METHODS: This retrospective CMR study included 11 patients with both AS and AR (BAV-ASR), 30 with AS (BAV-AS), 28 with AR (BAV-AR), 47 with neither AS nor AR (BAV-no_AS/AR), and 40 with trileaflet aortic valve (TAV-no_AS/AR). CMR analysis included the LV end-diastolic volume index (LVEDVi), mass index (LVMi), and extracellular volume fraction (ECV). The Sokolow-Lyon and Cornell products by ECG and TTE-derived E/e' were measured. RESULTS: There were no differences in the ECG, TTE, and CMR parameters between BAV-no_AS/AR and TAV-no_AS/AR. However, the presence of aortic valve dysfunction resulted in an elevated Sokolow-Lyon product for BAV-ASR (p = 0.017) and BAV-AR (p = 0.001), as well as increased Cornell product (p = 0.04) and E/e' (p < 0.001) for BAV-AS compared with BAV-no_AS/AR. LVEDVi and LVMi were elevated in patients with BAV-ASR and BAV-AR compared with those with BAV-no_AS/AR (LVEDVi: 101 ± 29 ml/m2 and 112 ± 32 ml/m2 vs. 74 ± 15 ml/m2, p = 0.005 and p < 0.001, LVMi: 75 ± 7 g/m2 and 64 ± 14 g/m2 vs. 47 ± 9 g/m2, respectively; p < 0.001). There was no difference in ECV between the BAV and TAV-no_AS/AR subgroups. CONCLUSION: Normally functioning BAV did not result in LV remodeling. However, concomitant AV dysfunction was associated with statistically significant morphological remodeling.