Magnetic Resonance Imaging Characterization and Clinical Outcomes of Dilated and Arrhythmogenic Left Ventricular Cardiomyopathies.

BACKGROUND: Nondilated left ventricular cardiomyopathy (NDLVC) has been recently differentiated from dilated cardiomyopathy (DCM). A comprehensive characterization of these 2 entities using cardiac magnetic resonance (CMR) and genetic testing has never been performed. OBJECTIVES: This study sought to provide a thorough characterization and assess clinical outcomes in a large multicenter cohort of patients with DCM and NDLVC. METHODS: A total of 462 patients with DCM (227) or NDLVC (235) with CMR data from 4 different referral centers were retrospectively analyzed. The study endpoint was a composite of sudden cardiac death or major ventricular arrhythmias. RESULTS: In comparison to DCM, NDLVC had a higher prevalence of pathogenic or likely pathogenic variants of arrhythmogenic genes (40% vs 23%; P < 0.001), higher left ventricular (LV) systolic function (LV ejection fraction: 51% ± 12% vs 36% ± 15%; P < 0.001) and higher prevalence of free-wall late gadolinium enhancement (LGE) (27% vs 14%; P < 0.001). Conversely, DCM showed higher prevalence of pathogenic or likely pathogenic variants of nonarrhythmogenic genes (23% vs 12%; P = 0.002) and septal LGE (45% vs 32%; P = 0.004). Over a median follow-up of 81 months (Q1-Q3: 40-132 months), the study outcome occurred in 98 (21%) patients. LGE with septal location (HR: 1.929; 95% CI: 1.033-3.601; P = 0.039) was independently associated with the risk of sudden cardiac death or major ventricular arrhythmias together with LV dilatation, older age, advanced NYHA functional class, frequent ventricular ectopic activity, and nonsustained ventricular tachycardia. CONCLUSIONS: In a multicenter cohort of patients with DCM and NDLVC, septal LGE together with LV dilatation, age, advanced disease, and frequent and repetitive ventricular arrhythmias were powerful predictors of major arrhythmic events.

Guidelines for the Evaluation of Prosthetic Valve Function With Cardiovascular Imaging: A Report From the American Society of Echocardiography Developed in Collaboration With the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography.

In patients with significant cardiac valvular disease, intervention with either valve repair or valve replacement may be inevitable. Although valve repair is frequently performed, especially for mitral and tricuspid regurgitation, valve replacement remains common, particularly in adults. Diagnostic methods are often needed to assess the function of the prosthesis. Echocardiography is the first-line method for noninvasive evaluation of prosthetic valve function. The transthoracic approach is complemented with two-dimensional and three-dimensional transesophageal echocardiography for further refinement of valve morphology and function when needed. More recently, advances in computed tomography and cardiac magnetic resonance have enhanced their roles in evaluating valvular heart disease. This document offers a review of the echocardiographic techniques used and provides recommendations and general guidelines for evaluation of prosthetic valve function on the basis of the scientific literature and consensus of a panel of experts. This guideline discusses the role of advanced imaging with transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance in evaluating prosthetic valve structure, function, and regurgitation. It replaces the 2009 American Society of Echocardiography guideline on prosthetic valves and complements the 2019 guideline on the evaluation of valvular regurgitation after percutaneous valve repair or replacement.

Sustained monomorphic ventricular tachycardia as the presenting sign of Chagas' cardiomyopathy in a low prevalence setting, diagnosis and management challenges. A case report.

Approximately 300,000 people in the United States are estimated to have Chagas' disease, with cardiac manifestations including arrhythmias occurring in 20%-30% of patients. We report a patient diagnosed with Chagas' cardiomyopathy after presenting in ventricular tachycardia. This patient was asymptomatic before her presentation with recurrent episodes of ventricular tachycardia, which motivated us to screen her since she was an immigrant from an endemic Chagas region. This manuscript highlights some of the characteristic cardiac magnetic resonance imaging (MRI) and electrophysiology findings present in patients with Chagas' cardiomyopathy. We also detail the management of patients with Chagas' cardiomyopathy who have suffered from ventricular tachycardia.

Right Ventricular Dysfunction During Endurance Exercise as Determined by Pressure-Volume Analysis.

A 37-year-old athlete completed invasive endurance (90 km) bicycle exercise testing for right ventricular pressure-volume analysis. Increased right ventricular afterload caused declines in ventricular-arterial coupling and cardiac output, causing increased arteriovenous oxygen difference to maintain oxygen uptake. These findings demonstrate effects of changes in right ventricular performance on exercise capacity. (Level of Difficulty: Intermediate.).

Impact of advanced imaging techniques on preoperative planning in cardiac surgery.

BACKGROUND AND AIM OF THE STUDY: Review how advanced imaging techniques and a multidisciplinary heart team approach are used to evaluate complex cardiac structural pathology. METHODS: Single-center retrospective case series. RESULTS AND CONCLUSIONS: Cardiac computed tomography angiography in addition to transthoracic and transesophageal echocardiography impacts pre-procedural planning and procedural success.

Clinical and genetic features of arrhythmogenic cardiomyopathy: diagnosis, management and the heart failure perspective.

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an emerging new concept of a life-threatening heart muscle disorder due not only to desmosome gene mutations, but also to non-desmosome genes, such as filamin C, lamin A/C, phospholamban, transmembrane protein 43, titin, SCN5A and RNA binding motif protein 20.Multi-modality imaging along with genetic testing are important tools for risk stratification to tailor treatment to a single patient. Cardiac magnetic resonance imaging (CMR) with late gadolinium enhancement (LGE) is the gold standard for evaluating left and right ventricular structure and function, edema, and fibrosis. The identification of regional fibrosis with LGE has prognostic value. The management of ACM involves several aspects: treatment of arrhythmias and heart failure, risk stratification, implantable cardioverter-defibrillator (ICD) placement, exercise restrictions, and life-style changes. The decision for ICD placement in ACM patients is not well established and should be made weighing risks and benefits. However, the presence of specific genotypes can allow a precision medicine approach. In ACM patients with only mild left ventricular dysfunction but phospholamban, filamin C or lamin A/C mutations, an ICD is now considered a reasonable approach. AIM OF REVIEW: We sought to provide an overview of clinical and genetic feature of arrhythmogenic cardiomyopathy providing epidemiology, imaging, diagnostic and treatment information, using a systematic genetic approach.

Ischemic Heart Disease: Noninvasive Imaging Techniques and Findings.

Ischemic heart disease is a leading cause of death worldwide and comprises a large proportion of annual health care expenditure. Management of ischemic heart disease is now best guided by the physiologic significance of coronary artery stenosis. Invasive coronary angiography is the standard for diagnosing coronary artery stenosis. However, it is expensive and has risks including vascular access site complications and contrast material-induced nephropathy. Invasive coronary angiography requires fractional flow reserve (FFR) measurement to determine the physiologic significance of a coronary artery stenosis. Multiple noninvasive cardiac imaging modalities can also anatomically delineate or functionally assess for significant coronary artery stenosis, as well as detect the presence of myocardial infarction (MI). While coronary CT angiography can help assess the degree of anatomic stenosis, its inability to assess the physiologic significance of lesions limits its specificity. Physiologic significance of coronary artery stenosis can be determined by cardiac MR vasodilator or dobutamine stress imaging, CT stress perfusion imaging, FFR CT, PET myocardial perfusion imaging (MPI), SPECT MPI, and stress echocardiography. Clinically unrecognized MI, another clear indicator of physiologically significant coronary artery disease, is relatively common and is best evaluated with cardiac MRI. The authors illustrate the spectrum of imaging findings of ischemic heart disease (coronary artery disease, myocardial ischemia, and MI); highlight the advantages and disadvantages of the various noninvasive imaging methods used to assess ischemic heart disease, as illustrated by recent clinical trials; and summarize current indications and contraindications for noninvasive imaging techniques for detection of ischemic heart disease. Online supplemental material is available for this article. Published under a CC BY 4.0 license.

Practical cardiovascular imaging approach to diagnose immune checkpoint inhibitor myocarditis.

Immuno-oncology employs various therapeutic strategies that harness a patient's own immune system to fight disease and has been a promising new strategy for cancer therapy over the last decade. Immune checkpoint inhibitors (ICI), are monoclonal antibodies, that increase antitumor immunity by blocking intrinsic down-regulators of immunity, such as cytotoxic T-lymphocyte antigen 4 (CTLA-4) and programmed cell death 1 (PD-1) or its ligand, programmed cell death ligand 1 (PD-L1). Seven ICIs are currently approved by the Food and Drug Administration and have increased the overall survival for patients with various cancer subtypes. These are used either as single agents or in combination with other checkpoint inhibitors, small molecular kinase inhibitors or cytotoxic chemotherapies. There are also many other immune modifying agents including other checkpoint inhibitor antibodies that are under investigation in clinical trials.

Performance of single-energy metal artifact reduction in cardiac computed tomography: A clinical and phantom study.

BACKGROUND: To investigate the performance of a reconstruction algorithm, single-energy metal artifact reduction (SEMAR), against standard reconstruction in cardiac computed tomography (CT) studies of patients with implanted metal and in a defibrillator lead phantom. METHODS: From a retrospective, cross-sectional clinical study with institutional review board approval of 118 patients with implanted metal, 122 cardiac CT studies from November 2009 to August 2016 performed on a 320-detector row scanner with standard and SEMAR reconstructions were included. The maximum beam hardening artifact radius, artifact attenuation variation surrounding the implanted metal, and image quality on a 4-point scale (1-no/minimal artifact to 4-severe artifact) were assessed for each reconstruction. A defibrillator lead phantom study was performed at different tube potentials and currents with both reconstruction methods. Maximum beam hardening artifact radius and average artifact attenuation variation were measured. RESULTS: In the clinical study, SEMAR markedly reduced the maximum beam hardening artifact radius by 77% (standard: 14.8 mm [IQR 9.7-22.2] vs. SEMAR: 3.4 mm [IQR 2.2-7.1], p < 0.0001) and artifact attenuation variation by 51% (standard: 130.0 HU [IQR 75.9-184.4] vs. SEMAR: 64.3 HU [IQR 48.2-89.2], p < 0.0001). Image quality improved with SEMAR (standard: 3 [IQR 2-3.5] vs. SEMAR: 2 [IQR 1-2.5], p < 0.0001). The defibrillator lead phantom study confirmed these results across varying tube potentials and currents. CONCLUSIONS: SEMAR reconstruction achieved superior image quality and markedly reduced maximum beam hardening artifact radius and artifact attenuation variation compared to standard reconstruction in 122 clinical cardiac CT studies of patients with implanted metal and in a defibrillator lead phantom study.

Comparison of professional medical society guidelines for appropriate use of coronary computed tomography angiography.

BACKGROUND: Multiple appropriate use criteria (AUC) exist for the evaluation of coronary artery disease (CAD), but there is little data on the agreement between AUC from different professional medical societies. The aim of this study is to compare the appropriateness of coronary computed tomography angiography (CCTA) exams assessed using multimodality AUC from the American College of Cardiology Foundation (ACCF) versus the American College of Radiology (ACR). METHODS: In a single-center prospective cohort study from June 2014 to 2016, 1005 consecutive subjects referred for evaluation of known or suspected CAD received a contrast-enhanced CCTA. The primary outcome was the agreement of appropriateness ratings using ACCF and ACR guidelines, measured by the kappa statistic. A secondary outcome was the rate of obstructive CAD by appropriateness rating. RESULTS: Among 1005 subjects, the median (5-95th percentile) age was 59 (37-76) years with 59.0% male. The ACCF criteria classified 39.6% (n = 398) appropriate, 24.2% (n = 243) maybe appropriate, and 36.2% (n = 364) rarely appropriate. The ACR guidelines classified 72.3% (n = 727) appropriate, 2.6% (n = 26) maybe appropriate, and 25.1% (n = 252) rarely appropriate. ACCF and ACR appropriateness ratings were in agreement for 55.0% (n = 553). Overall, there was poor agreement (kappa 0.27 [95% confidence interval 0.23-0.31]). By both AUC methods, a low rate of obstructive CAD was observed in the rarely appropriate exams (ACCF 7.1% [n = 26 of 364] and ACR 13.5% [n = 34 of 252]). CONCLUSIONS: Compared to ACCF criteria, the ACR guidelines of appropriateness were broader and classified significantly more CCTA exams as appropriate. The poor agreement between appropriateness ratings from the ACCF and ACR AUC guidelines evokes implications for reimbursement and future test utilization.

Allergy and the Heart: Eosinophilic Myocarditis With Biventricular Thrombi.

A 40-year-old woman with history of atopy and peripheral eosinophilia presented with clinical signs of heart failure. Echocardiography revealed a restrictive cardiomyopathy with biventricular thrombi. Hypereosinophilic syndrome resulting in eosinophilic myocarditis (Loeffler's syndrome) was diagnosed. This case highlights the workup, diagnosis, and management of hypereosinophilic syndrome with eosinophilic myocarditis. (Level of Difficulty: Advanced.).

A novel method for evaluating regional RV function in the adult congenital heart with low-dose CT and SQUEEZ processing.

BACKGROUND: Measuring local RV function in adult congenital heart disease (ACHD) with echocardiography or MRI is challenging because of the complex geometry and existing pacing devices. Visual assessment of ventricular function via low-dose cardiac CT has been recently performed. This pilot study assessed whether low-dose 4D cine CT combined with automatic measurement of regional shortening could quantify right-ventricular function in ACHD patients. METHODS: Seven patients with Tetralogy of Fallot either contraindicated for MRI or assessed for coronary artery disease and seven non-congenital patients were imaged with ECG-gated cardiac CT utilizing a 320-detector row scanner. Right ventricular global function and regional shortening were quantified. RESULTS: Non-congenital patients were imaged with 2.9±2.1mSv and 395±359 HU blood-myocardium contrast. The ACHD patients were imaged with 2.1±1.3mSv and 726±296 HU contrast. Right ventricles of the ACHD patients had higher end-diastolic volume (297±107mL vs 123±34mL, p=0.001), lower ejection fraction (32.0±4.9% vs 45.0±6.0%, p=0.001), and higher dyskinetic fraction (10.9±3.7% vs 2.6±2.8%, p<0.001) relative to the non-congenital controls. CONCLUSIONS: In this initial pilot study, right ventricular global and regional systolic function were measured using low-dose cine CT with SQUEEZ quantification in non-congenital patients as well as ACHD patients with Tetralogy of Fallot. Unique regional features of RV dyskinesia were identified in the ACHD patients which could yield a more precise quantification of RV function.

Feasibility of low radiation dose retrospectively-gated cardiac CT for functional analysis in adult congenital heart disease.

BACKGROUND: The use of cardiac computed tomography (CT) in the evaluation of adult congenital heart disease patients is limited due to concerns of high radiation doses. The purpose of this study was to prospectively assess whether low radiation dose cardiac CT is feasible to evaluate ventricular systolic function in adults with congenital heart disease. METHODS: The study group included 30 consecutive patients with significant congenital heart disease who underwent a total of 35 ECG-gated cardiac CT scans utilizing a 320-detector row CT scanner. Each study included a non-contrast scan and subsequent contrast-enhanced retrospectively-gated acquisition. Effective radiation dose was estimated by multiplying the dose length product by a k-factor of 0.014mSv/mGycm. RESULTS: The mean age of the patients was 34.4±8.9years, 60% were men, and mean body mass index was 24.2±4.3kg/m2. A majority of patients (n=28, 93.3%) had contraindications to cardiac MRI. A tube potential of 80kV was used in 27 (77.1%) of the contrast-enhanced scans. The mean signal-to-noise and contrast-to-noise ratios were 11.5±3.9 and 10.3±3.7, respectively. The median radiation dose for non-contrast and contrast-enhanced images were 0.1mSv (0.07-0.2mSv) and 0.94mSv (0.5-2.1mSv), respectively. All 35 CT scans were successfully analyzed for ventricular systolic function. CONCLUSIONS: A low radiation contrast-enhanced, retrospectively-gated cardiac CT with a median radiation dose of less than 1mSv was successful in evaluating ventricular systolic function in 30 consecutive adult congenital heart disease patients who underwent a total of 35 scans.