Delayed Gadolinium Enhancement Research Articles

Left atrial wall thickness measured by a machine learning method predicts AF recurrence after pulmonary vein isolation

Abstract Background Left atrial (LA) remodeling plays a significant role in the progression of atrial fibrillation (AF). Although LA wall thickness (LAWT) has emerged as an indicator of structural remodeling, its impact on AF outcomes remains unclear. We aimed to determine the association between LAWT and AF recurrence after pulmonary vein isolation (PVI). Methods Single-center registry of patients enrolled for radiofrequency PVI from 2016 to 2018. In all cases, a pre-ablation CT scan was performed within less than 48 hours. Mean LAWT was retrospectively determined by a semi-automated machine learning method with minimal human intervention (ADAS 3D®). Additionally, regional tissue thickness was assessed in four locations: roof and inferior, posterior, and anterior walls. In a subgroup of patients, a pre-ablation cardiac magnetic resonance (CMR) was also performed within the same week. LA functional parameters and fibrosis, using 3D delayed gadolinium enhancement, were analyzed. The primary endpoint was AF recurrence after a 3-month blanking period. Results A total of 439 patients (mean age 61±12years, 62% male, 78% with paroxysmal AF) were included. The mean LAWT was 1.4±0.2mm (ranging from 0.9 to 1.9mm). Software processing duration was 8.2±0.4 min, and the mean human input time was 1.3±0.1 min. There was no correlation between LAWT and CT-derived LA-indexed volume (Spearman –0.01, p=0.845). During a median follow-up of 5.8 (IQR 4.9–6.6) years, 238 patients (54%) had an AF relapse. LAWT was an independent predictor of recurrence, after adjusting for known confounders including age, non-paroxysmal AF, LA-indexed volume, and chronic kidney disease (adjusted HR 6.49 [95% CI 2.70-15.49], p<0.001). AF recurrence rates were 11%, 15%, and 21%/year across terciles of increasing LAWT (log-rank p<0.001) – Figure. The posterior LAWT revealed the strongest association with the study endpoint (HR 1.93 [95% CI 1.24-3.02], p=0.004). In the cohort of 62 patients with both CT and CMR, LAWT showed weak correlations with LA ejection fraction and LA coupling index (Spearman <0.25; p=0.054 and p=0.093, respectively), and a moderate correlation with LA fibrosis (Spearman 0.468; p<0.001). Conclusions Mean LAWT, easily assessed by commercially available machine learning software, is an independent predictor of AF recurrence after PVI in the long-term follow-up. This association is mainly driven by the posterior LAWT. Whether patients with increased LAWT should receive tailored therapy deserves further investigation.Figure

A case for genetic testing: Arrhythmogenic cardiomyopathy presenting as myocarditis

ABSTRACT Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiomyopathy associated with fibrofatty tissue replacement of the ventricular tissue. The disease can cause ventricular dysfunction and arrhythmias and can increase the risk of sudden cardiac death. This cardiomyopathy can have variable clinical presentations, especially in the pediatric and young adult populations. In this report, we describe the case of an 18-year-old female with myocarditis as the initial presentation of ACM. She presented following a resuscitated cardiac arrest due to ventricular arrhythmia. On arrival, myocardial edema and delayed gadolinium enhancement were present on cardiac magnetic resonance imaging, with no ventricular changes observed, making the diagnosis consistent with myocarditis. Genetic testing revealed a pathogenic mutation in the desmoplakin gene consistent with ACM. Given the unconventional initial presentation of this patient’s disease, early consideration of genetic testing may be beneficial to aid in the early diagnosis and management of ACM in young patients.

Abstract 13651: Pembrolizumab Associated Myocarditis, Myasthenia Gravis, and Myositis

Case: A 71 year old man presented with acute generalized muscle weakness, double vision, sagging eye lids, weakness with swallowing, shortness of breath, and dark urine. History was notable for stage IV non-small cell lung cancer with second dose of pembrolizumab therapy completed 12 days earlier. Exam showed bilateral ptosis and muffled speech. He had increased work of breathing, tachypnea, 3/5 strength in the bilateral lower extremities, and 5/5 strength in the upper extremities. His initial NIF was -25. Labs were notable for troponin of 1532, CK 16229, ESR 100, CRP 8.8, ALT 225, and AST 648. EKG revealed a new right bundle branch block and left posterior fascicular block. TTE showed EF of 65% without regional wall motion abnormalities. Cardiac MRI showed mildly elevated T1 and T2 relaxation times, suggestive of diffuse myocardial edema and inflammation without delayed gadolinium enhancement. He was treated with high dose steroids, fluid resuscitation, IVIG, and briefly pyridostigmine. On hospital day 8, he was found to have complete AV block with junctional escape rhythm. He had urgent placement of a temporary pacemaker. He was started on abatacept, ruxolitinib, and oral prednisone. Over the next 14 days he made gradual improvement. Discussion: While ICI-associated myocarditis is rare, it is worth investigating due to the high mortality. The clinical presentation can range from asymptomatic troponin elevations to cardiogenic shock and ventricular arrhythmias/AV block. Diagnosis requires a high index of suspicion and multiple diagnostic modalities, including ECG, echocardiography, serum biomarkers, and often cardiac MRI and endomyocardial biopsy. There is a large overlap between myocarditis and myositis. The mainstay treatments of ICI-induced myocarditis include holding the offending immune-checkpoint inhibitor, high-dose corticosteroids, and guideline-directed medical therapy for associated heart failure or arrhythmias. Abatacept, a cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) immunoglobulin fusion protein, and ruxolitinib, a JAK 1/2 inhibitor, have recently been suggested as a novel combination to reduce mortality from ICI-associated myocarditis with concomitant myositis.

Abstract 17626: Apolipoprotein A-IV-Associated Cardiac Amyloidosis: A Classic Presentation of a Rare Condition

Introduction: A 65-year-old male with type one diabetes mellitus underwent a pancreas and kidney transplant approximately seven years ago and has received chronic tacrolimus. He presented with subacute dyspnea on exertion, orthopnea, and edema. His background also includes ongoing chronic kidney disease stage 3A, orthostatic hypotension, and spinal stenosis. On exam, he had abdominal and lower extremity swelling. An echocardiogram showed preserved LVEF, concentric hypertrophy, hyperrefractile myocardium, reduced longitudinal strain with apical sparing, and a tiny pericardial effusion. Hypothesis: His echocardiogram indicated an infiltrative process such as amyloidosis or tacrolimus cardiotoxicity or possibly hypertrophic cardiomyopathy. Methods: Free light chains and protein studies with mass-fixation demonstrated no monoclonal gammopathy. PYP scan was negative for transthyretin amyloidosis. A cardiac MRI showed patchy subendocardial and midmyocardial delayed gadolinium enhancement, supporting the clinical suspicion for cardiac amyloidosis. With the need to exclude tacrolimus toxicity as well, subsequent endomyocardial biopsy revealed apolipoprotein A-IV-associated cardiac amyloidosis (Figure 1). Results: Apolipoprotein A-IV-associated cardiac amyloidosis occurs in about 0.6% of patients with cardiac amyloid and has classic manifestations of LV outflow obstruction, microvascular disease, and especially renal insufficiency. When these features occur in patients with clinical and imaging findings suggesting cardiac amyloidosis despite negative testing for more common types of amyloidosis, clinicians should proceed with endomyocardial biopsy to evaluate for less common forms. Conclusions: We report a rare case of apolipoprotein A-IV-associated cardiac amyloidosis. This case highlights the need for endomyocardial biopsy when clinical suspicion for cardiac amyloidosis persists despite negative testing.

Abstract 18687: Cardio-Rheumatology: Coronary and Cerebrovascular Thromboses in Antiphospholipid Antibody Syndrome

Description of Case: A 27-year-old male with antiphospholipid antibody syndrome (APLS) with a high-risk antibody profile presented for cardiac evaluation. APLS was diagnosed after presentation with acute cardioembolic stroke requiring thrombolysis. Echocardiography showed a normal ejection fraction, with multiple small echo-densities attached to the anterior and posterior leaflet tips of the mitral valve suggesting non-bacterial thrombotic endocarditis (NBTE). The patient was treated with warfarin and aspirin was recommended but declined. As the patient reported lightheadedness, a cardiac rhythm monitor was placed and showed two episodes of non-sustained ventricular tachycardia (NSVT). A cardiac MRI was then obtained, showing multifocal, subendocardial and mesocardial delayed gadolinium enhancement suggesting fibrosis, in a distribution most consistent with a multivessel embolic event. A CT coronary angiogram was performed to further evaluate the coronary arteries, revealing normal coronary anatomy, free from atherosclerotic plaque or thrombus. Beta-blocker therapy was initiated for arrhythmic episodes, with resolution of symptoms and no recurrence of NSVT on follow-up monitoring. Left ventricular (LV) function continued to remain preserved on follow-up imaging, with no progression of mitral valve vegetations on anticoagulation. Discussion: Cardiac involvement in APLS has been well described but largely centers around valvular disease and de novo venous and arterial thromboses. This case of cerebrovascular and coronary pathology in APLS as a likely consequence of thromboembolic phenomena is an unusual presentation and highlights the potential downstream consequences of left-sided valvular NBTE. A high index of suspicion is required to specifically evaluate for myocardial scarring despite normal LV function on echocardiography. The presence of myocardial fibrosis in these cases can act as a focus for scar-related ventricular arrhythmias, which if diagnosed and treated in a timely manner can limit further cardiovascular complications for the patient.

Abstract 14394: Complete Atrioventricular Block in a Young Patient With Alport Syndrome: A Case Report

Background: Atrioventricular block (AVB) in young adults is rare, and the etiologies and mechanisms in most cases remain uncertain. Alport syndrome is a heterogeneous, hereditary disorder characterized by nephropathy, hearing loss, and ocular abnormalities. Complete AVB has never been reported in Alport syndrome patients with normal kidney function. Case report: A 20-year-old male patient was admitted to hospital with “asymptomatic bradycardia for years”. On admission, the patient’s blood pressure was normal and electrocardiogram (ECG) showed third-degree AVB with atrial rate of 63 bpm and ventricular rate of 36 bpm. He was diagnosed with Alport syndrome at the age of 10. Genetic testing at that time showed single nucleotide variants (c.2372T>C, c.2072T>G) and deletion (c.1781-1949(Exon25)) in COL4A5 gene. During the subsequent 10-year follow-up, the patient was presented with consistent hematuria and proteinuria, but his renal function has never been found impaired. He denied other medical history as well as family history of arrhythmia. Laboratory tests indicated hypoproteinemia, hyperuricemia, hyperlipidemia, and hyperhomocysteinemia. The 24-hour ambulatory ECG revealed a heart rhythm consisting of sinus rhythm with second-degree AVB (2:1 downward transmission) and complete AVB with junctional escape. The mean ventricular rate was 36 bpm (ranged 25-60 bpm). Neither echocardiography nor cardiac magnetic imaging with delayed gadolinium enhancement found any significant abnormality. We then did whole exome sequencing (WES), which did not identify specific pathogenic variant (Table1). 3mg atropine was admitted to the patient for an atropine test to rule out the possibility of vagally mediated AVB and the pacemaker implantation was then performed. Conclusion: We report a case of complete AVB with unknown etiology in a young patient with Alport syndrome.

Clinical Analysis of 3D-Fluid Attenuated Inversion Recovery and T1volume interpolated body examination Sequences on Delayed Gadolinium-Enhanced Scanning in Ramsay Hunt Syndrome.

Through the clinical analysis of 4 clinically confirmed cases of delayed gadolinium enhancement of Ramsay Hunt syndrome 3D-Fluid Attenated Inversion Recovery'and 'T1volume interpolated body examination (3D-FLAIR and T1VIBE) sequences, the more suitable sequences and pathologically damaged tissue sites of deep tissues of Ramsay Hunt syndrome by magnetic resonance imaging gadolinium enhancement were preliminarily explored. From October 2020 to March 2021, 4 clinically confirmed patients with Ramsay Hunt syndrome, 2 males and 2 females, aged 27-63, were continuously collected in the hospital otology clinic. Siemens Avento 1.5T magnetic resonance imaging 3D-FLAIR and T1VIBE sequencedelayed gadolinium enhancement scans and serological laboratory tests were performed, respectively, and corresponding antiviral and antiinflammatory therapy was given. The magnetic resonance imaging gadolinium enhancement of 4 cases of Ramsay Hunt syndrome was as follows: 3D-FLAIR sequence delay of 4.5 hours scanning 4 patients labyrinthine and/or middle ear signal was enhanced at the same time as the healthy side; T1VIBE sequence scanning disease in 3 cases of vestibular nerve development was enhanced than the healthy side, 2 cases of facial nerve development was enhanced than the healthy side, and 2 cases of cochlear nerve development was enhanced than the healthy side. All 4 patients were cured with related treatment. Through the comparison of 3D-FAIR and T1VIBE sequence of 4.5 hours delay before intravenous gadolinium injection and 4.5 hours delay after intravenous gadolinium injection in 4 patients with Ramsay Hunt syndrome, it was found that (i) 3D-FLAIR sequence delay of 4.5 hours scan was more likely to show whether the inner ear labyrinth barrier permeability increased and (ii) Ramsay Hunt syndrome deep ear tissue damage can be manifested as labyrinthitis, vestibular cochlear neuritis, facial neuritis, and otitis media.

Prognostic implications of unrecognized myocardial infarction and periprocedural myocardial injury on cardiac magnetic resonance imaging in patients with chronic coronary syndrome

This study sought to evaluate the prognostic implications of the presence of preprocedural unrecognized myocardial infarction (UMI) and periprocedural myocardial injury (PMI) evaluated by delayed gadolinium enhancement cardiac magnetic resonance (DE-CMR) in patients with chronic coronary syndrome (CCS) undergoing elective percutaneous coronary intervention (PCI). We enrolled 250 CCS patients scheduled for elective PCI. UMI was defined as the presence of late gadolinium enhancement (LGE) detected by pre-PCI CMR in the region without medical history of revascularization and/or MI. Periprocedural new occurrence or increased volume of LGE in the target territory detected by post-PCI CMR (PPL) were used to assess PMI. In the final analysis of 235 patients, UMI and PPL were detected in 43 patients (18.3%) and 45 patients (19.1%), respectively. During follow-up for a median of 2.2 years, major adverse cardiac events (MACE) occurred in 31 (13.2%) patients. On multivariable analysis, UMI and PPL remained as significant predictors of MACE after adjusting confounding factors (HR 4.62, 95% CI 2.24–9.54, P < 0.001, HR 2.33, 95% CI 1.11–4.91, P = 0.026). In patients with CCS who underwent elective PCI, UMI and PPL were independent predictors of worse outcomes. UMI and PPL on DE-CMR might provide additional potential insight for the risk stratification of patients undergoing elective PCI.

Cardiac magnetic resonance features associated with the risk of cardiac arrest in patients with acute myocardial infarction

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): This work was supported by the George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Târgu Mureș, Research Grant number NR. 164 / 26 / 10.01.2023. Background Cardiac arrest (CA) is the most severe complication of acute myocardial infarction (AMI). The role of different factors related to the site and severity of coronary occlusion in the pathogenesis of AMI-related cardiac arrest is still under investigation. The aim of the study was to investigate the association between (1) different cardiac magnetic resonance (CMR) features associated with the location and severity of the myocardial injury, and (2) the risk of CA accompanying an AMI. Methods In total, 54 patients AMI undergoing post-AMI CMR imaging with delayed gadolinium enhancement were enrolled in the study. The study lot was divided into 2 groups: group 1–8 patients who survived a CA in the acute phase of AMI and group 2–46 patients, matched for age and gender, with AMI but without CA. In all patients, infarct mass, the proportion of high transmural extent, and scar mass at different myocardial segments were calculated using the QMap software (Medis BV). Results Compared to patients without CA, those with CA had a significantly higher infarct mass (47.9 +/- 38 g versus 23.3 g, p = 0.03), infarct mass % (26.9 +/ 17.3% vs 15.1 +/- 8.6 %, p = 0.02), and a higher degree of transmurality (29.28 +/- 20.2 % vs 14.1 +/- 9.2 %, p = 0.01). Location of myocardial injury at the level of latero-apical, anterolateral, and bazal anterior segments seemed to be more frequently associated with the risk of CA in the acute phase of AMI: infarct mass 33.9 +/- 30.6 g in group 1 vs 13.6 +/- 17.3 g in group 2, p = 0.02 for the latero-apical segment, 26.5 +/- 29.0 g in group 1 vs 8.9 +/- 12.8 g in group 2, p = 0.02 for the anterolateral segment, and 20.1 +/- 21.5 g in group 1 vs 7.8 +/- 14.7 g in group 2, p = 0.02 for anterobazal segment. Conclusions Myocardial mas, high transmural extent at CMR imaging, and a large myocardial injury identified by CMR at the level of the anterior and lateral ventricular segments seems to be associated with an increased risk of CA in the acute phase of AMI.

Myocardial edema at CMR imaging end evolution of ventricular function following COVID-19 myocarditis - insights from the CARDIOCOV study

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): The CARDIOCOV project - Prototype for personalized assessment of cardiovascular risk and post-Covid myocarditis based on artificial intelligence, advanced medical imaging and cloud computing - financed by UEFISCDI PN-III-P2-2.1-PTE-2021-0450 (Contract Number 108PTE/2022). Background Myocarditis following SARS-COV-2 infection has recently become a subject of concern, being detected in a significant number of post-COVID-19 patients who undergo cardiac magnetic resonance(CMR) examination. While the role of CMR in detecting presence of viral myocarditis has been well established, the CMR features associated with significant alteration of ventricular function in post-COVID patients are not clearly identified yet. Purpose The aim of the study was to investigate the role of myocardial edema (ME) at CMR for predicting the evolution of ventricular function in patients with COVID myocarditis. Methods In total, 55 patients with CMR signs of viral myocarditis following COVID-19 infection (52.72% males, mean age 36.85+/-16.29) were enrolled in the study. The delayed gadolinium enhancement phase-sensitive inversion recovery sequences were used for characterization of the myocardial tissue, and inversion recovery images showing high signal intensity were considered suggestive of edematous changes. Patients were divided into 2 study groups, according to the presence of myocardial edema at the moment of CMR evaluation: group 1 (n=18, 32.72%) - patients without ME, and group 2 (n=37, 67.27%)-patients with ME identified by CMR. In all patients, end-distolic and end-systolic volume indexes (EDVI and ESVI), ejection fraction (EF) and stroke volume (SV) were calculated. Results Compared to patients without ME, those with ME present at CMR were older n (42.9+/-14.8 vs. 36.7+/-16.6, p = 0.145) and more frequently males(59.4% vs. 38.8%). At the same time, EF was significantly lower in patients with ME(50.9+/-14.5% in group 2 vs. 58.4+/-10.2% in group 1, p = 0.03). Patients from group 2 also exhibited a more pronounced dilatation of ventricular cavities, as reflected by a significant increase of ventricular volumes and especially of the ESVI(109.8+/-125.9 versus 80.1+/-14.2, p = 0.5 for EDVI, and 65.2+/-109.1 vs. 30.0+/-11.4, p = 0.02 for ESVI). Compared to the group without ME, those with ME presented a significantly lower stroke volume index: 44.2+/-17.3 in group 1 vs. 47.7+/-7.5, p = 0.03 in group 2. Conclusions In patients with COVID-19 myocarditis, presence of ME at CMR imaging is associated with a worse evolution of the left ventricular function, as reflected by increased ventricular dilatation and decreased cardiac output in the post-COVID period.

Prognostic implications of non-infarct-related territory unrecognized myocardial infarction in patients with non-ST-segment-elevation acute coronary syndrome

BackgroundPrognostic value of non-infarct-related territory (non-IR) unrecognized myocardial infarction (UMI) in patients with non-ST-elevation acute coronary syndrome (NSTE-ACS) remains to be elucidated. We sought to evaluate the prognostic impact of non-IR UMI in patients with first NSTE-ACS presentation. MethodsThis retrospective single-center analysis was conducted in patients with NSTE-ACS without prior history of coronary artery disease, who underwent uncomplicated urgent percutaneous coronary intervention (PCI) within 48 h of admission between August 2014 and January 2018. All patients underwent postprocedural cardiac magnetic resonance imaging (CMR) within 30 days after PCI. Non-IR UMI was defined as the presence of non-IR delayed gadolinium enhancement with an ischemic distribution pattern. We investigated the association of non-IR UMI, other CMR findings and baseline clinical characteristics with major adverse cardiac events (MACE), defined as death, non-fatal myocardial infarction, stroke, late revascularization, and hospitalization for congestive heart failure. ResultsA total of 168 NSTE-ACS patients were included [124 men (73.8 %); 66 ± 11 years]. Non-IR UMI was detected in 28 patients (16.7 %). During a median follow-up of 32 (15–58) months, MACE occurred in 10 (35.7 %) patients with non-IR UMI, and 20 (14.3 %) patients without (p = 0.013). Cox's proportional hazards analysis showed that the presence of non-IR UMI was an independent predictor of MACE (hazard ratio [HR], 2.37, 95 % confidence interval [CI], 1.09–5.18, p = 0.030). In patients with NSTE-ACS undergoing urgent PCI, the prevalence of non-IR UMI was 16.7 %. ConclusionsNon-IR UMI provided prognostic information independent of conventional risk factors and the extent of myocardial injury caused by NSTE-ACS.

PO-04-053 OUT-OF-HOSPITAL CARDIAC ARREST ASSOCIATED WITH EARLY REPOLARIZATION

Early repolarization syndrome (ERS) is characterized by J-point elevation presenting as slurring or notching of the terminal part of the QRS complex. Of the several congenital and acquired disorders that encompass J-wave syndromes, ERS has been linked to increased risk of developing polymorphic VT and idiopathic VF that can lead to sudden cardiac death. To describe a patient who presented after a cardiac arrest with initial ECG criteria consistent with Type III ERS who recieved a dual-chamber ICD. n/a We present a case of a 42-year-old man without any prior history that presented to the emergency room after successful resuscitation from out-of-hospital cardiac arrest due to VF. After noticing agonal respiration during early morning hours, his wife called emergency medical services (EMS) and promptly performed cardiopulmonary resuscitation. Upon arrival, EMS found the patient to be in VF. He was shocked once by an automatic external defibrillation leading to ROSC with restoration of sinus rhythm. In the ED, his initial vitals were 122/93 mmHg, 104 bpm, 95% oxygen saturation on room air, and temperature of 36.4 °C. He denied any family history of sudden cardiac death. Wife reported prior history of nocturnal agonal respirations. Initial diagnostic work-up was significant for a leukocytosis of 19,000 and serial high-sensitivity cardiac troponins of 130, 305, and 364 with a 0/3-hour delta change of 234. A12-lead ECG upon presentation is shown in Figure 1 without previous ECGs available for comparison. An emergent coronary angiography was performed which revealed no significant coronary obstruction or anomaly. With both his ECG findings and a Shanghai Score of 7, a diagnosis of Type III ERS was highly suspected. Further diagnostic testing included a TTE showing left ventricular dysfunction with an ejection fraction of 40-45%. CMR showed no delayed gadolinium enhancement. Ultimately, a dual-chamber ICD was implanted during the admission. Follow-up ECGs revealed resolution of slurred terminal QRS complexes. He was discharged on beta blocker and low-dose losartan with close outpatient follow-up with electrophysiology. ECG changes of ERS can be dynamic and may sometimes be concealed, particularly in the setting of an out-of-hospital cardiac arrest. It is important to identify early repolarization patterns due to the adverse prognostic significance especially of Type III ERS which is associated with the highest level of risk for development of malignant arrhythmia.

Emerging Role of 3.0 Tesla Cardiac Magnetic Resonance Imaging in Differentiating Cardiac Masses

Objective: To evaluate the role of Cardiac Magnetic Resonance Imaging in differentiating cardiac masses (thrombus vs tumor and benign tumor vs malignant tumor) in local population.&#x0D; Study Design: Analytical cross-sectional study.&#x0D; Place and Duration of Study: Tertiary Cardiac Care Center, Department of Cardiac Magnetic Resonance Imaging, Rawalpindi Pakistan, from Oct 2017 to Jun 2021.&#x0D; Methodology: This retrospective study included (n=56) patients via universal sampling, enrolled for Cardiovascular magnetic resonance imaging with a suspicion of cardiac masses either on echocardiography or Computed Tomography scan. Cardiovascular magnetic resonance imaging sequences were reviewed as SSFP cine images for mass location, size and mobility.T1 weighted turbo spin echo, T2 weighted turbo spin echo with and without fat saturation and TIRM sequences told their intensity as compared to normal myocardium, myomaps evaluated the relaxation time, while contrast first pass perfusion indicated the vascularity and delayed gadolinium enhancement images with standard and long TI were analyzed for contrast enhancement.&#x0D; Results: Total n=56 patients with a confirmed diagnosis of mass were included for the analysis. Mean age of participants was found to be 45.21(18.3%), height 168.4(8.69%) and weight 68.6(14.94%). There were 47(83.9%) males and females were 9(16.1%). 22(39.3%) had hypertension, 12(21.4%) had diabetes mellitus, 24(42.9%) had previous myocardial infarction. Maximum number of masses were found in LV 31(55.4%) followed by RV 11(19.6%). Sensitivity of T1 map to detect fibrosis intumor is 100%. Sensitivity of T2 map to detect edema in tumor is 82%.&#x0D; Conclusion: Cardiac masses like thrombi and tumors accurately diagnosed by cardiac MRI with etiology..

Dilated Cardiomyopathy Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy in a 35-Year-Old Woman

While dilated cardiomyopathy (DCMP) typically involves the left ventricle (LV), it can also impact the right ventricle (RV). Arrhythmogenic right ventricular cardiomyopathy is a heritable myocardial disorder that mainly affects the RV. A 35-year-old woman presented to the emergency department complaining of chest discomfort and palpitations. The patient had ingested 100 g of alcohol daily for more than 5 years. Transthoracic echocardiography showed dilation of the four cardiac chambers with global systolic dysfunction of the prominent myocardial recesses of both ventricles. Non-sustained ventricular tachycardia was observed on 24-hour Holter monitoring. On magnetic resonance imaging (MRI), the ratio between noncompacted and compacted layers did not meet the criteria for non-compaction, and delayed gadolinium enhancement was only observed in the inferior LV myocardium. Endomyocardial biopsy revealed no fibrosis or fatty changes. Consequently, a diagnosis of alcohol-induced DCMP was reached. Various diagnostic modalities, including MRI and biopsy, can contribute to accurate diagnoses.

MRI Evaluation of Gene Therapy in the Canine Model of Duchenne Muscular Dystrophy.

Magnetic resonance imaging (MRI) is a well-established and widely used technique to characterize and quantify skeletal and cardiac muscle changes in Duchenne muscular dystrophy (DMD). Recently, MRI has been explored to study disease progression and response to gene therapy in the canine DMD model. Using traditional sequences, delayed gadolinium enhancement, novel sequences, and spectroscopy, investigators have begun to (i) establish the baseline MRI characteristics of the muscles in normal and affected dogs and (ii) evaluate gene therapy outcomes in treated dogs. As a noninvasive assay, MRI offers an excellent opportunity to study longitudinal muscle changes in long-term gene therapy studies in the canine model. In this chapter, we outline the MRI method used to study DMD in the canine model.

Abstract 13359: Added Benefit of Parametric Mapping in Anthracycline-Induced Cardiomyopathy - Avoiding a Misdiagnosis

Case Presentation A 72 year old man presented with progressive dyspnea on exertion, only able to climb 4 stairs prior to symptom onset. He had a history of diffuse large B-cell lymphoma for which he had completed chemotherapy with cyclophosphamide, doxorubicin, rituximab, vincristine, and prednisone a year prior to presentation with recent PET scan showing complete remission. EKG showed rate-controlled atrial fibrillation. TTE confirmed a severely reduced systolic function with LVEF 25% and global hypokinesis. Coronary angiogram revealed no obstructive disease. Cardiac MRI (CMR) was then performed with no evidence of myocardial infarction, infiltration, or scarring on delayed gadolinium enhancement (DGE). T2 mapping, DIR, and TIR sequencing did not show any myocardial edema. However, baseline pre-contrast T1 relaxation time was increased at 1140-1153ms [Figure 1A] with a calculated ECV [Figure 1B] elevated at 37-40%, concerning for anthracycline-induced cardiomyopathy (AIC). Discussion Anthracyclines, such as doxorubicin, are important chemotherapeutic agents; however, their utility is often limited by development of progressive cardiotoxicity. Cumulative dose-dependent exposure can lead to apoptotic myocyte loss, myocardial fibrosis, and dysmotility. DGE usually identifies focal myocardial fibrosis; however, anthracycline-induced fibrosis is frequently diffuse and can be missed by qualitative analysis and DGE burden assessment. Diffuse fibrosis is better demonstrated with use of hematocrit adjusted ECV and pre-/post-T1 mapping. CMR provides additional information about decrease in the LV mass, aortic stiffness, myocardial strain, early fibrosis detection, and myocardial edema that can aid in early detection of cardiotoxicity during anthracycline therapy. Use of CMR parametric imaging is complementary and essential in accurately diagnosing AIC in patients with prior exposure and merits widespread consideration in cardio-oncology.

Abstract 15424: The Toxic Tango: Tyrosine Kinase Inhibitors (TKI) and Immune Checkpoint Inhibitors (ICI) Toxicities

Introduction: ICI and TKI have several cardiotoxicity side effects. We present a case of TKI-ICI toxicity resulting in multiorgan inflammatory syndrome with myocarditis and thrombotic STEMI that was treated with high-dose steroids and PCI. Case Presentation: 72-year-old man presented with acute SOB and inferior STEMI. He complains of severe DOE for the past 3 months, chronic diarrhea, and altered mental status. PMH of renal cell carcinoma (RCC) limited to kidney s/p nephrectomy. Follow-up CT scan with 4cm inferior left adrenal gland mass avid for FDG-PET and IR-biopsy positive for RCC. He was started on Pembrolizumab 200mg IV q3 weeks and axitinib 5mg PO q12h for the past 5 months. Investigation: LHC showed a thrombotic lesion in RCA with normal rest of coronaries without evidence of atherosclerosis or bystander plaque with successful PCI-DES. TTE with inferior wall hypokinesis and moderate pericardial effusion with organized material. Elevated CRP/ESR. Despite PCI, he is unable to walk 200ft due to DOE and needs supplemental O2. VQ scan low % for PE, PFT mild obstructive disease with severe reduction DLCO, and HRCT moderate emphysema, bronchial/ septal thickening, and normal hemodynamics in RHC. CMR demonstrated moderate pericardial effusion with internal debris, LVEF 38%, myocardial edema, and delayed gadolinium enhancement from base to apical inferior wall. Treatment: Empirical trial of IV-methylprednisolone 500mg IV q12 was started with drastic improvement of shortness of breath, resolution of AMS, ESR/CRP, and discontinuation of oxygen. Follow-up TTE with normal LVEF and resolution of pericardial effusion. Conclusions: ICI-Myocarditis is a severe irAE. The diagnosis requires clinical suspicion and exclusion of other cardiac conditions due to the high fatality rate. Early recognition and prompt treatment are needed to improve mortality. TKI-related arterial-thromboembolic events is not common but if present different therapy options should be offered.

Abstract 12675: A Modified Classification Scheme for Chaga's Cardiomyopathy Incorporating Cardiac Magnetic Resonance Imaging

Introduction: The public health concerns from Chaga’s disease warrant improved cardiovascular imaging efforts. We present a novel and modified Chaga’s staging classification incorporating cardiac magnetic resonance imaging (CMR) to aid in diagnosing cardiomyopathies (CMs). (Figure 1) Case Presentation: An 18 year old asymptomatic military basic trainee screened serology positive for Trypanosoma Cruzi. A native of South Texas, he had frequent exposure to many triatome bugs without any history of chagoma or bites. Electrocardiogram showed sinus bradycardia, first degree atrioventricular delay, right bundle branch block with a left anterior fascicular block. Transthoracic echocardiogram (TTE) demonstrated a preserved left ventricular ejection fraction (LVEF), no wall motion abnormalities or valvular disease. Cardiopulmonary exercise testing demonstrated the trainee prematurely reaching anaerobic threshold. CMR was performed to evaluate specifically for delayed gadolinium enhancement and chamber size derangements for prognostication and military disposition purposes. CMR demonstrated mild left ventricular dilation, LVEF 65%, no late gadolinium enhancement, normal T1 relaxation time of 932 milliseconds, and normal T2 weighted images. The exposure history, positive serologies, electrocardiogram, and early ventricular remodeling were consistent with a Chaga’s dilated CM revealed with CMR improved spatial resolution. Case Outcome: Military standards currently prohibit cardiomyopathies from entering active duty service. The trainee was discharged and referred for medical therapy. Conclusions: We propose that CMR be incorporated into the Chaga’s classification schemes to (1) identify structural disease not apparent by TTE, (2) recognize fibrotic or inflammatory myocardial involvement which is prognosis altering, and (3) diagnose early involvement of CM which could benefit from earlier anti-parasitic therapy.

Multimodality imaging predictors of appropriate ICD shock and mortality in adults with cardiac sarcoidosis

Abstract Background Identifying patients with cardiac sarcoidosis (CS) who are at increased risk of sudden cardiac death (SCD) is imperative. Current guideline recommendations for implantable cardioverter-defibrillator (ICD) implantation in patients with CS are based on small observational studies and have not been validated in contemporary cohorts using multimodality cardiac imaging. Purpose The aim of this study was to characterize a cohort of patients with tissue-proven cardiac sarcoidosis who underwent multimodality cardiac imaging and identify predictors of appropriate ICD shock and mortality. Methods We retrospectively identified subjects with a diagnosis of CS established by clinical/imaging criteria, and tissue biopsy (N=273) seen at our tertiary care center between 2001 and 2021. Clinical characteristics and outcomes were collected from electronic medical records. The primary endpoint of interest was a composite of appropriate ICD shock and all-cause mortality. Secondary endpoints were individual rates of appropriate ICD shock and all-cause mortality. Cox proportional hazard regression analysis was used to identify independent predictors of the outcomes. Results Mean age was 59±11 years and 40% were female. Isolated CS was found in 49 subjects (17.9%). The prevalence of traditional cardiovascular risk factors was low. Atrial fibrillation prevalence was high (41%). After a median follow-up of 7.9 years, the rate of appropriate ICD shock and all-cause mortality was 29% (N=79). The 5-year overall survival rate of 97.5%. Age, left ventricular ejection fraction (LVEF), and delayed gadolinium enhancement (DGE) in cardiac magnetic resonance (CMR) were independent predictors of the primary composite endpoint; LVEF and DGE in CMR were independent predictors of appropriate ICD-shock; and LVEF and baseline serum NT proBNP were independent predictors of overall mortality. An LVEF of 47% was identified as the optimal cutoff in predicting the primary composite endpoint. Presence of scar, inflammation or mismatch pattern in positron emission tomography were not significant predictors of the outcomes. Conclusion In this large cohort of subjects with CS, we found that the presence of DGE in CMR was the strongest independent predictor of the composite endpoint of appropriate ICD-shock and mortality and of appropriate ICD-shock individually; LVEF by echocardiogram was an independent predictor of the primary and secondary endpoints with an optimal LVEF cutoff for predicting the composite endpoint of 47%. Funding Acknowledgement Type of funding sources: None.

Prognostic implication of unrecognized myocardial infarction in patients with non-ST-segment-elevation acute coronary syndrome

Abstract Background Prognostic value of unrecognized non-infarct-related territory (non-IR) myocardial infarction (UMI) in patients with non-ST-segment-elevation acute coronary syndrome (NSTE-ACS) remains to be elucidated. Purpose This study sought to evaluate the prevalence of non-IR UMI and its prognostic value in patients with first NSTE-ACS presentation. Methods This retrospective single-center analysis was conducted in patients with NSTE-ACS without prior history of coronary artery disease, who underwent uncomplicated urgent percutaneous coronary intervention (PCI) within 48 hours of admission between August 2014 and January 2018. All patients underwent postprocedural cardiac magnetic resonance imaging (CMR) within 30 days after PCI. Non-IR UMI was defined as the presence of non-IR delayed gadolinium enhancement with an ischemic distribution pattern. We investigated the association of non-IR UMI, other CMR findings and baseline clinical characteristics with major adverse cardiac events (MACE), defined as all-cause death, non-fatal myocardial infarction, ischemic stroke, late revascularization and hospitalization for congestive heart failure. Results A total of 168 NSTE-ACS patients were included (124 males (73.8%); 66±11 years). Non-IR UMI was detected in 28 patients (16.7%). During a median follow-up of 32 months (15–58), MACE occurred in 10 (35.7%) patients with non-IR UMI, and 20 (14.3%) patients without (P=0.013). Patients with MACE showed higher frequency of non-IR UMI in RCA territory and multi vessel disease, higher level of NT-proBNP at admission, higher Genisini score, and greater extent of UMI. Cox's proportional hazards analysis showed that the presence of non-IR UMI was an independent predictor of MACE (HR 2.34, 95% CI 1.02–5.37, P=0.045), after adjusting confounding factors, such as multi vessel disease and serum levels of NT-proBNP at admission. The discriminant efficacy (IDI and NRI) of predicting MACE was significantly improved when the presence of non-IR UMI added to the reference clinical risk model. Kaplan-Meier analysis revealed that patients with non-IR UMI were significantly associated with poor prognosis. (Figure 1). Conclusions In patients with NSTE-ACS undergoing urgent PCI, the prevalence of non-IR UMI was 16.7%. Non-IR UMI provided prognostic information independent of conventional risk factors. Funding Acknowledgement Type of funding sources: None.