Advanced Cardiac Imaging Research Articles

Procedural Adaptations to Avoid Haemodynamic Instability During Catheter Ablation of Scar-related Ventricular Tachycardia.

Classically, catheter ablation for scar-related ventricular tachycardia (VT) relied upon activation and entrainment mapping of induced VT. Advances in post-MI therapies have led to VTs that are faster and haemodynamically less stable, because of more heterogeneous myocardial fibrosis patterns. The PAINESD score is one means of identifying patients at highest risk for haemodynamic decompensation during attempted VT induction, who may, therefore, benefit from alternative ablation strategies. One strategy is to use temporary mechanical circulatory support, although this warrants formal assessment of cost-effectiveness. A second strategy is to minimise or avoid VT induction altogether by employing a family of 'substrate'-based approaches aimed at identifying VT isthmuses during sinus or paced rhythm. Substrate mapping techniques are diverse, and focus on the timing, morphology and amplitude of local ventricular electrograms - sometimes aided by advanced non-invasive cardiac imaging modalities. In this review, the evolution of VT ablation over time is discussed, with an emphasis on procedural adaptations to the challenge of haemodynamic instability.

Windows, Wings and Wonder: Advanced Cardiac Imaging Via Transesophageal Two-Dimensional and Transthoracic Three-Dimensional Echocardiography for the Accurate Diagnosis of Double-Orifice Mitral Valve in the Background of Primum Atrial Septal Defect: A Case Report

The assessment of mitral valve anatomy can be difficult or even misleading on standard cardiac imaging procedures. Such is the scenario in a 40-year-old woman with an incidental finding of atrial septal defect (ASD) and consideration of a trileaflet versus double-orifice mitral valve (DOMV) on transthoracic two-dimensional echocardiography. Further interrogation using transesophageal two-dimensional and transthoracic three-dimensional (3D) echocardiography (to allow more accurate assessment and simultaneous viewing of multiple imaging planes) revealed the typical “seagull wing” configuration of the mitral valve on long-axis view and two distinct but nearly equal-sized orifices on short-axis view, one oriented anteriorly and the other posteriorly. The patient’s DOMV was classified as an incomplete bridge type as confirmed on 3D imaging, with each orifice having its own set of papillary muscles and chordal attachments. The mitral regurgitation was graded as severe, resulting from leaflet prolapse of the anterior orifice. No left ventricular outflow tract obstruction was demonstrated in this case, and the overall left ventricular systolic function was preserved. The ASD was of the primum type, with the configuration and exact location verified through 3D imaging. The accurate detection of congenital anomalies via noninvasive techniques and complementary advanced modalities is vital for preoperative planning, as well as anticipation of potential complications related to the structural anomalies. To our knowledge, this is the first local report of DOMV with primum ASD in an adult. KEYWORDS: double-orifice mitral valve, primum atrial septal defect, transesophageal and three dimensional echocardiography

Management of the arrhythmic manifestations of cardiac sarcoidosis.

Cardiac sarcoidosis (CS) is characterised by a high burden of arrhythmic manifestations and cardiac electrophysiologists play an important role in both the diagnosis and management of this challenging condition. CS is characterised by the formation of noncaseating granulomas within the myocardium, which can subsequently lead to fibrosis. Clinical presentations of CS are varied and depend on the location and extent of granulomas. Patients may present with atrioventricular block, ventricular arrhythmias, sudden cardiac death or heart failure. CS is being increasing diagnosed through use of advanced cardiac imaging, however endomyocardial biopsy is often still required to confirm the diagnosis. Due to the low sensitivity of fluoroscopy-guided right ventricular biopsies, three-dimensional electro-anatomical mapping and electrogram-guided biopsies are being investigated as a means to improve diagnostic yield. Cardiac implantable electronic devices are often required in the management of CS, either for pacing or for primary or secondary prevention of ventricular arrhythmias. Catheter ablation for ventricular arrythmias may also be required, although this is often associated with high recurrence rates due to the challenging nature of the arrhythmogenic substrate. This review will explore the underlying mechanisms of the arrhythmic manifestations of CS, provide an overview of current clinical practice guidelines, and examine the important role that cardiac electrophysiologists play in managing patients with CS.

Advanced Cardiac Imaging in the Assessment of Aortic Stenosis.

Aortic stenosis is the most common form of valve disease in the Western world and a major healthcare burden. Although echocardiography remains the central modality for the diagnosis and assessment of aortic stenosis, recently, advanced cardiac imaging with cardiovascular magnetic resonance, computed tomography, and positron emission tomography have provided invaluable pathological insights that may guide the personalized management of the disease. In this review, we discuss applications of these novel non-invasive imaging modalities for establishing the diagnosis, monitoring disease progression, and eventually planning the invasive treatment of aortic stenosis.

Impact of physician performed point of care ultrasound (POCUS) during first outpatient cardiology consultation on patient management and downstream testing.

Goal directed point of care ultrasound (POCUS) is a bedside tool to assist with clinical diagnosis. We examined the impact of POCUS performed by consulting cardiologist (CC) during initial cardiology consult on clinical management and downstream testing. Sixty-nine study patients (pts) seen in a general cardiology outpatient clinic of a tertiary center by an expert imaging CC were compared to a control group of 65 pts seen by three different CCs without POCUS during the same time-period, in whom the first standard echo (SE) was performed after the initial visit. Baseline characteristics were similar between the two groups for age, cardiac risk factors, and referral diagnoses. Echo findings on POCUS and by SE (mean delay of 17.2days after visit) in the control group were comparable for RV size and function and for valvular heart disease. More patients with lower LVEF, higher LV filling pressures, new regional wall motion abnormalities, and increased aortic root size were present among POCUS group resulting in greater yield of echo abnormalities. There were more cardiovascular medication changes at the first visit (15.3% vs. 5.7%, p<.01), less referral for noninvasive stress testing (10% vs. 29%, p<.01), more advanced cardiac testing and subspecialty referrals (29% vs. 18% pts, p=.06), in the study compared to the control group after cardiology visit. POCUS at time of consultation detects more abnormal echo findings, results in more medication adjustments, less referral for noninvasive stress testing, earlier referral for advanced cardiac diagnostic imaging, and subspecialty cardiac referrals.

PO-05-005 SEPTAL LATE ENHANCEMENT BY CARDIAC CT IS SIGNIFICANTLY ASSOCIATED WITH VT RECURRENCE AFTER CATHETER ABLATION IN PATIENTS WITH NONISCHEMIC CARDIOMYOPATHY

Scar substrate in patients with nonischemic cardiomyopathy (NICM) is often difficult to identify and characterize. Advances in cardiac imaging, especially using late iodine-enhanced CT (LIE-CT), allow better characterization of scars that give rise to the arrhythmia. Currently, there is limited data on clinical correlates of CT-derived scar substrates in NICM patients and outcomes. To assess relationship between scar location on LIE-CT and outcomes. Consecutive patients from 2018-2020 with non-ischemic cardiomyopathy undergoing index ventricular tachycardia (VT) radiofrequency ablation (RFA) and pre procedural cardiac CT scar modeling (InHeart models, Pessac, France) at Brigham and Women’s Hospital and Medical University of South Carolina were included. 3 patients had VT ablations at an outside facility prior to index ablation at our centers. 5 patients had CT data that could not be interpreted and were excluded. Primary endpoints were need for repeat procedure post index ablation and VT recurrence. 63 patients, mean age 63+/-12y, 90% male, mean EF 39+/-14%. 33 patients (52%) required repeat procedures. 49 patients (78%) had VT recurrence with average time to recurrence of 59 days. CT derived total scar volume was also found to be positively correlated with intrinsic QRS duration (r=0.35, p=0.005) similar to what has been shown in prior studies of cardiac MRI-detected late gadolinium enhancement. 35 patients (56%) had septal scar on cardiac CT and 42 (67%) had lateral scar (figure 1). Univariate logistic regression showed that septal scar on CT was predictive of need for repeat ablation with OR 2.9 (p=0.049; 1.0-8.4). In contrast, lateral scar did not show a statistically significant relationship (p=0.364; OR = 0.6; 0.1-1.8). Septal scar better predicted VT recurrence when compared to lateral scar, but neither were statistically significant (p=0.089; OR 2.9; 0.5-10.6 and p=0.547;OR 1.48; 0.4-5.3, respectively). In this tertiary care referral population, patients with NICM undergoing VT catheter ablation with septal LIE-CT have nearly 3-fold increased risk of VT recurrence.

Cardiac Calcified Amorphous Tumor: An Unusual Etiology for Recurrent Cardioembolic Strokes (P3-5.026)

<h3>Objective:</h3> To report a patient with recurrent cardioembolic strokes, in whom an underlying calcified amorphous tumor (CAT) of the heart was detected as the etiology, 2 years after initial presentation. <h3>Background:</h3> CAT of the heart is a rare intracavitary cardiac mass characterized histologically by amorphous fibrinous material and calcification. It has a tendency for embolism. We use this illustrative case to bring awareness for this rare entity and utility of advanced cardiac imaging. <h3>Design/Methods:</h3> An 87-year-old woman with recurrent recent strokes was evaluated for her 5^th stroke. Her neuroimaging had suggested an embolic etiology, but routine cardiac workup had not identified a source. After her 5^th stroke happened despite being on anticoagulation, a trans-esophageal ECHO (TEE) was done followed by cardiac MRI. <h3>Results:</h3> TEE and MRI showed a left atrial non-enhancing calcified mass attached by a stalk to the interatrial septum suspicious for CAT. The tumor was surgically resected. Histological examination showed nodular amorphous calcification against the background of degenerating fibrinous material consistent with CAT. The immediate post-operative period was uneventful, and the patient was discharged to a rehabilitation facility on aspirin monotherapy. <h3>Conclusions:</h3> CAT is a rare cardiac tumor, and its presence is suggested by the presence of calcification of an intracardiac mass on cardiac imaging. The presence of mitral annular calcification (MAC) and renal disease can be additional clues to CAT being the cause for an intracardiac mass, which can then be identified by advanced cardiac imaging. A definitive diagnosis can be made only on pathology, and the definitive treatment is surgical. MAC-related CAT can grow rapidly, has a high embolic risk, and should be removed expeditiously to prevent embolic strokes, even in asymptomatic individuals. <b>Disclosure:</b> Mr. Sharma has nothing to disclose. Dr. Annam has nothing to disclose. Bilal Ali has nothing to disclose. Dr. Guler has nothing to disclose. Dr. Hussein has nothing to disclose.

Diagnosis and Treatment of Cardiac Sarcoidosis.

About 5% of sarcoidosis patients develop clinically manifest cardiac features. Cardiac sarcoidosis (CS) typically presents with conduction abnormalities, ventricular arrhythmias and heart failure. Its diagnosis is challenging and requires a substantial degree of clinical suspicion as well as expertise in advanced cardiac imaging. Adverse events, particularly malignant arrhythmias and development of heart failure, are common among CS patients. A timely diagnosis is paramount to ameliorating outcomes for these patients. Despite weak evidence, immunosuppression (primarily with corticosteroids) is generally recommended in the presence of active inflammation in the myocardium. The burden of malignant arrhythmias remains important regardless of treatment, thus leading to the recommended use of an implantable cardioverter defibrillator in most patients with clinically manifest CS.

Cardiovascular Magnetic Resonance Imaging in Familial Dilated Cardiomyopathy.

Dilated cardiomyopathy (DCM) is a common cause of non-ischaemic heart failure, conferring high morbidity and mortality, including sudden cardiac death due to systolic dysfunction or arrhythmic sudden death. Within the DCM cohort exists a group of patients with familial disease. In this article we review the pathophysiology and cardiac imaging findings of familial DCM, with specific attention to known disease subtypes. The role of advanced cardiac imaging cardiovascular magnetic resonance is still accumulating, and there remains much to be elucidated. We discuss its potential clinical roles as currently known, with respect to diagnostic utility and risk stratification. Advances in such risk stratification may help target pharmacological and device therapies to those at highest risk.

Myocardial injury after major non-cardiac surgery evaluated with advanced cardiac imaging: a pilot study

BackgroundMyocardial injury after non-cardiac surgery (MINS) is a frequent complication caused by cardiac and non-cardiac pathophysiological mechanisms, but often it is subclinical. MINS is associated with increased morbidity and mortality, justifying the need to its diagnose and the investigation of their causes for its potential prevention.MethodsProspective, observational, pilot study, aiming to detect MINS, its relationship with silent coronary artery disease and its effect on future adverse outcomes in patients undergoing major non-cardiac surgery and without postoperative signs or symptoms of myocardial ischemia. MINS was defined by a high-sensitive cardiac troponin T (hs-cTnT) concentration > 14 ng/L at 48–72 h after surgery and exceeding by 50% the preoperative value; controls were the operated patients without MINS. Within 1-month after discharge, cardiac computed tomography angiography (CCTA) and magnetic resonance imaging (MRI) studies were performed in MINS and control subjects. Significant coronary artery disease (CAD) was defined by a CAD-RADS category ≥ 3. The primary outcomes were prevalence of CAD among MINS and controls and incidence of major cardiovascular events (MACE) at 1-year after surgery. Secondary outcomes were the incidence of individual MACE components and mortality.ResultsWe included 52 MINS and 12 controls. The small number of included patients could be attributed to the study design complexity and the dates of later follow-ups (amid COVID-19 waves). Significant CAD by CCTA was equally found in 20 MINS and controls (30% vs 33%, respectively). Ischemic patterns (n = 5) and ischemic segments (n = 2) depicted by cardiac MRI were only observed in patients with MINS. One-year MACE were also only observed in MINS patients (15.4%).ConclusionThis study with advanced imaging methods found a similar CAD frequency in MINS and control patients, but that cardiac ischemic findings by MRI and worse prognosis were only observed in MINS patients. Our results, obtained in a pilot study, suggest the need of further, extended studies that screened systematically MINS and evaluated its relationship with cardiac ischemia and poor outcomes.Trial registration Clinicaltrials.gov identifier: NCT03438448 (19/02/2018).

Patients with Very High Risk of Cardiovascular Adverse Events during Carfilzomib Therapy: Prevention and Management of Events in a Single Center Experience.

Carfilzomib (CFZ) improves the prognosis of multiple myeloma (MM) patients but has shown cardiovascular toxicity. The risk stratification of cardiovascular adverse events (CVAEs) now seems well established, while little is known about the course and management of patients with a high-cardiovascular-risk profile or experiencing CVAEs during therapy. Therefore, we aimed to describe our experience in decision making to support health professionals in selecting the best management strategies to prevent and treat CVAEs. A total of 194 patients with indication to CFZ underwent baseline evaluation of CVAEs risk and were prospectively followed. We propose a novel approach, which includes advanced cardiac imaging testing for patients at high baseline CV risk to rule out clinical conditions that could contraindicate starting CFZ. After baseline evaluation, 19 (9.8%) patients were found at high risk of CVAEs: 13 (6.7%) patients underwent advanced cardiac testing and 3 (1.5%) could not receive CFZ due to CV contraindications. A total of 178 (91.7%) patients started CFZ: 82 (46%) experienced arterial-hypertension-related events and 37 (20.8%) major CVAEs; 19 (10.7%) patients had to discontinue or modify the CFZ dosing regimen. Along with baseline risk stratification, subsequent cardiovascular clinical events and diagnostic follow-up both provided critical data to help identify conditions that could contraindicate the anticancer therapy.

Abstract TP94: Optimizing The Utilization Of Cardiac Computed Tomography Angiography Among Patients With Ischemic Stroke

Background: The current AHA guidelines recommend (class IIb) advanced cardiac imaging in embolic stroke of unknown source (ESUS). We aim to better characterize the role of cardiac CTA (cCTA) in the evaluation of stroke patients. Method: A retrospective review of stroke patients at a comprehensive stroke center, who had cCTA between 12/2016 and 11/2020 was conducted using institutional registries. Ischemic stroke patients with ESUS, cryptogenic (with ESUS as a competing etiology) and cardio-embolic etiologies were included. Only cardioembolic etiologies in which a suspicion of intracardiac thrombus with either a known diagnosis of atrial fibrillation or presence of LVAD were included. Cases with TIA, large vessel disease, small vessel disease, dissection or hemodynamic etiologies were excluded. TTE and cCTA results from all cases were reviewed for remarkable findings, most notably intracardiac thrombus, and analyzed if a change in anti-thrombotic therapy was directly attributable to cCTA results. Results: As per our criteria, 96 cases (Female 42.7%, Black 37.5%, Age mean: 63.7 years) were included, of whom 45 (46.9%) were ESUS, 21 (21.9%) were cryptogenic, and 30 (31.2%) were cardioembolic. cCTA revealed intracardiac thrombus in 5 (5.2%) cases, all with LA thrombus. Of these, 3 (60%) had escalation of anti-thrombotic therapy. Interestingly, in one patient who had both cMRI and cCTA imaging, the cMRI revealed an apical LV thrombus which was not detected on cCTA. Conclusion: In this retrospective analysis of 96 select stroke cases, escalation of anti-thrombotic therapy from anti-platelets to full dose anti-coagulation was indicated in 3 patients (3.1%) with addition of cCTA. Compared to a similar cohort of patients from the same institutional registry using cMRI as the advanced cardiac imaging modality, cCTA did not reveal any LV thrombi and even missed a case of LV thrombus seen on cMRI suggesting lower sensitivity for cCTA to detect LV thrombus. Further analysis of data is in process to determine the subset of stroke patients who would benefit the most from cCTA.

Serum neurofilament light chain in functionally relevant coronary artery disease and adverse cardiovascular outcomes

Background: Functionally relevant coronary artery disease (fCAD), causing symptoms of myocardial ischemia, can currently only be reliably detected with advanced cardiac imaging. Serum neurofilament light chain (sNfL) is a biomarker for neuro-axonal injury known to be elevated by cardiovascular (CV) risk factors and cerebrovascular small-vessel diseases. Due to their pathophysiological similarities with fCAD and the link to CV risk factors, we hypothesised that sNfL may have diagnostic and prognostic value for fCAD and adverse cardiovascular outcomes. Methods: Of the large prospective Basel VIII study (NCT01838148), 4’016 consecutive patients undergoing cardiac work-up for suspected fCAD were included (median age 68 years, 32.5% women, 46.9% with history of CAD). The presence of fCAD was adjudicated using myocardial perfusion imaging single-photon emission tomography (MPI-SPECT) and coronary angiography. sNfL was measured using a high-sensitive single-molecule array assay. All-cause and cardiovascular death, myocardial infarction (MI), and stroke/transient ischaemic attack (TIA) during 5-year follow-up were the prognostic endpoints. Results: The diagnostic accuracy of sNfL for fCAD as quantified by the area under the curve (AUC) was low (0.58, 95%CI 0.56–0.60). sNfL was strongly associated with age, renal dysfunction, and body mass index and was a strong and independent predictor of all-cause death, cardiovascular death, and stroke/TIA but not MI. Time-dependent AUC for cardiovascular-death at 1-year was 0.85, 95%CI 0.80–0.89, and 0.81, 95%CI 0.77–0.86 at 2-years. Conclusion: While sNfL concentrations did not show a diagnostic role for fCAD, in contrast, sNfL was a strong and independent predictor of cardiovascular outcomes, including all-cause death, cardiovascular death and stroke/TIA.

Precision therapy in dilated cardiomyopathy: Pipedream or paradigm shift?

Precision medicine for cardiomyopathies holds great promise to improve patient outcomes costs by shifting the focus to patient-specific treatment decisions, maximising the use of therapies most likely to lead to benefit and minimising unnecessary intervention. Dilated cardiomyopathy (DCM), characterised by left ventricular dilatation and impairment, is a major cause of heart failure globally. Advances in genomic medicine have increased our understanding of the genetic architecture of DCM. Understanding the functional implications of genetic variation to reveal genotype-specific disease mechanisms is the subject of intense investigation, with advanced cardiac imaging and mutliomics approaches playing important roles. This may lead to increasing use of novel, targeted therapy. Individualised treatment and risk stratification is however made more complex by the modifying effects of common genetic variation and acquired environmental factors that help explain the variable expressivity of rare genetic variants and gene elusive disease. The next frontier must be expanding work into early disease to understand the mechanisms that drive disease expression, so that the focus can be placed on disease prevention rather than management of later symptomatic disease. Overcoming these challenges holds the key to enabling a paradigm shift in care from the management of symptomatic heart failure to prevention of disease.

591 SEQUENCE OF DIAGNOSTIC TESTING IN CARDIAC AMYLOIDOSIS: EARLY MONOCLONAL PROTEIN STUDY IS ASSOCIATED WITH BETTER OUTCOMES IN AL AMYLOIDOSIS

Abstract Background Isolated cardiac amyloidosis (CA) is a complex diagnostic scenario, highlighting the need for physician awareness in differential diagnosis. Among the different types of amyloidosis, nearly all cases of CA are caused by light chain (AL) and transthyretin amyloidosis (ATTR). Patients with suspect CA without monoclonal components (MC) in both serum and urine and normal free light chain (FLC) ratio can have a non-biopsy diagnosis of ATTR amyloidosis with bone scintigraphy (1). However, in all suspected CA with a MC (approximately 20% of patients with ATTR amyloidosis), amyloid typing is mandatory. Thus, the diagnostic pathway of CA diverges based on MC-studies. We aim to assess if different sequence of diagnostic tests can affect outcomes in patients with cardiac AL amyloidosis. Methods Pavia Amyloidosis prospectively maintained database was searched for patients with isolated cardiac AL amyloidosis referred to our Centre from January 2016 to December 2020. Patients with a known monoclonal gammopathy (MG) and with multiple organ involvement were excluded. We searched for the date of symptom onset and first suspect of CA (i.e. recognition of clinical, imaging or laboratory signs of CA). In addition, we recorded the date of the different diagnostic tests performed: i.e. echocardiogram; serum and urine immunofixation and FLC measurement (MC-study); bone scintigraphy and cardiac magnetic resonance (defined as advanced cardiac imaging). We calculated the interval between those time-points and the final diagnosis: (a) from symptom onset to diagnosis, (b) from first suspect to final diagnosis, (c) from first suspect to MC-study and (d) to advanced cardiac imaging tests. We then searched for possible intervals of time amongst those, that were able to predict death at 3 months, by means of a ROC analysis. Results A total of 94 patients were included in the analysis (25% of all patients with cardiac AL amyloidosis diagnosed in the study period). Six (6%) patients died &amp;lt;1 month from diagnosis, and 27 (29%) died &amp;lt;3 months. Median overall survival (OS) of the whole cohort was 8 months, and the median follow-up of living patients was 39 months (range 16-73). The median time from symptom onset to diagnosis was 9 months (range 1-44) and the median time from the first suspect to diagnosis was 2 months (range 0-9). An interval from the first suspect to MC-study ≥6 weeks was the only predictor of death at 3 months. None of the other tested periods were associated with a significant ability to predict survival. A delay in MC-study ≥6 weeks identified patients with more advanced cardiac stage (50% vs. 25% were stage IIIb, P=0.02) and was associated with a significantly worse outcome (median survival 13 months vs. 4 months, P=0.012). In the whole cohort, a total of 76 (81%) patients underwent at least one advanced imaging examination. Amongst those, 37 (49%) performed the imaging tests before MC-study with a higher percentage of patients who had a delay in MC-studies evaluation (69% vs. 27%; P&amp;lt;0.001). Conclusion In patients who present with isolated cardiac AL amyloidosis with previously unknown MG, a relatively short delay in identifying the amyloid MC results in a considerable reduction of survival. A delay in MC-study was associated with more advanced cardiac stage. MC studies should be the first step in the work-up of patients with suspected CA to guide biopsy vs. non-biopsy diagnostic approach.

β3 adrenergic agonist treatment in chronic pulmonary hypertension associated with heart failure (SPHERE-HF): a double blind, placebo-controlled, randomized clinical trial.

Pulmonary hypertension (PH) associated with left heart disease is an increasingly prevalent problem, orphan of targeted therapies, and related to a poor prognosis, particularly when pre- and post-capillary PH combine. The current study aimed to determine whether treatment with the selective β3 adrenoreceptor agonist mirabegron improves outcomes in patients with combined pre- and post-capillary PH (CpcPH). The β3 Adrenergic Agonist Treatment in Chronic Pulmonary Hypertension Secondary to Heart Failure (SPHERE-HF) trial is a multicentre, randomized, parallel, placebo-controlled clinical trial that enrolled stable patients with CpcPH associated with symptomatic heart failure. A total of 80 patients were assigned to receive mirabegron (50 mg daily, titrated till 200 mg daily, n=39) or placebo (n=41) for 16 weeks. Of them, 66 patients successfully completed the study protocol and were valid for the main analysis. The primary endpoint was the change in pulmonary vascular resistance (PVR) on right heart catheterization. Secondary outcomes included the change in right ventricular (RV) ejection fraction by cardiac magnetic resonance or computed tomography, other haemodynamic variables, functional class, and quality of life. The trial was negative for the primary outcome (placebo-corrected mean difference of 0.62 Wood units, 95% confidence interval [CI] -0.38, 1.61, p=0.218). Patients receiving mirabegron presented a significant improvement in RV ejection fraction as compared to placebo (placebo-corrected mean difference of 3.0%, 95% CI 0.4, 5.7%, p=0.026), without significant differences in other pre-specified secondary outcomes. SPHERE-HF is the first clinical trial to assess the potential benefit of β3 adrenergic agonists in PH. The trial was negative since mirabegron did not reduce PVR, the primary endpoint, in patients with CpcPH. On pre-specified secondary outcomes, a significant improvement in RV ejection fraction assessed by advanced cardiac imaging was found, without differences in functional class or quality of life.

Multi-modality imaging to guide the implantation of cardiac electronic devices in heart failure: is the sum greater than the individual components?

Heart failure is a clinical syndrome with an increasing prevalence and incidence worldwide that impacts patients' quality of life, morbidity, and mortality. Implantable cardioverter-defibrillator and cardiac resynchronization therapy are pillars of managing patients with HF and reduced left ventricular ejection fraction. Despite the advances in cardiac imaging, the assessment of patients needing cardiac implantable electronic devices relies essentially on the measure of left ventricular ejection fraction. However, multi-modality imaging can provide important information concerning the aetiology of heart failure, the extent and localization of myocardial scar, and the pathophysiological mechanisms of left ventricular conduction delay. This paper aims to highlight the main novelties and progress in the field of multi-modality imaging to identify patients who will benefit from cardiac resynchronization therapy and/or implantable cardioverter-defibrillator. We also want to underscore the boundaries that prevent the application of imaging-derived parameters to patients who will benefit from cardiac implantable electronic devices and orient the choice of the device. Finally, we aim at providing some reflections for future research in this field.

Exercise Stress Echocardiography in Kawasaki Disease Patients with Coronary Aneurysms

The most significant sequelae of Kawasaki disease (KD) are coronary artery aneurysms, which can lead to risk of future myocardial ischemia. Exercise stress echocardiography allows for non-invasive assessment of myocardial dysfunction. We reviewed our single center experience with exercise stress echocardiography in patients with previous history of KD with coronary aneurysms. We reviewed the records of 53 KD patients who underwent exercise stress echocardiography from 2000 to 2020. Abnormal stress echocardiograms were defined as those showing no increase in biventricular systolic function post-exercise or regional wall motion abnormalities. Computed tomography angiography and cardiac magnetic resonance imaging were reviewed for patients with abnormal stress echocardiograms. Clinical data were reviewed and correlated with stress echocardiogram results. Of the 53 patients, three (5.7%) had an abnormal exercise stress echocardiogram. All three patients were classified as AHA Risk Level 4 or 5 by coronary Z-score (internal dimension normalized for body surface area) and were confirmed to have coronary aneurysms, stenosis, or myocardial tissue perfusion defects on advanced cardiac imaging that could account for the results seen on stress echocardiogram. Exercise stress echocardiography detected signs of myocardial ischemia in a subset of high-risk patients with Kawasaki disease and coronary aneurysms and may be considered as a useful screening tool for this complex patient cohort.

Abstract 10063: Changes in Clinical Phenotype of Patients With ATTR Cardiac Amyloidosis During the Past 20 Years

Introduction: Diagnostic and therapeutic advances have led to increased awareness of transthyretin cardiac amyloidosis (ATTR-CA). We sought to characterise the change in clinical phenotype of patients with ATTR-CA over the last 20-years. Methods: We studied 1967 patients diagnosed with ATTR-CA at the National Amyloidosis Centre (NAC) from 2002-2021. Results: Analysis by 5-year quartiles revealed a substantial incremental increase in patients diagnosed, with the most marked increases seen in wild-type ATTR. Increased diagnoses were accompanied by greater proportions of patients referred following bone scintigraphy and cardiac magnetic resonance imaging (3% vs 44% vs 67% vs 76%, P&lt;0.001). Over time, median duration of symptoms prior to diagnosis diminished from 36-months between 2002-2006 to 12-months between 2017-2021 (P&lt;0.001) and a greater proportion of patients had milder disease at the time of diagnosis across the 5-yearly quartiles (NAC stage 1: 34% vs 42% vs 44% vs 53%, P&lt;0.001). The latter was associated with more favourable echocardiographic parameters of structure and function, including an incremental decrease in interventricular septal thickness(18.0±3.8mm vs 17.2±2.6mm vs 16.9±2.3mm vs 16.6±2.4mm, P=0.01), which was associated with improved survival. The improved survival associated with the year of diagnosis remained significant when censoring at trial or disease modifying medication start date (2012-2016 vs. 2017-2021: HR=1.05, 95%CI[1.03-1.07], P&lt;0.001). Conclusions: Increased awareness and advances in cardiac imaging have been associated with a substantial increase in diagnosis of ATTR-CA and at a progressively earlier stage of the disease, which has contributed to improved survival in recent years. These changes may have important implications for initiation and outcome of therapy and urgently need to be factored into clinical trial design.

Abstract 11198: Intra-Myocardial Dissecting Haematoma Presented as a Ventricular Tachycardia in a Patient With Remote Silent Myocardial Infarction

Intramyocardial dissecting haematoma (IMDH), a rare form of myocardial rupture, is recognised as a complication of acute myocardial infarction (MI) but can also occur as a result of a thoracic trauma or spontaneous rupture of intramural coronary vessels. Before the advent of advanced multi-modality cardiac imaging, IMDH was an autopsy diagnosis. In this report, we present a patient with recent onset of palpitation due to non-compromising ventricular tachycardia (VT). Post cardioversion ECG showed anterior-apical deep Q waves in keeping with prior silent MI. Transthoracic echocardiography showed severely reduced left ventricular ejection fraction due to extensive apical aneurysm and raised the possibility of protruding apical thrombus. Subsequent contrast echocardiography showed features compatible with extensive LV apical thrombus and raised the differential diagnosis of contained myocardial rupture and IMDH. During the two years follow-up guideline directed medical therapy for heart failure and antiarrhythmic medication stabilised patient at NYHA class I-II with no recurrence of VT. Multimodality imaging including cardiac CT and MRI confirmed the suspicion of IMDH. With this case we are highlighting the difficulties in the diagnosis and the controversies of the management of IMDH. Figure: A: ECG shows monomorphic VT. B: after chemical cardioversion, ECG shows sinus rhythm with old transmural extensive anterior MI. C: Transthoracic echocardiography, apical 4 chamber view shows aneurysmatic left ventricular apex with echo lucent space with mobile intraventricular membrane separating the true LV lumen and the dissecting hematoma (Asterix). D: Contrast enhanced echocardiography delineating the true LV cavity from the IMDH. E,F: Contrast enhanced CT showing the low attenuation hematoma located in the anterior LV apex. Note the calcification in the LV apical myocardium indication of prior transmural MI.