Subepicardial Late Gadolinium Enhancement Research Articles

Non-dilated left ventricular cardiomyopathy with arrhythmias is commonly caused by the nonsense variant DSP:c.3793G>T in Slovenian patients.

DSP-cardiomyopathy has recently been recognised as a specific type of cardiomyopathy. Using an in-house Mendelian disease registry, we aimed to identify probands with likely pathogenic or pathogenic DSP variants. We detected these variants in 4.8% and 77.8% of genotype-positive probands referred for dilated and non-dilated left ventricular cardiomyopathy (NDLVC), respectively. We identified six Slovenian probands with the DSP:c.3793G>T and characterised them along with further eight of their relatives at the molecular and phenotypic level. Medical records revealed NDLVC with arrhythmia in six individuals (five probands, one relative; 33 ± 14 years; three males, three females). All had subepicardial late gadolinium enhancement on cardiac MRI (CMRI), and five received an ICD. Four individuals (one proband, three relatives; 48 ± 14 years; all female) had no ECG and/or cardiac abnormalities on CMRI detected. Our analysis presents a Slovenian-specific molecular pathology of DSP cardiomyopathy, delineates the clinical manifestation of DSP:c.3793C>T, and thereby improves the understanding of the clinical outcomes associated with truncating DSP variants.

YOUNG WOMAN WITH LEFT VENTRICULAR DYSFUNCTION AND MITRAL LAMBL EXCRESCENCE: WHAT TO DO WHEN ISCHEMIC STROKE IS RECURRENT?

Abstract Lambl excrescence describing rare cardiac growths that develop at the valvular coaptation sites of the heart which are seen as a thin, hypermobile and filiform strand on an echocardiogram. These filiform strands can be noted in association with embolic stroke. Also heart failure (HF) and stroke frequently coexist because of an overlap of shared risk factors and subsequent mechanisms and a higher risk of stroke is present also in HF patients in sinus rythm (SR). The clinical case concerns a 54–year–old woman. In March 2020 she was admitted for ischemic stroke by occlusion of middle cerebral artery subjected to thrombolysis and mechanical thromboctomy. Electrocardiogram showed SR and left branch block. Echocardiogram and echocardiogram transesophageal showed left ventricular (LV) dysfunction (EF 40%) withouth clear presence of emboligen sources. Thrombophilic screening and neurological checks were negative. Single antiaggregant therapy has been started. New hospitalization in October 2023 for recurrent ischemic stroke. Echocardiogram showed worsening of LV dysfunction (EF 20%). Transesophageal echocardiogram showed the presence of a filiform filament on the mitral leaflets on the atrial side (Fig 1) without the presence of intracavitary thrombi. Coronary TC angiography (Fig 2) was negative. Cardiac magnetic resonance (Fig 3) confirmed reduced ejection fraction and showed subepicardial late gadolinium enhancement in lateral wall. There are no clear evidence–based guidelines for the treatment of Lambl excrescences. When associated with stroke, an exhaustive stroke workup to identify the potential cause of stroke should always be undertaken. If the workup remains negative without any identifiable cause, then the patients can be treated with antiplatelet agents such as aspirin and clopidogrel or anticoagulation with warfarin. Also the indication to antithrombotic strategies in patients with HF, SR and stroke is controversial. There are no data to support a routine strategy of anticoagulation in patients with HF with LV dysfuncion in SR who do not have history of paroxysmal atrial fibrillation (ESC 2022 HF guidelines). So which therapy to start in young patient, with recurrent strokes, heart failure /severe LV dysfunction and mitral lambl excrescence? Oral anticoagulant? Double platelet antiaggregation? Oral anticoagulant and single platelet antiaggregation? Definitely the choice is not simple but we decided to start warfarin and continue follow–up.

Description of the Two-Dimensional Layer-Specific Strain Echocardiography Phenotype of Arrhythmogenic Left Ventricular Cardiomyopathy

Arrhythmogenic left ventricular cardiomyopathy (ALVC) is characterized by fibrofatty myocardial replacement demonstrated on cardiac magnetic resonance by late gadolinium enhancement (LGE) mainly involving the subepicardium. The aims of this study were to describe the layer-specific strain (LSS) echocardiography phenotype of ALVC and to compare it with LGE features. All consecutive ALVC pathogenic genetic variant carriers and noncarrier relatives were separated into four prespecified groups (overt ALVC [group 1], isolated LGE [group 2], pathogenic genetic variant carrier without ALVC phenotype [group 3], and no genetic variant carrier [group 4]) and studied accordingly using cardiac magnetic resonance and LSS echocardiography. Eighty-five individuals were included. Endocardial global longitudinal strain (GLS)-epicardial GLS (GLSepi) gradient was altered predominantly in group 1, illustrating transmural strain alteration in overt ALVC (3.8±1.1 in group 1, 4.3±2.2 in group 2, 5.2±1.2 in group 3, and 5.4±1.6 in group 4; P=.0017), whereas GLSepi was impaired predominantly in group 2 (endocardial GLS and GLSepi were 15.0±4.1% and 11.2±3.3%, respectively, in group 1; 20.5±2.8% and 16.2±5.5% in group 2; 23.4±3.3% and 18.2±2.7% in group 3; and 24.6±2.8% and 19.2±1.9% in group 4; P<.0001 for all). GLSepi was able to detect subepicardial LGE in genetic variant carriers without overt ALVC with an area under curve of 0.84 (95% CI, 0.73-0.95). However, segmental epicardial and endocardial strain behaved similarly and showed comparable diagnostic values for segmental LGE detection (areas under the curve, 0.72; [95% CI, 0.69-0.76] and 0.73 [95% CI, 0.70-0.76], respectively, P=.40). LSS alteration in ALVC progresses from the epicardium to the endocardium along with disease severity. Irrespective of LSS analysis, which did not provide incremental diagnostic value for the detection and localization of LGE, strain echocardiography was shown to be a potential surrogate marker of LGE, including in apparently healthy individuals with isolated LV fibrosis.

3-Tesla cardiac magnetic resonance imaging in primary dilated cardiomyopathy.

Cardiac magnetic resonance imaging (CMR) is an excellent non-invasive imaging tool in the assessment of patients with dilated cardiomyopathy (DCM). Few studies have analysed the findings in primary (idiopathic) DCM. To study the CMR features in primary DCM. We conducted a descriptive observational study on 20 adult patients with suspected or confirmed primary DCM. Each patient underwent a dedicated 3-Tesla CMR scan, and the findings were evaluated. Seventeen patients had systolic dysfunction with a reduced ejection fraction and elevated end-diastolic volume, 19 patients had contractile dysfunction in the form of global left ventricular hypokinesia, 13 patients showed no abnormal delayed contrast enhancement with gadolinium administration, and 7 patients showed abnormal late gadolinium enhancement patterns. In patients with primary DCM, CMR is a powerful diagnostic tool that can definitively establish the diagnosis, assess the severity of the disease, predict the risk of future adverse cardiovascular outcomes, check for complications, and assist in future follow-ups. What the study adds. Cardiac magnetic resonance imaging (CMR) is an excellent non-invasive imaging tool in the assessment of patients with primary dilated cardiomyopathy (DCM). Findings include global ventricular enlargement, systolic dysfunction (ejection fraction <40%), and elevated end-diastolic (≥140 mL) and end-systolic volumes. Global abnormal wall contractility is often seen. In DCM there is either no abnormal gadolinium enhancement or curvilinear mid-myocardial or subepicardial late gadolinium enhancement, unrelated to a coronary artery distribution.Implications of the findings. In patients with primary DCM, CMR provides powerful diagnostic and prognostic information. Enhanced awareness and understanding of this relatively uncommon condition among clinicians and radiologists would be of benefit in patient management and treatment.

Risk stratification in patients with structurally normal hearts: Does fibrosis type matter?

The study sought to assess the prognostic significance of nonischemic myocardial fibrosis (MF) on cardiovascular magnetic resonance (CMR)-both macroscopic MF assessed by late gadolinium enhancement (LGE) and diffuse microscopic MF quantified by extracellular volume fraction (ECV)-in patients with structurally normal hearts. The clinical relevance of tissue abnormalities identified by CMR in patients with structurally normal hearts remains unclear. Consecutive patients undergoing CMR were screened for inclusion to identify those with LGE imaging and structurally normal hearts. ECV was calculated in patients with available T1 mapping. The associations between myocardial fibrosis and the outcomes of all-cause mortality, new-onset heart failure [HF], and an arrhythmic outcome were evaluated. In total 525 patients (mean age 43.1±14.2 years; 30.5% males) were included. Over a median follow-up of 5.8 years, 13 (2.5%) patients died and 18 (3.4%) developed new-onset HF. Nonischemic midwall /subepicardial LGE was present in 278 (52.9%) patients; isolated RV insertion fibrosis was present in 80 (15.2%) patients. In 276 patients with available T1 mapping, the mean ECV was 25.5 ± 4.4%. There was no significant association between LGE and all-cause mortality (HR: 1.36, CI: 0.42-4.42, p = 0.61), or new-onset HF (HR: 0.64, CI: 0.25-1.61, p = 0.34). ECV (per 1% increase) correlated with all-cause mortality (HR: 1.19, CI: 1.04-1.36, p = 0.009), but not with new-onset HF (HR: 0.97, CI: 0.86-1.10, p = 0.66). There was no significant association between arrhythmic outcomes and LGE (p = 0.60) or ECV (p = 0.49). In a multivariable model after adjusting for covariates, ECV remained significantly associated with all-cause mortality (HR per 1% increase in ECV: 1.26, CI: 1.06-1.50, p = 0.009). Nonischemic LGE in patients with structurally normal hearts is common and does not appear to be associated with adverse outcomes, whereas elevated ECV is associated with all-cause mortality and may be an important risk stratification tool.

Cardiac MRI and Clinical Outcomes in TMEM43 Arrhythmogenic Cardiomyopathy.

Arrhythmogenic cardiomyopathy is an inherited cardiomyopathy that can involve both ventricles. Several genes have been identified as pathogenic in arrhythmogenic cardiomyopathy, including TMEM43. However, there are limited data on cardiac MRI findings in patients with TMEM43 variants to date. In this case series, cardiac MRI findings and clinical outcomes are described in 14 patients with TMEM43 variants, including eight (57%) with the pathogenic p.Ser358Leu variant (six female patients; mean age, 33 years ± 15 [SD]) and six (43%) with a TMEM43 variant of unknown significance (three female patients; mean age, 38 years ± 11). MRI findings demonstrated left ventricular systolic dysfunction in eight (57%) patients and right ventricular dysfunction in four (29%) patients. Among the nine patients with late gadolinium enhancement imaging, left ventricular late gadolinium enhancement was present in seven (78%; all subepicardial) patients. In summary, TMEM43 variants are associated with high prevalence of subepicardial late gadolinium enhancement and left ventricular dysfunction. Keywords: Arrhythmogenic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy, TMEM43, Cardiac MRI, Genetic Variants Supplemental material is available for this article.

Cardiac Magnetic Resonance Imaging in COVID-19 Vaccine-Associated Myocarditis Compared With Classical Myocarditis

BackgroundStudies comparing COVID-19 vaccine-associated and classical myocarditis (CM) are lacking. ObjectivesThe purpose of this study was to compare cardiac magnetic resonance (CMR) imaging findings and short-term clinical outcomes in patients with messenger RNA COVID-19 postvaccination myocarditis (PVM) and CM. MethodsThis was a retrospective study of patients with myocarditis: 31 with PVM and 46 with CM. Patients underwent a CMR protocol scan including T1 and T2 sequences. Late gadolinium enhancement (LGE) was expressed as percentage of left ventricular myocardial mass and the extracellular volume was calculated based on precontrast and postcontrast T1 images. Clinical outcomes included heart failure hospitalizations and mortality. ResultsStudy patients were predominantly male (81% in PVM vs 89% in CM, P = 0.330). Patients with PVM had lower T1 values compared with CM (1,064.2 ± 67.0 ms vs 1,081.6 ± 41.9 ms, P = 0.032), although T2 and extracellular volume values were similar in both groups. Left ventricular ejection fraction and LGE were similar in both groups. The most frequent location of LGE was the basal inferolateral wall. PVM more commonly demonstrated a mid-wall LGE pattern while CM demonstrated a subepicardial LGE pattern. Compared with CM, patients with PVM were more likely to have a pericardial effusion (42% vs 17%, P = 0.018) and pericardial LGE (38% vs 13%, P = 0.009). During short-term follow-up (median 300 days for PVM, 319 days for CM), there were no deaths or heart failure hospitalizations in either group. ConclusionsOur study shows similar CMR imaging findings and short-term outcomes in PVM and CM, although PVM was associated with milder myocardial abnormalities and more frequent pericardial involvement.

Risk stratification in patients with structurally normal hearts: does fibrosis type matter?

Abstract Background The clinical relevance of tissue abnormalities identified by CMR in patients with structurally normal hearts remains unclear. Purpose The study sought to assess the prognostic significance of nonischemic myocardial fibrosis (MF) on cardiovascular magnetic resonance (CMR) – both macroscopic MF assessed by late gadolinium enhancement (LGE) and diffuse microscopic MF quantified by extracellular volume fraction (ECV) – in patients with structurally normal hearts. Methods Consecutive patients undergoing CMR were screened for inclusion to identify those with LGE imaging and structurally normal hearts. ECV was calculated in patients with available T1 mapping. The associations between myocardial fibrosis and the outcomes of all-cause mortality, new-onset heart failure [HF], and an arrhythmic outcome were evaluated. Results In total 525 patients (mean age 43.1±14.2 years; 30.5% males) were included. Over a median follow-up of 5.8 years, 13 (2.5%) patients died and 18 (3.4%) developed new-onset HF. Nonischemic midwall (Figure 1B-C)/subepicardial LGE (Figure 1D) was present in 278 (52.9%) patients; isolated RV insertion fibrosis was present in 80 (15.2%) patients (Figure 1A). In 276 patients with available T1 mapping, the mean ECV was 25.5 ± 4.4%. There was no significant association between LGE and all-cause mortality (HR: 1.36, CI: 0.42-4.42, p=0.61), or new-onset HF (HR: 0.64, CI: 0.25-1.61, p=0.34). ECV (per 1% increase) correlated with all-cause mortality (HR: 1.19, CI: 1.04-1.36, p=0.009), but not with new-onset HF (HR: 0.97, CI: 0.86-1.10, p=0.66)(Figure 2). There was no significant association between arrhythmic outcomes and LGE (p=0.60) or ECV (p=0.49). In a multivariable model after adjusting for covariates, ECV remained significantly associated with all-cause mortality (HR per 1% increase in ECV: 1.26, CI: 1.06-1.50, p=0.009). Conclusion Nonischemic LGE in patients with structurally normal hearts is common and does not appear to be associated with adverse outcomes, whereas elevated ECV is associated with all-cause mortality and may be an important risk stratification tool.

Abstract 11836: A Rare Cause of Recurrent Myocardial Infarction: Genetic Testing Cracks the Case

A 29-year-old man presented to our institution with acute, severe substernal chest pain. An ECG on arrival revealed 3mm ST-elevations in the lateral leads with reciprocal depressions in the inferior leads. Troponin-I was above assay (&gt;50 ng/mL). Emergent coronary angiography showed normal coronary arteries, and left ventriculogram showed no wall motion abnormalities. A transthoracic echocardiogram showed normal biventricular size, wall thickness, and ejection fraction (EF). This was his fifth episode of chest pain over the past 10 years, consistent with prior presentations of ST-elevation myocardial infarction and angiographically normal coronary arteries. A prior cardiac magnetic resonance imaging (MRI, 6 years before the current presentation) revealed increased T2 signal intensity and subepicardial late gadolinium enhancement (LGE) in the septum, inferior, and inferolateral walls, suggestive of nonischemic acute myocardial injury. A repeat cardiac MRI during the current presentation showed mildly reduced biventricular systolic function (LVEF 52%, RVEF 36%), and again showed increased T2 signal intensity and subepicardial LGE involving the septum, inferior and inferolateral walls. The anterior and anterolateral walls were also now involved. Endomyocardial biopsy (EMB) showed focal interstitial fibrosis with no evidence of active myocarditis. On telemetry he was noted to have runs of non-sustained ventricular tachycardia (NSVT). Serologic evaluation was negative for parvovirus, cytomegalovirus, and Chagas disease. Erythrocyte sedimentation rate and C-reactive protein levels were normal. Genetic testing was performed at our newly established cardiovascular genetics program, which showed a pathogenic truncating mutation in the desmoplakin gene (DSP). DSP cardiomyopathy (CM) is an LV-predominant arrhythmogenic CM, which can present with episodic myocardial injury, and has a high disposition for ventricular arrhythmias with subepicardial LGE pattern often proceeding LV systolic dysfunction. A primary prevention implantable cardioverter-defibrillator was placed given extent of LGE and NSVT burden in the context of DSP variant. This case illustrates the importance of genetic testing in identifying rare CM phenotypes.

Abstract 16249: A Case of Myocarditis and Pneumonitis Associated With Pembrolizumab Therapy

Introduction: Immune checkpoint inhibitors (ICI) are a novel therapy that activates antitumor immunity but can have varied immune-related adverse effects. As ICI therapy is used to treat malignancy, cases of autoimmune myocarditis and heart failure increase, thought to be due to PD-1 inhibition, which may be potentially fatal. Case presentation: A 37 yo female with history of autoimmune hepatitis and stage III melanoma on Pembrolizumab (ninth cycle 1.5 months prior) presented with chest pain, tachycardia, fatigue, dyspnea, and fever. Lab studies were notable for elevated troponin, lactic acidosis, and disseminated intravascular coagulation (DIC). CT thorax showed asymmetric ground glass opacities, concerning for ICI pneumonitis. Echocardiogram showed an LVEF of 15% with global hypokinesis. She was treated for severe sepsis, DIC, and respiratory failure, temporarily requiring intra-aortic balloon pump. Heart failure guideline-directed medical therapy (GDMT) was started. Clinical presentation was concerning for acute myocarditis related to ICI therapy as a cause for new cardiomyopathy. A cardiac MRI (CMR) revealed subepicardial late gadolinium enhancement (LGE) along the basal inferior/inferolateral wall segments concerning for myocarditis. Corticosteroids with a prolonged taper were started. Endomyocardial biopsy of the septum (EMB) was negative. Her clinical status improved and repeat echo showed LVEF recovery up to 45%. Discussion: Endomyocardial biopsy (EMB) provides definitive diagnosis of myocarditis and is recommended (Class I) for new-onset heart failure with hemodynamic compromise and/or arrhythmias. Although our patient’s EMB was negative (likely due to limitation of sample location), she met the 2013 ESC guideline diagnostic criteria for myocarditis with elevated troponin, functional and structural abnormalities on cardiac imaging. With increased utilization of ICI therapy in the management of aggressive malignancies, it is important to monitor for potentially adverse effects such as autoimmune myocarditis. Standard treatment is prompt initiation of corticosteroids, though newer studies report successful treatment with immunomodulators, such as abatacept and ruxolitinib, if steroids are unsuccessful.

Eosinophilic myocarditis as presentation of hypereosinophilic syndrome.

A 67-year-old female with history of eosinophilic gastritis, was admitted with a first episode of heart failure (HF). The X-ray showed a bilateral interstitial infiltrate with a large left pleural effusion (Panel A), with abundant eosinophils in the pleural drainage and eosinophilia in blood stream (22 × 109, normal value 0,5–1 × 109). The transthoracic echocardiogram observed a normal left ventricular function (53%) (see Supplementary Video S1) with restrictive mitral filling pattern (Panel B) and a severely diminished global longitudinal strain of—9.6% (Panel C). The cardiac resonance (see Supplementary Video S2) revealed a septal intramyocardial and anterolateral subepicardial late gadolinium enhancement (Panels D and E) with normal right ventricle function, non-thickened pericardium, and no effusions. Due to high suspicious of a restrictive myocardiopathy (RMC) secondary to a hyperosinophilic syndrome, a right heart catheterization (RHC) and a cardiac biopsy were scheduled. The RHC was compatible with RMC (Panel F), with pulmonary hypertension (mean pulmonary pressure of 28 mmHg), ‘dip & plateau pattern’, and pressure equalization between chambers. Finally, the endomyocardial biopsy (Panel G) revealed an important infiltration of eosinophils in myocardial tissue, confirming the diagnosis of eosinophilic myocarditis (EM), in the context of hypereosinophilic syndrome. High doses of intravenous corticoids plus HF treatment were started, and the patient evolved favourably.

PO-01-110 ATRIAL TORSADES DE POINTES POST COVID MYOCARDITIS

Among patients with COVID-19 infection, the risk of adverse cardiovascular outcome, particularly myocarditis and dysrhythmias remain elevated at least up to one year after infection. We present a case of atrial tachycardia and atrial Torsades de Pointes from COVID myocarditis, persisted 6 months after infection, which was successfully managed by ablation. A 25-year-old female presented with mild COVID-19 infection, Omicron variant, in May 2022. One month after, her Covid infection resolved; she presented with symptomatic atrial tachycardia, paroxysmal atrial fibrillation and flutter. ECG showed multiple blocked premature atrial contractions (PAC) (Figure 1A). Holter monitor showed PAC triggered atrial tachycardia degenerating to paroxysmal atrial fibrillation, atrial Torsades de Pointes. She has mild persistent troponin elevation. Echocardiography was normal. Cardiac MRI showed evidence of mild myocarditis with subepicardial late Gadolinium enhancement (LEG) along the lateral mid-apical left ventricular wall and edema. (Figure 1B). She was treated with Colchicine for 2 months. Repeat cardiac MRI 4 months after COVID infection showed resolution of edema and LGE. However, her symptomatic PAC and atrial tachycardia did not respond to betablocker and amiodarone. She underwent electrophysiology study. Activation mapping of PAC using CARTO revealed earliest activation at the right anterior atrial wall, with close proximity to tricuspid valve; unipolar signal showed QS pattern, bipolar signal showed 16 msec pre-PAC (Figure 1C and 1D). Mechanical pressure from ThermoCool SmartTouch ablation catheter (Biosense Webster Inc.) at this site suppressed the PAC. Radiofrequency ablation resulted with an initial acceleration and then disappearance of the PAC. We did not isolate pulmonary veins or ablate cavotricuspid isthmus. Post ablation, PAC and atrial fibrillation were not inducible on Isoproterenol. N/A Covid myocarditis can result in dysrhythmia that lingers long after Covid myocarditis has resolved. Covid myocarditis can be caused by direct viral invasion of myocytes or more commonly is inflammatory related to cytokine release and edema. Our case demonstrates that dysrhythmias can persist despite resolution of myocarditis. Catheter ablation can successfully to treat these arrhythmias. This case highlights the importance of recognizing cardiac dysrhythmia as possible the long-term cardiac complications of COVID-19, requiring specific treatment such as catheter ablation.

Successful treatment of acute myocardial injury of Duchenne muscular dystrophy with steroids: a case report

BackgroundDuchenne muscular dystrophy (DMD) is an X-linked muscular disease which is caused by the absence of dystrophin. Troponin elevation with acute chest pain may indicate acute myocardial injury in these patients. We report a case of DMD that presented with ACP and troponin elevation, who was diagnosed with acute myocardial injury, and successfully treated with corticosteroids.Case presentationA 9-year-old with DMD was admitted to the emergency department with the complaint of acute chest pain. His electrocardiogram (ECG) revealed inferior ST elevation and serum troponin T was elevated. The transthoracic echocardiography (TTE) demonstrated inferolateral and anterolateral hypokinesia with depressed left ventricular function. An ECG-gated coronary computed tomography angiography ruled out acute coronary syndrome. Cardiac magnetic resonance imaging revealed mid-wall to sub-epicardial late gadolinium enhancement at the basal to the mid inferior lateral wall of the left ventricle and corresponding hyperintensity on T2-weighted imaging, consistent with acute myocarditis. A diagnosis of acute myocardial injury associated with DMD was made. He was treated with anticongestive therapy and 2 mg/kg/day of oral methylprednisolone. Chest pain resolved the next day, and ST-segment elevation returned to normal on the third day. Troponin T decreased in the sixth hour of oral methylprednisolone treatment. TTE on the fifth day revealed improved left ventricular function.ConclusionDespite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death in patients with DMD. Acute chest pain attacks with elevated troponin in patients with DMD without coronary artery disease may indicate acute myocardial injury. Recognition and appropriate treatment of acute myocardial injury episodes in DMD patients may delay the development of cardiomyopathy.

Long-term impact of COVID-19 on cardiovascular health of healthcare workers of a tertiary care hospital of Bangladesh

Background: The coronavirus disease (COVID-19) is a multisystem disease that sometimes affects the cardiovascular system (CVS) during acute illness and follows a protracted course in a proportion of patients causing ‘post-COVID syndrome’. Exercise tolerance test (ETT) can assess functional capacity and imaging modalities like echocardiography and cardiac magnetic resonance (CMR) can assess structural and functional status of cardiovascular system (CVS) efficiently. However, the pattern of cardiovascular involvement and its effect on functional capacity in COVID-19 survivors long after the index illness are inadequately known. This study was carried out to detect the functional status and imaging findings in COVID-19 recovered healthcare workers.&#x0D; Methods: This cohort study was carried out in the Department of Cardiology of the National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh from July 2021 to December 2021. Thirty male healthcare workers, previously not known to have any cardiac disease, who suffered from reverse transcriptase polymerase chain reaction (RT-PCR)-confirmed COVID-19 of mild severity at least 3 months back were included. Besides clinical evaluation, ETT, echocardiography and CMR were done.&#x0D; Results: The mean age was 37.2±4.7 years. The mean time interval from RT-PCR positivity was 317.4±85.7 days. The functional capacity was good in the study subjects; the achieved metabolic equivalents (METs) were 13.0±1.5. The mean left ventricular ejection fraction (LVEF) was 66.9% ± 6.2%; all were within the normal range. The mean LV global longitudinal strain (LVGLS) was -19.2% ± -1.9%; 6 patients had LVGLS &lt;-18%. One patient had mildly reduced tricuspid annular plane systolic excursion (TAPSE) of 16 mm but 3 patients had reduced lateral tricuspid annulus peak systolic velocity (RVS’). CMR revealed subtle abnormalities in 14 (46.7%) patients. Eight patients (26.67%) had increased T2 signal indicating myocardial oedema, 2 (6.7%) had subepicardial late gadolinium enhancement (LGE).&#x0D; Conclusion: In healthcare workers who recovered from mild COVID-19, despite preserved functional capacity, subclinical cardiac abnormalities detected by echocardiography and CMR may be present. Long-term follow-up is warranted to define the clinical significance of these findings.&#x0D; BIRDEM Med J 2023; 13(1): 3-11

291 BIVENTRICULAR ARRHYTMOGENIC CARDIOMYOPATHY: COULD ELECTROCARDIOGRAPHY SEE BEYOND THE CARDIAC MRI?

Abstract We described the case of a 34 year-old young athlete who was referred to our Department because of palpitations and fatigue, arising after hard effort but also present at rest. Family history was unremarkable for sudden cardiac death or cardiomyopathies. Electrocardiography (ECG) evidenced multifocal premature ventricular contractions (PVCs): left bundle branch block (LBBB) morphology and superior/inferior axis; right bundle branch block (RBBB) morphology and undetermined axis. Cardiac MRI showed extensive mid wall and subepicardial late gadolinium enhancement in the postero-lateral left ventricular wall. We decided to perform electroanatomic mapping (EAM) because we didn't explain the origin of the PVCs with LBBB configuration. During EAM we found an area extending from the right ventricular outflow tract to the subtricuspidal lateral inferior wall which it had not described by cardiac MRI. In the low voltage areas, the electrograms inscribed by the catheter's electrodes was fractionated with different waveforms, a finding that corresponds to a deficiency of healthy myocardial tissue. We performed an endomyocardial biopsy in the interventricular septum close to the ventricular out flow tract. The histological examination of the biopsy samples showed fibro-fatty replacement. Therefore genetic testing revealed a pathogenic desmoplakin mutation. According to “Padua Criteria”, we made a diagnosis of biventricular arrhythmogenic cardiomyopathy. We finally implanted a transvenous cardioverter defibrillator. Conclusion Diagnosis of arrhythmogenic cardiomyopathy (ACM) is complex and requires the combination of multiple clinical, histological and genetic parameters. This clinical case demonstrated that ECG represent an essential tool in diagnostic work-up of the disease, and electroanatomic mapping is useful in selected cases for the documentation of the diagnosis of arrhytmogenic cardiomyopathy.

433 A PRO-ARRHYTHMIC DOUBLE HIT

Abstract A 42-year-old man went to the emergency room complaining about dyspnea and palpitations. He reported an episode of atrial fibrillation (AF) with symptoms of dizziness 8 years before. He had a family history of ischemic cardiopathy, his father has suffered from AF since a young age.Acute course: The ECG revealed AF at high ventricular frequency. The echocardiography demonstrated mild reduction of the left ventricular function (EF 45%) with diffuse hypokinesis, and mitral valve prolapse (MVP) with mild mitral regurgitation. The blood tests showed a troponin peak of 750 ng/L (n.v. 0–34 ng/L). The suspect was acute coronary syndrome. Thus, he was treated with dual antiplatet therapy and low doses of beta-blockers, and he underwent coronarography, which demonstrated no significant lesions. So, after a transesophageal echocardiography that excluded thrombosis, and a loading dose of amiodarone, a successful electrical cardioversion was performed. The discharge diagnosis was AF with mild left ventricular disfunction. Three months later: He came back to the emergency room because of suffering some days of progressive epigastric and retrosternal pain radiating to the shoulders. The ECG and the echocardiography were unchanged, the troponin-I values were 79 ng/L-74 ng/L. Therefore, he underwent cardiac magnetic resonance (CMR), which showed normal biventricular dimensions and function (LVEF 64%, LVEDV 80 ml/mq), MVP with mitral annular disjunction (MAD) and curling of the infero-lateral basal wall (Figure A). The native T1, T2 and ECV values were increased, and there was a wide left ventricular subepicardial late gadolinium enhancement (LGE). The conclusion was MVP and active myocarditis. Moreover, he underwent endomyocardial biopsy that revealed focal lymphocytic infiltration, PCRs for common cardiotropic viruses or bacteria were negative. Six months later: The basal ECG was characterized by low QRS voltages in limb leads (Figure B). The CMR revealed a wide left ventricular non-ischemic fibrosis with a ring-like pattern (Figure C). The 24-hours Holter ECG showed premature ventricular beats with a right bundle branch block/superior and inferior axis morphology (Figure D), in keeping with MVP. The next step was the genetic testing that resulted positive for a Filamin-C (FLNC) mutation associated with cardiomyopathy. The final diagnosis was “Hot-phase of Left Ventricular Arrhythmogenic Cardiomyopathy (ACM), MVP and paroxysmal AF”. Discussion: The ACM is a rare hereditary heart muscle disease, and the “hot phase” represents its uncommon clinical presentation. Mutations in FLNC gene could be involved in the pathogenesis of ACM. Moreover, there are few cases in literature about the correlation between FLNC mutations and MVP. FLNC mutation may be a cross-sectional substrate for both these pro-arrhythmic conditions.

310 CLINICAL COURSE AND CHARACTERISTICS OF ADVANCED HEART FAILURE ASSOCIATED WITH ARRHYTHMOGENIC CARDIOMYOPATHY

Abstract Background The prevalence and course of heart failure (HF) in arrhythmogenic cardiomyopathy (ACM) is unresolved, and previous studies have mostly focused on the right-dominant variant of the disease, less prone to HF. Conversely, ACM variants with left ventricular (LV) involvement are now increasingly recognized, often initially ‘mis-diagnosed’ as dilated cardiomyopathy. Aim of this study was therefore to describe the prevalence and clinical course of advance HF in the full clinical spectrum of ACM. Methods We retrospectively reviewed records of all ACM patients diagnosed before 2021 from 3 Italian Cardiomyopathy Referral Centres (Azienda Ospedaliero Universitaria Careggi; Policlinico Sant’Orsola; Azienda Ospedaliero Universitaria Sant’Andrea). LV involvement was diagnosed in the presence of subepicardial late gadolinium enhancement (LGE) in at least 3 contiguous segments in the same short-axis slice at cardiac magnetic resonance, independent of whether it fulfilled the 2010 Task Force criteria for right-dominant ACM (biventricular ACM) or not (left-dominant ACM); in this latter case, diagnosis was reached after careful exclusion of other differential diagnosis, and only when at least one of the following features was present: likely pathogenic/pathogenic genetic variant associated with ACM; familial history of ACM; electrocardiographic abnormalities suggestive of ACM with LV involvement. Advanced HF was defined as NYHA functional class III/IV and/or referral for heart transplantation. Median follow-up was 6 years. Results Forty-four out of 174 ACM patients (25%) developed advanced HF: 10 right-dominant ACM and 34 with LV involvement. Twenty ACM patients were initially diagnosed with dilated cardiomyopathy; as such, development of advanced HF in 17 cases preceded the diagnosis of ACM. Five patients were diagnosed with ACM after heart transplantation, by histological examination. In the other 22, median time from ACM diagnosis to advanced HF development was 4 years. As compared to those without, advanced HF patients were more likely to present LV involvement. During clinical course, 20 (46%) advanced HF patients received at least one appropriate ICD intervention, with 13 experiencing an electrical storm. Twenty-six (59%) patients required HF-related hospitalization, and 32 (72%) were referred for heart transplantation with 25 ultimately receiving it. ACM patients with advanced HF, compared to those without, experienced a higher rate of mortality (36% vs. 6%; OR 3.5 [95%CI: 1.4-8.7], p=0.01) and ventricular arrhythmic events (41% vs. 11%; OR 2.4 [95%CI: 1.1-5.0], p=0.02). Conclusions Advanced HF progression in ACM is not rare, and occurs more frequently in variants with LV involvement. Advanced HF is associated with increased mortality and arrhythmic risk.

Cardiac adverse reactions of COVID-19 vaccination: cardiac MRI findings.

The rapid development of COVID-19 vaccines in the wake of the COVID-19 pandemic has led to an equally expediently deployed vaccination campaign with more than 12billion vaccinations administered worldwide. Reports of vaccine-associated adverse reactions (VAARs) have ranged from headaches and pain at the injection site to potentially life-threatening events such as cerebral venous sinus thrombosis. The heart has also not been spared of VAARs, as vaccine-associated myocardial infarction and more commonly, albeit still rare, myocarditis and perimyocarditis have been reported in predominantly young male recipients. Cardiac magnetic resonance imaging findings of vaccine-associated myocarditis such as prolonged T1 and T2 relaxation times, increased T2 signal intensity ratio, and subepicardial late gadolinium enhancement have been demonstrated to be similar to those in virus-induced myocarditis, enabling the use of the modified 2018 Lake Louise Criteria for diagnostic purposes to confirm vaccination-associated myocardial inflammation. Other reported cardiac findings such as cardiomyopathies and arrhythmias were confined to case reports. The incidence of myocardial infarction was not noted to be higher than in the overall population. The overall preliminary prognosis of vaccine- associated myocarditis seems to be good as suggested by initial reports, but long-term follow-up is needed to sufficiently assess possible sequelae and consequences.

Post COVID-19 cardiac magnetic resonance findings among inpatients vs outpatients

Abstract Background Myocardial injury and myopericarditis constitute an important complication after viral infection. The prevalence of myocardial injury among patients that survived COVID – 19 infections and its causes are still not clear. The purpose of this study is to estimate whether there is a difference in the prevalence of cardiac magnetic findings between patients treated in a hospital vs patients treated at an outpatient clinic. Methods We evaluated 360 cardiac magnetic resonance examinations, performed from 1st of June 2020 until the 31st of August, 2021. Out of them, 141 patients (39%) underwent cardiac magnetic resonance due to persistent symptoms after a SARS-CoV-2 infection. A conventional CMR protocol was performed to rule out myocarditis. Revised 2018 Lake Louise Criteria were used to diagnose myocarditis. All scans were performed by Phillips Medical Systems Ingenia 1.5T. T1 native values were estimated elevated when mapping values measured above 1030ms, T2 mapping values were estimated elevated when greater than 55 ms. Mid wall or subepicardial late gadolinium enhancement, pericardial effusions and extracardiac findings were evaluated. Chi-square test was used. Results Out of 141 patients, 78 patients (55%) had at least one cardiac magnetic resonance finding: either increased T1 (22%), T2 mapping (7%), T2 STIR (1.4%), left gadolinium enhancement (30%), small pericardial effusion (26%) or lung parenchymal changes (12%) after COVID-19. Twenty out of 141 patients (14%) fulfilled the criteria for myocarditis. Out of these 20 patients, 14 patients (70%) received treatment at an outpatient clinic, while 6 patients (30%) were treated from COVID-19 in a hospital (p&amp;lt;0.053). The most prevalent symptoms were effort intolerance and palpitations (50% and 26% respectively). There was no statistical difference in myocarditis prevalence, between hospitalized patients treated with or without corticosteroids (p=0.65), as well as between patients treated with hemodiafiltration (Oxiris filter) and patients without hemodiafiltration (p=0.95). Also, there was no statistical difference between T1 mapping among the inpatients and outpatients (p=0.58), as well as the severity of the clinical picture (p=0.72). There was no statistical difference between the in- and outpatient groups according to age (p=0.46). None of these patients had signs of fulminant myocarditis. Conclusion The prevalence of myopericardial and/or lung involvement after SARS-CoV-2 infection is present in every other cardiac magnetic resonance examination performed for persistent symptoms after a survived COVID-19. Myocarditis after SARS-CoV-2 infection develops regardless of the severity of the symptoms or the treatment method. We can conclude that we have to look for the reasons for myocarditis, beyond the clinical picture and the treatment strategies. Funding Acknowledgement Type of funding sources: None.

Characteristics and long-term survival of patients with left ventricular non-compaction cardiomyopathy.

Left ventricular non-compaction cardiomyopathy (LVNC) is a poorly understood entity resulting in heart failure. Whether it is a distinct form of cardiomyopathy or an anatomical phenotype is a subject of discussion. The current diagnosis is based on morphologic findings by comparing the compacted to non-compacted myocardium. The study aimed to compare demographic and prognostic variables of patients with dilated cardiomyopathy (DCM) and LVNC. Emphasis was given to cardiac magnetic resonance (CMR) imaging analysis. Data on survival were also assessed. We retrospectively evaluated the characteristics and outcomes of 262 non-ischaemic cardiomyopathy patients with LVNC and DCM phenotypes. Petersen's CMR criteria of non-compacted to the compacted myocardial ratio 2.3 were used to diagnose LVNC. The primary endpoint was a composite endpoint of major adverse cardiovascular events comprising cardiovascular-related death, left ventricular assisted device implantation, or heart transplantation. A total of 262 patients with CMR data were included in the study. One hundred fifty-five patients who fulfilled CMR criteria were diagnosed as LVNC. CMR findings revealed that LVNC patients had higher left ventricular end-diastolic (137.2±51.6, 116.8±44.6, P=0.002) and systolic volume index (98.4±49.5, 85.9±42.7, P=0.049). Cardiac haemodynamics, cardiac output (5.61±2.03, 4.96±1.83; P=0.010), stroke volume (73.9±28.8, 65.1±25.1; P=0.013), and cardiac index (2.85±1.0, 2.37±0.72; P<0.0001), were higher in LVNC patients. Of all the 249 patients, 102 (40.9%) patients demonstrated late gadolinium enhancement (LGE). According to Petersen's criteria, the Kaplan-Meier survival outcome did not reveal significant differences (hazard ratio [HR]: 1.53, 95% confidence interval [CI]: [0.89-2.63], P=0.11). The presence or pattern of LGE did not show significant importance for endpoint-free survival. Most of the sub-epicardial LGE pattern was found in LVNC patients (94.4%). When receiver operator characteristics analysis was applied to NC/C ratio to discriminate the primary endpoint, a higher NC/C ratio of 2.57 was associated with adverse events (HR: 1.90, 95% CI: [1.12-3.24], P=0.016). Our study questions the criteria being used for the diagnosis of LVNC. Further evaluation of CMR variables and association of these findings with demographic variables and survival is mandatory.