Mid-wall Late Gadolinium Enhancement Research Articles

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.

Incremental prognostic value of late gadolinium enhancement extent, transmurality, and pattern in patients with ischaemic cardiomyopathy

Abstract Background Presence and extent of late gadolinium enhancement (LGE) using cardiac magnetic resonance (CMR) are strong prognosticators of outcomes in ischaemic cardiomyopathy (ICM), but the prognostic value of multiple location of LGE, LGE transmurality, and midwall LGE area in addition to ischaemic LGE is not well established. Purpose To assess the incremental prognostic value of presence, extent, transmurality, and pattern of LGE in patients with ICM and reduced left ventricular ejection fraction (LVEF)­­. Methods Between 2008 and 2022, all consecutive patients referred for myocardial viability assessment using CMR and a history of ICM (≥70% stenosis in ≥1 epicardial coronary vessel on angiography and/or history of myocardial infarction and/or coronary revascularization) and LVEF value <50% were prospectively recruited. The outcome was all-cause death using the electronic French National Registry of Death. Nested Cox proportional hazard models were used to assess the prognostic value of the following LGE features: the extent, the transmurality of subendocardial ischaemic LGE (<50%, ≥50%, or transmural), the presence of LGE in multiple areas, and the presence of midwall LGE. The additional predictive value of the LGE features was assessed by the C-statistic increment, the continuous net reclassification improvement (NRI), the integrative discrimination index (IDI) and the global Chi-2. Results Among the 6,082 included patients (mean age 65±12 years, 73% male, mean LVEF 43.8±5.7%), 3,591 (59%) had ischaemic LGE; 652 (10.7%) patients died during a median follow up of 9 [6.6-11.5] years. After adjustment for risk factors in the overall population, the presence of ischaemic LGE was associated with a higher risk of death (adjusted HR=3.57; 95%CI: 2.89-4.40, p<0.001). Figure 1 shows survival according to the different LGE features. Patients with both multiple localisations of ischaemic LGE area and additional LGE midwall exhibited the highest risk of death (p<0.001). After adjustment in the subgroup of patients with ischaemic LGE (n=3,591), LGE extent (HR=1.55; 95%CI: 1.47-1.63), multiple localisations of ischaemic LGE (HR 1.61 95%CI: 1.32-1.97), LGE transmurality (HR=4.48; 95%CI: 3.39-5.93), and presence of additional midwall LGE (HR=1.77; 95%CI: 1.44-2.17, all p<0.001) were independently associated with a higher risk of death. A model combining all LGE features showed the best improvement in model discrimination and reclassification above traditional factors (C-statistic improvement: 0.17; NRI=54.1%; IDI=12.6%, Chi-2 global=1133, all p<0.001; LR-test p<0.001, Figure 2). Conclusion In a large cohort of ICM patients with reduced LVEF, ischaemic LGE extent, transmurality, multiple ischaemic LGE localisations, and additional presence of midwall LGE were independent prognosticators of death. A model combining all these LGE features had an incremental prognostic value over traditional prognostic factors to predict all-cause death.Kaplan-Meier survival curvesIncremental prognostic value

A high-risk late gadolinium enhancement pattern is associated with arrhythmic events in patients with dilated cardiomyopathy

Abstract Introduction Myocardial fibrosis constitutes a substrate for the development of ventricular arrhythmias in patients with non-ischaemic dilated cardiomyopathy (DCM). Late gadolinium enhancement (LGE)-cardiac magnetic resonance (CMR) imaging represents a powerful tool for arrhythmic risk stratification. Moreover, arrhythmic risk is determined by the extent and localization of LGE. A high-risk LGE pattern, defined as the presence of epicardial, transmural or septal plus free-wall LGE, has been recently associated with a higher incidence of arrhythmic events comparing with other LGE patterns. Purpose To evaluate the prevalence of a high-risk LGE pattern in patients with DCM and its association with arrhythmic events. Methods From 2014 to 2021, all patients (N = 229) with DCM were prospectively evaluated in our tertiary care hospital. 174 patients underwent a 1.5 Tesla scanner CMR as part of the diagnostic workup and were included in the present study. The primary endpoint was a composite of sudden cardiac death or sustained monomorphic ventricular tachycardia. Results Mean age of our cohort was 61 years (standard deviation 14.5), and 64.2% were male. Median follow-up was 41 (interquartile range 26-62) months. LGE was present in 96 (55.2%) patients of our cohort (LGE+), and a high risk LGE pattern was found in 41 (42.7% of all LGE+ patients). LGE was more frequently located in the basal segments (78.6%). Mid-wall septal LGE was found in 46.9% of LGE+ patients, and it was the most common pattern. 21.4% of LGE+ patients had combined septal and free-wall LGE, 23.5% had epicardial LGE and 13.3% had transmural LGE. Baseline characteristics of LGE+ patients with and without a high-risk LGE pattern are shown at the Table. The incidence of ventricular arrhythmic events during follow-up was 31.7% in patients with a high-risk LGE pattern, and 9.1% in LGE+ patients but without a high-risk pattern (p=0.005). In patients without a high-risk LGE, including LGE- patients and LGE+ patients with other LGE patterns, incidence of ventricular arrhythmic events was 8.3% (p<0.001). The survival free curves for the combined arrhythmic endpoint comparing LGE+ patients with and without a high-risk pattern are shown in the Figure. Conclusion The presence of a high-risk LGE pattern in patients with DCM its associated with a higher incidence of ventricular arrhythmic events, comparing with LGE+ patients without a high-risk pattern. We suggest that this high-risk pattern should be taken into account when performing arrhythmic risk stratification in patients with DCM.TableFigure

Cardiac Magnetic Resonance in Patients with Suspected Tachycardia-Induced Cardiomyopathy: The Impact of Late Gadolinium Enhancement and Epicardial Fat Tissue.

Tachycardia-induced cardiomyopathy (TIC) is a reversible subtype of dilated cardiomyopathy (DCM) resulting from sustained supraventricular or ventricular tachycardia and diagnosed by the normalization of left ventricular ejection fraction (LVEF) after stable sinus rhythm restoration. The aim of this study was to determine the contribution of cardiac magnetic resonance (CMR) to the differential diagnosis of TIC and DCM with persistent atrial arrythmias in patients hospitalized for the first time with heart failure (HF) with reduced LVEF of nonischemic origin. A total of 29 patients (age: 58.2 ± 16.9 years; males: 65.5%; average EF: 37.0 ± 9.5%) with persistent atrial tachyarrhythmia and first decompensation of HF without known coronary artery diseases were included in this study. The patients successfully underwent cardioversion and were observed for 30 days. The study population was divided into groups of responders (TIC patients; N = 16), which implies achieving FF > 50% or its increase > 10% in 30 days of TIC, and non-responders (N = 13). The increase in left ventricle (LV) volumes measured using CMR was significantly higher in the non-responder group when compared with the responders (114.8 mL ± 25.1 vs. 68.1 mL ± 10.5, respectively, p < 0.05). Non-responders also demonstrated decreased interventricular septum thickness (9.1 ± 0.8 vs.11.5 ± 1.3, respectively, p < 0.05). Late gadolinium enhancement (LGE) was observed in 12 patients (41.4%). The prevalence of LGE was increased in the non-responder group (25.0% vs. 65.1%, respectively, p = 0.046). Notably, a septal mid-wall LGE pattern was found exclusively in the non-responders. Epicardial adipose tissue thickness was decreased in the non-responder group versus the TIC patients. Conclusion: Patients with TIC were found to have smaller atrial and ventricular dimensions in comparison to patients with DCM. In addition, LGE was more common in DCM patients.

Circulating biomarker- and magnetic resonance-based nomogram predicting long-term outcomes in dilated cardiomyopathy.

Dilated cardiomyopathy (DCM) has a high mortality rate and is the most common indication for heart transplantation. Our study sought to develop a multiparametric nomogram to assess individualized all-cause mortality or heart transplantation (ACM/HTx) risk in DCM patients. The present study is a retrospective cohort study. The demographic, clinical, blood test, and cardiac magnetic resonance imaging (CMRI) data of DCM patients in the tertiary center (Fuwai Hospital) were collected. The primary endpoint was ACM/HTx. The least absolute shrinkage and selection operator (LASSO) Cox regression model was applied for variable selection. Multivariable Cox regression was used to develop a nomogram. The concordance index (C-index), area under the receiver operating characteristic curve (AUC), calibration curve, and decision curve analysis (DCA) were used to evaluate the performance of the nomogram. A total of 218 patients were included in the present study. They were randomly divided into a training cohort and a validation cohort. The nomogram was established based on eight variables, including mid-wall late gadolinium enhancement, systolic blood pressure, diastolic blood pressure, left ventricular ejection fraction, left ventricular end-diastolic diameter, left ventricular end-diastolic volume index, free triiodothyronine, and N-terminal pro-B type natriuretic peptide. The AUCs regarding 1-year, 3-year, and 5-year ACM/HTx events were 0.859, 0.831, and 0.840 in the training cohort and 0.770, 0.789, and 0.819 in the validation cohort, respectively. The calibration curve and DCA showed good accuracy and clinical utility of the nomogram. We established and validated a circulating biomarker- and CMRI-based nomogram that could provide a personalized prediction of ACM/HTx for DCM patients, which might help risk stratification and decision-making in clinical practice.

Septal Midwall Late Gadolinium Enhancement in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy—Characteristics and Prognosis

Septal midwall late gadolinium enhancement (LGE) is a characteristic finding on cardiac magnetic resonance imaging (CMR) in nonischemic dilated cardiomyopathy (DCM) and is associated with adverse events. Its significance in ischemic cardiomyopathy (ICM) is unknown. With this multicenter observational study, we aimed to study the characteristics of septal midwall LGE and evaluate its prognostic value in ICM. A total of 1,084 patients with an impaired left ventricular (LV) ejection fraction (<50%) on LGE-CMR, either because of ICM (53%) or DCM, were included retrospectively. Septal midwall LGE was defined as midmyocardial stripe-like or patchy LGE in septal segments and was present in 10% of patients with ICM compared with 34% of patients with DCM (p<0.001). It was significantly associated with larger LV volumes and lower LV ejection fraction, irrespective of etiology. The primary endpoint was all-cause mortality and secondary endpoint was ventricular arrhythmias (VAs), including resuscitated cardiac arrest, sustained VA, and appropriate implantable cardioverter-defibrillator (ICD) therapy. During a median follow-up of 2.7years, we found a significant association between septal midwall LGE and mortality in patients with DCM (hazard ratio [HR] 1.92, p=0.03), but not in patients with ICM (HR 1.35, p=0.39). Risk of VAs was significantly higher in patients with septal midwall LGE on CMR, both in DCM (HR 2.80, p<0.01) and in ICM (HR 2.70, p<0.01). In conclusion, septal midwall LGE, typically seen in DCM, was also present in 10% of patients with ICM and was associated with increased LV dilation and worse function, irrespective of etiology. When present, septal midwall LGE was associated with adverse outcome.

Cardiac magnetic resonance findings and prognosis in type 1 myotonic dystrophy.

Cardiac involvement is a major determinant of prognosis in type 1 myotonic dystrophy (DM1), but limited information is available about myocardial remodeling and tissue changes. The aim of the study was to investigate cardiac magnetic resonance (CMR) findings and their prognostic significance in DM1. We retrospectively identified all DM1 patients referred from a neurology unit to our CMR laboratory from 2009 to 2020. Thirty-four patients were included (aged 45 ± 12, 62% male individuals) and compared with 68 age-matched and gender-matched healthy volunteers (43 male individuals, age 48 ± 15 years). At CMR, biventricular and biatrial volumes were significantly smaller (all P < 0.05), as was left ventricular mass (P < 0.001); left ventricular ejection fraction (LVEF) and right ventricular ejection fraction (RVEF) were significantly lower (all P < 0.01). Five (15%) patients had a LVEF less than 50% and four (12%) a RVEF less than 50%. Nine patients (26%) showed mid-wall late gadolinium enhancement (LGE; 5 ± 2% of LVM), and 14 (41%) fatty infiltration. Native T1 in the interventricular septum (1041 ± 53 ms) was higher than for healthy controls (1017 ± 28 ms) and approached the upper reference limit (1089 ms); the extracellular volume was slightly increased (33 ± 2%, reference <30%). Over 3.7 years (2.0-5.0), 6 (18%) patients died of extracardiac causes, 5 (15%) underwent device implantation; 5 of 21 (24%) developed repetitive ventricular ectopic beats (VEBs) on Holter monitoring. LGE mass was associated with the occurrence of repetitive VEBs (P = 0.002). Lower LV stroke volume (P = 0.017), lower RVEF (P = 0.016), a higher LVMi/LVEDVI ratio (P = 0.016), fatty infiltration (P = 0.04), and LGE extent (P < 0.001) were associated with death. DM1 patients display structural and functional cardiac abnormalities, with variable degrees of cardiac muscle hypotrophy, fibrosis, and fatty infiltration. Such changes, as evaluated by CMR, seem to be associated with the development of ventricular arrhythmias and a worse outcome.

A Young Patient Presenting with Dilated Cardiomyopathy and Renal Infarction during Treatment with Isotretinoin: Mere Coincidence or Serious Side Effect of a Drug Commonly Used in Adolescence?

Isotretinoin or 13-cis-retinoic acid (RA) is one of the most effective and widely used drugs for the treatment of severe acne vulgaris. Despite being deemed safe, no definite consensus has been reached on the cardiovascular risk of RA derivatives. We report a case of heart failure due to dilated cardiomyopathy (DCM) and concomitant renal infarction occurring after 5 months of isotretinoin use in a previously healthy 18-year-old male. The patient, with a history of acne vulgaris, presented to our emergency department with left iliac fossa pain and effort dyspnea. A trans-thoracic echocardiogram showed DCM and severely reduced left ventricle ejection fraction (LVEF: 29%). During hospitalization, a total body computed tomography (CT) showed an ischemic lesion in the left kidney. Ischemic, autoimmune, infective, and heritable causes of DCM were ruled out. Cardiac magnetic resonance (CMR) evidenced LV circumferential mid-wall late gadolinium enhancement. Heart failure therapy was promptly started and up-titrated, but only poor LVEF improvement was detected overtime. Our case aims to raise awareness on rare life-threatening cardiovascular events possibly associated with isotretinoin use. To the best of our knowledge, this is the first described case of renal thromboembolism and severe DCM leading to implantable cardioverter-defibrillator (ICD) implantation occurring during isotretinoin treatment.

Pathophysiologic mechanism for MYOcarditis in COVID-19 VAccinations ("MYOVAx" Study)

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): NMRC COVID-19 Research Fund Objective This is the first prospective cohort study in Singapore to investigate the COVID-19 vaccine-associated myocarditis to understand its pathophysiology. Introduction Acute myocarditis and other cardiovascular symptoms have been observed to be associated with the two mRNA-based coronavirus disease 2019 (COVID-19) vaccines—namely Pfizer-BioNTech BNT162b2 and Moderna mRNA-1273)—currently in-use in Singapore. The mechanisms through which myocarditis occurs are unknown, hence our study aims to understand the pathophysiology of myocarditis associated with COVID-19 vaccines. Methods Patients with onset of cardiac manifestations were recruited from multiple hospital outpatient clinics between November 2021 and September 2022. Clinical history and physical examination data was collected with blood sample collection, echocardiography, 12-lead electrocardiogram (ECG), coronary angiography and magnetic resonance imaging (MRI) at recruitment and 6-month follow-up. Analysis of biomarkers, genetic, serological and MRI data was conducted. Results As of 6 September 2022, a total of 5 patients have been enrolled (4 males, 1 female). The most commonly reported symptoms across all patients were chest pain/discomfort (80%), followed by palpitations (40%). MRI evidence of myocarditis has been detected in 2 (50%) of the male patients, of which both reported two or more symptoms occurring 1-2 days post-vaccination. Both patients have each received at least two doses of either the Pfizer-BioNTech BNT162b2 vaccine or Moderna mRNA-1273 vaccine. Their MRI findings were consistent with myocarditis. On late gadolinium enhancement (LGE) imaging, epicardial enhancement at the basal inferolateral segment and mid-wall enhancement at the apical anterior, lateral and inferior walls were observed in one patient. Patchy, mid-wall LGE in the basal inferior/inferolateral wall was observed in the other patient. No MRI evidence of myocarditis was available for the sole female patient. Conclusion While more data is needed to definitively prove the association of the two mRNA-based Pfizer-BioNTech BNT162b2 and Moderna mRNA-1273 COVID-19 vaccines with post-vaccination myocarditis, we believe our findings may support further investigations to enable risk stratification for vaccine-associated myocarditis and identify potential preventative strategies accordingly.

240 A PEDIATRIC CASE OF BIVENTRICULAR END-STAGE ARRHYTHMOGENIC CARDIOMYOPATHY

Abstract Background Arrhythmogenic cardiomyopathy (ACM) is a rare heart muscle disease characterized by cardiomyocyte necrosis and fibro-adipose substitution which contribute both to the genesis of malignant ventricular arrhythmias and the morpho-functional alterations of the ventricles. ACM has always been considered a right ventricular disease with secondary involvement of the left ventricle, but recently a left dominant phenotype has been also described. Case report A 14-year-old patient was admitted to our hospital after out-of-hospital cardiac arrest due to ventricular fibrillation, which occurred when she was at rest. A family history of sudden death and dilated cardiomyopathy was retrieved (her brother died at the age of 18 with a dilated heart), but other family members had never been screened to date. Cardiac magnetic resonance imaging (cMRI) appearance was consistent with biventricular dilatation along with severe systolic dysfunction (left ventricular ejection fraction 20%; right ventricular ejection fraction 25%). It showed also thinning and bulging of the basal free wall of the right ventricle proximal to the outflow tract (up to 4 mm) and multiple foci of subepicardial, mid-wall and transmural late gadolinium enhancement (LGE) in both ventricles. Signs of acute decompensated heart failure and a high arrhythmic burden were present. After cardiopulmonary stabilization, she underwent the implantation of a cardioverter-defibrillator (ICD). Conclusion We herein report an uncommon case of biventricular AC (‘definite diagnosis’ according to both Task force criteria 2010 and Padua criteria) with advanced morpho-functional dysfunction occurring already in pediatric age, without any alerting symptom until the cardiac arrest (the typical onset of the ACM in children). It shows how severe biventricular involvement may affect young patients with a rapid progression of the disease. It also stresses the need for a strict follow-up of the family members of a proband since his first diagnosis, highlighting the pivotal role of cMRI in the diagnosis of pediatric disease. This has not been thoroughly studied and lacks sensitive and specific diagnostic criteria.

Abstract 12981: Non-Conventional Causes of ST-Elevation Myocardial Infarction: Essential Thrombocytosis

Case: A 61-year-old female with no significant past medical history, except hospitalization for COVID-19 pneumonia complicated by myocarditis ten months prior, presented to the emergency department with worsening chest pain over the last two weeks. The admission electrocardiogram (ECG) was without ischemic changes. The initial impression was that the patient's symptoms were attributed to her prior myocarditis. However, a repeat ECG obtained six hours later, due to worsening chest pain, demonstrated ST-segment elevations in leads V2, I and AVL. Emergent coronary angiography was performed, showing a 90% occlusion of the proximal LAD, which was managed with a drug-eluting stent. Admission platelet count was significantly elevated at 1,049,000mm 3 , which resulted in a hematology consultation. Her further work up included a positive JAK2-V617F mutation and negative BCR-ABL1 translocation, which confirmed the diagnosis of essential thrombocythemia. During the prior hospitalization for her COVID pneumonia, she underwent invasive coronary angiogram for suspected myocardial infarction in the setting of elevated troponin, which showed a mild, non-obstructive lesion in the proximal LAD. She underwent a cardiac MRI that demonstrated both sub-epicardial and mid-wall late gadolinium enhancement consistent with myocarditis. Discussion: Essential thrombocythemia is a clonal myeloproliferative disease caused by a pathological expansion of megakaryocytes, which results in excess platelet production. Essential thrombocythemia leads to a hematologic hyper-viscosity and subsequent increased risk of vasomotor, thrombotic and hemorrhagic manifestations. Myocardial infarction as the primary clinical presentation of essential thrombocythemia is rare but an important risk factor that should be considered in patients presenting with acute coronary syndromes and elevated platelet counts, both with and without traditional cardiovascular risk factors.

Abstract 15052: A Young Patient Who Presented With Dilated Cardiomyopathy and Renal Infarction During Treatment With Isotretinoin

Background: Isotretinoin or 13-cis-retinoic acid (RA) is one of the most effective and widely used drug for the treatment of severe acne vulgaris. Despite deemed safe, no definite consensus has been reached on the cardiovascular risk of RA derivatives. We describe the first case of concomitant dilated cardiomyopathy (DCM) and renal infarction after 5 months of isotretinoin use in a previously healthy 18-years old man. Case Summary: An18-years-old man, with history of acne vulgaris, presented in August 2021 to our emergency department because of left iliac fossa pain and effort dyspnea. Computed tomography scan revealed left renal infarction and echocardiogram demonstrated global left ventricular (LV) dilatation with severely reduced LV-ejection fraction (LVEF: 29%). Coronary artery disease, autoimmune, infective, or heritable causes of DCM were ruled out. Cardiac magnetic resonance evidenced LV circumferential mid-wall late gadolinium enhancement and ECG monitoring revealed several non-sustained ventricular tachycardia episodes. Thus, Bisoprolol, Sacubitril/Valsartan, and Eplerenone were started and up titrated until maximum tolerated doses, with only poor LVEF improvement. Conclusion: Our case aims to raise awareness on rare life-threatening cardiovascular events associated with isotretinoin use for the treatment of severe acne in young patients. To the best of our knowledge, this is the first described case of renal thromboembolism and DCM requiring S-ICD implantation and cardiac transplant program listing occurring in the course of isotretinoin treatment.

Impact of late gadolinium enhancement extent, location, and pattern on ventricular tachycardia and major adverse cardiac events in patients with ischemic vs. non-ischemic cardiomyopathy.

Left ventricular late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) has been associated with increased risk for life-threatening ventricular tachyarrhythmias. The differences in association between LGE characteristics and prognosis in patients with ischemic (ICM) vs. non-ischemic (NICM) cardiomyopathy is incompletely understood. A total of 168 consecutive patients who underwent CMR imaging with either ICM or NICM were included in our study. LGE extent, location and pattern were examined for association to the primary endpoint of ventricular tachycardia (VT) and secondary endpoint of major adverse cardiac events (MACE). Of 68 (41%) patients with ICM and 97 (59%) patients with NICM, median LGE mass was 15% (IQR 9-28) for the ICM group and 10% (IQR 6-15) for the NICM group. On multivariate analysis for both groups, LGE characteristics were prognostic while LVEF was not. In patients with ICM, septal and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median) and inferior wall LGE independently predicted the primary endpoint. In patients with NICM, anterior, inferior and apical segment LGE, and involvement of multiple walls predicted both endpoints on multivariate analysis. LGE extent (≥median, number of LGE segments, LGE stratified per 5% increase) and midwall LGE were independent predictors of the primary endpoint. Although LGE was an independent predictor of prognosis in both groups, LGE extent, location, and pattern characteristics were more powerful correlates to worse outcomes in patients with NICM than ICM.

Prognostic Value of Late Gadolinium Enhancement in Left Ventricular Noncompaction: A Multicenter Study.

Current diagnostic criteria for left ventricular noncompaction (LVNC) may be poorly related to adverse prognosis. Late gadolinium enhancement (LGE) is a predictor of major adverse cardiovascular events (MACE), but risk stratification of LGE in patients with LVNC remains unclear. We retrospectively analyzed the clinical and cardiovascular magnetic resonance (CMR) data of 75 patients from three institutes and examined the correlation between different LGE types and MACE based on the extent, pattern (including a specific ring-like pattern), and locations of LGE in LVNC. A total of 51 patients (68%) presented LGE. A specific ring-like pattern was observed in 9 (12%). MACE occurred in 29 (38.7%) at 4.3 years of follow-up (interquartile range: 2.1–5.7 years). The adjusted hazard ratio (HR) for patients with ring-like LGE were 6.10 (95% CI, 1.39–26.75, p < 0.05). Free-wall or mid-wall LGE was associated with an increased risk of MACE after adjustment (HR 2.85, 95% CI, 1.31–6.21; HR 4.35, 95% CI, 1.23–15.37, respectively, p < 0.05). The risk of MACE in LVNC significantly increased when the LGE extent was greater than 7.5% and ring-like, multiple segments, and free-wall LGE were associated with MACE. These results suggest the value of LGE risk stratification in patients with LVNC.

Late gadolinium enhancement patterns in severe symptomatic high-gradient aortic stenosis

Abstract Background Left ventricular (LV) remodeling in patients with severe aortic valve stenosis (AS) is a complex process that goes beyond hypertrophic response and may involve reparative/replacement fibrosis. Currently, cardiac magnetic resonance (CMR) is the gold-standard imaging technique for detecting focal myocardial fibrosis through late gadolinium enhancement (LGE). However, myocardial fibrosis prevalence and distribution is quite variable among series. Our goal was to assess LGE prevalence and distribution pattern in severe symptomatic high-gradient AS. Methodology Single-center prospective cohort of 132 patients with severe symptomatic high-gradient AS (mean age 73±11 years; 48% male, mean valvular transaortic gradient 60±20 mmHg; mean aortic valve area 0.7±0.2 cm2/m2; mean LV ejection fraction by 2D echocardiogram 58±9%), all with normal flow (except one) undergoing surgical aortic valve replacement. Those with previous history of acute myocardial infarction, ischemic cardiomyopathy or other cardiomyopathy were excluded. All patients performed 1.5T CMR assessment with LV myocardium tissue characterization prior to surgery. Segmental LGE presence was assessed by two independent operators and classified according to the AHA 16 segment model, using 5-standard deviations from remote myocardium as the signal intensity cut-off for LGE identification and quantification. Results Overall, 96 patients (74%) had non-ischemic LGE (median LGE mass 3.2 g [IQR 0.2–8.3] g; median percentage of LGE myocardial mass 2.5% [IQR 0.1–6.1]%); 22 patients [17%] with exclusively junctional LGE); in one patient an incidental ischemic scar (subendocardial distribution) was identified. No cases of subepicardial distribution were found. Intramyocardial LGE was most frequently observed in basal and mid-anterior and inferior interventricular septum – see Figure 1. In these segments, LGE was most often junctional at right-ventricular insertion points (54%), followed by mid-wall LGE (32%) or both sites involvement (14%). Conclusion LGE is frequent in symptomatic high-gradient AS patients with preserved left ventricular ejection fraction, most often presenting as junctional enhancement in basal/mid-anterior and inferior interventricular septum. Future studies may address whether distinct LGE patterns may impact patient prognosis. Funding Acknowledgement Type of funding sources: None.

CMR-driven immunosuppressive therapy in systemic sclerosis patients with suspected myocarditis: a single-center experience

Abstract Background Systemic sclerosis (SSc) myocarditis is common, but often underrecognized due to absence of symptoms at early stages. Cardiac Magnetic Resonance (CMR) is the imaging modality of choice for detection of SSc myocarditis. Current guidelines recommend CMR in case of alterations in first level cardiologic diagnostic tests. Immunosuppressive therapy is only indicated for patients with organ involvement, i.e. most frequently heart and lung, or with diffuse cutaneous systemic sclerosis. Purpose To explore the impact of CMR in the diagnostic and therapeutic pathway of SSc patients. Methods Of 286 SSc patients referred to our hospital, we retrospectively enrolled patients who received CMR during an observational period of 6 years (2016–2022). Exclusion criteria were previous immunosuppressive therapy and cardiopathy from other cause. Clinical data including Holter-ECG performed within 90 days from CMR were available. Results 39 patients (67 [58–72] years; females 87%) were identified. 15 patients were excluded due to previous immunosuppressive therapy (9), lack of information on therapy (3), cardiopathy from other causes (3). 24 patients were finally included in the study. 14 patients (58%) did not show myocardial inflammation at CMR (LVEF 65.5 [61–69]%, LVEDV 114.5 [92–141] ml, T2 ratio 1.6 [1.4–1.9]). 1 patient presented with mid-wall inferior late gadolinium enhancement (LGE) indicative of previous myocarditis. 1 patient received immunosuppressive therapy due to SSc lung involvement in follow-up. In this group premature ventricular contractions (PVCs) during 24-hours Holter-ECG were rare (29 [3–48]). When measured, high-sensitivity Troponin I (hsTnI) was 2.4 [0.8–5] ng/L. 10 patients (42%) showed myocardial inflammation at CMR: LVEF 60 [56–65] %, LVEDV 137,5 [122–166] ml, T2 ratio 2.1 [1.7–2.3], T1 mapping 1022.5 [1010–1036] msec and T2 mapping 50.5 [50–52] msec. Myocardial LGE with non-ischemic pattern was seen in 6 patients. The number of PVCs at 24-hours Holter-ECG was collectively higher in these patients (354 [1–4245]). When measured, hsTnI was 8 [6–18] ng/L. With detection of myocardial inflammation at CMR all 10 patients eventually received immunosuppressive therapy (mycophenolate mofetil (MMF) or azathioprine in case of MMF intolerance). At follow-up CMR performed 4 [3–6] months later on therapy various parameter differences were observed: LVEF (+2 [−2; 4] %), T1 mapping (−23 [−27; −12] msec), T2 mapping (−2 [−4; −1] msec), T2 ratio (−0.5 [−0.6; −0,2]). Of 6 patients with LGE, 4 displayed LGE persistence at follow-up. Conclusions In our experience, CMR was the tool of choice for diagnosis and therapeutic management in SSc patients with suspected cardiac involvement. SSc myocarditis patients tended to display more arrhythmic burden at Holter-ECG and higher cardiac biomarkers. Our data suggest to intensify first level cardiological screening, in order to identify suitable candidates to CMR. Funding Acknowledgement Type of funding sources: None.

Global longitudinal strain by CMR improves prognostic stratification in acute myocarditis presenting with normal LVEF.

Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization. Left ventricular peak global longitudinal strain (LV-GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF. Data of patients undergoing cardiac magnetic resonance (CMR) for clinically suspected AM in seven European Centres (2013-2020) were retrospectively analysed. Patients with AM confirmed by CMR and LVEF ≥50% were included. LGE was visually characterized: localized versus. non-localized, subepicardial versus midwall. LV-GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including cardiac death, life-threatening arrhythmias, development of heart failure or of LVEF <50%. Of 389 screened patients, 256 (66%) fulfilled inclusion criteria: median age 36 years, 71% males, median LVEF 60%, median LV-GLS -17.3%. CMR was performed at 4 days from hospitalization. At 27 months, 24 (9%) patients experienced ≥1 ACE (71% developed LVEF <50%). Compared to the others, they had lower median LV-GLS values (-13.9% vs. -17.5%, p=.001). At Kaplan-Meier analysis, impaired LV-GLS (both considered as > -20% or quartiles), non-localized and midwall LGE were associated with ACEs. Patients with LV-GLS ≤-20% did not experience ACEs. LV-GLS remained associated with ACEs after adjustment for non-localized and midwall LGE. In AM presenting with LVEF ≥50%, LV-GLS provides independent prognostic value over LGE characterization, improving risk stratification and representing a rationale for further studies of therapy in this cohort.

Global longitudinal strain by CMR improves prognostic stratification in acute myocarditis presenting with normal LVEF

Abstract Funding Acknowledgements Type of funding sources: None. Background Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization (1). Purpose Left ventricular peak global longitudinal strain (LV-GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF (2,3). Methods Data of patients undergoing cardiac magnetic resonance (CMR) for clinically suspected AM in seven European Centres (2013-2020) were retrospectively analysed. Patients with AM confirmed by CMR and LVEF ≥50% were included. LGE was visually characterized: localized vs. diffuse, subepicardial vs midwall. LV-GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including cardiac death, life-threatening arrhythmias, development of heart failure or of LVEF &amp;lt;50%. Results Of 389 screened patients, 256 (66%) fulfilled inclusion criteria: median age 36 years, 71% males, median LVEF 60%, median LV-GLS -17.3%. CMR was performed at 4 [2-12] days from hospitalization. At 27 months, 24 (9%) patients experienced ≥1 ACE (71% developed LVEF &amp;lt;50%). Compared to the others, patients experiencing ACEs had lower median LV-GLS values (-13.9% vs -17.5%, p=0.001). At Kaplan-Meier analysis, impaired LV-GLS (both considered as &amp;gt;-20% or quartiles), diffuse and midwall LGE were associated with ACEs (Figure 1). Patients with LV-GLS ≤-20% did not experience ACEs. LV-GLS remained associated with ACEs after adjustment for diffuse and midwall LGE. Conclusions In AM presenting with LVEF ≥50%, LV-GLS provides independent prognostic value over LGE characterization, improving risk stratification and representing a rationale for further studies of therapy in this cohort (Figure 2).

Left and Right Ventricular Morphology, Function and Myocardial Deformation in Children with Left Ventricular Non-Compaction Cardiomyopathy: A Case-Control Cardiovascular Magnetic Resonance Study.

Background: Left ventricular non-compaction (LVNC) is a rare cardiomyopathy typically involving the left ventricle (LV); however, the right ventricle (RV) can also be affected. This case-control study aimed to assess the morphology and function of LV and RV in children with LVNC. Methods: Sixteen children (13 ± 3 years, six girls) with LVNC were compared with 16 sex- and age-matched controls. LV and RV morphology and function were evaluated in cardiovascular magnetic resonance (CMR) studies. Additionally, LV and RV global radial (GRS), circumferential (GCS), and longitudinal strain (GLS) were assessed using tissue-tracking analysis. Results: Patients with LVNC did not differ from the healthy controls in terms of age, height, weight, and body surface area (BSA). In total, 4/16 subjects with LVNC had mid-wall late gadolinium enhancement (LGE). Compared to the control group, patients with LVNC had higher end-diastolic volume (EDV) indexed for body surface area (BSA), lower ejection fraction (EF), and lower LV strain parameters (all p < 0.05). Children with LVNC also presented with thicker RV apical trabeculation, whereas there were no differences in RV EF and EDV/BSA between the groups. Nevertheless, children with LVNC had impaired RV GRS and GCS (both p < 0.05). Conclusions: LVNC in pediatric patients is associated with LV enlargement and impaired LV systolic function. Additionally, children with LVNC have increased RV trabeculations and subclinical impairment of RV myocardial deformation.

100 Global longitudinal strain by CMR improves prognostic stratification in acute myocarditis presenting with normal LVEF

Abstract Aims Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular (LV) ejection fraction (EF) relies mostly on late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) imaging. In this specific AM population, LV peak global longitudinal strain (LV-GLS) measured by feature tracking (FT) analysis might provide further prognostic information. Methods and results Data of patients undergoing CMR for clinically suspected AM in seven European Centres, between January 2013 and August 2020, were retrospectively analysed. Those patients fulfilling CMR Lake Louise Criteria (LLC) for the diagnosis of AM and presenting with normal LVEF (≥50%) were included. Patients presenting with heart failure (HF) or significant arrhythmic events, LVEF &amp;lt;50% or haemodynamic instability were excluded. CMR-LGE extent (localized vs. diffuse), localization (subepicardial vs. mid-wall), and distribution (anteroseptal vs. inferolateral) were visually assessed. LV-GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including a composite of cardiac death, development of heart failure, life-threatening arrhythmias, or development of LVEF &amp;lt;50%. In patients experiencing more than one event, the first one was considered for the outcome analysis. Of 389 patients with clinically suspected AM, 256 (66%) had confirmed AM with LVEF ≥50% and were included. Median age was 36 years, 71% were males, median LVEF was 60%, and median LV-GLS −17.3%. CMR was performed at a median time of 4 (IQR: 2–12) days from hospital admission. At a median follow-up of 27 months, 24 (9%) patients experienced at least one ACE with development of LVEF &amp;lt;50% accounting for 17 [71%]. Compared to the others, patients experiencing ACEs had lower median LV-GLS values at baseline (−13.9% vs. −17.5%, P = 0.001). At Kaplan–Meier analysis, impaired LV-GLS (both considered as &amp;gt;-20% or quartiles), diffuse and mid-wall LGE were associated with a significantly higher rate of ACEs. LV-GLS remained independently associated with ACEs after adjustment for diffuse or mid-wall LGE as covariate at bivariable analysis. Conclusions In AM with LVEF ≥50%, LV-GLS provides independent prognostic value over LGE, improving risk stratification and providing a rationale for further studies of therapy in this cohort. 100 Figure