Transmural Late Gadolinium Enhancement Research Articles

855 PREDICTORS OF LATE GADOLINIUM ENHANCEMENT DEVELOPMENT AND EXTENSION IN MYOCARDIAL INFARCTION WITH NON-OBSTRUCTIVE CORONARY ARTERIES

Abstract Background Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a heterogeneous clinical entity and in its complex diagnostic approach cardiovascular magnetic resonance (CMR) plays a pivotal role. Purpose To characterize the differences of MINOCA patients with and without late gadolinium enhancement (LGE) at CMR and to identify the predictors for ischemic LGE development and extension. Methods We assessed 461 MINOCA cases from January 2016 to June 2021. MINOCA were defined according to the current European guidelines criteria. We excluded acute myocarditis, Tako-tsubo syndromes, cardiomyopathies, or non-pathological CMR. According to CMR imaging findings, our cohort was divided into two CMR phenotypes based on regional myocardial necrosis detected throughout LGE (“LGE-positive MINOCA”) or regional ischemic injury without LGE (“LGE-negative MINOCA”). Extended LGE was considered as the presence of >2 segments with transmural LGE. Multivariate logistic regression analysis was used to determine the predictors of LGE and extended LGE. Results The final cohort included 175 MINOCA: 121 (69.1%) constituted the LGE-positive group. The mean time delay between acute clinical presentation and CMR was 6 ± 2.9 days. At admission, MINOCA LGE-patients more frequently presented angina and ST segment elevation (24% vs 7.4%, p = 0.01), compared to the LGE negative ones. Furthermore, the LGE positive group had a significantly greater infarct size, measured by peak hs-Troponin I values and left ventricular function. The only predictor of LGE was the peak troponin value (OR 1.64, 95% CI 1.18–2.28, p = 0.003), while predictors of extended LGE were ST-segment elevation at admission (OR 7.44, 95% CI 1.57–35.22, p = 0.01), peak troponin values (OR 1.07, 95% CI 1.02–1.13, p = 0.01) and the presence of non-obstructive coronary artery disease at coronary angiography (OR 5.49, 95% CI 1.20–25.09, p = 0.028). Conclusion The presence and extension of LGE at early CMR evaluation is an important feature in the setting of MINOCA. In addition, simple baseline characteristics (such as ST elevation, peak troponin value and LVEF) may aid the identification of a greater ischemic necrosis burden at CMR and therefore these high-risk MINOCA subjects could be benefit from a stricter management effort.

225 PROGNOSTIC ROLE OF EARLY CARDIAC MAGNETIC RESONANCE IN MYOCARDIAL INFARCTION WITH NON-OBSTRUCTIVE CORONARY ARTERIES (MINOCA)

Abstract Background Myocardial infarction with non-obstructive coronary arteries (MINOCA) represents a significant proportion of acute myocardial infarction (AMI) population. MINOCA is a working diagnosis and an accurate investigation of the underlying causes should always be performed. In this setting, cardiac magnetic resonance (CMR) imaging plays a pivotal diagnostic role. However, a prognostic stratification based on the CMR findings in ischemic MINOCA is still unavailable. Purpose To evaluate the potential prognostic impact of specific CMR findings - especially ischemic late gadolinium enhancement (LGE) patterns - in order to look for measurable parameters that may guide the management of this still troubled clinical entity. Methods We assessed 461 MINOCA from January 2016 to June 2021. We excluded acute myocarditis, Tako-tsubo syndromes, cardiomyopathies, or non-pathological CMR. According to CMR findings, MINOCA were classified in two phenotypes: LGE-positive (an ischemic subendocardial or transmural LGE pattern) or LGE-negative (cases without LGE but exhibiting regional myocardial injury defined by myocardial edema in a coronary territory with a typically ischemic "wave-front" and/or regional wall motion abnormality consistent with coronary distribution). All-cause mortality, re-infarction, stroke, heart failure (HF) and the composite endpoint (MACE) were evaluated. Extended LGE was considered as the presence of >2 segments with transmural LGE. The mean follow-up was 36.1 ± 15.2 months and CMR was performed at a mean of 6 ± 2.9 days from the acute presentation. Results The final cohort included 175 MINOCA with a likely-ischemic etiology: 121 (69.1%) constituted the LGE-positive group. The mean age of the study population was 62.3 ± 12.9 years and more than 61% were females. During follow-up, HF (15.7% vs 1.9%, p=0.008) and MACE (20.7% vs 7.4%, p=0.029) occurred more frequently in MINOCA "LGE-positive" compared to the "LGE-negative" ones. Extended LGE was significantly more prevalent in patients with versus without subsequent HF. On multivariable Cox regression, extended LGE was an independent predictor of HF occurrence (HR 18.49, 95%CI 4.65–73.61, p < 0.001) and MACE (HR 14.64, 95%CI 3.91–54.86, p < 0.001). Conclusions Our data suggest that in MINOCA patients the detection of LGE is correlated with the incidence of major cardiovascular events and heart failure during long-term follow-up. In fact, LGE extension was identified as the strongest predictor of cardiac adverse events. The early execution of CMR is useful in the prognostic stratification of MINOCA and this could guide the subsequent clinical and therapeutic management.

996 INCIDENCE AND PREDICTORS OF HEART FAILURE DEVELOPMENT IN A COHORT OF PATIENTS WITH MYOCARDIAL INFARCTION WITH NON-OBSTRUCTIVE CORONARY ARTERIES

Abstract Background A higher risk of death and major cardiovascular events, including Heart Failure (HF), after a Myocardial Infarction with Non-Obstructive Coronary Arteries (MINOCA) has been described. In particular, HF has a negative impact not only on survival but also on quality of life. In this context, strategies to identify subjects with a greater risk of developing HF are needed. Aims To assess the incidence and predictors of HF developing subsequently a MINOCA. Methods 461 subjects with suspected MINOCA from 2016 to 2021 were assessed. Acute myocarditis, Takotsubo syndromes, cardiomyopathies and patients with incomplete data were excluded. The final cohort consisted of 188 patients with a confirmed MINOCA diagnosis. After a mean follow-up of 36±14.8 months, 20 patients (10.6%) had hospital readmission for HF. Demographic, clinical, laboratory, and instrumental data were collected and analyzed. Results None among demographic characteristics, cardiovascular risk factors or comorbidities showed correlation with HF occurrences. Paroxysmal or permanent atrial fibrillation was more frequent among patients who developed HF (p .005), such as ST-elevation at onset (p .001). Higher glycemia at admission correlated with HF (p .012). In this regard, hyperglycemia could act as a direct toxic agent or as an indirect marker of a greater stress response. Cardiac troponin I (cTnI) at peak, but not first cTnI measurement, was significantly higher in patients who developed HF (p<.001), confirming the prognostic value of troponin, even in this context. Concerning echocardiography, HF incidence was higher in patients with any wall motion abnormalities (p .037), lower ejection fraction (EF; p .046), especially if EF≤40% (p<.001), higher atrial maximum diameter (p .005), end-diastolic diameter (p. 001) or normalized end-diastolic volume (p<.001). Among cardiac magnetic resonance findings, HF occurred more often (p<.001) in patients with a transmural pattern of late gadolinium enhancement (LGE), which resulted as an independent risk factor for HF developing at the multivariate analysis (HR 15.32, 95% CI 5.13-45.75, p<.001). Finally, 32 patients (17%) were affected by non-obstructive coronary arteries stenosis (i.e., <50%). This finding, but not re-infarction, was also associated with HF development (p .004). Notably, only 2 out of the 68 patients dismissed with DAPT developed HF (p .010). In our study beta-blockers, RAS inhibitors and statins at dismission had not reduced the risk of HF. Nevertheless, the low sample size forbids inferring conclusions about the therapy. Conclusion In our cohort of patients with MINOCA, several clinical, laboratory and echocardiographic characteristics correlated with a higher frequency of HF occurrences. Moreover, a broader extent of cardiac damage, as testified by cTnI, wall motion abnormalities, reduced EF and transmural LGE, could predict HF development. Further studies to establish adequate follow-up programs and therapy to prevent HF progression in these patients are needed.

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 11573: Refractory Ventricular Tachyardia Storm: Treatment With Sympathetic Denervation in Patient With Non-Ischemic Cardiomyopathy

Introduction: Ventricular tachycardia (VT) is a potentially life-threatening arrhythmia commonly present in cardiomyopathy. Implantable cardioverter-defibrillator (ICD) placement can decrease mortality in patients with VT. However, when an ICD shocks it can cause direct myocardial injury, contraction band necrosis, fibrosis, and significant morbidity. Antiarrhythmic medications and catheter ablation can decrease arrhythmia burden and ICD shocks. Cardiac sympathetic denervation has been effective in a few refractory cases, but prospective data is scarce, especially in non-ischemic cardiomyopathy. Case description We present the case of a 59-year-old gentleman with a past medical history of hypertension, diabetes, hypothyroidism, atrial fibrillation, and non-ischemic cardiomyopathy, who presented to the hospital with recurrent ICD shocks. The patient had an ICD placed due to an episode of non-sustained VT resulting in presyncope. The patient had an arrhythmia-free period of 10 years followed by frequent VT. He was managed in the outpatient setting with amiodarone, dofetilide, and mexiletine which did not suppress his arrhythmia burden. Cardiac MRI showed multi-segment transmural late gadolinium enhancement consistent with a non-ischemic pattern. He underwent three endocardial and epicardial ablations over eight months before this admission. He was started on amiodarone and lidocaine infusions. With his history of recurrent VT despite multiple ablations, the patient underwent bilateral thoracoscopic sympathetic denervation. He was discharged from the hospital six days post-op without any episodes of VT. His latest ICD check which was ten months post-operatively did not show any VT or VT-induced ICD shock. Conclusion VT storm may be associated with a catecholaminergic surge and be refractory to traditional therapy. Due to this, sympathetic denervation is a viable option to prevent morbidity of frequent defibrillator therapy. This case highlights the utility of sympathetic denervation as a treatment of refractory ventricular arrhythmias in non-ischemic cardiomyopathy.

Abstract 9482: Coronary Spasm in a Patient With Argininosuccinic Aciduria

Case presentation: A 39-year-old male patient was referred for the treatment of hypertension. The patient had been treated for argininosuccinic aciduria (ASA) since eight months of age. Therapeutic drugs including, L-arginine, sodium phenylbutyrate and antiepileptic drugs had been administered. A detailed medical history taking revealed that the patient sometimes complained of chest discomfort under psychological stress. A 12-lead electrocardiogram showed abnormal q waves in lead III and aVF. Transthoracic echocardiography showed hypokinesis of left ventricular posterior wall. Endothelial function was quantified with brachial artery flow-mediated dilatation (FMD). The patient’s FMD value was 2.4% (normal cut off value 7.0%). Coronary computed tomography angiography showed no significant coronary stenosis. Cardiac magnetic resonance revealed transmural late gadolinium enhancement in the mid-ventricular level of the left ventricular posterior wall. The patient was suspected to have prior myocardial infarction. The patient was started on amlodipine and irbesartan, however, he continued to complain of chest discomfort at rest. Cardiac catheterization was performed to make a definitive diagnosis. The acetylcholine spasm provocation test revealed diffuse left and right coronary vasospasm. Transient ST-segment elevation with chest discomfort was observed. The patient was diagnosed as having coronary spastic angina. Chest discomfort was disappeared after additional administration of isosorbide mononitrate. Discussion: ASA is a genetic disorder of the urea cycle, caused by deficiency of argininosuccinate lyase (ASL). Specific laboratory findings are hyperammonemia, argininosuccinic acidemia and low plasma arginine levels. ASL is required to utilize extracellular arginine for NOS-dependent NO synthesis in endothelial cell. Reduction of ASL enzymatic activity leads to decline in NO production even if arginine is supplemented. Efficacy of NO supplementation for treatment of long-term complications in ASA patients has been reported. The precise mechanisms of coronary spasm remain to be elucidated. Our case report might support the significance of endothelial function in the pathogenesis of coronary spasm.

Prognostic implications of clinical phenotype and severity of cardiac involvement in patients presenting with immunoglobulin light chain amyloidosis

Abstract Background Patients with systemic immunoglobulin light chain (AL) amyloidosis may present with a wide array of signs and symptoms due to the multi-systemic organ involved. The presence of cardiac involvement is the key determinant of survival. Cardiac magnetic resonance (CMR) has the unique ability to measure the continuum of cardiac amyloidosis (CA) infiltration providing a deep characterisation from early CA involvement to severe degree of CA burden. Purpose The aim of this study was to characterise the clinical profiles and the severity of organ involvement in patients presenting with AL amyloidosis and to investigate implications for long-term outcome. Methods Patients newly diagnosed with AL amyloidosis at the National Amyloidosis Centre underwent comprehensive clinical, laboratory and instrumental work up, including CMR imaging with left ventricular (LV) mass, late gadolinium enhancement (LGE) and extracellular volume (ECV). The clinical phenotypes were classified in cardiac, renal and other according to the symptoms at presentation. The degree of CA was investigated by CMR: 0= no features of CA (normal LV mass, no LGE and normal ECV); 1=early cardiac amyloid infiltration (normal LV mass, raised ECV no LGE); 2= characteristic of CA with normal mass (diffuse subendocardial or transmural LGE, altered gadolinium kinetics and raised ECV); 3= characteristic of CA with elevated mass (diffuse subendocardial or transmural LGE and raised ECV). The study outcome was all-cause mortality. Results The study population included 241 AL patients presenting with cardiac and renal (22.8%, n=55), cardiac (28.2%, n=68), renal (33.2%, n=80) and other (15.8% n=38) phenotypes. During a median follow up of 33 (IQR 7–52) months, cardiac phenotype either in isolation or in combination with renal phenotype was associated with a higher rate of all-cause mortality compared to the others (p<0.001) (Figure). On CMR imaging, 43.2% of patients without cardiac phenotype (49%, n=118/241) had characteristic scans of CA (CMR grade 2 and 3) whilst 13.8% of patients with cardiac phenotype (51%, n=123/241) had no features of CA on CMR images (CMR grade 0) in (p<0.001). With Kaplan Meier analysis, the risk of all-cause death increased in patients with characteristic features of CA on CMR scan (Figure 1) and in patients with cardiac phenotype and features of CA on CMR scans compared to the others (both p<0.001) (Figure). At multivariable analysis, age at diagnosis (hazard ratio [HR] 1.03, p=0.009), clinical phenotype at presentation (HR 1.35, p=0.014) and ECV measured by CMR (HR 56, p<0.001) emerged as independent prognostic parameters. Conclusions Patients with newly diagnosed AL amyloidosis present most frequently with renal and cardiac phenotypes. CMR detects CA in >40% of patients with non-cardiac phenotype. ECV is an independent predictor of all-cause mortality across the full clinical spectrum of AL amyloidosis. Funding Acknowledgement Type of funding sources: None.

Prognostic role of early cardiac magnetic resonance in myocardial infarction with non-obstructive coronary arteries

Abstract Background Myocardial infarction with non-obstructive coronary arteries (MINOCA) represents a significant proportion of acute myocardial infarction (AMI) population. MINOCA is a working diagnosis and an accurate investigation of the underlying causes should always be performed. In this setting, cardiac magnetic resonance (CMR) imaging plays a pivotal diagnostic role. However, a prognostic stratification based on the CMR findings in ischemic MINOCA is still unavailable. Purpose To evaluate the potential prognostic impact of specific CMR findings - especially ischemic late gadolinium enhancement (LGE) patterns - in order to look for measurable parameters that may guide the management of this still troubled clinical entity. Methods We assessed 461 MINOCA from January 2016 to June 2021. We excluded acute myocarditis, Tako-tsubo syndromes, cardiomyopathies, or non-pathological CMR. According to CMR findings, MINOCA were classified in two phenotypes: LGE-positive (an ischemic subendocardial or transmural LGE pattern) or LGE-negative (cases without LGE but exhibiting regional myocardial injury defined by myocardial edema in a coronary territory with a typically ischemic “wave-front” and/or regional wall motion abnormality consistent with coronary distribution). All-cause mortality, re-infarction, stroke, heart failure (HF) and the composite endpoint (MACE) were evaluated. Extended LGE was considered as the presence of >2 segments with transmural LGE. The mean follow-up was 36.1±15.2 months and CMR was performed at a mean of 6±2.9 days from the acute presentation. Results The final cohort included 175 MINOCA with a likely-ischemic etiology: 121 (69.1%) constituted the LGE-positive group. The mean age of the study population was 62.3±12.9 years and more than 61% were females. During follow-up, HF (15.7% vs 1.9%, p=0.008) and MACE (20.7% vs 7.4%, p=0.029) occurred more frequently in MINOCA “LGE-positive” compared to the “LGE-negative” ones. Extended LGE was significantly more prevalent in patients with versus without subsequent HF. On multivariable Cox regression, extended LGE was an independent predictor of HF occurrence (HR 18.49, 95% CI 4.65–73.61, p<0.001) and MACE (HR 14.64, 95% CI 3.91–54.86, p<0.001). Conclusions Our data suggest that in MINOCA patients the detection of LGE is correlated with the incidence of major cardiovascular events and heart failure during long-term follow-up. In fact, LGE extension was identified as the strongest predictor of cardiac adverse events. The early execution of CMR is useful in the prognostic stratification of MINOCA and this could guide the subsequent clinical and therapeutic management. Funding Acknowledgement Type of funding sources: None.

Predictors of late gadolinium enhancement development and extension in myocardial infarction with non-obstructive coronary arteries

Abstract Background Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a heterogeneous clinical entity and in its complex diagnostic approach cardiovascular magnetic resonance (CMR) plays a pivotal role. Purpose To characterize the differences of MINOCA patients with and without late gadolinium enhancement (LGE) at CMR and to identify the predictors for ischemic LGE development and extension. Methods We assessed 461 MINOCA cases from January 2016 to June 2021. MINOCA were defined according to the current European guidelines criteria. We excluded acute myocarditis, Tako-tsubo syndromes, cardiomyopathies, or non-pathological CMR. According to CMR imaging findings, our cohort was divided into two CMR phenotypes based on regional myocardial necrosis detected throughout LGE (“LGE-positive MINOCA”) or regional ischemic injury without LGE (“LGE-negative MINOCA”). Extended LGE was considered as the presence of >2 segments with transmural LGE. Multivariate logistic regression analysis was used to determine the predictors of LGE and extended LGE. Results The final cohort included 175 MINOCA: 121 (69.1%) constituted the LGE-positive group. The mean time delay between acute clinical presentation and CMR was 6±2.9 days. At admission MINOCA LGE-patients more frequently presented angina and ST segment elevation (24% vs 7.4%, p=0.01), compared to LGE negative ones. Furthermore, the LGE positive group had a significantly greater infarct size, measured by peak hs-Troponin I values and left ventricular function (LVEF). The only predictor of LGE was the peak troponin value (OR 1.64, 95% CI 1.18–2.28, p=0.003), while predictors of extended LGE were ST-segment elevation at admission (OR 7.44, 95% CI 1.57–35.22, p=0.01), peak troponin values (OR 1.07, 95% CI 1.02–1.13, p=0.01) and the presence of non-obstructive coronary artery disease at coronary angiography (OR 5.49, 95% CI 1.20–25.09, p=0.028). Conclusion The presence and extension of LGE at early CMR evaluation is an important feature in the setting of MINOCA. In addition, simple baseline characteristics (such as ST elevation, peak troponin value and LVEF) may aid the identification of a greater ischemic necrosis burden at CMR and therefore these high-risk MINOCA subjects could be benefit from a stricter management effort. Funding Acknowledgement Type of funding sources: None.

A Starry Myocardium at CT: Cardiac Cysticercosis.

Supplemental material is available for this article.

Collateral presence and extent do not predict myocardial viability and ischemia in chronic total occlusions: A stress-CMR study

BackgroundWell-developed collaterals are assumed as a marker of viability and ischemia in chronic total occlusions (CTO). We aim to correlate viability and ischemia with collateral presence and extent in CTO patients by cardiac magnetic resonance (CMR). MethodsMulticentre study of 150 CTO patients undergoing stress-CMR, including adenosine if normal systolic function, high-dose-dobutamine for patients with akinetic/>2 hypokinetic segments and EF ≥35%, otherwise low-dose-dobutamine (LDD); all patients underwent late gadolinium enhancement (LGE) imaging. Viability was defined as mean LGE transmurality ≤50% for adenosine, as functional improvement for dobutamine-stress-test, ischemia as ≥1.5 segments with perfusion defects outside the scar zone. ResultsRentrop 3/CC 2 defined well-developed (WD, n = 74) vs poorly-developed collaterals (PD, n = 76). Viability was equally prevalent in WD vs PD: normo-functional myocardium with ≤50% LGE in 52% vs 58% segments, p = 0.76, functional improvement by LDD in 48% vs 52%, p = 0.12. Segments with none, 1–25%,26–50%,51–75% LGE showed viability by LDD in 90%,84%,81%,61% of cases, whilst in 12% if 76–100% LGE (p < 0.01). There was no difference in WD vs PD for ischemia presence (74% vs 75%, p = 0.99) and extent (2.7 vs 2.8 segments, p = 0.77). ConclusionsIn a large cohort of CTO patients, presence and extent of collaterals did not predict viability and ischemia by stress-CMR. Scar extent up to 75% LGE was still associated with viability, whereas ischemia was undetectable in 25% of patients, suggesting that the assessment of CTO patients with CMR would lead to a more comprehensive evaluation of viability and ischemia to guide revascularization.

Myocardial strain analysis by cardiac magnetic resonance 3D feature-tracking identifies subclinical abnormalities in patients with neuromuscular disease and no overt cardiac involvement.

Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich's ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne's muscular dystrophy (BMD/DMD, n = 15), Duchenne's carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement.

The prevalence of ischemic pattern of heart damage in patients with acute myocardial infarction depending on the presence of obstructive coronary artery disease: Magnetic resonance study

Currently, there are practically no works that are devoted to the analysis of different types of damage to ischemic and nonischemic patterns in patients with acute myocardial infarction (AMI), depending on the nature of atherosclerotic lesions of the coronary arteries.In this regard, the aim of this work was to evaluate the frequency of ischemic pattern of heart damage in patients with AMI depending on the presence of obstructive coronary artery disease.Material and Methods. The study retrospectively included patients with a working diagnosis of acute coronary syndrome (ACS) who underwent contrast-enhanced cardiac magnetic resonance (MR) imaging (CMR) during the first week after hospitalization. A 1.5 T CMR was performed using a standard protocol. T2-WI, T1-WI, and IR-images were visually assessed for edema and scar/necrosis, respectively. The pattern of acute ischemic damage to the left ventricular (LV) myocardium was an increase in the signal intensity on T2-WI as a sign of myocardial edema and late gadolinium enhancement (LGE) with a typical ischemic nature of contrast enhancement: subendocardial/subtransmural/transmural. The non-ischemic nature of myocardial injury was defined as intramyocardial/subepicardial LGE.Results. Based on invasive coronary angiography (ICA) data, patients were divided into two groups: group of myocardial infarction (MI) and coronary artery disease (MICAD group) and group of MI and non-obstructive coronary arteries (MINOCA group). It was found that the frequencies of occurrence of subendocardial pattern of cardiac LGE in early CMR did not differ in the groups of AMI patients against the background of obstructive and non-obstructive coronary artery disease (CAD). The most characteristic MR-pattern in MICAD patients was transmural LGE, including that in combination with microvascular obstruction (MVO). The most characteristic MR-pattern in MINOCA patients was mid-wall/insertion point LGE. Subepicardial LGE occurred with equal frequency in MICAD and MINOCA patients.Conclusion. The most pathognomonic MR-pattern of myocardial damage in AMI against the background of obstructive CAD was the transmural type of LGE with or without the phenomenon of MVO. Identification of the subendocardial type of LGE in patients with MINOCA allowed to stratify these patients into a higher risk group with a further change in treatment tactics.

Diagnostic Value of 11C-PIB PET/MR in Cardiac Amyloidosis.

BackgroundThe thioflavin T derivative, 11C-Pittsburgh-B (PIB), is used for Alzheimer's disease imaging because it specifically binds to β-amyloid protein deposits in the brain. The aim of this study was to estimate the diagnostic value of combined 11C-PIB positron emission tomography/magnetic resonance (PET/MR) in cardiac amyloidosis (CA).MethodsWe enrolled 23 heart failure patients with suspected CA based on echocardiographic and electrocardiograph findings. All patients underwent cardiac 11C-PIB PET/MR and non-cardiac biopsy within one week. We also enrolled eight healthy volunteers that underwent cardiac 11C-PIB PET/MR as a control group. The cardiac magnetic resonance (CMR) protocol included cine imaging, late gadolinium enhancement (LGE), and native and post-contrast T1 mapping. Extracellular volume (ECV) was measured using pre- and post-contrast T1 mapping images. LVEF, IVSD, LVPW, LVmass, LVESV, LVEDV, native T1 value, ECV, and maximum uptake of myocardial tissue-to-blood background ratio (TBR) values were obtained from PET/MR images in all patients and healthy subjects.ResultsThirteen out of twenty-three heart failure patients were clinically diagnosed with CA. The remaining 10 patients were CA-negative (non-CA patient group). Twelve of the thirteen CA patients showed diffuse transmural LGE patterns, whereas LGE was either absent or patchy in the non-CA patients. The diagnostic sensitivity and specificity of TBRmax were 92.3 and 100%, respectively, at a cut-off value of 1.09. Several CMR imaging parameters (LVEF, IVSD, LVmass, LVEDV, LVESV, LVPW, native T1 value and ECV) and TBR showed significant differences between CA patients, non-CA patients, and healthy controls (P < 0.05). Native T1 mapping values positively correlated with TBRmax values in CA and non-CA patients (r = 0.38, P = 0.0004).Conclusions11C-PIB PET/MRI is a valuable tool for the accurate and non-invasive diagnosis of CA because it distinguishes CA patients from non-CA patients and healthy subjects with high specificity and sensitivity. Moreover, native T1 mapping values positively correlated with TBRmax values in CA and non-CA patients. In the future, larger cohort studies are necessary to confirm our findings.

Strain-based characterization of electromechanical tissue properties using patient-specific simulation of dyssynchronous hearts: a pilot study

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): Dutch Heart Foundation (ERA-CVD JTC2018 grant 2018T094; Dr. Dekker Program grant 2015T082 to J.L.) and the Netherlands Organisation for Scientific Research (NWO- ZonMw, VIDI grant 016.176.340 to J.L.) Background/Introduction Interpretation of regional strain patterns in patients with failing hearts is often challenging due to presence of different underlying tissue abnormalities, including electrical conduction delay and myocardial infarction (MI). Purpose To test whether an automatic strain-based computer simulation algorithm can differentiate between electrical and ischemic contractile dysfunction in patients with mechanical dyssynchrony. Methods Three heart failure patients with reduced left ventricular ejection fraction (33 ± 3%) and wide QRS (160 ± 7ms, LBBB morphology) were retrospectively included, two with and one without MI. Longitudinal strain patterns obtained in 18 myocardial segments using speckle tracking echocardiography (STE) and left ventricular (LV) volume measurements were used as an input to the patient-specific simulation algorithm, which automatically optimizes a set of global and regional myocardial tissue parameters such that the regional strain patterns and global volumes simulated by the CircAdapt model of the human heart and circulation were similar to the strains and volumes measured in the patients. As output, the patient-specific simulations revealed onset time of mechanical activation (relative to first activated segment) and relative severity of contractile dysfunction in all 18 segments. We compared predicted mechanical activation delays and segmental contractile dysfunction values with independent measurements of QRS width and transmurality of late gadolinium enhancement (LGE), respectively. Results The model was able to reproduce the dyssynchronous regional strain patterns as measured in the patients (Figure 1: representative example). In all patients, significant septal—to-lateral wall mechanical activation delays (33-55ms) were predicted (Figure 2: blue bullseye plot of same patient used for figure 1), matching the presence of electrical activation delays on the patient’s ECGs (i.e. wide QRS and LBBB morphology). Furthermore, all simulations predicted a global loss of contractile dysfunction, matching the low ejection fractions reported in those patients. The simulations of the two patients with MI predicted more severe contractile dysfunction (&amp;gt;60%) in the regions with transmural LGE (Figure 2: red bullseye plot versus gold standard LGE-MRI image of same patient used for figure 1). Conclusion This pilot study demonstrated the ability of an automatic patient-specific simulation algorithm to differentiate between electrical and ischemic myocardial disease substrates in heart failure patients with electrical conduction abnormalities with and without MI. Future studies will investigate whether this computer algorithm can improve diagnosis of patients with mechanical dyssynchrony. Abstract Figure 1 Abstract Figure 2

593 Spontaneous coronary artery dissection in young

Abstract Aims We present the case of a 25 years-old patient, presented to the Emergency Department for chest pain and epigastralgia complicated by vomiting. Symptoms started about 30 h before, afterwards an effort. The patient did not have any known cardiovascular risk factors (except for sporadic consumption of cannabis) and family medical history was negative for premature cardiovascular disease or sudden cardiac death. His past medical history was unremarkable. Methods and results Clinical examination was normal. ECG was suggestive for subacute anterior ST-elevation myocardial infarction (QS in V1–V4 leads with ST segment elevation and biphasic T-waves in V4–V6) and was accompanied by significant elevation of myocardial necrosis biomarkers (hsTnT 3416 ng/l, n.v. &amp;lt;15). Echocardiography revealed a severely reduced left ventricular ejection fraction (LVEF = 30%) with regional wall motion abnormalities (apical and septal akinesia, and anterior mid-ventricular segment hypokinesia). Coronary angiography was suggestive for a dissection of the proximal-to-mid left anterior descending artery. Dissection flap, subocclusion of the first septal branch, and intramural thrombosis were noted. Direct stenting with drug-eluting stent was successful, with thrombolysis in myocardial infarction (TIMI) flow grade 3. The patient was started on dual antiplatelet treatment (aspirin and ticagrelor), ACE-inhibitors, beta blockers, and spironolactone. Further testing ruled out other conditions that may be associated with spontaneous coronary artery dissection (autoimmune diseases, including vasculitis, connective tissue disease, coagulative disorders). The clinical course was complicated by pericarditis and the development of LV apical thrombus. So, patient was started on anticoagulation (warfarin) with subsequently downgrading from ticagrelor to clopidogrel. Echocardiographic follow-up documented a progressive improvement of LVEF (50%), and global longitudinal strain (GLS −15%) with persistence of wall motion abnormalities in the apical and septal segments. Partial resolution of intracavitary thrombi was documented. CMR revealed LV apical aneurysm. Moreover, the use of dedicated sequences showed transmural late gadolinium enhancement in akinetic segments, and fatty metaplasia of the apical portion of the anterior wall and anterior septal wall. Finally, early gadolinium enhancement imaging, confirmed the persistence of LV stratified apical thrombus. Conclusions Spontaneous coronary artery dissection (SCAD) is an underdiagnosed and frequent cause of acute coronary syndrome (ACS) in young-to-middle aged patients, particularly women; therefore, even in young people, a chest pain episode suggestive for an ischaemic cause should never be underestimated. Cardiac magnetic resonance is the most accurate method to evaluate the sequelae of myocardial infarction (including LV size and function, presence of fatty metaplasia, extent and transmurality of ischaemic scar, presence of thrombi), to aid in differential diagnosis and to define prognosis and guide the therapeutic management.

Myocardial viability with chronic total occlusion assessed by hybrid positron emission tomography/magnetic resonance imaging

BackgroundThe present study was performed to compare the relationship of 18F-fluorodeoxyglucose (FDG) uptake and late gadolinium enhancement (LGE) transmurality with the improvement of left ventricular function in patients with coronary chronic total occlusion (CTO) assessed by hybrid FDG positron emission tomography (PET)/magnetic resonance imaging (MRI). MethodsThirty-eight consecutive patients with CTO underwent FDG PET/MRI. Twenty-three patients then underwent percutaneous coronary intervention (PCI), and the final study population comprised 15 patients who underwent both initial and follow-up MRI. The degree of wall motion abnormality in each of the 17 myocardial segments was evaluated based on the extent of wall thickening on cine MRI using a 5-point scale. ResultsAmong all 646 myocardial segments at baseline, FDG uptake significantly decreased as the transmurality of LGE is advanced. Of the 15 patients who underwent PCI, 152 segments showed wall motion abnormalities at baseline. The functional recovery of the wall motion abnormality of the PET-viable/MRI-viable segments was highest, and that of the PET-nonviable/MRI-nonviable segments was lowest. There were no differences in functional recovery between the PET-viable/MRI-nonviable and PET-nonviable/MRI-viable segments. ConclusionSimultaneous assessment of FDG and LGE using a hybrid PET/MRI system can help to predict functional recovery after PCI in patients with CTO.

SPONTANEOUS CORONARY ARTERY DISSECTION RESULTING IN MITRAL VALVE REPLACEMENT

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Spontaneous coronary artery dissection (SCAD) is a rare condition accounting for only 0.1%–4% of acute coronary syndromes and 0.4% of sudden cardiac deaths. It predominantly affects young women with few or no traditional cardiovascular risk factors and is commonly associated with fibromuscular dysplasia (FMD), connective tissue disorders, and recreational drug use. Herein, we report a rare case of Type 2A SCAD in a male patient further complicated by the development of severe mitral valve regurgitation requiring mitral valve replacement (MVR). CASE PRESENTATION: A 47-year-old male with a history of hypertension, hyperlipidemia, and renal artery stenosis presented with complaints of fatigue, dyspnea, and chest pain. The initial EKG showed nonspecific ST changes, and troponin was elevated to 10 ng/dL (normal: <0.49 ng/dL). An urgent cardiac catheterization demonstrated non-obstructive coronary artery disease. Computed tomography (CT) of the chest ruled out a pulmonary embolism. The patient's troponin following cardiac catheterization continued to trend up with a peak of 23 ng/dL, at which point a formal transthoracic echocardiogram (TTE) was found to be normal. He had one recurrence of chest pain which correlated with another troponin spike and was found to have a decreased ejection fraction on emergent repeat TTE (EF: 45-50%). A third TTE was performed due to a new systolic murmur which showed a further decrease in EF (40-45%) and new mild MR. At this point, our leading differential diagnosis was myocarditis. Cardiac MRI showed an area of transmural late gadolinium enhancement involving the mid left ventricular inferolateral wall with islands of sparing which raised suspicion for a myocardial infarction (Figure 1), however, on further retrospective review of the cardiac angiogram, the findings (in correlation with the MRI) were consistent with narrowing in the obtuse marginal (OM) 1, a branch of the left circumflex artery, concerning for SCAD type 2. The patient was discharged home in stable condition with the diagnosis of SCAD of the OM-1 branch. Unfortunately, the patient returned with worsening dyspnea and was found to have severe MR which had progressed rapidly from the prior TTE. Given the circumflex distribution, and rapidly progressive MR, the patient was emergently referred for cardiothoracic surgery evaluation and ultimately underwent MVR. DISCUSSION: Any epicardial artery can be involved in SCAD, the left anterior descending artery is the most commonly affected. In this case, the involvement of the OM-1 resulted in impaired perfusion of the lateral wall and the anterolateral papillary muscle, subsequently leading to papillary rupture and MR. CONCLUSIONS: Even though this condition most commonly affects the female population, and is further primarily associated with connective tissue disorders, our work up revealed no such underlying conditions. REFERENCE #1: https://www.ahajournals.org/doi/10.1161/CIR.0000000000000564 DISCLOSURES: No relevant relationships by Nicole Anthony, source=Web Response no disclosure on file for Erdal Cavusoglu; no disclosure on file for Muhammad Dogar; No relevant relationships by Dilpat Kumar, source=Web Response No relevant relationships by Kamal Preet Singh, source=Web Response

Magnetic Resonance Imaging in Cardiac Amyloidosis: Unraveling the Stealth Entity.

Amyloidosis is a systemic disease involving many organs. Cardiac involvement is a significant cause of morbidity and mortality in these patients. Diagnosis of cardiac amyloidosis is based on endomyocardial biopsy which however is invasive and associated with complications. Noninvasive methods of diagnosis include magnetic resonance imaging (MRI) with various methods and sequences involved. Our study aims at describing MRI features of cardiac amyloidosis including new imaging sequences and to prognosticate the patients based on imaging features. We included 35 patients with suspected cardiac amyloidosis who underwent MRI at our center over 4 years. All images were retrieved from our archive and assessed by an experienced radiologist. Common morphological features in our patients included increased wall thickness of left ventricle (LV) (16. 1 ± 4.1 mm), right ventricle (RV) (6.3 ± 1.1 mm), and interatrial septum (6.2 ± 0.8 mm). Global late gadolinium enhancement (LGE) ( n = 21 [65%]) including subendocardial or transmural was the most common pattern followed by patchy enhancement. Global transmural LGE was associated with worse prognosis. Four types of myocardial nulling patterns were observed on postcontrast time to invert (TI) scout imaging: normal nulling pattern (myocardium nulls after blood and coincident with spleen) and abnormal nulling pattern (ANP) which is further divided into three types: Type 1-myocardium nulls before blood pool but coincident with spleen, Type 2-myocardium nulling coincident with blood but not coincident with spleen, and Type 3-features of both Type 1 and Type 2. Type 3 ANP was the most common ( n = 23) nulling pattern in our patients. Cardiac MRI is an essential in noninvasive diagnosis of cardiac amyloidosis. Transmural global LGE serves as a poor prognosticator in these patients. "Three-tier" TI scout imaging is essential to avoid false-negative enhancement results. Type 3 ANP is the most specific nulling pattern in cardiac amyloidosis.

Myocardial perfusion quantification by CMR for detection of obstructive coronary artery disease in patients with previous coronary artery bypass surgery

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): British Heart Foundation Background Coronary artery bypass grafting (CABG) is an established treatment for patients with advanced coronary artery disease (CAD). A subsequent recurrence of symptoms can cause the need for re-assessment of the coronary circulation. The accuracy of visually assessed stress perfusion cardiovascular magnetic resonance (CMR) for the detection of obstructive CAD is reduced in patients with prior CABG. In patients with complex multi-vessel CAD, myocardial perfusion quantification by CMR is superior to visual assessment (VA) for detection of obstructive disease however patients with CABG have been absent from previous studies. Purpose This study sought to assess the performance of myocardial perfusion quantification by CMR against invasive coronary angiography (ICA) for detecting obstructive CAD in patients with previous CABG. Methods Twenty-nine patients with a history of previous CABG and subsequent clinically indicated perfusion CMR study and invasive coronary angiography were recruited. Patients underwent a dual bolus stress perfusion CMR with late gadolinium enhancement (LGE) imaging at 3 Tesla. Stress myocardial blood flow (MBF) was estimated at the coronary territory level according to the AHA 16 segment model using Fermi function-constrained deconvolution. Segments with transmural LGE were excluded from MBF analysis. Stress perfusion images were analysed visually alongside LGE images and matched perfusion-LGE defects were considered negative. On ICA, coronary territories with lumen stenosis &amp;gt;70% without an unobstructed bypass graft (&amp;lt;70% stenosis) were considered positive. Results 86/87 coronary territories were suitable for analysis. Sixty-five territories had at least one bypass graft including 32 territories with arterial grafts. 28/86 territories (33%) had obstructive disease on angiography. Territories with obstructive CAD had significantly lower stress MBF than unobstructed territories (1.21 [IQR: 0.96–1.45] vs 1.58 [1.40–1.84] ml/g/min, p &amp;lt; 0.001, Figure 1). Stress MBF had good accuracy to detect coronary territories with obstructive CAD (sensitivity 71%, specificity 84%, area under the curve (AUC) 0.83, p &amp;lt; 0.001, Figure 2A). For visual assessment, sensitivity was 79%, specificity 78% and diagnostic accuracy 78%. When analysis was confined to only territories with bypass grafts, stress MBF had 78% sensitivity, 81% specificity and AUC of 0.85, p &amp;lt; 0.001 (Figure 2B).. In this subgroup, VA had a sensitivity of 78%, specificity of 76% and a 77% diagnostic accuracy. Conclusions In patients with previous surgical revascularisation, quantification of stress myocardial blood flow by CMR offers good diagnostic accuracy for the detection and localisation of anatomically significant stenoses. Accuracy is reduced compared with published data in patients without coronary grafts but remains comparable to expert visual assessment.