Structural and functional cardiac changes in endomyocardial fibrosis treated with endomyocardial resection: Disease progression captured by multimodality imaging.

Eosinophilic myocarditis, a rare and under-recognized disease process, occurs due to cytotoxic inflammation of the endomyocardium that over time may lead to a restrictive cardiomyopathy. We report clinical, multimodality imaging, and pathologic findings in a 45-year-old woman over a 17-month period as she progressed from suspected acute eosinophilic myocarditis to phenotypic endomyocardial fibrosis resulting in recurrent ascites. Interval echocardiograms demonstrate definitive pathologic structural changes that reflect the hemodynamic consequences of the underlying cardiomyopathy. Despite a negative myocardial biopsy, characteristic findings on cardiovascular magnetic resonance imaging clarified the diagnosis which led to successful treatment with endomyocardial resection and valve replacements.

State of the art: Evaluation and prognostication of myocarditis using cardiac MRI.

Myocarditis encompasses both primary and secondary processes causing inflammation of the myocardium. Viral infections are a common secondary cause of myocarditis with important clinical relevance. Viral myocarditis has a varied clinical presentation, potentially resulting in significant morbidity and mortality. Acutely, systolic dysfunction and sudden cardiac death may ensue; chronically, myocarditis may result in a dilated cardiomyopathy requiring heart transplantation. Myocarditis is thought to be one of the most common causes of myocardial infarction with nonobstructive coronary arteries (MINOCA), with important consequences for cardiovascular outcomes. Patients with myocarditis are currently underdiagnosed. Cardiac MRI has evolved as the noninvasive test of choice, with cardiac MRI-specific diagnostic requirements defined in the Lake Louise Criteria (LLC). Detecting the presence of tissue edema, hyperemia, and necrosis in both acute and chronic stages form the foundation of the LLC. Cardiac MR for chronic myocarditis (greater than 8 weeks from symptom onset) has decreased sensitivity for diagnosis. Emerging sequences such as T1 and T2 parametric maps provide tissue characterization regarding inflammation without reliance on reference tissue, overcoming limitations of the LLC. Beyond diagnostic criteria, these imaging techniques have proven useful in further characterizing the diseased tissue, prognostication, and clinical decision-making. This review describes the utility and evolving use of cardiac MRI in clinical practice. Level of Evidence: 1 Technical Efficacy Stage: 5 J. Magn. Reson. Imaging 2018;47:1061-1071.

Three-dimensional echocardiography demonstrates a skewered left ventricular thrombus in a patient with a heart transplant.

A left ventricular (LV) false tendon is a frequently visualized structure in echocardiography with unclear clinical significance. We present the case of a false tendon serving as a nidus for thrombus in a post-orthotopic heart transplantation patient. Three-dimensional transthoracic echocardiography (3DTTE) was utilized to visualize a LV mass and facilitate its identification as a thrombus as well as the surrounding structures. Using datasets from 3DTTE, the lack of ventricular wall attachment and circumferential formation of the thrombus around the false tendon was identified. Serial imaging demonstrated resolution of the thrombus with anticoagulation.

A novel and practical cardiovascular magnetic resonance method to quantify mitral annular excursion and recoil applied to hypertrophic cardiomyopathy.

BACKGROUND: We have developed a novel and practical cardiovascular magnetic resonance (CMR) technique to evaluate left ventricular (LV) mitral annular motion by tracking the atrioventricular junction (AVJ). To test AVJ motion analysis as a metric for LV function, we compared AVJ motion variables between patients with hypertrophic cardiomyopathy (HCM), a group with recognized systolic and diastolic dysfunction, and healthy volunteers. METHODS: We retrospectively evaluated 24 HCM patients with normal ejection fractions (EF) and 14 healthy volunteers. Using the 4-chamber view cine images, we tracked the longitudinal motion of the lateral and septal AVJ at 25 time points during the cardiac cycle. Based on AVJ displacement versus time, we calculated maximum AVJ displacement (MD) and velocity in early diastole (MVED), velocity in diastasis (VDS) and the composite index VDS/MVED. RESULTS: Patients with HCM showed significantly slower median lateral and septal AVJ recoil velocities during early diastole, but faster velocities in diastasis. We observed a 16-fold difference in VDS/MVED at the lateral AVJ [median 0.141, interquartile range (IQR) 0.073, 0.166 versus 0.009 IQR -0.006, 0.037, P < 0.001]. Patients with HCM also demonstrated significantly less mitral annular excursion at both the septal and lateral AVJ. Performed offline, AVJ motion analysis took approximately 10 minutes per subject. CONCLUSIONS: Atrioventricular junction motion analysis provides a practical and novel CMR method to assess mitral annular motion. In this proof of concept study we found highly statistically significant differences in mitral annular excursion and recoil between HCM patients and healthy volunteers.

Regional Analysis of Myocardial Strain to Wall Thickness Ratio in Cardiac Amyloidosis and Hypertrophic Cardiomyopathy.

BACKGROUND: Increased left ventricular wall thickness is a hallmark of cardiac amyloidosis (CA). Several other disease states, including hypertrophic cardiomyopathy (HCM), share this common feature. Myocardial strain has emerged as a diagnostic and prognostic tool to differentiate causes of increased left ventricular wall thickness. We sought to determine if regional strain differences were present in CA when compared with HCM when indexed to wall thickness as well as adjusting for important factors such as ejection fraction (EF), age, sex, and hypertension. METHODS: We performed a multicenter, retrospective analysis of 122 patients in 3 groups: CA (n=40), HCM (n=44), and controls (n=38). Using commercially available software, we determined peak systolic strain measurements in the base, mid, and apical segments in all 3 cardinal directions of radial strain, circumferential strain, and longitudinal strain. The regional strain was indexed to wall thickness to create a strain to wall thickness (STT) ratio. Analysis of Variance was performed to examine the association of each strain parameter with the disease group, adjusting for age, sex, hypertension, and EF. Multinomial logistic regression was performed to determine which combination of variables can potentially be used to best model the disease group. RESULTS: Ratios of STT at all 3 levels were significantly different with respect to the cardinal directions of radial, circumferential, and longitudinal strain in a multivariable analysis adjusting for age, sex, and hypertension. Specifically, with respect to the basal segments, the STT ratio across CA, HCM, and normal were significantly different in radial (1.13±0.34 vs. 3.79±0.22 vs. 4.12±0.38; P <0.0001), circumferential (-0.79±0.10 vs. -1.62±0.07 vs. -2.25±0.11; P <0.0001), and longitudinal directions (-0.41±0.09 vs. -1.03±0.06 vs. -1.41±0.10; P <0.0001). When adjusting for age, sex, hypertension and EF, only the base was significantly different between the CA and HCM groups in the radial (1.49±0.37 vs. 3.53±0.24; P <0.0001), circumferential -1.04±0.10 vs. -1.44±0.06; P <0.005), and longitudinal (-0.55±0.10 vs -0.94±0.06; P =0.007) directions. Using multinomial logistic regression, the use of age, left ventricular EF, global longitudinal strain, and basal radial strain yielded a diagnostic model with an area under the receiver operating characteristic curve (AUC) of 0.98. A model excluding age, despite being likely an independent predictor in our cohort, yielded an overall AUC of 0.90. When excluding age, the overall AUC was 0.91 and specifically when discriminating CA from HCM was 0.95. CONCLUSIONS: Regional myocardial strain indexed to wall thickness with an STT ratio can differentiate between etiologies of increased left ventricular wall thickness. Differences in myocardial deformation may be independent of wall thickness. Differences in basal strain when indexed to wall thickness in all 3 cardinal directions between CA and HCM are independent of EF. Multinomial logistic regression analysis using strain parameters differentiates CA and HCM with excellent diagnostic accuracy.

Hypertrophic cardiomyopathy - phenotypic variations beyond wall thickness.

Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy (LVH) in the absence of another causal disease. Several morphologic and histologic changes have been described. Given the morbidity and mortality associated with HCM, understanding these anatomic variations is key to interpreting imaging. This is especially important since many patients exhibit these associated findings in the absence of LVH and prompt early detection of these variations may lead to early diagnosis and treatment. This article describes the appearance of morphologic variations seen in HCM beyond myocardial thickening including: papillary muscle and mitral valve variants, myocardial crypts, left ventricular myocardial bands, and dystrophic calcification related to increased wall tension.

Pathophysiologic mechanisms of lupus-induced myocardial injury elucidated by cardiovascular magnetic resonance imaging.

Various pathophysiologic mechanisms may account for lupus-induced myocardial injury. Understanding the distinctions in the underlying disease process helps recognize variable clinical presentations and implement appropriate therapies. This report describes lupus-induced myocardial injury in three men less than 40 years old with diverse pathophysiologic mechanisms and presentations including acute myopericarditis with microvascular obstruction, acute coronary syndrome (ACS) and chronic myocarditis with systolic heart failure. Cardiovascular magnetic resonance (CMR) helped define the mechanism of disease, which included evidence of coronary microvascular obstruction in a patient without epicardial coronary artery disease. These findings highlight the cardiovascular effects of lupus, reveal coronary microvascular obstruction as potential consequence of acute myocarditis, and demonstrate the application of CMR in assessing the extent of disease.

Association between multimodality measures of aortic stenosis severity and quality-of-life improvement outcomes after transcatheter aortic valve replacement.

AIMS: Up to 40% of patients with aortic stenosis (AS) present with discordant grading of AS severity based on common transthoracic echocardiography (TTE) measures. Our aim was to evaluate the utility of TTE and multi-detector computed tomography (MDCT) measures in predicting symptomatic improvement in patients with AS undergoing transcatheter aortic valve replacement (TAVR). METHODS AND RESULTS: A retrospective review of 201 TAVR patients from January 2017 to November 2018 was performed. Pre- and post-intervention quality-of-life was measured using the Kansas City Cardiomyopathy Questionnaire (KCCQ-12). Pre-intervention measures including dimensionless index (DI), stroke volume index (SVI), mean transaortic gradient, peak transaortic velocity, indexed aortic valve area (AVA), aortic valve calcium score, and AVA based on hybrid MDCT-Doppler calculations were obtained and correlated with change in KCCQ-12 at 30-day follow-up. Among the 201 patients studied, median KCCQ-12 improved from 54.2 pre-intervention to 85.9 post-intervention. In multivariable analysis, patients with a mean gradient >40 mmHg experienced significantly greater improvement in KCCQ-12 at follow-up than those with mean gradient ≤40 mmHg (28.1 vs. 16.4, P = 0.015). Patients with MDCT-Doppler-calculated AVA of ≤1.2 cm2 had greater improvements in KCCQ-12 scores than those with computed tomography-measured AVA of >1.2 cm2 (23.4 vs. 14.1, P = 0.049) on univariate but not multivariable analysis. No association was detected between DI, SVI, peak velocity, calcium score, or AVA index and change in KCCQ-12. CONCLUSION: Mean transaortic gradient is predictive of improvement in quality-of-life after TAVR. This measure of AS severity may warrant greater relative consideration when selecting the appropriateness of patients for TAVR.

Paucicellular Fibroma of the Ascending Aorta.

Primary tumors of the aorta are extremely rare. To the best of our knowledge, herein, we present the first case in the literature of a paucicellular fibroma originating from the aortic wall.

Usefulness of Elevated Troponin to Predict Death in Patients With COVID-19 and Myocardial Injury.

Elevations in troponin levels have been shown to predict mortality in patients with coronavirus disease 2019 (COVID-19). The role of inflammation in myocardial injury remains unclear. We sought to determine the association of elevated troponin with mortality in a large, ethnically diverse population of patients hospitalized with COVID-19, and to determine the association of elevated inflammatory markers with increased troponin levels. We reviewed all patients admitted at our health system with COVID-19 from March 1 to April 27, 2020, who had a troponin assessment within 48 hours of admission. We used logistic regression to calculate odds ratios (ORs) for mortality during hospitalization, controlling for demographics, co-morbidities, and markers of inflammation. Of 11,159 patients hospitalized with COVID-19, 6,247 had a troponin assessment within 48 hours. Of these, 4,426 (71%) patients had normal, 919 (15%) had mildly elevated, and 902 (14%) had severely elevated troponin. Acute phase and inflammatory markers were significantly elevated in patients with mildly and severely elevated troponin compared with normal troponin. Patients with elevated troponin had significantly increased odds of death for mildly elevated compared with normal troponin (adjusted OR, 2.06; 95% confidence interval, 1.68 to 2.53; p < 0.001) and for severely elevated compared with normal troponin (OR, 4.51; 95% confidence interval, 3.66 to 5.54; p < 0.001) independently of elevation in inflammatory markers. In conclusion, patients hospitalized with COVID-19 and elevated troponin had markedly increased mortality compared with patients with normal troponin levels. This risk was independent of cardiovascular co-morbidities and elevated markers of inflammation.

Cardiotoxicity screening of long-term, breast cancer survivors-The CAROLE (Cardiac-Related Oncologic Late Effects) Study.

BACKGROUND: Long-term breast cancer survivors are at risk for cardiotoxicity after treatment, but there is insufficient evidence to provide long-term (~10 years) cardiovascular disease (CVD) screening recommendations. We sought to evaluate a tri-modality CVD screening approach. METHODS: This single-arm, feasibility study enrolled 201 breast cancer patients treated ≥6 years prior without CVD at diagnosis. Patients were sub-grouped: cardiotoxic (left-sided) radiation (RT), cardiotoxic (anthracycline-based) chemotherapy, both cardiotoxic chemotherapy and RT, and neither cardiotoxic treatment. Patients underwent electrocardiogram (EKG), transthoracic echocardiogram with strain (TTE with GLS), and coronary artery calcium computed tomography (CAC CT). The primary endpoint was preclinical or clinical CVD. RESULTS: Median age was 50 (29-65) at diagnosis and 63 (37-77) at imaging; median interval was 11.5 years (6.7-14.5). Among sub-groups, 44% had no cardiotoxic treatment, 31.5% had cardiotoxic RT, 16% had cardiotoxic chemotherapy, and 8.5% had both. Overall, 77.6% showed preclinical and/or clinical CVD and 51.5% showed clinical CVD. Per modality, rates of any CVD and clinical CVD were, respectively: 27.1%/10.0% on EKG, 50.0%/25.3% on TTE with GLS, and 50.8%/45.8% on CAC CT. No statistical difference was seen among the treatment subgroups (NS, χ2 test, p = 0.58/p = 0.15). CONCLUSION: This study identified a high incidence of CVD in heterogenous long-term breast cancer survivors, most >10 years post-treatment. Over half had clinical CVD findings warranting follow-up and/or intervention. Each imaging test independently contributed to the detection rate. This provides early evidence that long-term cardiac screening may be of value to a wider group of breast cancer survivors than previously recognized.

Myocardial hypoattenuation in cardiac sarcoidosis: CT correlation with CMR, PET and SPECT.

We report a marked abnormality in myocardial attenuation on non-gated contrast-enhanced CT in a patient with multiorgan sarcoidosis and correlate our findings with CMR, PET and SPECT. The noteworthy observation of myocardial hypoattenuation, in correspondence with the multimodality cardiovascular imaging findings, suggests that standard contrast-enhanced CT may provide information regarding tissue characterization. This report also demonstrates the independent clinical utility of CMR and PET in the evaluation and management of cardiac sarcoidosis.

Inadvertent anastomosis of the left internal mammary artery to the great cardiac vein.

We present an uncommon and underreported complication of coronary artery bypass graft surgery: erroneous anastomosis of the left internal mammary artery to the great cardiac vein. The iatrogenic aorto-coronary arteriovenous fistula with left-to-right shunting resulted in dilation of the coronary sinus, a supporting secondary finding. Factors predisposing to this inadvertent anastomosis include an intramyocardial segment of the left anterior descending coronary artery, demonstrated in this case, as well as epicardial fat and potentially fibrosis of the underlying myocardium.

Aortic Pseudoaneurysm Causing Compression of the Left Main Coronary Artery.

A 75-year-old man with a history of mechanical aortic valve replacement with aortic conduit for severe aortic insufficiency underwent routine screening computed tomography evaluation revealing right coronary anastomosis endoleak and proximal aortic root pseudoaneurysm.

Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis.

Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis. The severe progression of the disease required surgical intervention with endocardial stripping to treat the right-sided diastolic heart failure.

Histologic Sequelae of Apical Hypertrophic Cardiomyopathy: Dystrophic Calcification.

We present cardiac computed tomography (CT) findings demonstrating apical hypertrophic cardiomyopathy with dystrophic calcification of the left ventricular apex. The absence of significant epicardial coronary artery disease demonstrated by coronary CT angiography suggests that increased wall tension and decreased microvascular perfusion over time account for the dyskinetic apical myocardium, rather than myocardial infarction secondary to atherosclerotic plaque rupture. These observations support CT as the imaging modality of choice to visualize the deposition of calcium in injured myocardial tissue, a recognized occurrence in chronically infarcted myocardium.