Campylobacter-Associated Myocarditis in a 17-Year-Old Male.

Chest pain is a common presenting complaint in adolescent patients. Myocarditis is an important and potentially serious etiology of chest pain for clinicians who care for these patients to recognize. Myocarditis is commonly virally mediated, while extra-intestinal cardiac manifestations of bacterial enteritis, such as Campylobacter infections,are rare. Awareness of this uncommon, but potentially life-threatening pathophysiology is important for clinicians to understand.  In our case, a 17-year-old male presented with chest discomfort, chest pain on inspiration, headache, myalgias, vomiting, and diarrhea. He denied recent viral illnesses or immunizations. He lived in rural Ohio, swam recently in a freshwater lake, and had eaten home-prepared deer meat. His father had diarrhea as well. Presenting vital signs were within normal limits for age. The patient was obese (BMI 48.5), with an otherwise normal physical exam, including a thorough cardiopulmonary assessment. Laboratory workup revealed leukocytosis (16.1 x 109/L) and elevated high-sensitivity troponin (15,857 ng/L, >22,000 ng/L three hours later, ref range <20). Gastrointestinal polymerase chain reaction (PCR) panel detected Campylobacter spp., and stool culture was positive for Campylobacter jejuni. ECG, echocardiography, chest X-ray, and CT angiography were normal. Cardiac MRI revealed an increased T2 signal consistent with myocarditis. The patient was treated with non-steroidal anti-inflammatory drugs (NSAIDs) and azithromycin and had complete resolution in symptoms. He was exercise-restricted for six months.  Myocarditis is a potentially fatal pathology, representing a significant cause of sudden death in young adults. Myocarditis can present with a broad spectrum of signs and symptoms as well as variable clinical severity. Bacterial causes of myocarditis are uncommon, with Campylobacter among the least common. Campylobacter gastroenteritis, however, is quite common worldwide. Extra-intestinal and cardiac manifestations are rare; thus, it is important to maintain a high index of suspicion. Due in part to its rarity, treatment for Campylobacter-associated myocarditis is not well established. Treatment for myocarditis, regardless of etiology, is largely supportive in nature. Campylobacter-directed antibiotics, such as azithromycin, have been used successfully in adolescents with Campylobacter-associated myocarditis. Non-steroidal anti-inflammatory drugs (NSAIDs) are frequently used for symptom control, though their use remains controversial. Activity restriction is recommended for six months to reduce the risk of sudden cardiac death.  Myocarditis is an important cause of sudden death in young adults and is a rare extra-intestinal manifestation of Campylobacter bacterial gastroenteritis. Pediatric and adult providers should be aware of this presentation and its pathophysiology. They should also utilize a multi-modal workup, aggressive supportive care, appropriate subspecialty consultation, and appropriate antibiotics for patients with diarrheal illness and a high clinical suspicion for extra-intestinal involvement, such as myocarditis.

Hydroxychloroquine-induced cardiomyopathy: role of cardiac magnetic resonance for the diagnosis and follow-up of a very rare entity-a case report.

Background: Hydroxychloroquine (HCQ) is a disease-modifying antirheumatic used in rheumatological diseases such as systemic lupus erythematosus. Long-term exposure to HCQ results in drug accumulation and predisposes to adverse effects. Case summary: We present the case of a 45-year-old woman with long-term treatment with HCQ who presented to the Emergency Department with acute heart failure. Transthoracic echocardiogram, previously normal, showed severe biventricular hypertrophy and biventricular systolic dysfunction. Cardiac magnetic resonance (CMR) confirmed the previous findings and showed elevated native T1 and T2 values, elevated extracellular volume, and extensive mid-wall late gadolinium enhancement (LGE). Infiltrative cardiomyopathy was suspected, and endomyocardial biopsy performed. Light microscopy showed myocyte hypertrophy and vacuolar change and absence of lymphocytic inflammatory infiltrates. The diagnosis of HCQ-induced cardiomyopathy was established, and the drug was withdrawn. A CMR performed 1 year later showed normal systolic function of both ventricles and normalization of T2 values, reflecting resolution of myocardial oedema. However, severe hypertrophy, elevated native T1 values, and LGE persisted. Discussion: Our case shows that although discontinuation of the drug stops the progression of the disease, established myocardial structural damage persists. Early diagnosis of this entity is therefore essential to improve prognosis.

Mesalazine-induced myocarditis in a patient with ulcerative colitis: a case report.

Background: Mesalazine is an established first-line therapy for inflammatory bowel disease (IBD) and remains the mainstay of treatment for mild to moderate ulcerative colitis (UC). Both mesalazine and UC are rare but recognized causes of myopericarditis. Cardiac magnetic resonance (CMR) is a non-invasive method of assessing for myopericarditis. This case reports highlights the importance of early CMR in diagnosis, and management of myocarditis in a patient with IBD. Case summary: A 28-year-old male was admitted with a 2-day history of chest pain. Three weeks prior to this presentation, the patient was initiated on mesalazine for UC. Serum troponin T and C-reactive protein were elevated. An echocardiogram showed borderline low left ventricular systolic function (LVEF = 50-55%). A CMR showed extensive patchy late gadolinium enhancement (LGE) in the mid to epicardial basal and mid lateral wall. The findings were consistent with acute myocarditis, and a working diagnosis of mesalazine-induced myocarditis was made. Mesalazine was stopped and guideline-directed anti-inflammatories initiated. Oral prednisolone was also introduced for IBD control. Follow-up CMR at four months showed near complete resolution of LGE. Discussion: Myocarditis in the context of IBD may be infective, immune-mediated or due to mesalazine hypersensitivity. Histological conformation was not available in this case. This case report highlights the importance of access to early CMR in order establish the diagnosis and withdrawal of the culprit medication. In the majority of cases, CMR will replace the need for endomyocardial biopsy; however, this may still be required in the most severe cases.

Fat-free noncontrast whole-heart CMR with fast and power-optimized off-resonant water excitation pulses.

BACKGROUND: Cardiovascular MRI (CMR) faces challenges due to the interference of bright fat signals in visualizing structures like coronary arteries. Effective fat suppression is crucial, especially when using whole-heart CMR techniques. Conventional methods often fall short due to rapid fat signal recovery, leading to residual fat content hindering visualization. Water-selective off-resonant radiofrequency (RF) pulses have been proposed but come with tradeoffs between pulse duration, which increases scan time, and increased RF energy deposit, which limits their applicability due to specific absorption rate (SAR) constraints. The study introduces a lipid-insensitive binomial off-resonant (LIBOR) RF pulse, which addresses concerns about SAR and scan time, and aims to provide a comprehensive quantitative comparison with published off-resonant RF pulses for CMR at 3T. METHODS: A short (1ms) LIBOR pulse, with reduced RF power requirements, was developed and implemented in a free-breathing respiratory-self-navigated 3D radial whole-heart CMR sequence at 3T. A binomial off-resonant rectangular (BORR) pulse with matched duration, as well as previously published lipid-insensitive binomial off-resonant excitation (LIBRE) pulses (1ms and 2.2ms), were implemented and optimized for fat suppression in numerical simulations and validated in volunteers (n=3). Whole-heart CMR was performed in volunteers(n=10) with all four pulses. The signal-to-noise ratio (SNR) of ventricular blood, skeletal muscle, myocardium, and subcutaneous fat and the coronary vessel detection rates and sharpness were compared. RESULTS: Experimental results validated numerical findings and near homogeneous fat suppression was achieved with all four pulses. Comparing the short RF pulses (1ms), LIBOR reduced the RF power nearly two-fold compared with LIBRE, and three-fold compared with BORR, and LIBOR significantly decreased overall fat SNR from cardiac scans, compared to LIBRE and BORR. The reduction in RF pulse duration (from 2.2ms to 1ms) shortened the whole-heart acquisition from 8.5min to 7min. No significant differences in coronary arteries detection and sharpness were found when comparing all four pulses. CONCLUSION: LIBOR pulses enabled whole-heart CMR under 7minutes at 3T, with large volume fat signal suppression, while reducing RF power compared with LIBRE and BORR pulses. LIBOR is an excellent candidate to address SAR problems encountered in CMR sequences where fat suppression remains challenging and short RF pulses are required. AVAILABILITY OF DATA AND MATERIALS: An online repository containing the anonymized human MRI raw data, as well as RF pulse shapes used in this study is publicly available at: https://zenodo.org/records/8338079(PART 1: KNEE V1-V3, HEART V1-V5) https://zenodo.org/records/10715769 (PART 2: HEART V6-V10) Matlab code to 1) simulate the different RF pulses within a GRE sequence and 2) to read and display the anonymized raw data is available from: https://github.com/QIS-MRI/LIBOR_LIBRE_BORR_SimulationCode The compiled research sequence can be requested through the Teamplay platform of Siemens Healthineers.

Free-Running Cardiac and Respiratory Motion-Resolved Imaging: A Paradigm Shift for Managing Motion in Cardiac MRI?

Cardiac magnetic resonance imaging (MRI) is widely used for non-invasive assessment of cardiac morphology, function, and tissue characteristics due to its exquisite soft-tissue contrast. However, it remains time-consuming and requires proficiency, making it costly and limiting its widespread use. Traditional cardiac MRI is inefficient as signal acquisition is often limited to specific cardiac phases and requires complex view planning, parameter adjustments, and management of both respiratory and cardiac motion. Recent efforts have aimed to make cardiac MRI more efficient and accessible. Among these innovations, the free-running framework enables 5D whole-heart imaging without the need for an electrocardiogram signal, respiratory breath-holding, or complex planning. It uses a fully self-gated approach to extract cardiac and respiratory signals directly from the acquired image data, allowing for more efficient coverage in time and space without the need for electrocardiogram gating, triggering, navigators, or breath-holds. This review provides a comprehensive overview of the free-running framework, detailing its history, concepts, recent improvements, and clinical applications.

Comparison of left ventricular mass and wall thickness between cardiac computed tomography angiography and cardiac magnetic resonance imaging using machine learning algorithms.

Aims: Cardiac magnetic resonance imaging (MRI) is the gold standard in the assessment of left ventricle (LV) mass and wall thickness. In recent years, cardiac computed tomography angiography (CCTA) has gained widespread usage as an imaging modality. Despite this, limited previous investigations have specifically addressed the potential of CCTA as an alternative modality for quantitative LV assessment. The aim of this study was to compare CCTA derived LV mass and wall thickness with cardiac MRI utilizing machine learning algorithms. Methods and results: Fifty-seven participants who underwent both CCTA and cardiac MRI were identified. LV mass and wall thickness was calculated using LV contours which were automatically placed using in-house developed machine learning models. Pearson's correlation coefficients were calculated along with Bland-Altman plots to assess the agreement between the LV mass and wall thickness per region on CCTA and cardiac MRI. Inter-observer correlations were tested using Pearson's correlation coefficient. Average LV mass and wall thickness for CCTA and cardiac MRI were 127 g, 128 g, 7, and 8 mm, respectively. Bland-Altman plots demonstrated mean differences and corresponding 95% limits of agreement of -1.26 (25.06; -27.58) and -0.57 (1.78; -2.92), for LV mass and average LV wall thickness, respectively. Mean differences and corresponding 95% limits of agreement for wall thickness per region were -0.75 (1.34; -2.83), -0.58 (2.14; -3.30), and -0.29 (3.21; -3.79) for the basal, mid, and apical regions, respectively. Inter-observer correlations were excellent. Conclusion: Quantitative assessment of LV mass and wall thickness on CCTA using machine learning algorithms seems feasible and shows good agreement with cardiac MRI.

Diagnostic performance and relationships of structural parameters and strain components for the diagnosis of cardiac amyloidosis with MRI.

PURPOSE: The purpose of this study was to evaluate the diagnostic performance and relationships of cardiac MRI structural parameters and strain components in patients with cardiac amyloidosis (CA) and to estimate the capabilities of these variables to discriminate between CA and non-amyloid cardiac hypertrophy (NACH). MATERIALS AND METHODS: Seventy patients with CA (56 men; mean age, 76 ± 10 [standard deviation] years) and 32 patients (19 men; mean age, 63 ± 10 [standard deviation] years) with NACH underwent cardiac MRI. Feature tracking (FT) global longitudinal strain (GLS), radial strain (GRS), circumferential strain (GCS), strain AB ratio (apical strain divided by basal strain), myocardial T1, myocardial T2 and extracellular volume (ECV) were calculated. Comparisons between patients with CA and those with NACH were made using Mann-Whitney rank sum test. The ability of each variable to discriminate between CA and NACH was estimated using area under the receiver operating characteristic curve (AUC). RESULTS: Patients with CA had higher median GLS (-7.0% [Q1, -9.0; Q3, -5.0]), higher median GCS (-12.0% [Q1, -15.0; Q3, -9.0]), and lower median GRS (16.5% [Q1, 13.0; Q3, 23.0]) than those with NACH (-9.0% [Q1, -11.0; Q3, -8.0]; -17.0% [Q1, -20.0; Q3, -14.0]; and 25.5% [Q1, 16.0; Q3, 31.5], respectively) (P < 0.001 for all). Median myocardial T1 and ECV were significantly higher in patients with CA (1112 ms [Q1, 1074; Q3, 1146] and 47% [Q1, 41; Q3, 55], respectively) than in those with NACH (1056 ms [Q1, 1011; Q3, 1071] and 28% [Q1, 26; Q3, 30], respectively) (P < 0.001). Basal ECV showed the best performance for the diagnosis of CA (AUC = 0.975; 95% confidence interval [CI]: 0.947-1). No differences in AUC were found between AB ratio of GRS (0.843; 95% CI: 0.768-0.918) and basal myocardial T1 (0.834; 95% CI: 0.741-0.928) for the diagnosis of CA (P = 0.81). The combination of the AB ratio of FT-GRS and basal myocardial T1 had a diagnostic performance not different from that of basal ECV (P = 0.06). CONCLUSION: ECV outperforms FT-strain for the diagnosis of CA with cardiac MRI. The AB ratio of FT-GRS associated with myocardial T1 provides diagnostic performance similar to that achieved by ECV.

Flow quantification within the aortic ejection tract using 4D flow cardiac MRI in patients with bicuspid aortic valve: Implications for the assessment of aortic regurgitation.

PURPOSE: The purpose of this study was to evaluate the performance of four-dimensional (4D) flow cardiac MRI in quantifying aortic flow in patients with bicuspid aortic valve (BAV). MATERIALS AND METHODS: Patients with BAV who underwent transthoracic echocardiography (TTE) and 4D flow cardiac MRI were prospectively included. Aortic flow was quantified using two-dimensional phase contrast velocimetry at the sinotubular junction and in the ascending aorta and using 4D flow in the regurgitant jet, in the left ventricular outflow tract, at the aortic annulus, the sinotubular junction, and the ascending aorta, with or without anatomical tracking. Flow quantification was compared with ventricular volumes, pulmonary flow using Pearson correlation test, bias and limits of agreement (LOA) using Bland Altman method, and with multiparametric transthoracic echocardiography quantification using weighted kappa test. RESULTS: Eighty-eight patients (63 men, 25 women) with a mean age of 50.5 ± 14.8 (standard deviation) years (age range: 20.8-78.3) were included. Changes in flow with or without tracking were modest (< 5 mL). The best correlation was obtained at the aortic annulus for forward volume (r = 0.84; LOA [-28.4; 25.3] mL) and at the regurgitant jet and sinotubular junction for regurgitant volume (r = 0.68; LOA [-27.8; 33.8] and r = 0.69; LOA [-28.6; 24.2] mL). A combined approach for regurgitant fraction and net volume calculations using forward volume measured at ANN and regurgitant volume at sinotubular junction performed better than each level taken separately (r = 0.90; LOA [-20.7; 10.0] mL and r = 0.48, LOA [-33.8; 33.4] %). The agreement between transthoracic echocardiography and 4D flow cardiac MRI for aortic regurgitation grading was poor (kappa, 0.13 to 0.42). CONCLUSION: In patients with BAV, aortic flow quantification by 4D flow cardiac MRI is the most accurate at the annulus for the forward volume, and at the sinotubular junction or directly in the jet for the regurgitant volume.

Large left ventricular outflow tract mass in a young patient: uncommon presentation of a common disease! A case report.

Background: Infective endocarditis (IE) predominantly involves the cardiac valves. Timely diagnosis and initiation of therapy significantly reduce morbidity and mortality. Infective endocarditis presenting as a large left ventricular outflow tract (LVOT) mass is an atypical manifestation that provides significant challenges to the treating team. Case summary: A 19-year-young male presented with exertional shortness of breath, palpitations, and presyncope for 4 months with constitutional symptoms for the last 6 months. Two-dimensional echocardiogram showed a large LVOT mass arising from the mitral aortic intervalvular fibrosa causing dynamic severe aortic valve obstruction, moderate aortic regurgitation, and severe mitral regurgitation. He was managed on lines of IE and received intravenous antibiotics. In view of worsening heart failure and cardiogenic shock, he underwent mass excision, mechanical aortic valve replacement, and mitral valve repair. Histopathology confirmed it as vegetation. He was discharged and is doing well at 2-month follow-up. Discussion: An atypical presentation of IE as a large LVOT mass was observed in this young male. Sound clinical judgement, judicious use of ancillary imaging, and a multidisciplinary approach ensured timely diagnosis and appropriate treatment. Management included appropriate intravenous antibiotics followed by surgery.

Multi-modality imaging to assess rheumatic mitral stenosis severity.

Multi-modality imaging is the recommended approach to assess the severity of valvular heart diseases. Rheumatic mitral stenosis (MS), however, has yet to benefit from this approach. The aim of this study is to assess the added value of cardiac MRI (CMR) and computed tomography (CT) calcium score in assessing severity of MS when compared to 3D echocardiography. Patients with MS in sinus rhythm were included. Both CMR and 3D echo assessments of the mitral valve were performed. Subsequently, three radiologists and three cardiologists independently measured mitral valve area (MVA). In addition, CT of the mitral valve was conducted. Mitral calcium score was calculated according the Agatston method. A total of 41 patients were included. CMR significantly overestimated MVA when compared to 3D echo MVA regardless of the investigator [F (1, 40) = 23.3, p < 0.001, η2 = 0.36]. The more severe the MS, the greater the overestimation by CMR compared to 3D echo. Regarding CT of the mitral valve, 25 (61%) patients had an undetectable calcium. There was no significant difference in CT calcium scores between severe and non-severe MS (74 ± 282 HU vs. 65 ± 210 HU, p = 0.9). MVA measurement by CMR is overestimated when compared to 3D echo. Additionally, mitral valve calcium score is not correlated to MS severity.

Prognostic value of CMR-derived extracellular volume in AL amyloidosis: a multicenter study.

BACKGROUND: This study aimed to assess the prognostic value of cardiac magnetic resonance (CMR) variables and compare them with biological and echocardiographic markers in patients with AL cardiac amyloidosis (CA). METHODS: We conducted a prospective study across three tertiary centres, where patients underwent clinical examination, blood tests, echocardiography, and CMR. The primary endpoint was all-cause mortality. RESULTS: A total of 176 patients with AL CA were included, with a median age of 68 years (IQR 58-75). According to the 2004 Mayo Clinic staging, 121 patients (69%) were in stage 3. During a median follow-up of 22 months (IQR 8-48), 45 patients died, and 55 were hospitalized for heart failure. Patients who died had higher NT-proBNP and troponin levels, and lower LVEF, cardiac output, and longitudinal strain. Among CMR variables, extracellular volume (ECV) was most strongly associated with all-cause mortality. In multivariate Cox models, including Mayo Clinic staging, ECV ≥ 0.45 was independently associated with mortality (HR 2.36, CI 95% 1.47-5.60) and also with heart failure hospitalizations (HR 4.10, 95%CI 2.15-8.8). CONCLUSION: ECV is a powerful predictor of outcomes in AL CA, providing additional prognostic value on top of Mayo Clinic staging.

Fatty acid composition MRI of epicardial adipose tissue: Methods and detection of proinflammatory biomarkers in ST-segment elevation myocardial infarction patients.

PURPOSE: To develop a method for quantifying the fatty acid composition (FAC) of human epicardial adipose tissue (EAT) using accelerated MRI and identify its potential for detecting proinflammatory biomarkers in patients with ST-segment elevation myocardial infarction (STEMI). METHODS: A multi-echo radial gradient-echo sequence was developed for accelerated imaging during a breath hold using a locally low-rank denoising technique to reconstruct undersampled images. FAC mapping was achieved by fitting the multi-echo images to a multi-resonance complex signal model based on triglyceride characterization. Validation of the method was assessed using a phantom comprised of multiple oils. In vivo imaging was performed in STEMI patients (n = 21; 14 males/seven females). FAC was quantified in EAT, subcutaneous AT, and abdominal visceral AT. RESULTS: Phantom validation demonstrated strong correlations (r > 0.97) and statistical significance (p < 0.0001) between measured and reference proton density fat fraction and FAC values. In vivo imaging of STEMI patients revealed a distinct EAT FAC profile compared to subcutaneous AT and abdominal visceral AT. EAT FAC parameters had significant correlations with left ventricular (LV) end-diastolic volume index (p < 0.05), LV end-systolic volume index (p < 0.05), and LV mass index (p < 0.05). CONCLUSIONS: Accelerated MRI enabled accurate quantification of human EAT FAC. The relationships between the EAT FAC profile and LV structure and function in STEMI patients suggest the potential of EAT FAC MRI as a biomarker for adipose tissue quality and inflammatory status in cardiovascular disease.

Early detection of chemotherapy-induced cardiotoxicity in breast cancer patients: a comprehensive analysis using speckle tracking echocardiography.

Introduction: Chemotherapy-induced cardiotoxicity poses a significant challenge in the treatment of breast cancer, potentially compromising both the efficacy of cancer therapy and cardiac health of patients. This study aimed to enhance the early detection of cardiotoxic effects by integrating advanced imaging modalities and biomarker analysis, thereby facilitating timely interventions to mitigate cardiac risk. Methods: A prospective cohort design was employed, enrolling breast cancer patients scheduled for potentially cardiotoxic chemotherapy regimens. The study utilized a comprehensive diagnostic toolkit, including echocardiography with strain imaging, cardiac MRI, and serial measurements of cardiac biomarkers such as high-sensitivity troponins and natriuretic peptides. Results: The analysis revealed that subtle changes in myocardial strain parameters and early biomarker elevations were predictive of subsequent declines in left ventricular function, preceding conventional echocardiographic evidence of cardiotoxicity. Logistic regression analysis highlighted the additive predictive value of integrating biomarker data with advanced imaging findings to identify patients with the highest risk of significant cardiotoxicity. Discussion: The study concluded that an integrated diagnostic approach, combining detailed imaging assessments with sensitive biomarker analysis, offers a superior strategy for the early detection of chemotherapy-induced cardiotoxicity in breast cancer patients. This proactive diagnostic strategy empowers clinicians to tailor cancer therapy more precisely, balancing oncologic efficacy with cardiovascular safety and underscores the importance of a multidisciplinary approach in the management of patients undergoing potentially cardiotoxic chemotherapy.

Imaging for the diagnosis of acute myocarditis: can artificial intelligence improve diagnostic performance?

Myocarditis is a cardiovascular disease characterised by inflammation of the heart muscle which can lead to heart failure. There is heterogeneity in the mode of presentation, underlying aetiologies, and clinical outcome with impact on a wide range of age groups which lead to diagnostic challenges. Cardiovascular magnetic resonance (CMR) is the preferred imaging modality in the diagnostic work-up of those with acute myocarditis. There is a need for systematic analytical approaches to improve diagnosis. Artificial intelligence (AI) and machine learning (ML) are increasingly used in CMR and has been shown to match human diagnostic performance in multiple disease categories. In this review article, we will describe the role of CMR in the diagnosis of acute myocarditis followed by a literature review on the applications of AI and ML to diagnose acute myocarditis. Only a few papers were identified with limitations in cases and control size and a lack of detail regarding cohort characteristics in addition to the absence of relevant cardiovascular disease controls. Furthermore, often CMR datasets did not include contemporary tissue characterisation parameters such as T1 and T2 mapping techniques, which are central to the diagnosis of acute myocarditis. Future work may include the use of explainability tools to enhance our confidence and understanding of the machine learning models with large, better characterised cohorts and clinical context improving the diagnosis of acute myocarditis.

Cardiac manifestations and outcomes of COVID-19 vaccine-associated myocarditis in the young in the USA: longitudinal results from the Myocarditis After COVID Vaccination (MACiV) multicenter study.

Background: We aimed to study the clinical characteristics, myocardial injury, and longitudinal outcomes of COVID-19 vaccine-associated myocarditis (C-VAM). Methods: In this longitudinal retrospective observational cohort multicenter study across 38 hospitals in the United States, 333 patients with C-VAM were compared with 100 patients with multisystem inflammatory syndrome in children (MIS-C). We included patients ≤30 years of age with a clinical diagnosis of acute myocarditis after COVID-19 vaccination based on clinical presentation, abnormal biomarkers and/or cardiovascular imaging findings. Demographics, past medical history, hospital course, biochemistry results, cardiovascular imaging, and follow-up information from April 2021 to November 2022 were collected. The primary outcome was presence of myocardial injury as evidenced by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging. Findings: Patients with C-VAM were predominantly white (67%) adolescent males (91%, 15.7 ± 2.8 years). Their initial clinical course was more likely to be mild (80% vs. 23%, p < 0.001) and cardiac dysfunction was less common (17% vs. 68%, p < 0.0001), compared to MIS-C. In contrast, LGE on CMR was more prevalent in C-VAM (82% vs. 16%, p < 0.001). The probability of LGE was higher in males (OR 3.28 [95% CI: 0.99, 10.6, p = 0.052]), in older patients (>15 years, OR 2.74 [95% CI: 1.28, 5.83, p = 0.009]) and when C-VAM occurred after the first or second dose as compared to the third dose of mRNA vaccine. Mid-term clinical outcomes of C-VAM at a median follow-up of 178 days (IQR 114-285 days) were reassuring. No cardiac deaths or heart transplantations were reported until the time of submission of this report. LGE persisted in 60% of the patients at follow up. Interpretation: Myocardial injury at initial presentation and its persistence at follow up, despite a mild initial course and favorable mid-term clinical outcome, warrants continued clinical surveillance and long-term studies in affected patients with C-VAM. Funding: The U.S. Food and Drug Administration.

Contemporary Multimodality Imaging for Diagnosis and Management of Fabry Cardiomyopathy.

Fabry disease (FD) is an X-linked lysosomal storage disorder which leads to the accumulation of globotriaosylceramide (Gb3) in various organs, including the heart. FD can be subdivided into classic disease resulting from negligible residual enzyme activity and a milder, atypical phenotype with later onset and less severe clinical presentation. The use of multimodality cardiac imaging including echocardiography, cardiac magnetic resonance and nuclear imaging is important for the diagnostic and prognostic evaluation in these patients. There are gaps in the literature regarding the comprehensive description of cardiac findings of FD and its evaluation by multimodality imaging. In this review, we describe the contemporary practices and roles of multimodality cardiac imaging in individuals affected with Fabry disease.

Empagliflozin and left atrial function in patients with type 2 diabetes mellitus and coronary artery disease: insight from the EMPA-HEART CardioLink-6 randomized clinical trial.

BACKGROUND: Sodium-glucose cotransporter-2 (SGLT2) inhibitors have demonstrated reduction in heart failure outcomes in patients with type 2 diabetes mellitus, although the exact mechanism of benefit remains unclear. Alteration in left atrial (LA) function due to chronic pressure or volume overload is a hallmark of heart failure. OBJECTIVE: To evaluate the effect of the SGLT2 inhibitor empagliflozin on LA volume and function. METHODS: 90 patients with coronary artery disease and type 2 diabetes (T2DM) were randomized to empagliflozin (n = 44) or placebo (n = 46), and underwent cardiac magnetic resonance (CMR) imaging at baseline and after 6 months. The main outcome was change in LA volume; LA function, including active and passive components, was also measured by a blinded reader. RESULTS: At baseline, there was no significant difference in LA volumes between the empagliflozin (indexed maximum LA volume 26.4 ± 8.4mL/m2, minimum LA volume 11.1 ± 5.7mL/m2) and placebo (indexed maximum LA volume 28.7 ± 8.2mL/m2, minimum LA volume 12.6 ± 5.0mL/m2) groups. After 6 months, changes in LA volumes did not differ with adjusted difference (empagliflozin minus placebo): 0.99 mL/m2 (95% CI: -1.7 to 3.7 mL/m2; p = 0.47) for indexed maximum LA volume, and 0.87 mL/m2 (95% CI: -0.9 to 2.6 mL/m2; p = 0.32) for indexed minimum LA volume. Changes in total LA emptying fraction were also similar, with between-group adjusted mean difference - 0.01 (95% CI: -0.05 to 0.03, p = 0.59). CONCLUSION: SGLT2 inhibition with empagliflozin for 6 months did not have a significant impact on LA volume and function in patients with T2DM and coronary artery disease. (Effects of Empagliflozin on Cardiac Structure in Patients with Type 2 Diabetes [EMPA-HEART]; NCT02998970).