Background/Objectives: Fabry disease (FD) is a lysosomal storage disorder characterized by progressive renal and cardiac involvement and an increased burden of cardiovascular and cerebrovascular events. While cardiac magnetic resonance imaging (CMR) has significantly advanced structural assessment, circulating biomarkers reflecting disease-related cardiac manifestations remain incompletely understood. We therefore investigated adiponectin and leptin, two adipokines involved in inflammatory, metabolic, and fibrotic pathways, in relation to cardiac involvement and analyzed long-term lipid trajectories in FD. Methods: This longitudinal observational study included 49 patients with FD with 149 study visits. Circulating adiponectin, leptin, NT-proBNP, and conventional lipid parameters were assessed longitudinally and stratified by FD-specific therapy status and sex. Multivariable linear regression was performed to evaluate independent associations with log-transformed NT-proBNP values. Results: Adiponectin was positively associated with NT-proBNP, reflecting cardiac involvement, independent of age, sex, BMI, and eGFR (p < 0.001). Higher adiponectin levels were observed in patients with left ventricular hypertrophy or low T1 and those with fibrosis, detected by CMR (p = 0.009 and p < 0.001, respectively). This association was mainly seen in patients receiving FD-specific therapy, raising the question of whether this reflects underlying organ involvement or treatment effects. Leptin demonstrated weaker, inverse associations. Adiponectin, leptin, Triglycerides, total cholesterol, and HDL- and LDL-cholesterol levels remained stable over long-term follow-up, irrespective of FD-specific therapy or sex. Conclusions: In FD, adiponectin appears to be associated with cardiac involvement, and conventional lipid parameters remained unchanged over time. These findings suggest that alterations in adipokines, rather than progressive dyslipidemia, may reflect disease-related cardiac manifestations.

Purpose To evaluate pulmonary arterial pulse wave velocity (PWV) derived from phase-resolved functional lung (PREFUL) MRI in participants with suspected pulmonary hypertension (PH), assess its association with established markers, and investigate its prognostic value for 12-month mortality. Materials and Methods This post hoc analysis of a prospective multicenter Chronic Thromboembolic Pulmonary Hypertension Diagnosis Europe MRI (CHANGE MRI) study included participants with suspected chronic thromboembolic PH and complete PREFUL MRI between July 2016 and November 2023. PREFUL MRI-based two-dimensional images acquired at 1.5 T using spoiled gradient-echo sequences were used to calculate quantified perfusion, perfusion defect percentages, and PWV. Group comparisons were performed between PH and no PH and among PH groups using age- and sex-adjusted models (analysis of covariance framework). Spearman correlations with right heart catheterization and cardiac MRI parameters and associations with 12-month mortality were assessed using multivariable regression and Cox proportional hazard models. A P value less than .05 was considered statistically significant. Results A total of 632 participants (median age, 66 years; IQR, 22 years; 368 [58%] female) were included. Of these, 415 had confirmed PH, and 13 died within 12 months. PWV was higher in pulmonary arterial hypertension than in chronic thromboembolic PH (2.76 m/sec ± 1.32 vs 1.84 m/sec ± 1.29; P < .001). In participants with PH, PWV was associated with mortality (hazard ratio, 2.28 per SD increase [1.47 m/sec]; P < .001). PWV showed independent associations with right ventricular ejection fraction (β = -0.024 m/sec per percentage point; P < .001) and perfusion defect percentage (β = 0.014 m/sec per percentage point; P = .02). Conclusion Pulmonary arterial PWV derived from PREFUL MRI was higher in PH and independently associated with right ventricular function and 12-month mortality. Keywords: Functional Lung MRI, Pulmonary Arteries, Lung ClinicalTrials.gov identifier: NCT02791282 Supplemental material is available for this article. © RSNA, 2026.

Aims:Cardiovascular magnetic resonance imaging has become pivotal in the non-invasive assessment of the heart. Bright-blood sequences are used to retrieve information about cardiac anatomy and function. Concurrently, novel black-blood late gadolinium enhancement sequences have showcased potential for scar detection by uncovering scar patterns that may be confounded with blood. In the acute setting, T2 mapping allows for quantitative characterization of oedematous tissue. Nowadays, these images are acquired sequentially through multiple breath-holds, adding to the workload of medical professionals, reducing patient comfort, and hampering image analysis.Here, we assess the clinical value of SPOT-MAPPING, a sequence combining co-registered T2 mapping and joint black- and bright-blood imaging. Methods and results:Twenty-six patients (27% women, age 64 ± 12yo) with acute, chronic, ischaemic, non-ischaemic, and overlapping cardiomyopathies, prospectively underwent SPOT-MAPPING at 1.5T. Conventional PSIR images and T2 maps served as the reference standard. Left ventricular (LV) mass, scar mass, burden, and transmurality and T2 values were retrieved and compared between sequences. Acquisition times were recorded. Acquisition time for SPOT-MAPPING was in average twice shorter than combined reference sequences (5 min 55 s [5 min 14 s-6 min 30 s] vs. 11 min 56 s [10 min 39s-12 min 12 s]). High reproducibility was obtained with reference sequences for LV mass (ICC ≥ 0.93). Strong agreement was observed with PSIR in scar extraction (mean bias: mass +2.3 g, burden +1.1%LV mass, transmurality +1.4%). No significant difference with reference T2 mapping was observed in remote (P = 1.000) and oedematous myocardium (P = 0.883). Conclusion:SPOT-MAPPING demonstrated its efficacy in a wide range of patients, proving itself as a time-efficient and reproducible CMR method for the assessment of various cardiac diseases.

Abstract Persistent cardiac symptoms are common in post-COVID syndrome, even without structural heart disease. Evidence implicates immune dysregulation, endothelial dysfunction and low-grade cardiovascular inflammation. Yet no targeted treatment exists. Myoflame-19 is a multicenter, double-blind clinical trial of 279 participants with inflammatory cardiac involvement defined by cardiovascular magnetic resonance, randomized 1:1 to losartan plus prednisolone ( n = 139 ) or matching placebos ( n = 140 ) for 16 weeks. The modified intention-to-treat population comprised 124 and 122 participants. The primary endpoint, change in left ventricular (LV) ejection fraction, was neutral: between-group difference 0.74 percentage points (pp), 95%CI −0.14 to 1.62, p = 0.10, unpaired t-test; supportive baseline-adjusted ANCOVA 0.99, 95%CI 0.15-1.83, p = 0.021. Among prespecified secondary endpoints, several symptom and imaging measures showed numerical differences favoring intervention, including Average Symptom Score components (modified Canadian Chest Pain Scale −4.8 pp, 95%CI −17.3 to 7.6; NYHA class −8.1 pp, −20.6 to 4.3; Long COVID symptom burden −7.7 pp, −18.9 to 3.6), native T1 and T2 values (native T1 −2.46 ms, −8.35 to 3.42; native T2 −0.31 ms, −1.16 to 0.53), and LV end-diastolic volume ( + 1.45 ml/m², −0.09 to 3.00); however, confidence intervals included the null value and these findings should be regarded as hypothesis-generating.Treatment was safe and well-tolerated. These findings indicate a neutral treatment effect on the primary endpoint. They inform targeted immunomodulation and design of future trials in post-COVID syndrome and inflammatory cardiac involvement. Trial registration: EudraCT 2022-001682-12; NCT05619653.

BACKGROUND:Mitral annular disjunction (MAD) is increasingly recognised on cardiac magnetic resonance (CMR) imaging, yet its clinical significance remains under discussion. We aimed to determine the prevalence, prognostic impact, and association with native T1 relaxation time of MAD assessed in both end-systole and end-diastole in an all-comer CMR cohort. METHODS:We analysed data from 1969 patients enrolled in a prospective CMR registry at the Vienna General Hospital. MAD was defined as ≥ 1 mm separation between the left atrial wall at the mitral valve hinge point and the adjacent left ventricular myocardium. Native T1 relaxation times were measured at septal and lateral positions of the mitral annulus in the 4-chamber view. All-cause mortality was analysed by Cox proportional hazards regression. RESULTS:End-systolic MAD was present in 509 patients (25.9%), end-diastolic in 366 (18.6%). MAD patients were younger (59.6 ± 18.5 vs. 65.2 ± 17.9 years, p < 0.001), had fewer comorbidities, and had higher left ventricular ejection fractions (58.8% ± 10.4% vs. 56.8% ± 14.4%, p = 0.009). MAD distance correlated with native annular T1 relaxation time (r = 0.24, p < 0.001), and patients with MAD ≥ 5 mm showed higher T1 relaxation time than those < 5 mm (1041 ± 148 vs. 1010 ± 72 ms, p = 0.028), while mid-septal T1 relaxation time showed no difference (r = 0.06, p = 0.252). During 63.7 months median follow-up, 485 deaths occurred. After multivariable adjustment, end-diastolic MAD was not associated with mortality (HR 0.95, 95%-CI 0.74-1.23, p = 0.712). CONCLUSIONS:MAD is common and prognostically neutral after adjustment. However, larger disjunction distances are associated with higher native T1 relaxation time values at the annulus, suggesting local subclinical myocardial remodelling.

Background: Sub-clinical inflammation is considered a key mechanism in cardiovascular disease and myocardial remodeling. We therefore evaluated whether the neutrophil-lymphocyte ratio (NLR), a simple inflammatory marker derived from a routine full blood count, is associated with myocardial fibrosis. Methods: Consecutive patients from a cardiac magnetic resonance imaging (CMR) registry were included and stratified by the NLR tertile. The association of the NLR and the levels of C-reactive protein (CRP) with CMR-derived myocardial T1 time and the extracellular volume fraction (ECV) were assessed using linear regression analysis and compared using Z-scores. In addition, an association with outcome was tested utilizing the log-rank test. Results: 1152 patients (72.4 years, 53.1% male) constituted the final cohort. The median NLR was 3.11 [interquartile range (IQR): 2.145-4.67]. Tertiles were based on the cut-off values ≤ 2.5, >2.5 and <4.0, and ≥4.0. A higher NLR tertile was associated with lower biventricular ejection fraction, hypertrophy, and increased right ventricular volume. The myocardial native T1 time [tertile 1 vs. 3: 1010 ms (984-1038) vs. 1030 (1001-1059), p < 0.001] and ECV [tertile 1 vs. 3: 26.3% (24.2-28.6) vs. 28.1% (25.5-31.4), p < 0.001] also significantly differed between the NLR tertiles. The NLR's and the CRP's association with elevated myocardial T1 time and ECV were comparable; however, the proportion of variation in the target variables explained by either was generally low. Conclusions: In our CMR all-comer cohort, NLR and CRP were significantly associated with prolonged myocardial T1 times and increased ECV. However, only a modest variation observed in both parameters was explained by either variable.

INTRODUCTION:Cardiac amyloidosis (CA) is a progressive cardiomyopathy caused by amyloid deposition, leading to heart failure and increased mortality. Cardiac magnetic resonance (CMR) identifies myocardial involvement via elevated native T1 relaxation time and extracellular volume (ECV). Although amyloid infiltration has also been observed in thoracic skeletal muscle, the diagnostic and prognostic relevance of thoracic skeletal muscle T1 time and ECV remain unclear. AIM:To compare native thoracic skeletal muscle T1 time and ECV between CA patients and controls and assess their diagnostic and prognostic value. METHODS:In a prospective CMR registry, consecutive CA patients and controls underwent CMR with T1-mapping. Native and post-contrast T1 relaxation time and ECV were quantified in myocardium and thoracic skeletal muscles. Diagnostic performance was evaluated using ROC analysis and associations with mortality were assessed using Cox regression. RESULTS:Among 1976 participants (267 CA, 1709 controls), CA patients showed significantly higher native myocardial T1 time and ECV, as well as elevated native thoracic skeletal muscle T1 time (919.4 vs. 868.5 ms, p < 0.001) and ECV (16.4 vs. 12.9%, p < 0.001). Native thoracic skeletal muscle T1 time demonstrated moderate diagnostic performance for CA (AUC = 0.70), with an optimal cutoff of 895 ms yielding a sensitivity of 61% and specificity of 59%. Higher native thoracic skeletal muscle T1 time predicted increased mortality (HR = 1.65 per 100 ms, p < 0.001), and this association remained significant after adjusting for age, sex and ventricular function (HR = 1.21 per 100 ms, p = 0.008). CONCLUSION:Native skeletal muscle T1 relaxation time and ECV indicate systemic amyloid involvement and provide additional diagnostic and prognostic information beyond myocardial assessment, potentially supporting improved detection and risk stratification in CA.

Amyloidosis is a systemic disorder characterized by amyloid fibril deposition in multiple organs, including the heart, brain, liver, kidneys, and lungs, leading to organ dysfunction and affecting survival. In cardiac amyloidosis (CA), where the heart is primarily affected, the broader impact on other organs, particularly the brain, is not fully understood. This study aimed to assess the extent of amyloid deposition across systemic organs in CA, investigate brain-organ, brain-brain and organ-organ connections, and evaluate their effects on patients’ survival. A retrospective analysis was conducted on 70 patients, categorized into three groups by biopsy: cardiac amyloidosis (CA) (n = 31), non-cardiac amyloidosis (non-CA) (n = 12), and no amyloidosis control (NC) (n = 27). 11C-PIB PET/CT scans were performed, and standard uptake value mean (SUVmean) values were extracted using automated segmentation techniques. Brain-organ, brain-brain, organ-organ connectivity analyses were conducted using Spearman correlation. Survival outcomes and prognostic factors were evaluated using Kaplan–Meier analysis. Amyloid deposition in CA patients was prominent in the myocardium, liver, and kidneys, reflected by increased SUVmean values. Significant brain-organ connectivity was observed, particularly between the posterior cingulate cortex (PCC), parietal lobes, and myocardial amyloid burden. Additionally, CA patients demonstrated markedly stronger inter-organ connectivity, indicating systemic involvement. In contrast, Non-CA and NC patients demonstrated weaker brain-organ and inter-organ interactions. The CA group exhibited a higher mortality rate, highlighting the severe prognosis, particularly in systemic CA (SCA). Furthermore, elevated amyloid deposition in the bone marrow, lungs and spleen within SCA group was strongly associated with increased mortality. In CA patients, amyloidosis was associated with extensive systemic involvement, as shown by significant brain-organ and inter-organ connectivity patterns. Findings emphasize that cardiac amyloidosis substantially impacts survival, particularly when accompanied by amyloid deposition in the bone marrow, lungs, and spleen, indicating the need for a comprehensive diagnostic and management approach. Not applicable.

Background/Objectives: Cardiac amyloidosis (CA) as well as coronary artery disease (CAD) are both highly prevalent among the elderly. However, both the prevalence and risk factors associated with significant CAD among patients with CA, as well as potential outcome disparities, remain mainly unexplored. This study aimed to show the prevalence of CAD in patients with CA, as well as to assess outcomes and differences in late gadolinium enhancement (LGE) in comparison to patients with lone CA. Methods: We retrospectively assessed CA patients who underwent CAD assessment between 2013 and 2023. The primary endpoint was all-cause death. A subgroup underwent cardiac magnetic resonance imaging (CMR) with LGE assessment. Results: Of 255 consecutive patients with CA, 81 patients had significant CAD. Differences could be found with respect to age, sex, arterial hypertension, and hyperlipidemia. Significant differences in CMR features could only be found with respect to indexed left-ventricular end-diastolic volume, as well as left-ventricular mass. CAD-specific LGE was present only in 17.7% of patients with CAD, while most patients showed typical amyloid LGE, making a viability diagnosis difficult via CMR, especially in patients with end-stage CA. No differences in outcomes could be observed according to the prevalence of CAD. Conclusions: Concomitant obstructive CAD is highly prevalent among patients with CA. However, the presence of CAD does not influence patient outcomes. Furthermore, our data suggests that CAD viability testing by CMR might be complicated in patients with concomitant CA due to the high prevalence of amyloid-specific LGE.

BACKGROUND:Cardiac magnetic resonance (CMR) derived hepatic T1-time is associated with outcome. However, the interplay between tricuspid regurgitation (TR), which can cause congestive hepatopathy and liver T1-time is unclear. METHODS:We measured hepatic T1-time in CMR all-comers, who underwent echocardiography within 3 weeks of CMR. Kaplan-Meier estimates and Cox regression models were used to investigate the association between hepatic T1-time, TR severity and a composite endpoint of heart failure hospitalisation and all-cause death. RESULTS:1029 participants (67 ± 17 y/o, 44% female) had a mean hepatic T1-time of 605 ± 79 ms. Overall, 41% (417) presented with non/trace, 38% (391) with mild, 13% (135) with moderate and 8% (85) with severe/massive/torrential TR. Liver T1-time was significantly associated with TR severity (no/trace: 586 ± 72 ms; mild: 601 ± 74 ms; moderate: 634 ± 84 ms; severe/massive/torrential: 665 ± 83 ms; β = 25.4 ms, [95% CI:19.7-31.2, p < .001]). After adjustment for serum NT-proBNP and right ventricular function in a linear regression model, TR severity remained significantly associated with hepatic T1-time (p < .001). During follow-up (mean 53 ± 36 months) 326 (32%) events occurred. Hepatic T1-time (adj.HR 1.69 [95% CI: 1.49-1.92] per 100 ms increase, p < .001) and TR (adj.HR 1.66 [95% CI: 1.49-1.84], p < .001) were both associated with outcome. Even after adjustment for serum NT-proBNP, cardiac structure and function, age, sex and TR severity, hepatic T1-time remained significantly associated with event-free survival (adj.HR 1.42 [95% CI: 1.20-1.68] per 100 ms increase, p < .001). CONCLUSION:TR exerts a notable influence on hepatic T1-time. Nevertheless, after adjustment for serum NTproBNP, cardiac function and TR severity, hepatic T1-time still independently predicts outcomes. This underscores the importance of hepatic T1-time both as a marker of TR and prognosis.

Aims:Perivascular adipose tissue attenuation (PVAT) has emerged as a novel coronary computed tomography angiography (CCTA)-based biomarker predicting cardiovascular events by capturing inflammation around the coronary arteries. We assessed whether PVAT adds incremental prognostic value in patients undergoing transcatheter aortic valve implantation (TAVI). Methods and results:A total of 510 patients underwent CCTA imaging prior to TAVI between November 2015 and June 2020 at the Medical University of Vienna. PVAT was obtained from CCTA images and was measured around the right coronary artery [PVAT(RCA)] and the aortic valve [PVAT(valve)]. Following application of exclusion criteria, 372 patients [mean age 80.6 ± 6.8 years; 169 (45%) women] were analysed. Over a median follow-up of 3.0 (IQR 2.5-3.6) years, 52 (14%) individuals experienced a major adverse cardiovascular event (MACE, a composite of non-fatal stroke or myocardial infarction, cardiac death, or vascular intervention). Individuals exhibiting elevated PVAT[valve] displayed a heightened surgical risk according to European System for Cardiac Operative Risk Evaluation II, a lower body mass index, reduced left ventricular ejection fraction, prolonged hospitalization following TAVI, and elevated levels of circulating inflammatory markers compared with those in the low PVAT[valve] group (P < 0.05). However, neither PVAT[valve] nor PVAT[RCA] were independently associated with the occurrence of MACE in adjusted multi-variable analyses (PVAT[valve]: sub-distribution hazard ratio [SHR] 1.14, 95% CI:0.63-2.05, P = 0.672); PVAT[RCA]: SHR 1.16 [95% CI: 0.81-1.66], P = 0.417). Conclusion:Measuring PVAT around either the right coronary artery or the aortic valve does not provide additional prognostic value beyond established risk factors for the prediction of MACE in patients undergoing TAVI.

Background: Acute SARS-CoV-2 infection may induce cardiac arrhythmias associated with viral myocarditis, which typically disappear in the convalescent phase after healing of the myocardial inflammation. Methods: We report the case of a 37-year-old woman with a childhood history of atrial septal defect repair and stable normofrequent atrial rhythm, who presented two months post-COVID-19 with palpitations and dizziness. Diagnostic evaluation included cardiac magnetic resonance imaging (CMR), 24 h Holter electrocardiogram (ECG) monitoring, and laboratory assessments over a 3-year period. Results: CMR suggested subacute myocarditis, and Holter ECG revealed multiple discernible complex cardiac arrhythmias including atrial bradycardia, intermittent junctional rhythm (JR), atrial fibrillation (AF), and non-sustained ventricular tachycardia. Laboratory results showed a moderate but transient increase in lactate dehydrogenase, persistently mildly elevated N-terminal pro-B-type natriuretic peptide (NT-proBNP), and immunoglobulin A (IgA), with all other cardiac, inflammatory, immunologic, and organ function parameters remaining normal. In spite of chaotic cardiac rhythm with alternating JR, AF, and atrial normofrequent rhythm with frequent blocked supraventricular beats and increasing atrioventricular conduction time, no therapeutic intervention was necessary during follow-up, and a conservative treatment approach was agreed with the patient. Two years post-COVID-19 infection, the patient returned to a normofrequent atrial rhythm with a markedly prolonged PQ time (500 ms) and a different P wave morphology compared to pre-COVID, without other rhythm disturbances. Conclusions: This case demonstrates a rare pattern of post-viral arrhythmias first emerging in the convalescent phase and resolving spontaneously after two years. It underscores the need for long-term rhythm surveillance following COVID-19, even in patients with prior structural heart disease and a stable baseline rhythm.

Quantitative magnetic resonance imaging (MRI) is emerging as a non-invasive tool to measure tissue scarring in renal allografts. However, whether prolonged T1 relaxation time results in lower transplant survival rates is unknown. This retrospective cohort study analyzed the capability to predict renal allograft dysfunction based on median T1 time. Forty-six transplant recipients with non-contrast 1.5T MRI and allograft biopsy were included. The primary endpoint was the eGFR slope over 24 months. T1 relaxation time correlated significantly with eGFR levels at all follow-up stages. Patients with T1 relaxation time above the median (T1high) had a consistent decline in kidney function as compared to the patient group below the median (T1low): overall eGFR slope: 11.3 vs. 1.4 mL/min/1.73 m2 over 24 months, p = 0.016. Graft survival rates at 24 months were 52% in the T1high vs. 87% in the T1low group, p = 0.0015. ROC analysis discovered a positive predictive value of 52% and a negative predictive value of 91% for graft loss. T1 mapping identified patients with a persistent decline of allograft function and an increased risk of allograft loss. MRI could significantly influence monitoring strategies in transplant surveillance, offering a safe, non-invasive alternative to traditional diagnostic methods.

Left atrial (LA) fibrosis is a significant contributor to the pathophysiology of atrial fibrillation (AF) and a well-known predictor for arrhythmia recurrence post ablation. LA epicardial adipose tissue (EAT) plays a role in cardiac metabolism, can influence local inflammation and is associated with AF. Recent data suggests that beside LA EAT volume, its status of inflammation, as identified by higher values of attenuation, plays a significant role in the development and maintenance of AF as well. However, data on the association of LA EAT and LA fibrosis are scarce. To evaluate the correlation of LA EAT volume and inflammation with LA fibrosis in patients presenting for first time AF ablation of symptomatic AF. Consecutive patients presenting for first time AF ablation were included. All patients underwent computed tomography one day prior to the ablation procedure. LA area was manually traced and surrounding tissue within 1 cm of the LA, with Hounsfield units (HU) ranging from -195 to -45, was identified as EAT. For identification of study subjects with inflammatory LA EAT changes, the study population was divided by the median HU value. All participants underwent high density mapping of the LA in sinus rhythm for evaluation of general bipolar voltage and for identification of areas of low voltage (<0.5mV) prior to AF ablation. Follow-up and outcome data were collected. In total, 107 patients (44% female, age 65±11years, 45% persistent AF) were enrolled. The median LA bipolar voltage and low voltage area were 1.87mV (IQR 0.97-2.70) and 32.63 cm² (IQR 3.98-34.97). Elevated absolute volumes of LA EAT showed a correlation to decreased levels of LA bipolar voltage (r=-0.228; p=0.022) and to increased areas of low voltage (r=0.225; p=0.033). After adjustment for LA volume however, this correlation did not remain significant. Attenuation of LA EAT did not correlate with LA bipolar voltage. After stratification for the median HU value (-91.3), patients with a higher attenuation (≥-91.3 HU) (as a marker for inflammatory changes of the LA EAT) were younger (63±12 vs 68±9 years; p=0.039) and more often male (66 vs 46%, p=0.049), when compared to the study population with lower attenuation (<-91.3 HU). After a median follow up of 13 months (IQR 7-36) 44 patients (41%) experienced arrhythmia recurrence. Patients with higher LA EAT attenuation suffered from a higher rate of arrhythmia recurrence (p=0.030) (Figure 1). Higher tissue attenuation as a sign for LA EAT inflammatory changes did not correlate with LA fibrotic changes in patients presenting for AF ablation but is associated with higher rates of arrhythmia recurrence post ablation, when compared to patients with lower attenuation.

Background/Objectives: Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) often experience significantly reduced functional capacity due to myocardial involvement. Cardiopulmonary exercise testing (CPET) is the gold standard to quantify functional capacity, and 99mTc-DPD scintigraphy and SPECT/CT have proven to be highly effective tools for diagnostic and disease monitoring. We aimed to investigate the complementary role and correlation between both methods, focusing on their combined potential as a strong prognostic framework for monitoring disease progression and evaluating treatment efficacy. Methods: A total of 44 patients with diagnosed ATTR-CM, who underwent 99mTc-DPD scintigraphy and SPECT/CT imaging as well as CPET, were included. All patients were divided into two groups based on the median DPD retention index (low DPD uptake: ≤5.0, n = 22; high DPD uptake: >5.0, n = 22). Results: The mean age was 78 years, with 82% of participants being male. Significant correlations were observed between peak VO2 and DPD retention index (r = -0.355, p = 0.018) as well as between peak VO2 at anaerobic threshold with DPD retention index (r = -0.391, p = 0.009). Interestingly, there was no strong correlation between VE/VCO2 slope and the retention index. A strong association was identified between cardiac biomarkers and peak VO2, specifically for NT-proBNP (r = -0.530, p < 0.001) and Troponin T (r = -0.431, p < 0.001). Conclusions: In ATTR-CM, significant correlations were observed between key CPET parameters and quantitative cardiac DPD uptake, which further reflects on disease severity and functional impairment. Our findings highlight the utility of integrating CPET and SPECT/CT for comprehensive patient assessment in ATTR-CM.

Cardiac transthyretin amyloidosis (ATTR CA) has been increasingly recognized as an important heart failure (HF) entity, and cardiac magnetic resonance (CMR) imaging is a mainstay in the clinical evaluation of this disease. However, studies evaluating the prognostic values of longitudinal data in ATTR CA patients with disease-modifying therapies are lacking. We aimed to assess the prognostic significance of serial quantification of extracellular volume (ECV) in ATTR CA patients treated with tafamidis. The present study included ATTR CA patients who received ≥ 3 months of tafamidis treatment and underwent baseline and CMR, including ECV quantification. The primary endpoint was a composite of all-cause mortality, cardiac transplantation, or hospitalization due to HF. Between June 2016 and June 2020, 54 patients were included in the present analysis and were representative of a typical ATTR CA cohort (median age: 76.7 years, male participants: 79.6

BACKGROUND:Myocarditis typically affects ventricles, whereas isolated atrial myocarditis is rare, causing conduction abnormalities, atrial standstill, and right heart failure. CASE SUMMARY:Two weeks after a respiratory infection, a 35-year-old man developed dyspnea, leg edema, and bradycardia. Electrocardiogram revealed a narrow QRS escape rhythm without P waves, and N-terminal pro-B-type natriuretic peptide was elevated. Echocardiography showed severe right ventricular dysfunction with massive tricuspid regurgitation. Cardiac magnetic resonance demonstrated atrial late gadolinium enhancement and mild edema, whereas electrophysiological testing confirmed atrial standstill and atrioventricular block. DISCUSSION:This case highlights isolated atrial myocarditis as a rare cause of atrial dysfunction, marked by postcapillary pulmonary hypertension and prominent V waves, despite normal mitral valves. Rapid atrial fibrosis progression resulted in silent atria, confirmed by magnetic resonance imaging and electrophysiology. Further research is required to clarify pathophysiology and establish effective management. TAKE-HOME MESSAGES:Isolated atrial myocarditis is a rare cause of atrial standstill and atrioventricular block. Comprehensive multimodality imaging is essential for accurate diagnosis and management.

OBJECTIVES:In cardiac amyloidosis (CA) protein misfolding and consecutive storage into the extracellular myocardial compartment causes left ventricular hypertrophy and, in later stages of the disease, heart failure. The aim of this study was to compare extracellular volume (ECV) measurements obtained from photon-counting CT (PCCT) to the imaging reference cardiac magnetic resonance imaging (CMR) and to evaluate coronary artery disease (CAD) in a CA cohort. MATERIALS AND METHODS:Thirty CA patients (mean age 77.5 +/- 7.9 years) underwent clinically indicated coronary CT angiography (CCTA) for the evaluation of CAD on a first-generation PCCT including a late-phase scan for assessment of ECV. ECV in PCCT was derived using 2 different techniques: (I) a single-energy (SE) technique, based on attenuation changes between the precontrast calcium scoring scan and delayed CCTA in the equilibrium phase (II) a dual-energy (DE) technique, based on iodine density maps from the delayed scan. Both methods were compared with CMR-derived ECV. Statistical analysis included repeated-measures analysis of variance (RM-ANOVA) with Bonferroni-adjusted pairwise comparisons. Correlations between methods were assessed using Pearson's correlation coefficient, and agreement was evaluated using Bland-Altman analysis. RESULTS:CMR exhibited the highest mean ECV value (42.93 ± 10.14), followed by the SE method (42.5 ± 9.1), while the DE method yielded the lowest ECV values (40.7 ± 9.2). When compared with CMR, ECV obtained via the DE method was significantly lower ( MDiff  = -2.24, P =  0.04). In contrast, no significant difference was observed between CMR and the SE method ( MDiff  = 0.43, P  = 1.00). Differences between the DE and SE methods were significant ( MDiff  = -1.82, P <  0.001). Despite these differences, all 3 methods demonstrated excellent positive correlations. The strongest correlation was observed between the DE and SE methods ( r  = 0.98, P  < 0.001), indicating high consistency in their measurements. Comparatively, the correlation between CMR and DE ( r  = 0.892, P  < 0.001) was slightly stronger than that between CMR and SE methods ( r  = 0.882, P  < 0.001). CAD was present in 29 (97.0%) CA patients with a mean Agatston score of 1086 ± 1398 (range 0-6848.5). Despite this high mean plaque burden and 14 (47.6%) patients presenting with atrial fibrillation, image quality was preserved in 29 (97.0%) patients with 17 (57.6%) of the patients having nonobstructive CAD. CONCLUSIONS:Compared to the imaging reference standard CMR, ECV derived from the DE and SE methods via PCCT demonstrated excellent positive correlations with CMR. The DE method exhibited minor differences compared to CMR, which were clinically not relevant. CAD with an extensive burden of calcified plaque was highly prevalent in CA; however, 57.6% of patients presented with nonobstructive CAD. Therefore, PCCT is a valuable tool for imaging both the coronary arteries and myocardial structure in CA.

Background:Dual-source photon-counting detector computed tomography (PCDCT) offers the opportunity to perform cardiac examinations within one beat and simultaneously the acquisition of spectral information. This study, evaluated subjective and objective image quality of virtual monoenergetic image (VMI) reconstructions using data from a first-generation, dual-source PCDCT scanner, operated in high-pitch scanning mode. Methods:We retrospectively included 30 patients who underwent a clinically indicated CTA of the coronary arteries. VMI were reconstructed at five different energy levels. Subjective image quality was assessed by three radiologists according to a four-point Likert scale for four different quality features. To evaluate objective image quality, SNR and CNR were calculated via ROIs placed in the aorta, coronary arteries, myocardium, pectoral muscle, and epicardial fat. Results:VMI at 40, 50, 60, and 70 keV showed equal mean scores (4/4) for subjective vascular contrast, followed by 80 keV reconstructions with a mean score of 3/4. The 40 keV reconstruction yielded the lowest range (3-4) in Likert scores and highest percentage of reader agreement (80 %). Minor differences in subjective image noise, sharpness, and plaque visualization were observed with positive trends toward higher keV levels. SNR and CNR were superior for 40 keV, with a mean of 34.8 ± 1.7HU and 45.4 ± 2.7HU, respectively. Mean applied contrast volume was 65 ml, resulting in a mean CT value of 1150HU for 40 keV VMI. Conclusion:First-generation PCDCT-derived VMI at 40 and 50 keV offer satisfying subjective and objective image quality, even when acquired in high-pitch scanning mode.

Background: Extracellular volume (ECV) by cardiovascular magnetic resonance (CMR) imaging is associated with disease burden and clinical outcomes. Recent studies in patients with valvular heart disease (VHD) have suggested that the indexed total ECV (iECV) = ECVx(LVmass/1.05)/body surface area may supersede ECV in terms of prognostication. In this study, we aimed to compare the prognostic capability of conventional ECV and iECV in an all-comer CMR cohort. Methods: From January 2012 to 2023, ECV and iECV were measured in consecutive CMR patients. Adverse outcomes were defined as a composite of hospitalization for heart failure (HF) and/or death. All patients underwent transthoracic echocardiography within 3 weeks of CMR. Results: Overall, 1525 patients (44% female, mean age 65 ± 18 years) were included. The mean ECV was 29 ± 9% and the mean iECV was 21 ± 13 mL/m2. During 52 ± 36 months of follow-up, 414 (27%) events occurred. Both ECV (HR = 1.04, 95% CI = 1.04-1.05, p < 0.001) and iECV (HR = 1.03, 95% CI = 1.02-1.03, p < 0.001) were significantly associated with outcomes. Having been stratified for ECV and iECV tertiles, Kaplan-Meier analyses showed a significant association with event-free survival for both parameters (log-rank, p < 0.001 for both; central illustration). Regarding multivariate analysis, adjusted for age, sex, left ventricular function, and NT-proBNP, both ECV and iECV remained independently associated with the composite endpoint (ECV: HR = 1.31, 95% CI = 1.20-1.44, p < 0.001; iECV: HR = 1.17, 95% CI = 1.06-1.29, p = 0.002). In addition, ECV was significantly associated with aortic valve velocity (p < 0.001) pertaining to echocardiography, whereas iECV did not show an association (p = 0.41). Conclusions: Both conventional ECV and iECV provided profound prognostic information regarding the risk of HF hospitalizations and death. However, iECV, which is more complex to determine, did not add value.