Epicardial cavernous haemangioma; A case report of a unique incidental finding.

Background: Primary cardiac tumours are rare, accounting for only 0.002-0.03% at autopsy. Cardiac haemangiomas are benign vascular tumours and constitute for 0.28% of all primary cardiac tumours. Cavernous haemangiomas, capillary haemangiomas, and arteriovenous haemangiomas are three distinct types. Cardiac haemangiomas are often misdiagnosed as myxomas and must be differentiated from malignant angiosarcomas. Case summary: We present a 44-year-old Mediterranean male patient with a cavernous haemangioma in the inferior vena cava and right atrium, detected on transthoracic echocardiography. The patient experienced palpitations and dyspnoea on exertion. Computed tomography (CT) angiography revealed a 7.5 × 6 × 5 cm mass suspected to be perfused by the distal right coronary artery. A watch-and-wait approach was suggested, leading to a cardiac magnetic resonance imaging (MRI) with contrast 6 months later. T1 mapping exhibited a prolonged relaxation time and isointensity to the myocardium. T2 mapping revealed a homogenous hyperintense mass with heterogenous late enhancement. Surgical excision was performed using a bicaval cannulation technique on cardiopulmonary bypass. Intraoperatively, no connection to the coronaries was noted. At 1 year follow-up, the patient reported restored physical resilience, with no evidence of tumour recurrence. Discussion: Clinical symptoms of cardiac cavernous haemangiomas are unspecific and become evident once the tumour grows. To investigate the nature and vascular involvement of the tumour, a contrast-enhanced CT angiography or MRI can be performed. Cardiac haemangiomas are often misdiagnosed and must be differentiated from malignant angiosarcomas. Clear guidelines for the treatment of cardiac haemangiomas in adult patients are lacking. Primary cardiac tumours require thorough investigation, and surgical intervention should be tailored to the individual's case.

The individual relationship between atrial fibrillation sources from CARTOFINDER mapping and atrial cardiomyopathy: The catch me if you can trial.

BACKGROUND: Targeting individual sources identified during atrial fibrillation (AF) has been used as an ablation strategy with varying results. OBJECTIVE: Aim of this study was to evaluate the relationship between regions of interest (ROIs) from CARTOFINDER (CF) mapping and atrial cardiomyopathy from late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (CMR). METHODS: Twenty consecutive patients underwent index catheter ablation for persistent AF (PERS AF). Pre-processed LGE CMR images were merged with the results from CF mapping to visualize harboring regions for focal and rotational activities. Atrial cardiomyopathy was classified based on the four Utah stages. RESULTS: Procedural success was achieved in all patients (n = 20, 100%). LGE CMR revealed an intermediate amount of 21.41% ± 6.32% for LA fibrosis. ROIs were identified in all patients (mean no ROIs per patient n = 416.45 ± 204.57). A tendency towards a positive correlation between the total amount of atrial cardiomyopathy and the total number of ROIs per patient (regression coefficient, ß = 10.86, p = .15) was observed. The degree of fibrosis and the presence of ROIs per segment showed no consistent spatial correlation (posterior: ß = 0.36, p-value (p) = .24; anterior: ß = -0.08, p = .54; lateral: ß = 0.31, p = 39; septal: ß = -0.12; p = .66; right PVs: ß = 0.34, p = .27; left PVs: ß = 0.07, p = .79; LAA: ß = -0.91, p = .12). 12 months AF-free survival was 70% (n = 14) after ablation. CONCLUSION: The presence of ROIs from CF mapping was not directly associated with the extent and location of fibrosis. Further studies evaluating the relationship between focal and rotational activity and atrial cardiomyopathy are mandatory.

Assessment of pulmonary artery stiffness by multiparametric cardiac magnetic resonance-surrogate for right heart catheterization.

Background: Cardiac magnetic resonance (CMR) imaging allows for multiparametric assessment of healthy pulmonary artery (PA) hemodynamics. Gender- and aging-associated PA stiffness and pressure alterations have remained clinically unestablished, however may demonstrate epidemiological differences in disease development. The aim of this study is to evaluate the role of CMR as a surrogate for catheter examinations by providing a comprehensive CMR assessment of sex- and age-related reference values for PA stiffness, flow, and pressure. Methods and Results: PA hemodynamics were studied between gender and age groups (>/<50 years) using phase-contrast CMR. Corresponding correlation analyses were performed. 179 healthy volunteers with a median age of 32.6 years (range 11.3-68.2) were examined. Males demonstrated increased PA compliance (median [interquartile range] or mean ± standard deviation) (20.8 mm2/mmHg [16.6; 25.8] vs. 19.2 ± 7.1 mm2/mmHg; P < 0.033), higher pulse wave velocity (2.00 m/s [1.35; 2.87] vs. 1.73 m/s [1.19; 2.34]; P = 0.018) and a reduced full width half maximum (FWHM) (219 ± 22 ms vs. 235 ± 23 ms; P < 0.001) than females. Mean, systolic, diastolic PA pressure and pulmonary proportional pulse pressure were significantly elevated for males compared to females (P < 0.001). Older subjects (>50 years) exhibited reduced PA elasticity (41.7% [31.0; 52.9] vs. 66.4% [47.7; 83.0]; P < 0.001), reduced PA compliance (15.4 mm2/mmHg [12.3; 20.7] vs. 21.3 ± 6.8 mm2/mmHg; P < 0.001), higher pulse wave velocity (2.59 m/s [1.57; 3.59] vs. 1.76 m/s [1.24; 2.34]; P < 0.001) and a reduced FWHM (218 ± 29 ms vs. 231 ± 21 ms; P < 0.001) than younger subjects. Conclusions: Velocity-time profiles are dependent on age and gender. PA stiffness indices deteriorate with age. CMR has potential to serve as a surrogate for right heart catheterization.

Machine-Learning-Based Diagnostics of Cardiac Sarcoidosis Using Multi-Chamber Wall Motion Analyses.

BACKGROUND: Hindered by its unspecific clinical and phenotypical presentation, cardiac sarcoidosis (CS) remains a challenging diagnosis. OBJECTIVE: Utilizing cardiac magnetic resonance imaging (CMR), we acquired multi-chamber volumetrics and strain feature tracking for a support vector machine learning (SVM)-based diagnostic approach to CS. METHOD: Forty-five CMR-negative (CMR(-), 56.5(53.0;63.0)years), eighteen CMR-positive (CMR(+), 64.0(57.8;67.0)years) sarcoidosis patients and forty-four controls (CTRL, 56.5(53.0;63.0)years)) underwent CMR examination. Cardiac parameters were processed using the classifiers of logistic regression, KNN(K-nearest-neighbor), DT (decision tree), RF (random forest), SVM, GBoost, XGBoost, Voting and feature selection. RESULTS: In a three-cluster analysis of CTRL versus vs. CMR(+) vs. CMR(-), RF and Voting classifier yielded the highest prediction rates (81.82%). The two-cluster analysis of CTRL vs. all sarcoidosis (All Sarc.) yielded high prediction rates with the classifiers logistic regression, RF and SVM (96.97%), and low prediction rates for the analysis of CMR(+) vs. CMR(-), which were augmented using feature selection with logistic regression (89.47%). CONCLUSION: Multi-chamber cardiac function and strain-based supervised machine learning provides a non-contrast approach to accurately differentiate between healthy individuals and sarcoidosis patients. Feature selection overcomes the algorithmically challenging discrimination between CMR(+) and CMR(-) patients, yielding high accuracy predictions. The study findings imply higher prevalence of cardiac involvement than previously anticipated, which may impact clinical disease management.

Impact of pulmonary vein anatomy and ostial dimensions on long-term outcome after single-shot device-guided cryoablation for paroxysmal atrial fibrillation.

BACKGROUND: Cryoballoon (CB)-guided pulmonary vein isolation (PVI) is an established treatment for atrial fibrillation (AF). This observational study aimed to assess the role of individual anatomical characteristics to predict long-term freedom from arrhythmia recurrence after CB-guided PVI for paroxysmal AF (PAF). METHODS: Three hundred fifty three consecutive patients (58 ± 11 years, 56% males), undergoing PVI between 2012 and 2018 were analysed. Individual pulmonary vein (PV) anatomy was assessed using preprocedural cardiac magnetic resonance imaging (MRI). For each PV, the cross-sectional area (CSA) was calculated. The impact of PV characteristics and CSA on long-term AF-free survival was evaluated. RESULTS: Acute PVI was achieved in all patients. Two hundred twenty-three patients (63%) had a normal PV anatomy (2 left- and 2 right-sided PV). Variant PV anatomy was present in 130 patients (37%). During the observation period of 48 months, AF-recurrence was documented in 167 patients (47 %). Patients with AF-recurrence presented with significantly enlarged right-sided PVs and left superior PVs (LSPVs) (p < 0.001). The presence of left common PVs (LCPVs) (n = 75, Log-rank p < 0.001) as well as right variant PVs (n = 35, Log rank p < 0.001) was associated with a significantly impaired long-term AF-free survival rate as compared to patients with normal PV characteristics. CONCLUSION: Variant PV anatomy is a good predictor for AF-recurrence. A correlation between an enlarged CSA of right-sided PVs as well as LSPVs and AF-recurrence was documented.

Novel Filamin C Myofibrillar Myopathy Variants Cause Different Pathomechanisms and Alterations in Protein Quality Systems.

Myofibrillar myopathies (MFM) are a group of chronic muscle diseases pathophysiologically characterized by accumulation of protein aggregates and structural failure of muscle fibers. A subtype of MFM is caused by heterozygous mutations in the filamin C (FLNC) gene, exhibiting progressive muscle weakness, muscle structural alterations and intracellular protein accumulations. Here, we characterize in depth the pathogenicity of two novel truncating FLNc variants (p.Q1662X and p.Y2704X) and assess their distinct effect on FLNc stability and distribution as well as their impact on protein quality system (PQS) pathways. Both variants cause a slowly progressive myopathy with disease onset in adulthood, chronic myopathic alterations in muscle biopsy including the presence of intracellular protein aggregates. Our analyses revealed that p.Q1662X results in FLNc haploinsufficiency and p.Y2704X in a dominant-negative FLNc accumulation. Moreover, both protein-truncating variants cause different PQS alterations: p.Q1662X leads to an increase in expression of several genes involved in the ubiquitin-proteasome system (UPS) and the chaperone-assisted selective autophagy (CASA) system, whereas p.Y2704X results in increased abundance of proteins involved in UPS activation and autophagic buildup. We conclude that truncating FLNC variants might have different pathogenetic consequences and impair PQS function by diverse mechanisms and to varying extents. Further studies on a larger number of patients are necessary to confirm our observations.

Lesion formation following pulsed field ablation for pulmonary vein and posterior wall isolation.

BACKGROUND: Pulsed field ablation (PFA) results in unique lesion formation, but there is lack of in-vivo validation in terms of scar formation following atrial fibrillation (AF) ablation. OBJECTIVE: We aimed to access atrial lesion formation based on late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (CMR) following PFA for pulmonary vein (PV) and posterior wall isolation (PWI). METHODS: AF ablation was performed in 10 patients using a 31 mm pentaspline PFA catheter. After pulmonary vein isolation (PVI; n = 8 PFA-applications/ PV; n = 4 in basket and n = 4 in flower configuration), another eight applications in flower configuration were conducted for concomitant PWI. Patients underwent LGE CMR 3 months after ablation aiming for quantification of left atrial (LA) scar. RESULTS: Acute procedural success was achieved in all patients. Mean procedure duration was 62 ± 7 min. and mean LA dwell time of the PFA catheter was 13 ± 2 min. Mean post ablation total LA scar burden was 8.1 ± 2.1% and mean scar width was 12.8 ± 2.1 mm. At the posterior LA, 22.6 ± 2.2% of the anatomical segment resulted in chronic scar tissue, concentrated at the PW. Postablation CMR found no evidence for PV stenosis or collateral damage of adjacent structures. At 7 months of follow-up, 9/10 patients (90%) were free from arrhythmia recurrence. CONCLUSION: PFA for AF resulted in durable and transmural atrial scar tissue at the PVs and PW. LGE CMR found a very homogeneous and contiguous lesion pattern with no signs for collateral damage.

Magnetic resonance assessment of left atrial scar formation following a novel very high-power short-duration workflow for atrial fibrillation ablation.

AIMS: Atrial fibrillation (AF) ablation protocols using energy delivery with very high power and short duration (vHPSD) have been introduced to improve lesion formation. This study reports procedural data of vHPSD ablation in AF patients and analyses characteristics of ablation-induced left atrial (LA) scar formation from cardiac magnetic resonance imaging (MRI). METHODS AND RESULTS: Sixty consecutive patients undergoing index pulmonary vein isolation following our institutional Q4U-AF workflow were prospectively enrolled. Ablation was conducted using a contact force sensing catheter allowing for vHPSD ablation using a temperature-controlled ablation mode. Thirty patients underwent cardiac late gadolinium enhancement MRI of the LA 3 months after ablation to assess LA scar. Mean procedural duration was 66.5 ± 14.8 min. Mean ablation time was 4.7 ± 0.9 min with a mean number of 69.9 ± 14.2 applications. First-pass isolation was achieved in 51 patients (85%) for the right pulmonary veins (RPVs), in 37 patients (61.7%) for the left pulmonary veins (LPVs), and in 34 patients (56.7%) for both pulmonary veins (PVs). Magnetic resonance imaging at 3 months post-ablation demonstrated a mean scar width of 14.4 ± 2.6 mm around RPVs and 11.9 ± 1.9 mm at LPVs (P > 0.05). Complete PV encirclement was observed in 76.7% for RPVs, in 76.7% for LPVs, and in 66.7% for both PV pairs. During a mean follow-up of 4.7 ± 1.4 months, arrhythmia recurrence was observed in 3.3% of the patients. CONCLUSION: Pulmonary vein isolation following a novel vHPSD workflow resulted in short procedure duration and high acute and mid-term efficacy. Magnetic resonance imaging demonstrated durable and transmural PV lesions with homogeneous and contiguous scar formation.

CMR-based right ventricular strain analysis in cardiac amyloidosis and its potential as a supportive diagnostic feature.

Background: Right ventricular (RV) strain has provided valuable prognostic information for patients with cardiac amyloidosis (CA). However, the extent to which RV strain and strain rate can differentiate CA is not yet clinically established. CA underdiagnosis delays treatment strategies and exacerbates patient prognosis. Aims: Evaluation of cardiac magnetic resonance (CMR) quantified RV global and regional strain of CA and HCM patients along with CA subtypes. Methods: CMR feature tracking attained longitudinal, radial and circumferential global and regional strain in 47 control subjects (CTRL), 43 CA-, 20 hypertrophic cardiomyopathy- (HCM) patients. CA patients were subdivided in 21 transthyretin-related amyloidosis (ATTR) and 20 acquired immunoglobulin light chain (AL) patients. Strain data and baseline clinical parameters were statistically analysed with respect to diagnostic performance and discriminatory power between the different clinical entities. Results: Effective differentiation of CA from HCM patients was achieved utilizing global longitudinal (GLS: 16.5 ± 3.9% vs. -21.3 ± 6.7%, p = 0.032), radial (GRS: 11.7 ± 5.3% vs. 16.5 ± 7.1%, p < 0.001) and circumferential (GCS: -7.6 ± 4.0% vs. -9.4 ± 4.4%, p = 0.015) right ventricular strain. Highest strain-based hypertrophic phenotype differentiation was attained using GRS (AUC = 0.86). Binomial regression found right ventricular ejection fraction (RV-EF) (p = 0.017) to be a significant predictor of CA-HCM differentiation. CA subtypes had comparable cardiac strains. Conclusion: CMR-derived RV global strains and various regional longitudinal strains provide discriminative radiological features for CA-HCM differentiation. However, in terms of feasibility, cine-derived RV-EF quantification may suffice for efficient differential diagnostic support.

Cardiovascular Magnetic Resonance Imaging-Based Right Atrial Strain Analysis of Cardiac Amyloidosis.

Background: Cardiac amyloidosis (CA) manifests in a hypertrophic phenotype with a poor prognosis, making differentiation from hypertrophic cardiomyopathy (HCM) challenging and delaying early treatment. The extent to which magnetic resonance imaging (MRI) quantifies the right atrial strain (RAS) and strain rate (RASR), providing valuable diagnostic information, is not yet clinically established. Aims: This study assesses diagnostic differences in the longitudinal RAS and RASR between CA and HCM patients, control subjects (CTRL) and CA subtypes in addition to the impact of atrial fibrillation (AF) on the right atrial function in CA patients. The RAS and RASR of tricuspid regurgitation (TR) patients are used to assess the potential for diagnostic overlap. Methods: RAS and RASR quantification was conducted via MRI feature-tracking for biopsy-confirmed CA patients with subtypes identified. Strain parameters were compared for CTRL, HCM and TR patients. Post hoc testing identified intergroup differences. Results: In total, 41 CA patients were compared to 47 CTRL, 20 HCM and 31 TR patients. Reservoir (R), conduit and booster RAS and RASRs allow for significant differentiation (p < 0.001) between CA and HCM patients (R: 10.6 ± 14.3% vs. R: 33.5 ± 16.3%) and CTRL (R: 44.6 ± 15.7%). Booster and reservoir RAS and RASRs qualified as reliable diagnostic tests (AUC > 0.8). CA patients with AF, in contrast to sinus rhythm, demonstrated a significantly impaired reservoir RAS and RASR and booster RASR. The discriminative power of RAS for CA vs. TR was insufficient (R: 10.6% ± 14.3% vs. 7.0% ± 6.0%, p = 0.069). Differentiation between 21 transthyretin and 20 light-chain amyloidosis subtypes was not achievable (R: 0.7% ± 1.0% vs. 0.7% ± 1.0%, p = 0.827). Conclusion: The MRI-derived RAS and RASR are impaired in CA patients and may support noninvasive differentiation between CA, HCM and CTRL.

A Machine Learning Challenge: Detection of Cardiac Amyloidosis Based on Bi-Atrial and Right Ventricular Strain and Cardiac Function.

BACKGROUND: This study challenges state-of-the-art cardiac amyloidosis (CA) diagnostics by feeding multi-chamber strain and cardiac function into supervised machine (SVM) learning algorithms. METHODS: Forty-three CA (32 males; 79 years (IQR 71; 85)), 20 patients with hypertrophic cardiomyopathy (HCM, 10 males; 63.9 years (±7.4)) and 44 healthy controls (CTRL, 23 males; 56.3 years (IQR 52.5; 62.9)) received cardiovascular magnetic resonance imaging. Left atrial, right atrial and right ventricular strain parameters and cardiac function generated a 41-feature matrix for decision tree (DT), k-nearest neighbor (KNN), SVM linear and SVM radial basis function (RBF) kernel algorithm processing. A 10-feature principal component analysis (PCA) was conducted using SVM linear and RBF. RESULTS: Forty-one features resulted in diagnostic accuracies of 87.9% (AUC = 0.960) for SVM linear, 90.9% (0.996; Precision = 94%; Sensitivity = 100%; F1-Score = 97%) using RBF kernel, 84.9% (0.970) for KNN, and 78.8% (0.787) for DT. The 10-feature PCA achieved 78.9% (0.962) via linear SVM and 81.8% (0.996) via RBF SVM. Explained variance presented bi-atrial longitudinal strain and left and right atrial ejection fraction as valuable CA predictors. CONCLUSION: SVM RBF kernel achieved competitive diagnostic accuracies under supervised conditions. Machine learning of multi-chamber cardiac strain and function may offer novel perspectives for non-contrast clinical decision-support systems in CA diagnostics.

Clinically atypical abdominal manifestation of the lipoma localized in the right atrium: "Invagination hypothesis" revisited.

Cardiac lipomas are the second most common cardiac tumors. They are usually asymptomatic and diagnosed as incidental findings. We describe a 71-year-old patient with a tumor in the right atrium. In echocardiography and MRI scan, the diagnosis of a cardiac lipoma was suspected. Moreover, MRI demonstrated continuity of pericardial fat and the tumor in the right atrium by infolding of the atrial wall and epicardial adipose tissue in the space between the atrial walls, which might be a hint for the Waterstone groove hypothesis. An operative resection was performed which confirmed the suspected diagnosis.

Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers.

Left ventricular non-compaction (LVNC) describes the phenotypical phenomena characterized by the presence of excessive trabeculation of the left ventricle which forms a deep recess filled with blood. Considering the lack of a uniform definition of LVNC as well as the "golden standard" it is difficult to estimate the actual incidence of the disease, however, seems to be overdiagnosed, due to unspecific diagnostic criteria. The non-compacted myocardium may appear both as a disease representation or variant of the norm or as an adaptive phenomenon. This article covers different approaches to incidence, pathogenesis, diagnostics, and treatment of LVNC as well as recommendations for patients during follow-up.

Multi-parametric analyses to investigate dependencies of normal left atrial strain by cardiovascular magnetic resonance feature tracking.

Left-atrial (LA) strain is the result of complex hemodynamics, which may be better characterized using a multiparametric approach. Cardiovascular magnetic resonance (CMR) feature tracking was used to perform a comprehensive LA strain assessment of 183 enrolled healthy volunteers (11-70 years, 97 females, median 32.9 ± 28.3 years). Novel strain dependencies were assessed using multi-parametric regression (MPR) analyses. LA volumetric data, left ventricular strain, transmitral and pulmonary venous blood flow parameters were utilized to create clusters for MPR of all subjects and a heart rate controlled subgroup (pulse: 60-75/min, N = 106). The LA reservoir(r) and conduit(c) strains of the total cohort were significantly elevated (p ≤ 0.001) in women (r: 49.7 ± 12.9%, c: 32.0 ± 11.0%) compared to men (r: 42.9 ± 11.4%, c: 26.1 IQ 10.5%). In contrast, there were no gender-specific differences (p > 0.05) for subgroup LA reservoir, conduit and booster(b) strains (all, r: 47.3 ± 12.7%; c: 29.0 IQ 15.5%; b: 17.6 ± 5.4%) and strain rates (all, 2.1 IQ 1.0 s-1; - 2.9 IQ 1.5 s-1; - 2.3 IQ 1.0 s-1). MPR found large effect sizes (|R2|≥ 0.26) for correlations between strain and various cardiac functional parameters. Largest effect size was found for the association between LA conduit strain and LA indexed booster volume, LA total ejection fraction, left ventricular global radial strain and E-wave (|R2|= 0.437). In addition to providing normal values for sex-dependent LA strain and strain rate, no gender differences were found with modified heart rate. MPR analyses of LA strain/strain rate and various cardiac functional parameters revealed that heart rate control improved goodness-of-fit for the overall model.

Impact of left atrial appendage fibrosis on atrial fibrillation in patients following coronary bypass surgery.

OBJECTIVES: We aimed to assess the relationship of left atrial appendage (LAA) fibrosis with atrial fibrillation (AF) and postoperative events in patients receiving coronary artery bypass graft surgery (CABG). BACKGROUND: Increased atrial fibrosis has been associated with AF and worse outcome following catheter ablation. Only limited data exists focusing on the impact of LAA fibrosis on AF after CABG. METHODS: LAA tissue from 164 CABG-patients was stained with Masson-Goldner trichrome. The histological landscape was scanned and segmented into superpixels for software analysis (QuPath). A classification algorithm was extensively trained to detect fibrotic superpixels for quantification. In 43 propensity score matched pairs with AF or sinus rhythm (SR), LAA fibrosis was compared. Moreover, subgroups of mitral valve regurgitation (MR) were analyzed as follows: SR, SR + MR, AF and AF + MR. The predictive value of LAA fibrosis postoperative stroke, postoperative AF and mortality was assessed. RESULTS: Fibrotic remodeling (%) showed no significant difference for the total cohort between the SR and AF group (SR: 30.8 ± 11.4% and AF: 33.8 ± 16.0%, respectively, p = .32). However, significant fibrotic remodeling was observed for SR and AF subgroups (SR: 27.2 ± 12.2% vs. AF: 35.3 ± 13.7%; respectively, p = .049) and between SR and SR + MR subgroups (SR: 27.2 ± 12.2% vs. SR + MR: 34.9 ± 9.1%, respectively, p = .027). LAA fibrosis was not significantly associated with postoperative stroke, postoperative AF or overall mortality (all p > .05). CONCLUSION: LAA fibrosis may contribute to an individual arrhythmia substrate for AF in patients with AF but also in those with SR and coincidence of MR. LAA fibrosis was not found to be predictive for clinical events in patients after CABG.

Magnetic-Resonance-Imaging-Based Left Atrial Strain and Left Atrial Strain Rate as Diagnostic Parameters in Cardiac Amyloidosis.

Aims: The present study aims to evaluate magnetic-resonance-imaging (MRI)-assessed left atrial strain (LAS) and left atrial strain rate (LASR) as potential parameters for the diagnosis of cardiac amyloidosis (CA), the distinction of clinical subtypes and differentiation from other cardiomyopathies. Methods and results: LAS and LASR were assessed by MRI feature tracking in patients with biopsy-proven CA. LAS and LASR of patients with CA were compared to healthy subjects and patients with hypertrophic cardiomyopathy. LAS and LASR were also analyzed concerning differences between patients with transthyretin (ATTR) and light chain amyloidosis (AL). A total of 44 patients with biopsy-proven CA, 19 patients with hypertrophic cardiomyopathy and 24 healthy subjects were included. In 22 CA patients (50%), histological examination identified ATTR as CA subtype and AL in the remaining patients. No significant difference was observed for reservoir, conduit or booster LAS in patients with AL or ATTR. Reservoir LAS, conduit LAS and booster LAS were significantly reduced in patients with CA and HCM as compared to healthy subjects (p < 0.001). Reservoir LAS and booster LAS were significantly reduced in CA as compared to HCM patients (p < 0.001). A linear correlation was observed between LA global reservoir strain and LA-EF (p < 0.001, r = 0.5), conduit strain and global longitudinal LV strain (p < 0.001, r = 0.5), global booster strain rate and LA-EF (p < 0.001, r = 0.6) and between global booster strain rate and LA area at LVED (p < 0.0001, 0.5). Conclusions: LAS and LASR are severely impaired in patients with CA. The MRI-based assessment of LAS and LASR might allow non-invasive diagnosis and categorization of CA and its distinct differentiation from other hypertrophic phenotypes.

Left-Ventricular Reference Myocardial Strain Assessed by Cardiovascular Magnetic Resonance Feature Tracking and fSENC-Impact of Temporal Resolution and Cardiac Muscle Mass.

Aims: Cardiac strain parameters are increasingly measured to overcome shortcomings of ejection fraction. For broad clinical use, this study provides reference values for the two strain assessment methods feature tracking (FT) and fast strain-encoded (fSENC) cardiovascular magnetic resonance (CMR) imaging, including the child/adolescent group and systematically evaluates the influence of temporal resolution and muscle mass on strain. Methods and Results: Global longitudinal (GLS), circumferential (GCS), and radial (GRS) strain values in 181 participants (54% women, 11-70 years) without cardiac illness were assessed with FT (CVI42® software). GLS and GCS were also analyzed using fSENC (MyoStrain® software) in a subgroup of 84 participants (60% women). Fourteen patients suffering hypertrophic cardiomyopathy (HCM) were examined with both techniques. CMR examinations were done on a 3.0T MR-system. FT-GLS, FT-GCS, and FT-GRS were -16.9 ± 1.8%, -19.2 ± 2.1% and 34.2 ± 6.1%. fSENC-GLS was higher at -20.3 ± 1.8% (p < 0.001). fSENC-GCS was comparable at-19.7 ± 1.8% (p = 0.06). All values were lower in men (p < 0.001). Cardiac muscle mass correlated (p < 0.001) with FT-GLS (r = 0.433), FT-GCS (r = 0.483) as well as FT-GRS (r = -0.464) and acts as partial mediator for sex differences. FT-GCS, FT-GRS and fSENC-GLS correlated weakly with age. FT strain values were significantly lower at lower cine temporal resolutions, represented by heart rates (r = -0.301, -0.379, 0.385) and 28 or 45 cardiac phases per cardiac cycle (0.3-1.9% differences). All values were lower in HCM patients than in matched controls (p < 0.01). Cut-off values were -15.0% (FT-GLS), -19.3% (FT-GCS), 32.7% (FT-GRS), -17.2% (fSENC-GLS), and -17.7% (fSENC-GCS). Conclusion: The analysis of reference values highlights the influence of gender, temporal resolution, cardiac muscle mass and age on myocardial strain values.

The Effect of Diabetes Mellitus on the Recurrence of Atrial Fibrillation after Ablation.

Diabetes mellitus (DM) plays a crucial role in the regulation of atrial fibrillation (AF). This study aimed to evaluate the outcome of pulmonary vein isolation (PVI) using a single-shot device in patients with AF and DM. A total of 531 consecutive patients undergoing initial cryoballoon (CB)-guided PVI were evaluated. Two hundred eighty-one patients (53%) suffered from paroxysmal AF (PAF; mean age 51 ± 23.2 years, 26% female), 250 patients (48%) from persistent AF (PERS; 64 ± 10.0 years old, 30% female) and 80 patients (15%) were diagnosed with coincidental DM (68 ± 19.6 years old, 30% female). Follow-up visits were performed at 3, 6 and 12 months including 7-day Holter ECGs. Primary endpoint was the first documented recurrence of atrial tachyarrhythmia. AF recurrence occurred in 26% (140 patients). PAF patients with DM presented with a significantly higher risk for arrhythmia recurrence (Kaplan Meier analysis; Log rank p < 0.001 *). Multivariate analyses found DM to be an independent predictor (IP) for AF recurrence (p = 0.009 *, hazard ratio (HR) 4.363, confidence interval (CI) 1.456-13.074). In PERS, DM was associated with a 43% increase in AF recurrence (p = 0.320, HR 1.427, CI 0.707-2.879). DM has relevant effects on AF recurrence after PVI-only ablation approaches for AF. Major differences were observed in PAF as DM seems to favor the development of individual arrhythmia substrate, which is usually not yet present in PAF. In PERS, DM effects are less pronounced as individual fibrosis has already developed. Thus, personalized paths addressing individual arrhythmia substrates are needed in this specific cohort of patients.