Aims Blood flow rate quantification using two-dimensional phase-contrast MRI (PC-MRI) results in averaging of flow information due to long acquisition times precluding the examination of short-term effects. The aim of this study was to determine respiration-related flow rate variations by non-electrocardiographic triggered real-time phase-contrast MRI (PC-MRI). Methods and results Real-time PC-MRI was applied to study respiration-driven blood flow fluctuations in the ascending aorta (AAo), superior vena cava (SVC), and inferior vena cava (IVC) under normal and forced breathing in 33 healthy children and 10 Fontan patients. Respiration-dependent flow rates were virtually generated by dividing the respiration curve into four segments: expiration, end-expiration, inspiration, and end-inspiration. Whereas in volunteers aortic flow rate was elevated during end-expiration (5.6 ± 3.0%) and decreased during end-inspiration (−5.8 ± 3.5%) in relation to mean blood flow ( P < 0.05), highest flow was detected during inspiration in SVC (10.5 ± 14.1%) and IVC (22.5 ± 12.1%) and lowest flow during expiration (−11.6 ± 13.5%, −13.2 ± 14.1%, P < 0.05). Differences were increased under forced breathing in AAo (10.4 ± 5.5%, −7.4 ± 6.5%, P < 0.05) and SVC (40.0 ± 30.3%, −30.0 ± 19.2%, P < 0.05), whereas were unchanged in IVC (16.5 ± 23.6%, −13.7 ± 21.6%, P = n.s.). Regarding patients, respiratory-dependent flow rate variability was increased and had to be related to the patient's individual quality of Fontan circulation. Conclusion Real-time PC-MRI allows a physiological assessment of respiratory-related flow rate fluctuations in healthy subjects as well as in Fontan patients. Its capability for detection of short-term effects in clinical routine was demonstrated.

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). 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. 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.

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.

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.

Currently, little is known about the genetic background of restrictive cardiomyopathy (RCM). Herein, we screened an index patient with RCM in combination with atrial fibrillation using a next generation sequencing (NGS) approach and identified the heterozygous mutation DES-c.735G>C. As DES-c.735G>C affects the last base pair of exon-3, it is unknown whether putative missense or splice site mutations are caused. Therefore, we applied nanopore amplicon sequencing revealing the expression of a transcript without exon-3 in the explanted myocardial tissue of the index patient. Western blot analysis verified this finding at the protein level. In addition, we performed cell culture experiments revealing an abnormal cytoplasmic aggregation of the truncated desmin form (p.D214-E245del) but not of the missense variant (p.E245D). In conclusion, we show that DES-c.735G>C causes a splicing defect leading to exon-3 skipping of the DES gene. DES-c.735G>C can be classified as a pathogenic mutation associated with RCM and atrial fibrillation. In the future, this finding might have relevance for the genetic understanding of similar cases.

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.