RPS 503 - Imaging in structural heart disease

Author Block: P. Franceschi¹, F. Calo'², V. Russo¹, F. Niro¹, F. Buia¹, D. Attinà¹, C. Sportoletti¹, E. Rasciti¹, L. Lovato¹; ¹Bologna/IT, ²Brindisi/IT
Purpose: To assess the prevalence of mitral annular disjunction (MAD), mitral valve prolapse (MVP), and curling in healthy individuals using cardiac magnetic resonance (CMR), and to evaluate correlations between MAD length and native myocardial T1 mapping values.
Methods or Background: We prospectively enrolled 141 healthy volunteers (mean age 39 years; 57% female) without cardiovascular risk factors. CMR at 3T included ECG-triggered cine balanced steady-state free precession acquisitions in 2-, 3-, and 4-chamber long-axis views. Native T1 mapping was performed in three short-axis slices. Global and segmental values were extracted.
Results or Findings: MAD was detected in 63 of 140 subjects (45%), mostly in the inferior segment, with lengths between 1–12 mm. MVP was identified in 65 of 137 subjects (47%), predominantly monoleaflet (39%). MVP was more prevalent in subjects with MAD (56%), with significantly longer disjunction in bileaflet prolapse compared with monoleaflet or absent MVP (5±3 mm vs. 1±2 mm; p<0.001). Curling was observed in 3 of 140 cases (2%); two of these also presented with MAD and MVP. MAD length was greater in individuals with curling (7±2 mm vs. 1±2 mm; p<0.001). Native T1 showed no significant differences between participants with or without MAD, MVP, or curling.
Conclusion: MAD is a frequent finding in healthy individuals, predominantly at the inferior annulus. Native T1 values remained normal across groups, suggesting preserved myocardial tissue. While isolated MAD seems benign, longer disjunction associated with MVP and curling indicates structural remodeling of the atrioventricular junction and may represent an arrhythmogenic substrate. CMR enables simultaneous morphological and tissue characterization, supporting its role in early identification of at-risk phenotypes.
Limitations: The limitation of the study is the lack of correlation with echocardiographic, ECG and fibrosis data (contrast agent was not administered in healthy volunteers).
Funding for this study: No funding was received for this study.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: The study was approved by the Ethics Committee of Area Vasta Emilia Centro (CE-AVEC) (reference number 864/2022/Sper/AOUBo).

Author Block: Y. B. Uluman, E. İsmailov, F. Köse, M. Kadıoğlu, B. Tütüncüoğlu, F. Alpaslan, T. Banaz, K. B. Bekaroğlu, S. Baş Özkök; Istanbul/TR
Purpose: To investigate the association between cardiac arrhythmias and cardiac magnetic resonance (CMR) findings in mitral valve prolapse (MVP).
Methods or Background: MVP is usually benign, but some patients develop malignant arrhythmias and sudden cardiac death; CMR may help identify structural risk markers.
We retrospectively analyzed 27 MVP patients underwent 1.5 Tesla CMR with cine, T1/T2 mapping, and late-gadolinium-enhancement (LGE). Assessed parameters included ventricular volume/function, prolapse height, valve thickening, mitral annular disjunction, and LGE extent/localization. Prolapse height was defined relative to the atrioventricular junction, and measurements were performed using automated segmentation with manual correction. ECG/Holter were used to assessed arrhythmia. Continuous variables were presented as mean±SD and compared with the Mann–Whitney U test; categorical variables with the chi-square test. Correlations were examined using Pearson or Spearman coefficients, and multivariate logistic regression identified independent arrhythmia predictors. Significance was set at p<0.05.
Results or Findings: Mean prolapse height was 9.3±3.0 mm, and valve thickening was present in 29.6% of patients. MAD was identified in 66.7% (true/pseudo ratio: 11/8). Arrhythmia occurred in 44.4%, while biventricular systolic function was preserved. LGE was observed in 81.5% of patients, mostly limited (<5% of myocardial volume in 86.4%), involving the inferolateral wall in 29.6% and papillary muscles in 18.5%. Documented arrhythmias includes ventricular extrasystole (n=5), atrial fibrillation (n=2), and sustained ventricular tachycardia (n=5); no sudden death occurred. LGE extent and inferolateral wall involvement were not associated with arrhythmia, whereas papillary muscle fibrosis showed a significant correlation (OR=31.1,p=0.02,AUC=0.89).
Conclusion: This study demonstrated that papillary muscle fibrosis is a strong independent predictor of arrhythmia in patients with mitral valve prolapse. The findings suggest that papillary involvement, rather than global myocardial fibrosis, may represent the key structural substrate for arrhythmogenesis in this population.
Limitations: Relatively small, single-centre sample size.
Funding for this study: N/A
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Number: E-96317027-514.10-251444224 Subject: KAEK/26.06.2024.70

Author Block: C. Booz¹, S. Waldeck², L. S. Alizadeh¹, I. Yel¹, T. Vogl¹, A-I. Nica¹, V. Koch¹, L. D. Grünewald¹, D. Overhoff²; ¹Frankfurt/DE, ²Koblenz/DE
Purpose: To evaluate the diagnostic accuracy of high pitch coronary CT angiography (CCTA) spectral datasets derived from a single CTA for the assessment of coronary artery disease (CAD) in transcatheter aortic valve implantation (TAVI) imaging using invasive coronary angiography (ICA) as reference standard.
Methods or Background: Patients with severe aortic stenosis who underwent photon-counting CT TAVI pre-procedural planning between April 2022 and February 2023 were retrospectively selected. The spectral CCTA datasets were used for the evaluation of objective and subjective image quality. Additionally, the diagnostic accuracy was assessed by determining, sensibility, specificity, positive predictive value (PPV) and negative predictive value (NPV) using ICA as reference standard both on a per-vessel and a per-segment basis. Furthermore, patients were divided in two groups according to calcium score values to define the impact of calcium burden on the diagnostic performance.
Results or Findings: The final study population included 93 patients (mean age, 80±8.4 years; 58 men). The mean overall signal-to-noise ratio was 8.7±2.5 while the contrast-to-noise ratio set on mean values of 9.6±2.5. Image quality median value was 1 [1-2] for the subjective assessment. The diagnostic accuracy for the detection of CAD resulted in a sensitivity of 90.3% (95%CI: 82.9-95.2), specificity of 79.9% (95%CI: 74.5-84.6), PPV of 64.1% (95%CI: 58.2-69.7), NPV of 95.4% (95%CI: 92-97.4). On a per-segment basis sensitivity was 86.4% (95%CI: 79.1-91.9), specificity was 87.6% (95%CI: 85-90), PPV was 56% (95%CI: 50.7-61.1) and NPV was 97.3% (95%CI: 95.8-98.2).
Conclusion: Spectral datasets based on high pitch photon-counting single scan CCTA allow for high diagnostic accuracy in the evaluation of CAD in high-risk patients.
Limitations: Limitations of the study include its retrospective nature.
Funding for this study: No funding was obtained for this study.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: This study received approval from our Ethics Committee.

Author Block: M. Liberotti¹, A. Colombo¹, V. Dubreuil², C. Gnasso¹, D. Vignale¹, A. Palmisano¹, A. Esposito¹; ¹Milan/IT, ²London/UK
Purpose: Transcatheter aortic valve implantation (TAVI) is widely adopted treatment for severe aortic stenosis (AS) in high-risk patients. Accurate pre-TAVI risk stratification is crucial to predict post-procedural outcomes, and planning CT could provide opportunistic biomarkers that objectively capture patient frailty and comorbidities. This study aims to automatically extract opportunistic parameters from TAVI-planning CT to create a data-driven model to predict 1-year outcomes.
Methods or Background: In this single-center retrospective observational study, TAVI-planning CTs were automatically segmented using a pre-trained open-source nnUNet model. An automated pipeline extracted volume (mm³) and density metrics (HU) from over 120 anatomical structures per patient. From 1330 initial features, stable variables were identified via 5-fold cross-validated Random Survival Forest, filtered with univariate Cox, refined by Cox-LASSO and backward elimination. A multivariable Cox model predicting 1-year mortality was built and its performance assessed using the mean concordance index (C-index).
Results or Findings: The study included 458 severe AS patients undergoing TAVI (F/M=234/224; median age 82, IQR: 78-85). At 12-month follow-up post-TAVI, 70 deaths and 24 hospitalizations for heart failure occurred. Following multistage feature selection, five variables informed the final model with four independent event predictors: left atrial volume (HR 1.41s, p=0.03), HU standard deviation in left upper lung lobe (HR 1.30, p<0.005) and brachiocephalic arterial trunk (HR 1.20, p=0.01) and 95th percentile HU in intervertebral discs (HR 1.27, p<0.005). The multivariable Cox model, adjusted for demographic and clinical variables, achieved a mean C-index of 0.72 (± 0.06), demonstrating strong prognostic performance.
Conclusion: This study confirms the potential of total body CT-derived parameters to predict post TAVI outcomes for severe AS patients, by integrating multiple features into a unified predictive model.
Limitations: Retrospective and single-center design may introduce potential bias. The model was not externally validated.
Funding for this study: This study was partially granted by: European Union - Next Generation EU, Mission 4 Component 1, CUP D53D23 021100001”, Bando PRIN PNRR 2022 (P2022JBKN2), Italian Ministry of University and Research (MUR).
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: This monocentric observational study was approved by the institutional review board (CT-based myocardial characterization study: CTMyoC 112/INT/2019 ). The study is a retrospective analysis of prospectively collected data and was conducted according to the Declaration of Helsinki. Written informed consent was obtained for all participants.

Author Block: S. n. Li, X. Tong, W. Tian, N. Yang, B. Zhu, J. Nan, G. Wang, Z. Chen; Lanzhou, China/CN
Purpose: To assess the agreement in myocardial extracellular volume fraction(ECV) quantification between photon-counting detector CT (PCD-CT) and cardiovascular magnetic resonance (CMR) in patients with cardiovascular disease, and to identify the optimal PCD-CT reconstruction phase and myocardial layer for measurement.
Methods or Background: This retrospective study included 11 cardiovascular disease patients who underwent both PCD-CT(with late iodine enhancement) and CMR. ECV derived from CMR served as the reference standard, calculated via a standardized formula. PCD-CT-based ECV was measured at both systolic and diastolic reconstruction phases, with regions of interest placed in the endocardial, mid-myocardial, and epicardial layers, as well as their average.
Results or Findings: A significant correlation was found between PCD-CT-systolic-epicardial-ECV and CMR-ECV(r = 0.835, 95% CI: 0.471–0.956, p = 0.001), and a strong correlation between PCD-CT-diastolic-endocardial-ECV and CMR-ECV (r = 0.807, 95% CI: 0.402–0.948, p = 0.003). Overall correlation was higher in systole than diastole, with PCD-CT-systolic-epicardial-ECV demonstrating the strongest correlation across all phases and layers. The mean difference between PCD-CT-systolic-epicardial-ECV and CMR-ECV was –1.856% (95% LoA: –7.068 to 3.355).
Conclusion: PCD-CT-derived ECV measurements show strong agreement with CMR,particularly when acquired during the systolic phase at the epicardial layer, supporting its potential as a clinically viable alternative for myocardial interstitial fibrosis.
Limitations: Small sample size.
Funding for this study: This study was supported by Gansu Province Health Industry Research Project (GSWSKY2023-05), Gansu Province Science and Technology Plan Project (24JRRA310).
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: The First Hospital of Lanzhou University

Author Block: V. Vespucci, D. Boccetti, S. Ciambellini, F. Carusi, C. Confalone, S. Di Terlizzi, P. Palumbo, E. Di Cesare; L'Aquila/IT
Purpose: Mitral annular disjunction (MAD) is recognized as a potentially arrhythmogenic substrate, yet its clinical impact on disease progression and outcomes remains uncertain. Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) may help refine individual risk stratification in these patients. The objective of this study was to investigate the prognostic role of CMR findings in patients with MAD compared with individuals presenting a similar non-ischemic LGE phenotype but without MAD.
Methods or Background: In this retrospective-prospective study, 82 patients were enrolled and classified into three groups: MAD+/LGE- (n=26), MAD+/LGE+ (n=21), and MAD-/LGE+ (n=35, control group). The primary endpoint was the occurrence of major adverse cardiac events (MACE), defined as sudden cardiac death (SCD), uncontrolled ventricular arrhythmias (NCVA), catheter ablation or implantation of an implantable cardioverter defibrillator (ICD) or pacemaker (PMK).
Results or Findings: During a mean follow-up of 1.8 ± 1.1 years, 22 patients (32%) experienced MACE, with a comparable incidence across the three groups. Arrhythmic burden did not differ significantly among groups. Neither MAD presence/absence, MAD severity (MADmax, p=0.175), nor LGE extent (p=0.387) predicted adverse outcomes. However, MAD-/LGE+ patients showed lower GLS values, consistent with subclinical systolic dysfunction and a higher short- to mid-term MACE risk compared with MAD+/LGE+ patients (OR 4.463, 95% CI 1.506–13.230; p=0.007).
Conclusion: MAD should be regarded as a potential risk factor for arrhythmias and adverse cardiac events. Nonetheless, its prognostic weight appears lower than that of non-MAD disease with a comparable LGE phenotype.
Limitations: Study limitations include the small sample size, the retrospective–prospective design, and the absence of T1 mapping. As reported in previous studies, T1 mapping and extracellular volume (ECV) assessment could provide further insights into interstitial fibrosis and risk stratification in MAD.
Funding for this study: No fund
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information:

Author Block: T. Geyer, P. Franke, B. J. Wintersperger, R. Lorbeer; Munich/DE
Purpose: Cardiovascular magnetic resonance (CMR) strain imaging is increasingly applied for functional characterization of dilated cardiomyopathy (DCM). While left-sided strain has been extensively described, large-scale reference data for right atrial and ventricular strain in healthy populations remain limited, complicating clinical interpretation. Our objective was to establish normal RA and RV strain values in cardiovascular (CV) disease–free individuals and compare them with patients with DCM from a population-based registry using harmonized imaging and post-processing protocols.
Methods or Background: We studied 162 patients with non-ischemic DCM from the German Centre for Cardiovascular Research (DZHK) nationwide multicenter databank with standardized clinical and imaging protocols. Normal reference data were derived from 400 CV disease–free participants of the population-based KORA (Cooperative Health Research in Augsburg) MRI sub-study. All underwent 1.5T or 3T CMR. RA reservoir strain (RA-RS), RA longitudinal strain (RA-LS), RV radial strain (RV-RS), RV longitudinal strain (RV-LS), and RV circumferential strain (RV-CS) were quantified using feature tracking (cvi42, Circle CVI). Group differences were assessed by multivariable linear regression adjusted for age and sex.
Results or Findings: In controls, mean values were RA-RS 50.5%, RA-LS 20.0%, RV-RS 24.9%, RV-LS 21.6%, and RV-CS 14.3%. Patients with DCM showed significant reductions in all parameters (all p<0.001): RA-RS 40.8% (−9.7), RA-LS 15.4% (−4.6), RV-RS 16.1% (−8.8), RV-LS 17.8% (−3.8), and RV-CS 9.2% (−5.1). The largest adjusted differences were observed for RA-RS (β=−9.7%; 95%CI −13.5 to −4.7) and RV-RS (β=−8.8%; 95%CI −10.3 to −6.2).
Conclusion: This study provides robust, population-based reference values for right heart strain and demonstrates the utility of CMR-derived strain for detecting early dysfunction in DCM. Such reference values may improve diagnostic precision and support clinical decision-making.
Limitations: Limitations include the cross-sectional design and lack of longitudinal outcome data, which may restrict prognostic interpretation.
Funding for this study: None.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: The study was approved by the ethics committee of the Bavarian Chamber of Physicians and the ethics committee of the Ludwig-Maximilians-University Munich and complies with the Declaration of Helsinki.

Author Block: M. Dodaro, L. Conia, G. Marchionni, M. Alfarano, C. Chimenti, C. Catalano, N. Galea; Rome/IT
Purpose: Hypertrophic obstructive cardiomyopathy (HOCM), characterized by left ventricular (LV) hypertrophy and systolic anterior motion (SAM) of the mitral valve, remains a clinically and therapeutically challenging disease. Mavacamten, a selective cardiac myosin inhibitor, has shown clinical and functional benefits, but its structural effects assessed by cardiac magnetic resonance (CMR) imaging are not fully defined. This study explored structural and functional changes after Mavacamten therapy using advanced CMR techniques.
Methods or Background: Twelve patients with severe HOCM and SAM were prospectively enrolled and received Mavacamten for at least 17 weeks. CMR was performed at 3T, before and after therapy, including cine balanced steady-state free precession, pre- and post-contrast Modified Look-Locker Inversion Recovery, and T1-weighted inversion recovery imaging for late gadolinium enhancement (LGE). Measurements included ventricular wall thickness, LV and right ventricle (RV) volumes, LGE, global/segmental native T1 and extracellular volume fraction (ECV). Statistical analysis was performed with paired t-tests, with significance set at p≤0.05.
Results or Findings: After an average follow-up of 31±5.6 weeks, 9 of 12 patients demonstrated resolution of LV outflow tract obstruction and disappearance of SAM. Significant decreases in wall thickness were observed in septal and lateral basal-ventricular segments (p=0.04 and p=0.03), as well as in anterior and septal mid-ventricular walls (p=0.05 and p=0.03). RV anterior and inferior wall thickness also declined (p=0.004 and p=0.02). Significant reductions in indexed LV mass (p=0.004), RV stroke volume (p=0.004) and RV ejection fraction (p=0.01) were observed, although RV values ramained normal. No significant changes were seen in native T1, ECV, or LGE areas.
Conclusion: These preliminary data suggest that Mavacamten could induce early favorable reverse remodeling in patients with severe HOCM. Larger cohorts are required to better define myocardial tissue effects and remodeling.
Limitations: Monocentric study; small population; short-time follow-up
Funding for this study: None
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information: