Cao I, Cetera V, Viganò G
… +11 more, Marrone C, Torracchi L, Poli V, Celi S, Capellini K, Gasparotti E, Cantinotti M, Monteleone A, Festa P, Ait-Ali L, Pak V
Eur Heart J Imaging Methods Pract
· 2025 Aug · PMID 42325825
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AIMS: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial discordance and frequently associated with heterogeneous...AIMS: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial discordance and frequently associated with heterogeneous anatomical anomalies. This variability makes surgical indication and strategy particularly complex, requiring accurate preoperative anatomical and functional assessment. We report four paediatric patients with complex ccTGA who underwent anatomical repair at our institution between July 2022 and March 2024. Associated findings included dextrocardia, situs inversus, ventricular septal defects, pulmonary outflow tract obstruction, and atrioventricular valve abnormalities. METHODS AND RESULTS: All patients underwent stepwise multimodal imaging. Transthoracic echocardiography was complemented by cardiovascular magnetic resonance (CMR) to assess ventricular volumes and function, septal defects, outflow tract relationships, and systemic and pulmonary venous return. CMR datasets were processed to generate patient-specific virtual and 3D-printed models through dedicated segmentation, preserving real anatomical dimensions. These models were used for surgical simulation and multidisciplinary planning. Integration of CMR and 3D modelling directly influenced operative strategy in all cases. 3D reconstructions refined ventricular septal defect tunnelling towards the aorta, optimized ventriculotomy site selection, clarified atrial switch configuration, and anticipated the need for pulmonary root translocation. Anatomical repair was successfully achieved in all four patients, with no early mortality and preserved biventricular function at short follow-up. CONCLUSION: Structured integration of CMR and patient-specific 3D modelling enhances spatial understanding and supports individualized surgical planning in complex ccTGA. Incorporating advanced imaging into routine clinical workflow represents a valuable strategy to improve precision and decision-making in anatomically challenging congenital heart disease.
Minami K, Sato K, Machino-Ohtsuka T
… +24 more, Okada T, Kaneko T, Hoshino N, Izumi Y, Ota M, Sengoku K, Nishikawa R, Kato N, Sakamoto T, Eguchi N, Obokata M, Senoo M, Saijo Y, Amano M, Sato Y, Ohno Y, Abe Y, Morita K, Inaba T, Yamada Y, Kawamatsu N, Yamamoto M, Ishizu T, Kagiyama N
BACKGROUND: The vena contracta width (VCW) in parasternal long axis view (PLAX-VCW) and effective regurgitant orifice area (EROA) may underestimate the mitral regurgitation (MR) severity because of the elliptical-shaped...BACKGROUND: The vena contracta width (VCW) in parasternal long axis view (PLAX-VCW) and effective regurgitant orifice area (EROA) may underestimate the mitral regurgitation (MR) severity because of the elliptical-shaped effective orifice in atrial functional MR (AFMR). We aimed to assess the association between the vena contracta width by biplane method (Bi-VCW) and clinical outcomes in patients with AFMR and to compare the discriminative ability of Bi- VCW with those of other quantitative/semiquantitative metrics. METHODS: This was a substudy of a multicentre registry of moderate or severe AFMR. The Bi-VCW was evaluated as the average of the apical two-chamber and four-chamber views. The primary outcome was a composite of all-cause mortality, heart failure hospitalization, and mitral valve interventions. RESULTS: Among 423 studied patients, the Bi-VCW was higher than PLAX-VCW (8.1 ± 2.4 vs. 4.8 ± 1.9 mm, p < 0.001); the guideline-recommended threshold of Bi-VCW of >8.0 mm stratified 48% as severe MR. The Bi-VCW showed the highest discriminatory performance of the primary outcome (area under the time-dependent receiver operating characteristic curve of 0.76) compared with the PLAX-VCW and EROA and revealed an optimal threshold of 7.9 mm (sensitivity, 76%; specificity, 65%). In the multivariable Cox proportional hazards model, Bi- VCW >8.0 mm was independently associated with a higher risk of the primary outcome (adjusted hazard ratio=2.97, 95% confidence interval: 2.04-4.32, p < 0.001). CONCLUSIONS: The Bi-VCW was independently associated with poorer outcomes in patients with AFMR and demonstrated superiority over the guideline-recommended parameters. It could serve as an independent and first surrogate for risk stratification in patients with AFMR.
AIMS: Despite mavacamten's effect on left ventricular outflow tract (LVOT) obstruction, clinical response is heterogenous. Considering the central role of systolic anterior motion (SAM) in obstruction, we examined whethe...AIMS: Despite mavacamten's effect on left ventricular outflow tract (LVOT) obstruction, clinical response is heterogenous. Considering the central role of systolic anterior motion (SAM) in obstruction, we examined whether mitral geometry and SAM-related echocardiographic indices correlate with this variability in obstructive HCM patients. METHODS AND RESULTS: Total 128 patients with obstructive HCM were included in this multi-center observational study. SAM-related echocardiographic parameters-including anterior and posterior leaflet lengths, residual leaflet length (RL), protrusion height, and aortomitral angle (α)-were measured. A drag effect index was quantified by sin(α) × RL. Clinical response was defined as achieving a lowest observed Valsalva-induced LVOT gradient of less than 30 mmHg at any point during the 12-week follow-up period.Among the 128 patients, 33 (25.8%) were non-responders. Non-responders had a smaller α (115.3 ± 8.3° vs. 125.7 ± 9.9°; p < .001), longer RL (1.1 ± 0.2 vs. 0.8 ± 0.3, p < .001), and higher drag effect index values (sin(α) × RL; 1.0 ± 0.2 vs. 0.7 ± 0.3; p < .001). Multivariable logistic regression identified resting LVOT gradient, α, and drag effect index as independent factors associated with non-response. ROC analysis demonstrated moderate discriminative ability for α (AUC 0.787), RL (AUC 0.754), and strong discriminatory ability for the drag effect index (AUC 0.825), with the drag effect index providing the highest overall accuracy (73%). CONCLUSION: Drag-based echocardiographic indices offered high discriminatory performance for mavacamten response. Integrating these patient-specific metrics into routine assessment provides valuable pathophysiological insights into SAM mechanisms in obstructive HCM.
AIMS: To establish normal sex- and age-specific longitudinal strain curves, to quantify their morphological variation with age, and to demonstrate their utility by deriving novel measures from them with the aim of testin...AIMS: To establish normal sex- and age-specific longitudinal strain curves, to quantify their morphological variation with age, and to demonstrate their utility by deriving novel measures from them with the aim of testing prognostic value. METHODS AND RESULTS: Age- and sex-appropriate normal strain curves were derived from healthy participants of the Copenhagen City Heart Study (CCHS). Four novel measures were constructed: early (EDS) and late (LDS) diastolic strain, primarily to assess age-related variation in strain curve morphology, and mean and diastolic strain deviation. Their prognostic value was assessed using Cox proportional hazards regression and C-statistics internally in the CCHS and externally in the LOOP study against a composite endpoint of cardiovascular death and incident heart failure or atrial fibrillation.In total, 1,641 healthy subjects (mean±SD age 45.3±15.2 years, 62.3% female) from the CCHS and 1,307 (mean±SD age 74.4±4.0 years, 47.4% female) from the LOOP study were included. EDS decreased with age while LDS increased. During a median follow-up of 4.9 [IQI: 3.0, 5.6] years, 409 (31.3%) subjects met the outcome in the LOOP study. Mean strain deviation was independently associated with the outcome (adjusted HR = 1.02 (95% CI: 1.00, 1.05), p = 0.045), while diastolic strain deviation was not. CONCLUSION: We established normal sex- and age-specific longitudinal strain curves and furthermore demonstrated their utility by deriving novel measures from these with prognostic value beyond conventional measures. While promising, further validation in external populations is warranted. The normal curves and relevant code are publicly available.
Tamura T, Nagai T, Nakao M
… +16 more, Mizuguchi Y, Koya T, Tada A, George F, Yokota I, Kato Y, Imagawa S, Tokuda Y, Takahashi M, Matsumoto J, Motoi K, Wakamatsu Y, Machida M, Saito T, Anzai T, ELMSTAT-HF investigators
Eur Heart J Qual Care Clin Outcomes
· 2026 Jun · PMID 42320461
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AIMS: Optimisation of guideline-directed medical therapy (GDMT) is a cornerstone of management in heart failure (HF). The GDMT scoring system was developed to quantify both the use and intensification of GDMT. This study...AIMS: Optimisation of guideline-directed medical therapy (GDMT) is a cornerstone of management in heart failure (HF). The GDMT scoring system was developed to quantify both the use and intensification of GDMT. This study investigated the longitudinal changes in the GDMT score and its association with clinical outcomes in contemporary practice. METHODS AND RESULTS: This multicentre prospective cohort study included 680 patients with chronic HF with reduced ejection fraction. The patients were classified into the up-titration (n = 282) and no up-titration (n = 398) groups according to changes in the GDMT score over 9 months. The GDMT score incorporated the use and dose of guideline-recommended drugs including quadruple therapy, ivabradine, and vericiguat. The primary outcome was a composite of all-cause death and hospitalisation for worsening HF. Inverse probability of treatment weighting was used to adjust for baseline differences. Up-titration rates at 9 months were 31.8% for renin-angiotensin system blockers, 28.2% for β-blockers, 10.6% for mineralocorticoid receptor antagonists, and 11.9% for sodium-glucose cotransporter 2 inhibitors. Up-titration was independently associated with baseline GDMT score, age, left ventricular ejection fraction, blood pressure, and renal function. Furthermore, up-titration was associated with a lower incidence of the primary outcome than no up-titration (adjusted HR, 0.65; 95% CI, 0.43-0.98). CONCLUSION: In this contemporary chronic HF cohort, the GDMT score incorporating guideline-recommended drugs revealed suboptimal implementation and intensification of GDMT over 9 months. Greater increases in GDMT score were associated with improved clinical outcomes, supporting the clinical relevance of longitudinal GDMT optimisation.
Miyazaki S, Nitta J, Inaba O
… +21 more, Takahashi A, Hachiya H, Nagata Y, Hayashi T, Iwai S, Yamauchi Y, Mizukami A, Ono Y, Suzuki M, Handa K, Suzuki A, Nakajima J, Sekiguchi Y, Inamura Y, Tanaka Y, Kusa S, Negishi M, Tao S, Takigawa M, Hirakawa A, Sasano T
Zancanaro E, Giannopoulos A, Gimelli A
… +1 more, Kresoja KP
Eur Heart J Imaging Methods Pract
· 2026 Jan · PMID 42317395
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AIMS: Peer review is a cornerstone of scientific quality control, yet it is increasingly burdened by growing manuscript volumes and reviewer fatigue. Large language models (LLMs) have emerged as potential tools to suppor...AIMS: Peer review is a cornerstone of scientific quality control, yet it is increasingly burdened by growing manuscript volumes and reviewer fatigue. Large language models (LLMs) have emerged as potential tools to support scientific review, but it remains unclear whether AI-generated reviews are equivalent to human reviews on the endpoint that ultimately matters, agreement with the final editorial decision. METHODS AND RESULTS: We retrieved 40 manuscripts previously submitted to a cardiology journal (20 ultimately accepted, 20 rejected) along with all available historical human peer reviews ( = 77). For each manuscript, we generated a corresponding peer review using LLM in deep research mode ( = 41). All 118 reviews were reformatted into a single anonymous template by two unblinded investigators and scored independently by two blinded editors across seven domains (digestion, focus, balance, suggestions, precision, politeness, and conclusiveness; 0-2 scale). The primary endpoint was concordance between each reviewer recommendation (in favour of vs. against publication) and the final editorial decision. Secondary endpoints were domain-specific quality scores and AI-human inter-rater agreement (Cohen's κ). Concordance with the final editorial decision was 67.5% for AI-generated reviews (27/40) and 71.9% for the human consensus (23/32 evaluable; = 0.74). Stratified by editorial outcome, AI correctly recommended publication in 75% of accepted manuscripts and rejection in 60% of rejected manuscripts; the corresponding figures for the human consensus were 88% and 56%. AI-generated reviews scored significantly higher than human reviews in five of seven quality domains (focus, balance, suggestions, precision, and conclusiveness; all < 0.05), with a higher total sum score (13.2 ± 0.9 vs. 11.4 ± 2.0; < 0.001). AI-human inter-rater agreement was substantial (κ = 0.73), exceeding human-human agreement on the same articles (κ = 0.54). AI reviews were generated in 2-6 min vs. a median 17-day turnaround for human reviews. CONCLUSION: LLM-generated peer reviews are non-inferior to human reviews in terms of agreement with the final editorial decision, while showing higher internal consistency, comparable quality on structured domains, and substantially shorter turnaround. These findings support the integration of AI as a complementary tool in editorial workflows, rather than as a replacement for human peer review.
Christierson L, Revstedt J, Pozza A
… +6 more, Dragulescu A, Morgan C, Honjo O, Mertens LL, Isaksson H, Hakacova N
Eur Heart J Imaging Methods Pract
· 2026 Jan · PMID 42317394
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AIMS: Assessment of mitral valve (MV) function and haemodynamics is essential for optimizing surgical repair in children with mitral regurgitation. Patient-specific fluid-structure interaction (FSI) modelling can capture...AIMS: Assessment of mitral valve (MV) function and haemodynamics is essential for optimizing surgical repair in children with mitral regurgitation. Patient-specific fluid-structure interaction (FSI) modelling can capture the complex interplay between valvular mechanics and blood flow. In this study, we apply a patient-specific FSI framework to evaluate MV function and haemodynamics in paediatric patients before and after surgery. METHODS AND RESULTS: Seven paediatric patients with mitral regurgitation were analysed (age range: 2-17 years; median: 6 years; 57% female). Patient-specific MV apparatus geometries were segmented from pre- and postoperative 3D echocardiograms. Flow boundary conditions were derived from left ventricular volume measurements. Valve dynamics and haemodynamics were simulated using the FSI framework. Model performance was evaluated against echocardiographic data, pre- and postoperatively. The FSI model reproduced the angle of the regurgitant jet. Preoperatively, the regurgitation grade matched echocardiographic assessment in six of seven patients, and postoperatively in four of seven. The site of regurgitation was correctly identified in six of seven patients, pre- and postoperatively. The model reproduced the observed intraventricular flow patterns in most patients, and the simulated transvalvular pressure gradients agreed with Doppler measurements (mean difference: 0.38 ± 1.57 mmHg preoperatively, -0.42 ± 3.26 mmHg postoperatively). CONCLUSION: The proposed FSI framework captured MV function, haemodynamics, and disease-specific features in paediatric patients pre- and postoperatively, based on evaluation in one of the largest cohorts for the field. This computational framework has the potential to enable predictive simulations that could support surgical planning in the future and improve repair outcomes in children.