Journal of Cardiovascular Imaging最新文献

Hypertrophic cardiomyopathy (HCM) is a myocardial disorder characterized by unexplained myocardial hypertrophy. Although the diagnosis of HCM is traditionally based on increased left ventricular (LV) wall thickness, contemporary management requires a comprehensive multimodality imaging approach to accurately define disease phenotype, assess functional consequences, and guide risk stratification. Transthoracic echocardiography remains the first-line imaging modality, providing real-time evaluation of LV morphology, systolic and diastolic function, and LV outflow tract obstruction (LVOTO). However, its ability to assess myocardial tissue characteristics and complex morphologic variants may be limited in selected patients. Cardiac magnetic resonance (CMR) offers superior spatial resolution and allows detection of myocardial fibrosis using late gadolinium enhancement. Cardiac computed tomography serves as a complementary tool for evaluating coronary artery anatomy and detailed cardiac structure, particularly in patients with suboptimal echocardiographic windows or contraindications to CMR. This review summarizes the strengths and limitations of each imaging modality and highlights their complementary roles in the evaluation of cardiac morphology, systolic and diastolic function, LVOTO, and tissue characterization. An integrated imaging strategy is essential for optimized diagnosis, individualized risk stratification, and informed therapeutic decision-making in patients with HCM.

Background: Epicardial fat exerts both protective and deleterious effects on organs through diverse cytokine-mediated pathways. This study aimed to investigate computed tomography (CT)-based indexed epicardial fat volume (EFVi) in association with target organ damage parameters.

Methods: The prospectively enrolled cohort of 75 patients with nonobstructive coronary artery disease underwent electrocardiogram-gated CT and was evaluated for target organ damage parameters: estimated glomerular filtration rate, proteinuria, echocardiographic septal e' velocity, E/e' and tricuspid regurgitation velocity, brachial-ankle pulse wave velocity, and ankle-brachial index. EFVi was measured from semiautomated 3D segmentation of electrocardiogram-gated CT. Partial correlation, multiple linear regression, and receiver operating characteristic (ROC) analyses were conducted.

Results: Age and EFVi showed moderate positive linear correlation (r = 0.567, P < 0.001). After adjusting for age, EFVi was significantly correlated with the septal e' velocity (r = - 0.489, P < 0.001) and E/e' (r = 0.256, P = 0.034), but not with other target organ damage parameters (P > 0.05). Multiple linear regression analysis showed that the correlations of the EFVi with the septal e' velocity (β = -0.0003, P = 0.007) and E/e' (β = 0.0606, P = 0.024) remained significant after adjusting for potential confounders. ROC analysis identified optimal EFVi thresholds: 95.78 cm³/m² for reduced septal e' velocity (area under the ROC curve [AUC], 0.750; sensitivity, 88.2%; specificity, 56.8%) and 91.68 cm³/m² for elevated E/e' (AUC, 0.692; sensitivity, 71.4%; specificity, 64.8%).

Conclusions: EFVi was related to left ventricular diastolic function more than other target organ damage parameters, including renal function and arterial stiffness, which suggests that the epicardial fat may have a role in the pathogenesis of left ventricular diastolic dysfunction.

Background: The left atrial appendage (LAA) is a critical but frequently overlooked site of thrombus formation, reinforcing the need for accurate identification in routine cardiac imaging. This process is related to pathological dilation associated with endothelial injury and a proinflammatory status. This study assesses the performance of deep learning architectures based on U-Net, specifically UNet3D, Residual-UNet3D, 3D Attention-UNet, and Res16-PAC-UNet, in the semiautomated segmentation and volume measurement of LAA.

Methods: We retrospectively analyzed noncontrast cardiac computed tomography (NCCT) scans from 452 patients aged ≥ 60 years, acquired for chest pain evaluation, to compare the performance of four U-Net-based deep learning architectures (UNet3D, Residual-UNet3D, 3D Attention-UNet, and Res16-PAC-UNet) for semiautomated LAA segmentation and volume measurement. Segmentation accuracy was assessed with the Dice coefficient, and volumetric agreement with Pearson correlation and Bland-Altman analysis.

Results: Dice coefficients were 78.44 ± 1.93 for UNet3D, 78.97 ± 0.79 for Residual-UNet3D, 79.07 ± 1.43 for 3D Attention-UNet, and 77.68 ± 1.47 for Res16-PAC-UNet. All models showed strong correlations between predicted and manual volumes (P < 0.001), with the highest in 3D Attention-UNet (r = 0.800). Bland-Altman analysis indicated minimal bias and narrow limits of agreement for all architectures, confirming consistent reliability.

Conclusions: Deep learning-based segmentation on NCCT enables accurate, reproducible LAA morphological and volumetric assessment without contrast, offering a rapid and reliable tool to support cardiovascular risk stratification and treatment planning.

Background: Pulmonary hypertension (PH) is a progressive clinical condition that eventually leads to right ventricular (RV) failure. RV function is the primary determinant of morbidity and mortality in patients with PH. RV global longitudinal strain (RVGLS) is a promising echocardiographic metric used to assess RV function in this setting. Our study aimed to compare the ability of RVGLS, tricuspid annular plane systolic excursion (TAPSE) and fractional area change (FAC) to predict adverse outcomes in patients with PH.

Methods: We retrospectively evaluated 315 patients with PH of diverse etiologies with 62% constitute of WHO group 2 disease, who were followed at the PH clinic at the University of Virginia, from March 2012 to December 2018. We included all adult patients who met the hemodynamic definition of PH with right heart catheterization and who underwent echocardiography within 1 month of each other.

Results: Approximately half of the cohort was female, with a mean age of 64 ± 14 years. We found a strong correlation between RVGLS and FAC (r =  - 0.55, P < 0.001). Furthermore, there was a significant correlation between RVGLS and invasive hemodynamics. Compared with the TAPSE, the RVGLS stratified by quartiles was associated with mortality at 5 years and hospitalization.

Conclusion: RVGLS is an echocardiographic marker that correlates closely with FAC and invasive pulmonary hemodynamics. In this study, both RVGLS and FAC were associated with 5-year mortality, whereas TAPSE was not. Notably, only RVGLS showed a significant association with hospitalization, suggesting that it may provide additional prognostic value in patients with PH.

Hypertrophic cardiomyopathy has become a highly manageable condition due to recent therapeutic advances that have significantly reduced its overall mortality rate. However, sudden cardiac death continues to be a critical and unsolved threat, particularly in younger patients and competitive athletes. Even after recent updates to guidelines on sudden cardiac death risk evaluation in hypertrophic cardiomyopathy, new clinical evidence continues to emerge, further enriching our understanding of risk stratification and management. In this review, we summarize current research findings and explore recent advances to provide insights into future directions in the treatment of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a disease characterized by unexplained left ventricular hypertrophy and is caused by mutations in cardiac sarcomeric proteins. Despite advances in diagnostic modalities and risk stratification, therapeutic strategies have until recently mostly focused on the management of symptoms and the prevention of sudden cardiac death, rather than modifying the underlying sarcomeric dysfunction itself. Conventional pharmacological therapies such as β-blockers and nondihydropyridine calcium channel blockers are effective first-line treatments for obstructive HCM, and established invasive septal reduction therapies, such as surgical myectomy and alcohol septal ablation, provide effective relief of obstruction in refractory patients. However, these therapies address anatomical and hemodynamical consequences rather than the molecular etiology of the disease. In recent years, novel therapeutic approaches have emerged that target the pathophysiological mechanisms of HCM more directly. Sodium-glucose cotransporter 2 inhibitors have demonstrated clinical benefits in HCM through improvements in myocardial energetics. Cardiac myosin inhibitors directly attenuate sarcomeric hypercontractility and have shown improvements in symptoms, functional status, and hemodynamic parameters in obstructive HCM. Furthermore, preliminary gene-targeted therapies are under active investigation and offer the prospect of definitive cure. This review provides a comprehensive overview of current and emerging treatment modalities for HCM. Overall, the management of HCM is evolving toward a more mechanism-targeted approach spanning from gene to myocardium. Ongoing research will be essential to integrate the emerging molecularly targeted therapies with established management strategies into a personalized, multidisciplinary management of HCM.

Atrial fibrillation (AF) is the most common sustained arrhythmia in patients with hypertrophic cardiomyopathy (HCM), conferring a markedly increased risk of thromboembolic events. Conventional risk stratification tools such as the CHA₂DS₂-VASc (congestive heart failure, hypertension, age ≥ 75 years [doubled], diabetes mellitus, prior stroke or transient ischemic attack [doubled], vascular disease, age 65-74 years, female sex) score are often insufficient to predict thromboembolic events in patients with HCM and AF, as thromboembolic risk in HCM is driven by disease-specific structural, functional, and prothrombotic substrates. This review synthesizes current evidence on the epidemiology, pathophysiological mechanisms, and clinical impact of AF and thromboembolism in HCM. We discuss variable imaging modalities-including strain echocardiography, cardiac magnetic resonance, and cardiac computed tomography-that offer enhanced characterization of atrial remodeling and thromboembolic risk in patients with HCM. Furthermore, we outline current guideline-based anticoagulation strategies, the evolving role of direct oral anticoagulants, and adjunctive therapies such as left atrial appendage occlusion and catheter ablation. A comprehensive, multidisciplinary approach that incorporates advanced imaging, molecular profiling, and individualized management is ideal to optimize outcomes and reduce stroke burden in patients with HCM and AF.

Background: We aimed to find predictors of ejection fraction (EF) deterioration in heart failure with preserved EF (HFpEF) patients to prevent their further deterioration.

Methods: We studied 215 patients (mean age, 73 ± 8 years; 63% women) with HFpEF and with records of Charlson Comorbidity Index, glomerular filtration rate. Myocardial work, global longitudinal, radial, circumferential, and area strain. The global work index, global constructive work (GCW), wasted work, global work efficiency was obtained by echocardiography. Patients were followed up for 3 years.

Results: Five patients developed myocardial infarction and were excluded from the study. Baseline EF was higher in female patients (61.2% ± 3.1% vs. 56.4% ± 2.7%, P < 0.002), in patients aged > 70 years (62.4% ± 2.1% vs. 57.1% ± 2.3%, P < 0.005), and in patients with end-diastolic volume index < 60 mL/m² (56.1% ± 3.2% vs. 63.4% ± 2.3%, P < 0.001). EF decline compared to baseline was -7.3% ± 1.6% (P < 0.01). EF decline was significantly more in patients aged > 70 years, in patients with coronary artery disease and did not relate to sex, left ventricle size, cardiac index, and glomerular filtration rate. During follow-up 58 patients (27%) had EF < 50%, worsening in area strain (-27.9% ± 8.5% vs. -24.7% ± 5.3%, P < 0.003), global longitudinal strain (-19.7% ± 2.4% vs. -17.1% ± 1.6%, P < 0.005), and GCW (2,378% ± 117% vs. 2,102% ± 10%, P < 0.002). Patients with EF < 50% at the end of the study had less area strain and GCW baseline values compared with patients with EF > 50% (22.4% ± 7.2% vs. -27.6% ± 8.1%, P < 0.002; 2,081 ± 92 vs. 2,489 ± 127, P < 0.001). GCW was the predictor of EF deterioration (area under curve, 0.8853).

Conclusions: GCW predicts EF decline in HFpEF patients which may help identify this subset of patients and prevent their further deterioration earlier.

We conducted a comprehensive literature review of sarcomeric gene studies, registry analyses, and recent cohort investigations, focusing on genetic testing outcomes and clinical prognostication. Sarcomeric mutations account for approximately 60% of familial hypertrophic cardiomyopathy (HCM) cases and exhibit variable penetrance and expressivity. Additionally, mitochondrial DNA variants and nonsarcomeric genetic modifiers contribute to the phenotypic heterogeneity observed in HCM. Genetic testing facilitates diagnosis in atypical cases, guides cascade testing in families, and supports reproductive decision-making. Long-term follow-up data from registries indicate that sarcomere-positive patients are diagnosed approximately 13 years earlier and experience nearly double the 50-year incidence of adverse cardiovascular events compared to sarcomere-negative individuals. In Korean cohorts, the mutation detection rate is reported at 43.5%, with genotype-positive status independently associated with worse outcomes. However, for certain prognostic outcomes-particularly sudden cardiac death-more robust data are needed. Emerging therapies, including myosin inhibitors and gene-editing approaches, show promise in targeting the underlying molecular mechanisms of HCM. Therefore, integrating comprehensive genetic screening-including sarcomeric, mitochondrial, and modifier genes-is essential for precise risk stratification and personalized management of HCM. Future efforts should focus on refining variant interpretation and advancing genotype-guided therapeutic strategies.