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Background Cardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis. Methods Eighty-two patients (55.5 ± 8.5 years; 52 M) and 20 healthy subjects (53.2 ± 11.7 years; 10 M) were prospectively recruited. All subjects underwent CMR with LGE imaging and T1 mapping using a Modified Look-Locker Inversion-recovery (MOLLI) sequence on a 3 T scanner. Native T1 and ECV were measured semi-automatically using a dedicated CMR software. The left ventricular (LV) LGE pattern was classified as none, patchy, and global groups. Global LGE was considered when there was diffuse, transmural LGE in more than half of the short axis images. Follow-up was performed for all-cause mortality using Cox proportional hazards regression analysis and Kaplan-Meier survival curves. Results The patients demonstrated an increase in native T1 (1438 ± 120 ms vs. 1283 ± 46 ms, P  = 0.001) and ECV (43.9 ± 10.9% vs. 27.0 ± 1.7%, P  = 0.001) compared to healthy controls. Native T1, ECV and LGE showed significant correlation with Mayo Stage, and ECV and LGE showed significant correlation with echocardiographic E/E’ and LV ejection fraction. During the follow-up for a median time of 8 months, 21 deaths occurred. ECV ≥ 44.0% (hazard ratio [HR] 7.249, 95% confidence interval (CI) 1.751–13.179, P  = 0.002) and global LGE (HR 4.804, 95% CI 1.971–12.926, P  = 0.001) were independently prognostic for mortality over other clinical and imaging parameters. In subgroups with the same LGE pattern, ECV ≥ 44.0% remained prognostic (log rank P  = 0.029). Median native T1 (1456 ms) was not prognostic for mortality (Tarone-Ware, P  = 0.069). Conclusions During a short-term follow-up, both ECV and LGE are independently prognostic for mortality in AL amyloidosis. In patients with a similar LGE pattern, ECV remained prognostic. Native T1 was not found to be a prognostic factor.

Postinfarction mechanical dispersion (MD), that is, the regional heterogeneity of myocardial contraction throughout the cardiac cycle, has detrimental effects on left ventricular (LV) function and is related to the occurrence of heart failure and ventricular arrhythmias. However, its prevalence, pathophysiological determinants, and clinical utility are still unknown. The aim of the present study is to clarify these issues. In total, 130 consecutive patients (mean age 60 ± 12 years, 75% male) with a first ST-segment elevation myocardial infarction (STEMI) were included. Cardiac magnetic resonance (CMR) with late gadolinium enhancement imaging was performed to assess LV function, infarct size, and microvascular obstruction. Feature-tracking analysis was applied to cine-CMR short-axis images to assess MD, defined as the SD of the time-to-peak circumferential strain of the LV segments expressed as percent cardiac cycle. For comparison purpose, 40 control subjects similar in age and gender to the STEMI group were also included. Patients were followed-up for a median of 95 months; the outcome event was defined as a composite of cardiovascular death, aborted sudden cardiac death, and hospitalization for heart failure. STEMI patients had significantly higher MD compared with controls (12.0 ± 5.35% vs 3.85 ± 0.99%, p <0.001). At multivariate analysis, heart rate (β = 0.20, p = 0.008), LV end-systolic volume index (β = 0.37, p <0.001), and infarct size (β = 0.23, p = 0.017) were significantly and independently related to MD. The outcome event occurred in 26 (20%) patients. At multivariate Cox proportional hazards analysis, MD was significantly and independently related to the outcome event (p <0.001). MD provided significant incremental value over the other clinical and CMR variables in predicting the outcome event (p <0.001 for the chi-square change). In conclusion, MD after STEMI is a marker of the extent of myocardial damage; its assessment by feature-tracking CMR provides significant, independent, and incremental long-term prognostic information.

The presence and extent of left ventricular hypertrophy (LVH) is a major determinant of symptoms in patients with hypertrophic cardiomyopathy (HCM). There is increasing evidence to suggest that myocardial energetic impairment represents a central mechanism leading to LVH in HCM. There is currently a significant unmet need for disease-modifying therapy that regresses LVH in HCM patients. Perhexiline, a potent carnitine palmitoyl transferase-1 (CPT-1) inhibitor, improves myocardial energetics in HCM, and has the potential to reduce LVH in HCM. The primary objective is to evaluate the effects of perhexiline treatment on the extent of LVH, in symptomatic HCM patients with at least moderate LVH. RESOLVE-HCM is a prospective, multicenter double-blind placebo-controlled randomized trial enrolling symptomatic HCM patients with at least moderate LVH. Sixty patients will be randomized to receive either perhexiline or matching placebo. The primary endpoint is change in LVH, assessed utilizing cardiovascular magnetic resonance (CMR) imaging, after 12-months treatment with perhexiline. RESOLVE-HCM will provide novel information on the utility of perhexiline in regression of LVH in symptomatic HCM patients. A positive result would lead to the design of a Phase 3 clinical trial addressing long-term effects of perhexiline on risk of heart failure and mortality in HCM patients.

[2] However, the signs and symptoms of cardiomyopathy frequently are subtle and often overlooked during the ambulatory and early non-ambulatory stages. [...]non-invasive imaging methods are recommended for the assessment of myocardial involvement of DMD patients in the updated diagnosis and management statement, and echocardiography (Echo) and cardiac magnetic resonance (CMR) are two of the most commonly used imaging methods. Previous studies have reported the assessment of myocardial fibrosis in DMD patients by LGE,[4,5] but studies investigating the prevalence of myocardial fibrosis in DMD patients during the first decade of life is still lacking. [...]DMD is a multisystem disease, loss of dystrophy protein function could occur in skeletal muscle and cardiomyocytes. In practice, individuals with DMD start skeletal muscle management early but are not referred to a cardiac specialist until late in the disease. [...]it is important to clarify the association between skeletal muscle involvement and myocardial involvement in the early stages of disease, which may help clinicians start multidisciplinary management earlier and improve the quality of life of patients further. [...]myocardial involvement could occur in the first decade life of DMD patients.

Background Cardiovascular disease (CVD) is the leading cause of non‐cancer death in cancer survivors, but the risk of CVD varies between cancers. Objectives To synthesise available evidence on patterns and magnitude of CVD mortality risk. Methods A systematic search of Medline (OVID), CINAHL and Scopus databases from 01‐January‐2000 to 16‐July‐2023 of studies of people with cancer, reporting CVD mortality in cancer population compared with a reference population (e.g. general population) as standardised mortality ratios (SMR). Meta‐analysis of SMRs across cancer and CVD types were pooled using a random‐effects model to allow for heterogeneity of the true effect size across studies. Results We identified 136 studies from 16 countries. Sample sizes ranged from 157 to 7,529,481. The majority (n = 98; 72%) were conducted in the United States, followed by Europe (n = 22; 16%). The most common cancers studied were gastrointestinal (n = 34 studies), haematological (n = 31) and breast (n = 29). A total of 876 CVD SMRs were extracted across diverse CVD conditions. Of those, the majority (535; 61%) indicated an increased risk of CVD death (SMR >1), 109 (12%) a lower risk of CVD death (SMR <1) and 232 (27%) an equivalent risk (95% CI of SMR included 1) compared to the general population. The meta‐analysis of all reported SMRs showed an increased risk of CVD death (SMR = 1.55, 95% CI = 1.40–1.72) in cancer survivors compared with the general population. The SMR varied between CVD conditions and ranged from 1.36 (95% CI = 1.29–1.44) for heart diseases to 1.56 (95% CI = 1.39–1.76) for cerebrovascular diseases. SMR varied across cancer types, ranging from 1.14 (95% CI = 1.04–1.25) for testicular/germ cell tumours to 2.82 (95% CI = 2.20–3.63) for brain/central nervous system tumours. Conclusions Cancer survivors are at increased risk of premature CVD mortality compared to the general population, but the risk varies by cancer type and CVD. Future research should focus on understanding mechanisms behind the increased CVD risk to develop appropriate interventions. This study has summarised the existing evidence demonstrating higher cardiovascular (CVD) mortality in people with cancer compared to the general population across different CVD conditions and different cancer types. Reducing CVD mortality after cancer diagnosis should be an important priority for survivorship research and care.

Cardiovascular magnetic resonance (CMR) imaging is widely regarded as the gold-standard technique for myocardial tissue characterization, allowing for the detection of structural abnormalities such as myocardial fatty replacement, myocardial edema, myocardial necrosis, and/or fibrosis. Historically, the identification of abnormal myocardial regions relied on variations in tissue signal intensity, often necessitating the use of exogenous contrast agents. However, over the past two decades, innovative parametric mapping techniques have emerged, enabling the direct quantitative assessment of tissue magnetic resonance (MR) properties on a voxel-by-voxel basis. These mapping techniques offer significant advantages by providing comprehensive and precise information that can be translated into color-coded maps, facilitating the identification of subtle or diffuse myocardial abnormalities. As unlikely conventional methods, these techniques do not require a substantial amount of structurally altered tissue to be visually identifiable as an area of abnormal signal intensity, eliminating the reliance on contrast agents. Moreover, these parametric mapping techniques, such as T1, T2, and T2* mapping, have transitioned from being primarily research tools to becoming valuable assets in the clinical diagnosis and risk stratification of various cardiac disorders. In this review, we aim to elucidate the underlying physical principles of CMR parametric mapping, explore its current clinical applications, address potential pitfalls, and outline future directions for research and development in this field.

Late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging has enabled the accurate assessment of myocardial infarction (MI). However, LGE CMR has not been performed successfully in the fetus, where it could be useful for animal studies of interventions to promote cardiac regeneration. We believe that LGE imaging could allow us to document the presence, extent and effect of MI in utero and would thereby expand our capacity for conducting fetal sheep MI research. We therefore aimed to investigate the feasibility of using LGE to detect MI in sheep fetuses. Six sheep fetuses underwent a thoracotomy and ligation of a left anterior descending (LAD) coronary artery branch; while two fetuses underwent a sham surgery. LGE CMR was performed in a subset of fetuses immediately after the surgery and three days later. Early gadolinium enhancement (EGE) CMR was also performed in a subset of fetuses on both days. Cine imaging of the heart was performed to measure ventricular function. The imaging performed immediately after LAD ligation revealed no evidence of infarct on LGE (n=3). Two of four infarcted fetuses (50%) showed hypoenhancement at the infarct site on the EGE images. Three days after the ligation, LGE images revealed a clear, hyper-enhanced infarct zone in four of the five infarcted fetuses (80%). No hyper-enhanced infarct zone was seen on the one sham fetus that underwent LGE CMR. No hypoenhancement could be seen in the EGE images in either the sham (n=1) or the infarcted fetus (n=1). No regional wall motion abnormalities were apparent in two of the five infarcted fetuses. LGE CMR detected the MI three days after LAD ligation, but not immediately after. Using available methods, EGE imaging was less useful for detecting deficits in perfusion. Our study provides evidence for the ability of a non-invasive tool to monitor the progression of cardiac repair and damage in fetuses with MI. However, further investigation into the optimal timing of LGE and EGE scans and improvement of the sequences should be pursued with the aim of expanding our capacity to monitor cardiac regeneration after MI in fetal sheep.

Abstract Background Congenital disorders of glycosylation (CDG) are rare genetically inherited defects leading to enzyme deficiency or malfunction in the glycosylation pathway. Normal glycosylation is essential to the development of normal cardiac anatomy and function. Congenital disorders of glycosylation–related cardiomyopathy are often the first manifestation detected in early life and may lead to sudden cardiac death. Approximately one-fifth of CDG types are related to cardiac diseases that include cardiomyopathy, rhythm disturbances, pericardial effusions, and structural heart disease. Case summary We report a rare case of a 26-year-old lady with CDG-1 who presented with acute-onset dyspnoea. She had respiratory tract symptoms for the past 2 weeks. With the relevant clinical and biochemical findings, including supportive findings on echocardiogram and cardiac magnetic resonance imaging, we have managed to arrive at a diagnosis of severe pneumonia leading to acute decompensated heart failure, as well as the discovery of an underlying CDG-associated dilated cardiomyopathy (DCM) and acute myocarditis. Anti-failure medications and i.v. methylprednisolone were commenced, and she showed gradual clinical improvement with an increase of her left ventricular function. She was discharged home well with anti-failure therapy, prednisolone, and a follow-up echocardiogram with further review in the heart failure clinic. Discussion In conclusion, this case report highlights the need for accurate diagnosis and prompt management of CDG-associated DCM, leading to a successful recovery and discharge from hospital care. With this, we hope to add to the increasing number of reported cases of CDG-related cardiac disease in the medical literature to emphasize the importance of screening and follow-up for any underlying cardiac diseases in patients with CDG.

Background Although prior reports have evaluated the clinical and cost impacts of cardiovascular magnetic resonance (CMR) for low-to-intermediate-risk patients with suspected significant coronary artery disease (CAD), the cost-effectiveness of CMR compared to relevant comparators remains poorly understood. We aimed to summarize the cost-effectiveness literature on CMR for CAD and create a cost-effectiveness calculator, useable worldwide, to approximate the cost-per-quality-adjusted-life-year (QALY) of CMR and relevant comparators with context-specific patient-level and system-level inputs. Methods We searched the Tufts Cost-Effectiveness Analysis Registry and PubMed for cost-per-QALY or cost-per-life-year-saved studies of CMR to detect significant CAD. We also developed a linear regression meta-model (CMR Cost-Effectiveness Calculator) based on a larger CMR cost-effectiveness simulation model that can approximate CMR lifetime discount cost, QALY, and cost effectiveness compared to relevant comparators [such as single-photon emission computed tomography (SPECT), coronary computed tomography angiography (CCTA)] or invasive coronary angiography. Results CMR was cost-effective for evaluation of significant CAD (either health-improving and cost saving or having a cost-per-QALY or cost-per-life-year result lower than the cost-effectiveness threshold) versus its relevant comparator in 10 out of 15 studies, with 3 studies reporting uncertain cost effectiveness, and 2 studies showing CCTA was optimal. Our cost-effectiveness calculator showed that CCTA was not cost-effective in the US compared to CMR when the most recent publications on imaging performance were included in the model. Conclusions Based on current world-wide evidence in the literature, CMR usually represents a cost-effective option compared to relevant comparators to assess for significant CAD.

Background In pulmonary arterial hypertension (PAH), progressive right ventricular (RV) dysfunction is believed to be largely secondary to RV ischaemia. A recent pilot study has demonstrated the feasibility of Oxygen-sensitive (OS) cardiovascular magnetic resonance (CMR) to detect in-vivo RV myocardial oxygenation. The aims of the present study therefore, were to assess the prevalence of RV myocardial ischaemia and relationship with RV myocardial interstitial changes in PAH patients with non-obstructive coronaries, and corelate with functional and haemodynamic parameters. Methods We prospectively recruited 42 patients with right heart catheter (RHC) proven PAH and 11 healthy age matched controls. The CMR examination involved standard functional imaging, OS-CMR imaging and native T1 mapping. An ΔOS-CMR signal intensity (SI) index (stress/rest signal intensity) was acquired at RV anterior, RV free-wall and RV inferior segments. T1 maps were acquired using Shortened Modified Look-Locker Inversion recovery (ShMOLLI) at the inferior RV segment. Results The inferior RV ΔOS-CMR SI index was significantly lower in PAH patients compared with healthy controls (9.5 (– 7.4–42.8) vs 12.5 (9–24.6)%, p  = 0.02). The inferior RV ΔOS-CMR SI had a significant correlation to RV inferior wall thickness (r = – 0.7, p  < 0.001) and RHC mean pulmonary artery pressure (mPAP) (r = – 0.4, p  = 0.02). Compared to healthy controls, patients with PAH had higher native T1 in the inferior RV wall: 1303 (1107–1612) vs 1232 (1159–1288)ms, p  = 0.049. In addition, there was a significant difference in the inferior RV T1 values between the idiopathic PAH and systemic sclerosis associated PAH patients: 1242 (1107–1612) vs 1386 (1219–1552)ms, p  = 0.007. Conclusion Blunted OS-CMR SI suggests the presence of in-vivo microvascular RV dysfunction in PAH patients. The native T1 in the inferior RV segments is significantly increased in the PAH patients, particularly among the systemic sclerosis associated PAH group.