European Heart Journal - Cardiovascular Imaging最新文献

Anderson-Fabry disease (AFD) is a rare genetic disease with X-linked transmission characterized by a defect in the enzyme alpha-galactosidase A (alpha-GAL), which impairs glycosphingolipid metabolism and leads to an excessive storage of globotriaosylceramide (Gb3) within lysosomes. AFD involves renal, cardiac, vascular, and nervous systems and is mainly observed in male patients with onset in childhood, although cardiac manifestation is often shown in adults. AFD cardiomyopathy is caused by the accumulation of Gb3 within myocytes first showed by left ventricular (LV) hypertrophy and diastolic dysfunction, leading to restrictive cardiomyopathy and systolic heart failure with biventricular involvement. The diagnosis of AFD cardiomyopathy may be insidious in the first stages and requires accurate differential diagnosis with other cardiomyopathies with hypertrophic phenotype. However, it is fundamental to promptly initiate specific therapies that have shown promising results, particularly for early treatment. A careful integration between clinical evaluation, genetic tests, and cardiac imaging is required to diagnose AFD with cardiac involvement. Basic and advanced echocardiography, cardiac magnetic resonance, and nuclear imaging may offer pivotal information for early diagnosis (Central illustration) and the management of these patients is often limited to centres with high expertise in the field. This clinical consensus statement, developed by experts from the European Society of Cardiology (ESC) Working Group on Myocardial & Pericardial Diseases and the European Association of Cardiovascular Imaging of the ESC, aims to provide practical advice for all clinicians regarding the use of multimodality imaging to simplify the diagnostic evaluation, prognostic stratification, and management of cardiac involvement in AFD.

Aims: This study aims to assess the changes in cardiac damage stage in a real-world cohort of patients undergoing transcatheter aortic valve implantation (TAVI) and to investigate the prognostic value of cardiac damage stage evolution.

Methods and results: Patients with severe AS undergoing TAVI were retrospectively analyzed. A 5-stage system based on the presence and extent of cardiac damage assessed by echocardiography was applied before and 6 months after TAVI. Multivariable Cox regression analyses were used to examine independent prognostic value of the changes in cardiac damage after TAVI. A total of 734 patients with severe AS (mean age 79.8±7.4 years, 55% male) were included. Before TAVI, 32 (4%) patients did not show any sign of extra-valvular cardiac damage (Stage 0), 85 (12%) had left ventricular damage (Stage 1), 220 (30%) left atrial and/or mitral valve damage (Stage 2), 227 (31%) pulmonary vasculature and/or tricuspid valve damage (Stage 3), and 170 (23%) right ventricular damage (Stage 4). Six months after TAVI, 39% of the patients improved at least 1 stage in cardiac damage. Staging of cardiac damage at 6 months after TAVI (HR per 1-stage increase 1.391; P = 0.035) as well as worsening in the stage of cardiac damage (HR 3.729, P = 0.005) were independently associated with 2-year all-cause mortality.

Conclusion: More than one third of patients with severe AS showed an improvement in cardiac damage 6 months after TAVI. Staging cardiac damage at baseline and follow-up may improve risk stratification in patients undergoing TAVI.

Aims: Previous observational studies suggest that preoperative imaging in patients with chronic ischemic heart failure (iHF) may identify non-contractile, hypoperfused, yet metabolically viable (hibernating) myocardial segments that can regain function after coronary revascularization. Various imaging techniques, including positron emission tomography (PET) with retention tracers like 82Rb, have shown equivocal results. However, recent randomized studies have found limited value in these methods for predicting postoperative recovery and survival. This study, therefore, aims to assess whether PET viability imaging using the optimal perfusion tracer [15O]H2O, combined with [18F]FDG, provides better predictive accuracy.

Methods and results: Seventy-three patients with chronic iHF and reduced LVEF (mean baseline LVEF 31±9%) underwent [15O]H2O/[18F]FDG PET viability imaging before potential revascularization. The primary endpoint was a ≥5% absolute increase in LVEF from baseline to follow-up, assessed by echocardiography. In total, 31 of 73 (42%) patients were revascularized, with 16 of 31 (52%) experiencing a ≥5% LVEF improvement postoperatively. Baseline characteristics and revascularization type did not significantly differ between improvers and non-improvers. ROC analysis of PET metrics to predict LVEF improvement yielded AUC values ≤0.60, and no baseline characteristics or PET measures predicted survival in revascularized patients.

Conclusion: No [15O]H2O/[18F]FDG PET parameters predicted post-revascularization LVEF improvement or survival in patients with suspected chronic iHF. Thus, clinical use of PET viability imaging still warrants reconsideration, particularly if non-ischemic HF is present.

Background: Accurate risk stratification for patients with non-dilated left ventricular cardiomyopathy (NDLVC) remains challenging due to lack of dedicated clinical trials. This post-hoc analysis aims to delineate the arrhythmic risk and assess the incremental value of cardiac magnetic resonance (CMR) imaging in the DERIVATE (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy) study cohort meeting the NDLVC diagnostic criteria.

Methods: Patients with NDLVC from DERIVATE registry were identified in the absence of left ventricular (LV) dilatation and in the presence of non-ischaemic LV scarring ("fibrotic NDLVC") or isolated LV systolic dysfunction (LV ejection fraction <50%) without fibrosis ("hypokinetic NDLVC"). The primary endpoint was all-cause mortality. Major adverse arrhythmic cardiac events (MAACE) were the secondary endpoint and included sudden cardiac death (SCD) and aborted SCD.

Results: One hundred ninety-seven NDLVC patients were identified from the cohort of the DERIVATE study (Mean age: 59±14 years; Male: 135). Over a median follow-up of 2.7 years, 15 (8%) patients died, and 8 (4%) experienced MAACE. Patients with "hypokinetic" NDLVC had significantly lower rates of MAACE than non-ischaemic dilated cardiomyopathy (NIDCM) (p=0.001), while patients with "fibrotic" NDLVC had same rate of both primary (p=0.48) and secondary endpoints (p= 0.616) compared to NIDMC patients. Multivariable analysis identified LGE with midwall distribution as an independent predictor of MAACE in NDLVC patients (Hazard Ratio 6.7, 95% Confidence Interval: 1.33-33.67; p=0.021).

Conclusions: NDLVC patients exhibit a heterogeneous risk profile for arrhythmic events. The presence of midwall LGE, similarly to NIDCM, is a significant predictor of MAACE, highlighting the importance of CMR imaging for risk stratification.

Aims: Exercise stress echocardiography (ESE) is often used to identify heart failure with preserved ejection fraction (HFpEF) in patients presenting dyspnea. However, diagnostic criteria have not been standardized. Here, we sought to develop ESE-based criteria to diagnose HFpEF in dyspneic patients.

Methods and results: A total of 81 consecutive patients with dyspnea who underwent exercise right heart catheterization and ESE were evaluated. Diagnosis of HFpEF was ascertained by directly-measured hemodynamics (61 HFpEF and 20 controls). Logistic regression analysis was applied to develop an ESE-based scoring system to diagnose HFpEF. Multivariable logistic regression analysis identified resting left atrial reservoir strain <20%, exercise septal E/e' ratio >13, and increases in ultrasound B-lines as independent predictors of HFpEF. A weighted score was created with these variables (the ESE score) ranging from 0 to 5. The ESE score accurately discriminated HFpEF from controls (area under the curve [AUC] 0.90, p<0.0001), with a superior diagnostic ability to the ASE/ESCVI criteria (AUC comparison p<0.0001). The ESE score classified the HFpEF probability into three categories (probabilities: low risk 28%, intermediate risk 59-83%, and high risk 95-99%). In a cohort of 620 dyspneic patients, the predictive ability of the derived score was assessed. A higher ESE score was associated with an increased risk of all-cause mortality or worsening HF events even after adjusting for confounders (hazard ratio; 1.17 per 1-point increase, 95% confidence intervals; 1.00-1.37, p=0.04).

Conclusion: The ESE score, which is based on three echocardiographic variables, may be an effective tool for diagnosing HFpEF on exercise echocardiography.