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BackgroundDiagnosis of cardiac amyloidosis (CA) requires advanced imaging techniques. Typical surface ECG patterns have been described, but their diagnostic abilities are limited.ObjectiveThe aim was to perform a thorough electrophysiological characterisation of patients with CA and derive an easy-to-use tool for diagnosis.MethodsWe applied electrocardiographic imaging (ECGI) to acquire electroanatomical maps in patients with CA and controls. A machine learning approach was then used to decipher the complex data sets obtained and generate a surface ECG-based diagnostic tool.FindingsAreas of low voltage were localised in the basal inferior regions of both ventricles and the remaining right ventricular segments in CA. The earliest epicardial breakthrough of myocardial activation was visualised on the right ventricle. Potential maps revealed an accelerated and diffuse propagation pattern. We correlated the results from ECGI with 12-lead ECG recordings. Ventricular activation correlated best with R-peak timing in leads V1–V3. Epicardial voltage showed a strong positive correlation with R-peak amplitude in the inferior leads II, III and aVF. Respective surface ECG leads showed two characteristic patterns. Ten blinded cardiologists were asked to identify patients with CA by analysing 12-lead ECGs before and after training on the defined ECG patterns. Training led to significant improvements in the detection rate of CA, with an area under the curve of 0.69 before and 0.97 after training.InterpretationUsing a machine learning approach, an ECG-based tool was developed from detailed electroanatomical mapping of patients with CA. The ECG algorithm is simple and has proven helpful to suspect CA without the aid of advanced imaging modalities.

Several randomized, double-blind, placebo-controlled trials (RCT) explore disease-modifying therapeutics in transthyretin amyloid cardiomyopathy (ATTR-CM). However, it is currently unclear whether patients eligible to participate in the RCT are representative of real-world patients. ATTR-CM patients presenting to a tertiary referral center for cardiac amyloidosis at the Medical University of Vienna between March 2012 and May 2024 were included in a prospective registry. Inclusion and exclusion criteria of the ATTRACT, ATTRIBUTE, HELIOS-B, CARDIO-TTRANSFORM, and the DEPLETTR-CM trial were applied, and the baseline characteristics of the hypothetical trial cohorts as well as their survival were compared. 353 patients (80.3 years, IQR: 75.5–84.2, 17.6% female) were included, and 192 (57.8%) patients would have been eligible to participate in ATTRIBUTE, ATTRACT would have recruited 163 (49.1%) patients, HELIOS-B 105 (31.6%) patients, CARDIO-TTRANSFORM 80 (24.1%) subjects, and 71 (21.4%) patients would have been eligible for DEPLETTR-CM. Hypothetical ATTRIBUTE, ATTRACT, HELIOS-B, and CARDIO-TTRANSFORM patients demonstrated only minor differences regarding baseline characteristics, both among each other and compared to the real-world cohort. However, DEPLETTR-CM eligible patients exhibited more severely elevated biomarkers of heart failure (NT-proBNP: 2590pg/mL, IQR: 1614–4423, vs. 2339pg/mL, IQR: 1154–4250; p  < 0.001) and more advanced National Amyloidosis Centre stage ( p  < 0.001). Patients who could have been included in DEPLETTR-CM also showed significantly worse survival. Applied to our real-world ATTR-CM cohort, RCT inclusion and exclusion criteria would have selected patients comparable to the real-world cohort. Only the DEPLETTR-CM trial would have selected patients with more advanced disease and worse prognosis.

BackgroundPericardial and pleural effusion are common findings in patients with cardiac amyloidosis (CA). It is not known, whether effusions correlate with right ventricular (RV) function in these patients. Furthermore, data on the prognostic significance of pleural and pericardial effusion in CA is scarce.MethodsPatients with transthyretin (ATTR) and light chain (AL) CA were included in a clinical registry. All patients underwent transthoracic echocardiography at baseline. The presence of pericardial and pleural effusion was determined in every patient. The clinical endpoint was defined as cardiac death or heart failure hospitalization.ResultsIn total, 143 patients were analysed. Of these, 85 patients were diagnosed with ATTR and 58 patients with AL. Twenty-four patients presented with isolated pericardial effusion and 35 with isolated pleural effusion. In 19 patients, both pericardial and pleural effusion were found and in 65 patients no effusion was present at baseline. The presence of pleural effusion correlated well with poor RV function, measured by global RV free-wall strain (p = 0.007) in patients with AL, but not in ATTR. No such correlation could be found for pericardial effusion in either amyloidosis subtype. Patients with AL presenting with pleural effusion had worse outcomes compared to patients with pericardial effusion alone or no effusion at baseline. In the ATTR group, there was no difference in outcomes according to presence and type of effusion.ConclusionMore than 50% of patients with CA presented with pleural and/or pericardial effusions. While pleural effusion was clearly associated with poor RV function in AL, we were not able to detect this association with pericardial effusion.

Diastolic dysfunction of the left ventricle is common but frequently under-diagnosed. Particularly in advanced stages affected patients may present with significant functional tricuspid regurgitation (TR) as the most prominent sign on echocardiography. The underlying left ventricular pathology may eventually be missed and symptoms of heart failure are attributed to TR, with respective therapeutic consequences. The aim of the present study was to determine prevalence and mechanisms underlying TR evolution in heart failure with preserved ejection fraction (HFpEF). Consecutive HFpEF patients were enrolled in this prospective, observational study. Confirmatory diagnostic tests including echocardiography and invasive hemodynamic assessments were performed. Of the 175 patients registered between 2010 and 2014, 51% had significant (moderate or severe) TR without structural abnormalities of the tricuspid valve. Significant hemodynamic differences between patients with and without relevant TR were encountered. These included elevated pulmonary vascular resistance (p = 0.038), reduced pulmonary arterial compliance (PAC, p = 0.005), and elevated left ventricular filling pressures (p = 0.039) in the TR group. Multivariable binary logistic regression analysis revealed diastolic pulmonary artery pressure (p = 0.029) and PAC (p = 0.048) as independent determinants of TR. Patients were followed for 18.1±14.1 months, during which 32% had a cardiac event. While TR was associated with outcome in the univariable analysis, it failed to predict event-free survival in the multivariable model. The presence of ´isolated´ functional TR should prompt the suspicion of HFpEF. Our data show that significant TR is a marker of advanced HFpEF but neither an isolated entity nor independently associated with event-free survival.

Diffuse myocardial fibrosis is a key pathophysiologic feature in heart failure and can be quantified by cardiac magnetic resonance (CMR) T1 mapping. However, increases in myocardial free water also prolong native T1 times and may impact fibrosis quantification. Thus far, the impact of systemic patient volume status remains unclear. In this study, native T1 time by CMR was investigated in hemodialysis (HD) patients (n = 37) and compared with healthy controls (n = 35). Volume status was quantified by bioimpedance spectroscopy and correlated with CMR T1 time. While no differences between HD patients and controls were present with regard to age (p = 0.180), height (p = 0.535), weight (p = 0.559) and left ventricular (LV) ejection fraction (p = 0.273), cardiac size was significantly larger in HD patients (LV end-diastolic volume 164 ± 53 vs. 132 ± 26 ml, p = 0.002). Fluid overloaded HD patients had significantly longer native T1 times than normovolemic HD patients and healthy controls (1,042 ± 46 vs. 1,005 ± 49 vs. 998 ± 47 ms, p = 0.030). By regression analysis, T1 time was significantly associated with fluid status (r = 0.530, p = 0.009, post-HD fluid status). Our data strongly indicate that native CMR T1 time is significantly influenced by systemic volume status. As fluid overload is common in patients with cardiovascular diseases, this finding is important and requires further study.

Background: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiovascular disease and affects male patients more often than women. Prior studies, however, suggested that women are diagnosed later and at advanced stages of the disease, present with more pronounced symptoms, and experience worse outcomes. Objectives: To investigate sex-specific differences in clinical, laboratory, and comprehensive imaging characteristics in a contemporary cohort of HCM patients from a tertiary referral center in Austria. Methods: We retrospectively analyzed 321 HCM patients enrolled in a prospective registry (2018–2024). All patients underwent a comprehensive baseline evaluation, including medical history, laboratory assessment, transthoracic echocardiography, and cardiac magnetic resonance imaging. Results: At diagnosis, women were significantly older (62 vs. 53 years, p < 0.001) and presented with more advanced functional class (NYHA ≥ II: 80% vs. 49%, p < 0.001). Six-minute walking distance was lower and obstructive HCM was more prevalent in women (425 vs. 505 m, p < 0.001, and 55% vs. 32%, p < 0.001, respectively). Echocardiographic assessment revealed higher diastolic filling pressures (E/E′ 18 vs. 10, p < 0.001), larger indexed atrial volumes (29.5 vs. 26.6 mL/m2, p < 0.001), a higher left ventricular ejection fraction (70% vs. 62%, p < 0.001), and a larger indexed interventricular septal thickness in women (10.2 vs. 9.3 mm/m2, p = 0.004). Moreover, serum levels of NT-proBNP were significantly higher in women (760 vs. 338 pg/L, p < 0.001). Conclusions: Female patients with HCM were diagnosed at an older age, presented with more advanced symptoms, had higher rates of obstructive physiology, and a phenotype characterized by diastolic dysfunction and elevated biomarkers, closely resembling heart failure with preserved ejection fraction. Recognizing these sex-specific disparities is crucial in improving diagnostic awareness and individualized therapeutic management.

Background: Sodium–glucose cotransporter 2 inhibitors (SGLT2i) have been found to have potential hematopoietic effects in patients with heart failure (HF). However, these benefits have not been studied in patients with cardiac amyloidosis (CA). CA patients present with HF symptoms and often suffer from iron deficiency, which has a negative impact on erythropoiesis and leads to lower hemoglobin and hematocrit levels. We sought to determine the potential effects of SGLT2i on hematological parameters and functional capacity (FC) in CA patients. Methods: A prospective analysis was conducted to compare the effects of SGLT2i in patients who received the best medical therapy (BMT) along with SGLT2i (n = 20), versus patients receiving only BMT without SGLT2i (n = 20) (historical control group). All patients underwent blood testing and cardiopulmonary exercise testing (CPET) at baseline (BL) and after 6 months [interquartile range (IQR): 4.0 to 8.0]. Results: The SGLT2i-based therapy resulted in a significant improvement and difference in hematological parameters at 6 months follow-up compared to the control group. In the SGLT2i group, the mean hemoglobin level increased (+1.2 mg/dL), whereas in the control group, it decreased (–0.8 g/dL) (p < 0.001 for overall group comparison). The hematocrit showed a significant increase in the SGLT2i group (+4.4%) compared to a decrease in the control group (–1.8%) (p < 0.001). Additionally, the serum iron level improved in the SGLT2i-treated group (+ 5.5 [–5.0 to 17.5] μg/dL vs. –6.0 [–15.0 to 4.0] μg/dL, p = 0.121). Although there was no significant change in the peak oxygen consumption (peak VO2, (mL/min)/kg) (p = 0.206), as well as in pulmonary ventilation (VE)/carbon dioxide production (VCO2) slope in both groups (p = 0.964), the SGLT2i group maintained a peak VO2 and VE/VCO2 slope throughout the study. Conclusions: SGLT2i therapy improved hematological parameters and stabilized the FC of CA patients.

Background: Transthyretin amyloidosis is a multi-system disease that may manifest as cardiomyopathy (ATTR-CM) and/or polyneuropathy. Both disease manifestations are associated with autonomic dysfunction. However, the prevalence of autonomic dysfunction in ATTR-CM remains to be evaluated. Methods: Within the scope of a prospective ATTR-CM registry, the Composite Autonomic Symptom Score-31 (COMPASS-31) questionnaire was applied to consecutive patients between November 2022 and November 2024. Baseline characteristics are described, and associations of the COMPASS-31 score with markers of disease severity were assessed. Kaplan–Meier analysis was utilized to assess the COMPASS-31 score’s association with a combined endpoint of all-cause mortality and heart failure-related hospitalizations. Results: A total of 129 ATTR-CM patients [81.7 years (IQR: 77.4–84.3), 108 male (83.7%)] were included in the final study cohort. After stratification using the COMPASS-31 median [14 points, interquartile range (IQR): 6–29], statistically significant differences with regard to New York Heart Association (NYHA) stage and the Kansas City Cardiomyopathy Questionnaire (KCCQ) were observed. Furthermore, the COMPASS-31 score was moderately correlated with the KCCQ score in Spearman correlation analysis (r = −0.55, p < 0.001). The primary endpoint occurred in 16 patients (13 HF-hospitalizations/3 deaths) after 6.3 (IQR: 2.8–17.1) months. In Kaplan–Meier analysis, a COMPASS-31 score above the median of 14 was also associated with the primary endpoint of all-cause mortality and HF-related hospitalization (log-rank p = 0.047). Conclusions: Autonomic dysfunction is highly prevalent in ATTR-CM, affecting almost two-thirds of patients. As the presence of autonomic dysfunction is likely associated with more severely impaired quality of life, routine screening for this disease manifestation of transthyretin amyloidosis may be advisable.

Heart failure with preserved ejection fraction (HFpEF) is recognized as a major cause of cardiovascular morbidity and mortality. Thus, a profound understanding of the pathophysiologic changes in HFpEF is needed to identify risk factors and potential treatment targets in this specific patient population. Therefore, we aimed to comprehensively assess the impact of left- and right-ventricular function and hemodynamics on long-term mortality and morbidity in order to improve risk prediction in patients with HFpEF. We prospectively included 142 consecutive patients with HFpEF into our observational, non-interventional registry. Echocardiography, cardiac magnetic resonance imaging and invasive hemodynamic assessments including myocardial biopsy were performed at baseline. We detected significant correlations between left ventricular extracellular matrix and left ventricular end-diastolic diameter (r = -0.64;p = 0.03) and stroke volume (r = -0.53;p = 0.04). Hospitalization for heart failure and/or cardiac death was observed over a median follow up of 10 months. The strongest risk factors were reduced right ventricular function (adj. HR 6.62;95%CI 3.12- 14.02;p<0.001), systolic pulmonary arterial pressure (adj. HR per 1-SD 1.55;95%CI 1.15- 2.09;p = 0.004) and the pulmonary artery wedge pressure (adj. HR per 1-SD 1.51;95%CI 1.09-2.08; p = 0.012). The area under the ROC curve for right ventricular function was 0.63, for systolic pulmonary arterial pressure 0.75, and for pulmonary artery wedge pressure 0.68. The current study emphasizes the importance of right ventricular function and pulmonary pressures on outcome in patients with HFpEF providing pathophysiological insights into the hemodynamic changes in HFpEF.

: Cardiac amyloidosis (CA) as well as coronary artery disease (CAD) are both highly prevalent among the elderly. However, both the prevalence and risk factors associated with significant CAD among patients with CA, as well as potential outcome disparities, remain mainly unexplored. This study aimed to show the prevalence of CAD in patients with CA, as well as to assess outcomes and differences in late gadolinium enhancement (LGE) in comparison to patients with lone CA. : We retrospectively assessed CA patients who underwent CAD assessment between 2013 and 2023. The primary endpoint was all-cause death. A subgroup underwent cardiac magnetic resonance imaging (CMR) with LGE assessment. : Of 255 consecutive patients with CA, 81 patients had significant CAD. Differences could be found with respect to age, sex, arterial hypertension, and hyperlipidemia. Significant differences in CMR features could only be found with respect to indexed left-ventricular end-diastolic volume, as well as left-ventricular mass. CAD-specific LGE was present only in 17.7% of patients with CAD, while most patients showed typical amyloid LGE, making a viability diagnosis difficult via CMR, especially in patients with end-stage CA. No differences in outcomes could be observed according to the prevalence of CAD. : Concomitant obstructive CAD is highly prevalent among patients with CA. However, the presence of CAD does not influence patient outcomes. Furthermore, our data suggests that CAD viability testing by CMR might be complicated in patients with concomitant CA due to the high prevalence of amyloid-specific LGE.