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Aim: Persistent symptoms despite guideline‐directed medical therapy (GDMT) and poor tolerance of GDMT are hallmarks of patients with advanced heart failure (HF) with reduced ejection fraction (HFrEF). However, real‐world data on GDMT use, dose, and prognostic implications are lacking. Methods and results: We included 699 consecutive patients with HFrEF and at least one 'I NEED HELP' marker for advanced HF enrolled in a multicentre registry. Beta‐blockers (BB) were administered to 574 (82%) patients, angiotensin‐converting enzyme inhibitors, angiotensin receptor blockers or angiotensin receptor–neprilysin inhibitors (ACEi/ARB/ARNI) were administered to 381 (55%) patients and 416 (60%) received mineralocorticoid receptor antagonists (MRA). Overall, ≥50% of target doses were reached in 41%, 22%, and 56% of the patients on BB, ACEi/ARB/ARNI and MRA, respectively. Hypotension, bradycardia, kidney dysfunction and hyperkalaemia were the main causes of underprescription and/or underdosing, but up to a half of the patients did not receive target doses for unknown causes (51%, 41%, and 55% for BB, ACEi/ARB/ARNI and MRA, respectively). The proportions of patients receiving BB and ACEi/ARB/ARNI were lower among those fulfilling the 2018 HFA‐ESC criteria for advanced HF. Treatment with BB and ACEi/ARB/ARNI were associated with a lower risk of death or HF hospitalizations (adjusted hazard ratio [HR] 0.63, 95% confidence interval [CI] 0.48–0.84, and HR 0.74, 95% CI 0.58–0.95, respectively). Conclusions: In a large, real‐world, contemporary cohort of patients with severe HFrEF, with at least one marker for advanced HF, prescription and uptitration of GDMT remained limited. A significant proportion of patients were undertreated due to unknown reasons suggesting a potential role of clinical inertia either by the prescribing healthcare professional or by the patient. Treatment with BB and ACEi/ARB/ARNI was associated with lower mortality/morbidity. [ABSTRACT FROM AUTHOR]

Aims: The HFA‐PEFF and H2FPEF scores have been developed to diagnose heart failure with preserved ejection fraction (HFpEF), and hold prognostic value. Their value in patients with HFpEF caused by cardiac amyloidosis (CA) has never been investigated. Methods and results: We evaluated the diagnostic and prognostic value of the HFA‐PEFF and H2FPEF scores in 304 patients from three cohorts with HFpEF caused by transthyretin CA (n = 160, 53%) or immunoglobulin light‐chain CA (n = 144, 47%). A diagnosis of HFpEF was more likely using the HFA‐PEFF score with 2 (1%), 71 (23%), and 231 (76%) patients ranked as having a low (0–1), intermediate (2–4), or high (5, 6) probability of HFpEF, respectively. Conversely, 36 (12%), 179 (59%) and 89 (29%) of patients ranked as having a low (0–1), intermediate (2–5), or high (6–9) probability of HFpEF using the H2FPEF score. During a median follow‐up of 19 months (interquartile range 8–40), 132 (43%) patients died. The HFA‐PEFF score, but not the H2FPEF score, predicted a high risk of all‐cause death which remained significant after adjustment for age, AL‐CA diagnosis, high‐sensitivity troponin T, N‐terminal pro‐B‐type natriuretic peptide, and echocardiographic parameters, including left ventricular global longitudinal strain, left ventricular diastolic function and right ventricular function (hazard ratio 1.51, 95% confidence interval 1.16–1.95, p = 0.002 for every 1‐point increase in HFA‐PEFF). Conclusions: The HFA‐PEFF score has a higher diagnostic utility in HFpEF caused by CA and holds independent prognostic value for all‐cause mortality, while the H2FPEF score does not. [ABSTRACT FROM AUTHOR]

Aims Methods and results Conclusion Several scores were developed to help the diagnosis of cardiac amyloidosis (CA). The most recent one, being the Mayo transthyretin amyloidosis cardiomyopathy (ATTR‐CM) score, was not externally validated. We compared the diagnostic performance of the ATTR‐CM score with previous tools (increased wall thickness [IWT] score, AMYLoidosis Index [AMYLI] score, and cardiac biomarkers) in a cohort of patients evaluated for a suspicion of CA.We analysed 362 consecutive patients referred to a third‐level centre for suspected CA. Overall, 132 (36%) had transthyretin CA (ATTR‐CA), and 91 (25%) immunoglobulin light chain CA (AL‐CA); CA was excluded in 139 (38%). ATTR‐CM score had a good diagnostic performance to distinguish ATTR‐CA from AL‐CA or no CA, with an area under the curve (AUC) of 0.795 (95% confidence interval [CI] 0.747–0.842, p < 0.001), and ATTR‐CA from no CA (AUC 0.822, 95% CI 0.774–0.871, p < 0.001). Results were consistent in both patients with preserved (AUC 0.787, 95% CI 0.726–0.848, p < 0.001), and reduced or mildly reduced ejection fraction (AUC 0.790, 95% CI 0.709–0.871, p < 0.001). The ATTR‐CM score showed a better discrimination compared to IWT and AMYLI score to distinguish ATTR‐CA from AL‐CA or no CA (p = 0.002), but not to distinguish ATTR‐CA from no CA (p = 0.270). Diagnostic accuracy was significantly higher for the ATTR‐CM score as compared to the rule‐in cut‐off of high‐sensitivity troponin T.The Mayo ATTR‐CM score has a good performance in identifying patients with ATTR‐CA, with also better discrimination power when compared to other scores and biomarkers. [ABSTRACT FROM AUTHOR]

Aims: The HFA-PEFF and H 2 FPEF scores have been developed to diagnose heart failure with preserved ejection fraction (HFpEF), and hold prognostic value. Their value in patients with HFpEF caused by cardiac amyloidosis (CA) has never been investigated., Methods and Results: We evaluated the diagnostic and prognostic value of the HFA-PEFF and H 2 FPEF scores in 304 patients from three cohorts with HFpEF caused by transthyretin CA (n = 160, 53%) or immunoglobulin light-chain CA (n = 144, 47%). A diagnosis of HFpEF was more likely using the HFA-PEFF score with 2 (1%), 71 (23%), and 231 (76%) patients ranked as having a low (0-1), intermediate (2-4), or high (5, 6) probability of HFpEF, respectively. Conversely, 36 (12%), 179 (59%) and 89 (29%) of patients ranked as having a low (0-1), intermediate (2-5), or high (6-9) probability of HFpEF using the H 2 FPEF score. During a median follow-up of 19 months (interquartile range 8-40), 132 (43%) patients died. The HFA-PEFF score, but not the H 2 FPEF score, predicted a high risk of all-cause death which remained significant after adjustment for age, AL-CA diagnosis, high-sensitivity troponin T, N-terminal pro-B-type natriuretic peptide, and echocardiographic parameters, including left ventricular global longitudinal strain, left ventricular diastolic function and right ventricular function (hazard ratio 1.51, 95% confidence interval 1.16-1.95, p = 0.002 for every 1-point increase in HFA-PEFF)., Conclusions: The HFA-PEFF score has a higher diagnostic utility in HFpEF caused by CA and holds independent prognostic value for all-cause mortality, while the H 2 FPEF score does not., (© 2022 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)