Search Results (30)

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. Full article

Background: Cardiac contractility modulation (CCM) is an established therapy for patients with heart failure with a reduced ejection fraction (HFrEF) who are still symptomatic despite guideline-directed medical therapy. It has been described previously that CCM leads to both an improvement of heart failure symptoms as well as of the parameters of left ventricular (LV) function, including LVEF and global longitudinal strain (GLS). However, so far there are no reports describing the effects of CCM on right ventricular (RV) or left atrial (LA) function, respectively. This might be of particular interest as RV global strain (RV GS) and LA strain are important prognostic parameters in heart failure. Methods: Adult patients with heart failure with reduced left ventricular function (LVEF <45%) and a QRS complex <130 ms despite guideline-directed medical therapy and with an indication for CCM were eligible for inclusion into this study. Patients receive a follow-up examination every 3 months, including a standardized echocardiographic examination with a special focus on strain analysis. While the effects of CCM on LV global longitudinal strain have been described before, this analysis reports the findings on the RV and LA strain. Results: Between 30.12.2021 and 10.09.2024, 22 patients were prospectively included in the study. CCM implantation was performed in 19 patients. Under active CCM therapy, there was an improvement in right ventricular global strain (CCM: −13.7 ± 4.5 vs. no CCM: −10.1 ± 5.0; p < 0.05), free wall strain (CCM: −14.6 ± 7.3 vs. no CCM: −10.3 ± 10.2; p < 0.05), left atrium strain rate (CCM: 19.7 ± 1.0 vs. no CCM: 15.3 ± 10.2; p < 0.05), and left atrium strain contraction (CCM: −11.5 ± 7.0 vs. no CCM: −7.1 ± 8.5; p < 0.05), whereas there was no difference in left atrium strain conduit (CCM: −9.0 ± 5.0 vs. no CCM: −8.1 ± 5.4; n.s.). To determine which of these parameters are linked to an improvement of quality of life, as seen in the Kansas City Heart Failure Questionnaire (KCCQ), a regression analysis was performed. It turned out that only the parameters of left atrial (LA) strain (LAS_R and LAS_CT) were significantly associated with improved quality of life, while other echocardiographic parameters, such as LV-EF, LV-GLS, and RV-GS, showed no clear association. Conclusions: CCM therapy is not only associated with improvements of left ventricular function but also restores right ventricular and left atrial strain in patients with HFrEF. Regarding the improvement in quality of life, the increase of LA strain seems to be of special importance. Full article

Background: Sleep-disordered breathing (SDB), particularly Cheyne–Stokes respiration (CSR), is highly prevalent among patients hospitalized with acute decompensated heart failure (ADHF) and is associated with worse clinical outcomes. Sodium-glucose cotransporter-2 inhibitors (SGLT2i) have demonstrated cardiorenal benefits in heart failure, but their effects on nocturnal respiratory parameters remain underexplored. Objectives: This study aims to evaluate the impact of SGLT2i therapy on key respiratory and cardiac indices including CSR burden, oxygenation, and right heart function in patients with ADHF and reduced left ventricular ejection fraction. Methods: In this single-center prospective cohort study, 60 patients with ADHF, LVEF < 40%, and a baseline apnea–hypopnea index (AHI) > 5 were assessed before and three months after the initiation of SGLT2i therapy. Sleep respiratory parameters were measured using home polygraphy (ApneaLink^TM), while cardiac and renal indices were evaluated by echocardiography, NT-proBNP, and the estimated glomerular filtration rate (eGFR). Structural and functional echocardiographic changes were analyzed both at baseline and following the 3-month treatment period. Patient-reported outcomes were assessed using the Epworth Sleepiness Scale (ESS) and Kansas City Cardiomyopathy Questionnaire (KCCQ). Results: After 3 months of SGLT2i therapy, significant improvements were observed in daytime sleepiness (ESS: −2.68 points; p < 0.001), CSR index (−5.63 events/h; p < 0.001), AHI (−3.07 events/h; p < 0.001), ODI (−6.11 events/h; p < 0.001), and mean nocturnal SpO₂ (+1.95%; p < 0.001). KCCQ scores increased by 9.16 points (p < 0.001), indicating improved quality of life. Cardiac assessments revealed reductions in NT-proBNP (−329.6 pg/mL; p < 0.001) and E/e′ ratio (−1.08; p < 0.001), with no significant change in LVEF or chamber dimensions. Right ventricular function improved, as evidenced by the increased TAPSE/sPAP ratio (+0.018; p < 0.001). Renal function remained stable, with a non-significant upward trend in eGFR. Conclusions: This exploratory study suggests that SGLT2 inhibitors may be associated with the attenuation of Cheyne–Stokes respiration and an improvement in right heart function in patients with ADHF, warranting further investigation in controlled trials. These findings highlight the potential of SGLT2is to address overlapping cardio-respiratory dysfunction in this high-risk population. Full article

Background/Objectives: Cognitive impairment is a frequent and underrecognized comorbidity in elderly patients with heart failure with preserved ejection fraction (HFpEF), contributing to poor outcomes and complicating disease management. This study aimed to identify risk factors associated with cognitive impairment in elderly HFpEF patients from Western Romania and to develop a point-based risk score for clinical use. Methods: We conducted a cross-sectional analysis of HFpEF patients aged ≥65 years. Cognitive status was assessed using the Mini-Mental State Examination-2 (MMSE-2), with significant impairment defined as a score <24. Multivariable logistic regression analysis was performed to identify independent predictors of cognitive dysfunction. Results: A total of 326 HFpEF patients were included. Diabetes mellitus, prior stroke or transient ischemic attack (TIA), carotid artery disease, elevated N-terminal pro–B-type natriuretic peptide (NT-proBNP), and reduced estimated glomerular filtration rate (eGFR) were independently associated with cognitive impairment. Higher Kansas City Cardiomyopathy Questionnaire (12-KCCQ) scores and anticoagulant therapy for atrial fibrillation were associated with a lower risk. Based on these variables, a simple point-based cognitive risk score was developed, demonstrating strong discriminatory ability (area under the curve = 0.84). A threshold of ≥2 points identified cognitive impairment with 75% sensitivity and 83% specificity. Conclusions: Our findings underscore the importance of integrated cardiovascular and cognitive assessment in elderly HFpEF patients. The developed risk score offers a pragmatic tool for the early identification of cognitive dysfunction, potentially informing timely interventions and preventive strategies. Full article

Background: Managing acute decompensated heart failure (ADHF) is complex, particularly when combined with comorbidities like sleep apnea. Effective treatment requires personalized approaches, focusing on quality of life (QoL) and mental health outcomes. Purpose: This study explored the prevalence and characteristics of sleep apnea in patients with obesity and AHF exacerbations. It assessed how different sleep apnea phenotypes impact QoL and mental health, applying personalized medicine strategies. Methods: A prospective cohort study was conducted on 150 patients admitted for AHF exacerbation. Inclusion criteria included an Apnea–Hypopnea Index (AHI) > 5, an Epworth Sleepiness Scale (ESS) > 8, NT-proBNP > 900 pg/mL and informed consent obtained prior to participation. Optimized medical treatment was provided. QoL and mental health were evaluated using the Kansas City Cardiomyopathy Questionnaire (KCCQ) and the Beck Depression Inventory (BDI). Results: Among 81 patients with sleep apnea, 73% (n = 59) had obstructive sleep apnea (OSA) and 27% (n = 19) had central sleep apnea (CSA). OSA patients reported a higher QoL (61.12 ± 17.88) compared to CSA patients (37.18 ± 19.98, p < 0.001). CSA patients exhibited more severe depression (BDI: 26.18 ± 5.5 vs. 16.64 ± 4.1, p < 0.001). Significant correlations were noted between KCCQ and BDI scores (r = −0.849, p < 0.001) and central apnea events (r = −0.485, p < 0.001). Conclusions: Sleep apnea is common in ADHF patients, with CSA being linked to poorer QoL and greater depression. Personalized medicine offers promising strategies to enhance care and outcomes. Full article

Background: Cardiac contractility modulation (CCM) is a therapy for patients with chronic heart failure with reduced ejection fraction (HFrEF). However, so far, there is no data available as to whether the application of CCM leads to changes in left ventricular global strain (GLS). This might be of special interest because GLS is known to be a strong predictor of outcomes in patients with HFrEF. Methods: Patients over 18 years old with heart failure with impaired left ventricular function (LVEF < 45%), a QRS complex < 130 ms, and NYHA classes II–IV despite guideline-directed medical therapy who planned to receive CCM implantation within 6 months were prospectively included into this study. Every 3 months, the status regarding CCM therapy was determined, and a standardized echocardiographic examination including the determination of LV global longitudinal strain was performed. Results: Between 30.12.2021 and 10.09.2024, 22 consecutive patients were prospectively enrolled in the study. CCM implantation was performed for 19 patients at a mean time of 59 ± 65 days. Under active CCM therapy, there was an improvement in GLS, LV-EF, and the Kansas City Heart Failure Questionnaire (KCCQ; all p < 0.05). A linear regression analysis showed that the positive effect of CCM on GLS was especially pronounced in patients with a female sex, a non-ischemic etiology of heart failure, and age ≤ 69 years, respectively (all p < 0.05). Conclusions: CCM therapy is not only linked to an improvement in LV-EF but also increases the global longitudinal strain and quality of life of patients with HFrEF. Full article

Background: Mitral regurgitation (MR) is a common valvular disorder linked to high morbidity and mortality. For patients unsuitable for surgery, transcatheter mitral edge-to-edge repair (TEER) with the MitraClip^® G4 system offers an alternative. This study aims to evaluate procedural, echocardiographic, functional, and quality of life (QoL) outcomes in patients who underwent TEER with the MitraClip^® G4 system, along with possible predictors of New York Heart Association (NYHA) class I at 30 days and at 1 year. Methods: Patients with moderate-to-severe (3+) or severe (4+) degenerative MR (DMR) or functional MR (FMR), classified as NYHA class III or IV, and who underwent TEER with the MitraClip^® G4 system at our center between January 2021 and December 2023 were included. Results: A total of 83 patients [71% FMR, 66% male, median (IQR) age 70 (11) years] underwent TEER, with 100% procedural success. MR ≤ 2+ was achieved in 100% and 98% of patients at 30 days and 1 year, respectively. NYHA class I or II was achieved in 100% and 96.8% of patients at 30 days and 1 year, respectively. The Kansas City Cardiomyopathy Questionnaire (KCCQ) score improved from 51 ± 20 at baseline to 69 ± 15 at 30 days (p < 0.001) and 70.5 ± 15 at 1 year (p < 0.001). Lower baseline N-terminal pro-brain natriuretic peptide (NT-proBNP) predicted achieving NYHA class I at 30 days (HR: 0.63, 95% CI: 0.41–0.95, p = 0.030), while lower European System for Cardiac Operative Risk Evaluation II (EuroSCORE II) and NT-proBNP predicted it at 1 year [(HR: 0.50, 95% CI: 0.28–0.89, p = 0.019), (HR: 0.67, 95% CI: 0.44–0.99, p = 0.049), respectively]. Conclusions: The MitraClip^® G4 system provides significant improvements in MR severity, functional class, and QoL. Lower NT-proBNP and EuroSCORE II were strong predictors of achieving optimal functional status (NYHA class I). Full article

Background: Iron deficiency (ID) often coexists with heart failure (HF), and its prevalence increases with the severity of HF. Intravenous ferric carboxymaltose (FCM) has been associated with improvements in clinical outcomes, functional capacity, and quality of life (QoL) in patients with HF and ID. However, while earlier studies showed favorable results, more recent studies have failed to demonstrate significant improvements in outcomes for patients with heart failure with reduced ejection fraction (HFrEF) and ID. This meta-analysis seeks to provide updated insights into the effectiveness and safety of FCM compared to placebo/standard of care (SoC) among patients with HFrEF and ID/iron deficiency anemia (IDA). Methods: We performed a systematic review and meta-analysis of the literature from inception to December 2023, utilizing databases such as MEDLINE (via PubMed), Google Scholar, the Cochrane Library, ClinicalTrials.gov, and the ScienceDirect portal. A statistical analysis was carried out using RevMan 5.4 with a random-effects model. Dichotomous outcomes were reported as odds ratios (OR), while continuous outcomes were presented as the weighted mean difference (WMD) with corresponding 95% confidence intervals (CI), and heterogeneity was assessed using the I² test. Results: The final analysis included data from six randomized controlled trials (RCTs), comprising 5132 patients. Our findings indicate a significant reduction in total HF hospitalizations among patients with HFrEF and ID/IDA treated with FCM compared to those receiving the placebo or SoC, with an OR of 0.59 (95% CI: 0.40 to 0.88, p < 0.010). However, no statistically significant difference was observed in the total number of deaths between the FCM and placebo/SoC groups (OR: 0.85; 95% CI: 0.70 to 1.03, p = 0.09), non-HF hospitalizations (OR: 0.71; 95% CI: 0.41 to 1.25, p = 0.24), or the composite outcome of cardiovascular hospitalizations and cardiovascular deaths (OR: 0.65; 95% CI: 0.40 to 1.04, p = 0.07). Regarding functional capacity, as assessed by the change in 6-min walk test (6MWT) distance, no significant improvement was found, with a weighted mean difference (WMD) of 14.03 (95% CI: −10.94 to 38.99, p = 0.27). QoL, measured by the Kansas City Cardiomyopathy Questionnaire (KCCQ) score, also did not show significant enhancement, with a WMD of 3.85 (95% CI: −0.55 to 8.24, p = 0.09). Furthermore, the safety analysis revealed no significant difference in the incidence of serious adverse events between the FCM and placebo/SoC groups, with an OR of 0.73 (95% CI: 0.49 to 1.10, p = 0.13). Conclusions: In patients with HFrEF and IDA, treatment with intravenous FCM significantly lowers the risk of total HF hospitalizations but does not appear to affect functional capacity, QoL, or mortality. Full article

Background and Objectives: Previous studies showed that, paradoxically, obese patients with heart failure (HF) have better clinical outcomes compared to overweight, normal, or underweight patients. Scientific societies emphasize the importance of integrating quality of life (QoL) assessment in cardiovascular care. However, the association between QoL and weight remains understudied. Given the significant correlation between HF survival and QoL, it is essential to assess how obesity impacts patient-reported outcomes in this clinical setting. Methods: This cross-sectional multicenter study in 1028 HF patients with reduced ejection fraction (HFrEF) aims to evaluate the association between obesity and QoL, and whether the obesity paradox holds for HF patients regarding QoL. Specific and generic QoL questionnaires were administered alongside clinical parameters like body mass index (BMI) and body adiposity estimator (BAE). Results: Obese compared to non-obese reported worse QoL. In the adjusted linear regression models, neither BMI nor obesity were associated with QoL. Generalized additive models confirmed a strong non-parametric association between BMI, subdomain scores from Kansas City Cardiomyopathy Questionnaire (KCCQ) (OSS p = 0.004, CSS p = 0.006, TSS p = 0.02), and summary measurements of EQ-5D (EQ-5D index p = 0.003, visual analogue scale (VAS) p = 0.01). In contrast, BAE showed a statistically significant linear relation among QoL (OSS p ≤ 0.001, CSS p ≤ 0.001, TSS p ≤ 0.001) and EQ-5D summary measurements (EQ-5D index p ≤ 0.001, VAS p ≤ 0.001). Conclusions: Overall, obese patients have worse QoL; therefore, obesity cannot be considered a protective factor in terms of QoL in established HF. Full article

Background: Cardiac resynchronization therapy (CRT) is an essential treatment for patients with symptomatic heart failure and ventricular conduction abnormalities. Low-ejection-fraction (EF) cardiomyopathy often involves a wide QRS complex displaying a left bundle branch block (LBBB) morphology and markedly delayed activation of the LV lateral wall. Following CRT, patients with heart failure and LBBB have better outcomes and quality-of-life improvements. Various electrocardiographic and clinical parameters are thought to be able to predict this improvement. The Kansas City Cardiomyopathy Questionnaire (KCCQ) is a reliable tool for measuring these patients’ quality of life. Methods: This is an observational prospective study featuring over 69 individuals diagnosed with cardiac failure and dilatative cardiomyopathy with low-EF and major LBBB. This study analyzed the correlations between patient outcomes and demographic, clinical, and electrocardiographic parameters. Results: Following the analysis, we observed correlations between the QRS area, intraprocedural systolic blood pressure, Q-LV interval, the R-wave amplitude in the right precordial leads and the CRT outcomes indicated by the KCCQ score. Conclusions: The parameters found and their correlation with the KCCQ score show how CRT therapy impacts patients’ quality of life, symptom burden, and functional status. Full article

Symptomatic aortic stenosis and frailty reduce health-related quality of life (HrQoL). Transcatheter aortic valve implantation (TAVI) in patients at high to extreme risk has been proven to have a beneficial effect on HrQoL. Currently, TAVI is also considered in patients at intermediate risk. Our meta-analysis investigates whether benefits to HrQoL after TAVI is more pronounced in frail patients and patients at high to extreme vs. intermediate surgical risk. A systematic search of the literature was performed in November 2021 and updated in November 2023 in PUBMED, EMBASE, and the Cochrane Controlled Trials Register. Statistical analysis was performed according to the inverse variance method and the random effects model. A total of 951 studies were assessed, of which 19 studies were included. Meta-analysis showed a mean increase in the Kansas City Cardiomyopathy Questionnaire (KCCQ) score of 29.6 points (6.0, 33.1) in high to extreme risk patients versus 21.0 (20.9, 21.1) in intermediate risk patients (p < 0.00001) and 24.6 points (21.5, 27.8) in frail patients versus 26.8 (20.2, 33.4) in the general TAVI population (p = 0.55). However, qualitative analyses of non-randomized studies showed the opposite results. In conclusion, TAVI improves HrQoL more in high to extreme than intermediate risk patients. Frailty’s impact on HrQoL post-TAVI is inconclusive due to varying outcomes in RCTs vs. non-RCTs. Full article

Background: Chronic heart failure (CHF) is a major health problem, representing the main cause of hospitalization in people over 65 years of age. Several studies have associated the Mediterranean diet with a cardioprotective function, improving prognoses in patients with high cardiovascular risk. Our main objective is to determine whether higher adherence to the Mediterranean diet is associated with a lower severity of CHF, based on the number of decompensations and disease complications. Methods: This study was a single-center retrospective cohort study conducted at the Virgen del Rocío Hospital (Seville). Adherence to a Mediterranean diet was determined by the Mediterranean Diet Adherence Screener (MEDAS) in patients with chronic heart failure in a state of clinical stability, the number of decompensations in the 12 months before inclusion, cardiac biomarkers (NT-proBNP and CA125), evaluation of dyspnea, and quality of life assessment according to NYHA and KCCQ scales and analytical profiles. Results: Seventy-two patients were included (35 with high adherence to the Mediterranean diet and 37 with low adherence). The mean age was 81.29 ± 0.86 years. A trend towards fewer decompensations (1.49 ± 0.14 vs. 1.92 ± 0.17, p = 0.054) and lower NT-proBNP values (2897.02 ± 617.16 vs. 5227.96 ± 1047.12; p = 0.088) was observed in patients with high adherence compared to those with low adherence to the Mediterranean diet. Conclusions: Our results suggest that patients with CHF and high adherence to the Mediterranean diet have a tendency towards an improved cardiac profile, indicated by fewer decompensations and lower NT-proBNP levels. Future clinical trials are needed to substantiate these hypotheses. Full article

Background: In recent years, many advances have been made in the treatment of hereditary transthyretin amyloidosis (ATTRv). Patisiran is a small-interfering RNA used to treat ATTRv with only polyneuropathy or polyneuropathy and cardiomyopathy. The aim of our study was to assess the effect of patisiran on cardiac function in ATTRv patients using speckle tracking echocardiography (STE) analysis. Methods: A single-center prospective study was performed enrolling 21 patients with ATTRv (11 M—52% of the population; 10 F—48% of the population; median age 66 ± 8.4 years old). A total of 7 patients had cardiac amyloidosis and polyneuropathy, and 14 patients had only polyneuropathy without cardiac involvement. Cardiological evaluation including electrocardiograms, echocardiography with STE, and assessment of myocardial work parameters was performed in all patients before starting patisiran and after 9–18 months. Functional capacity was assessed using the 6 min walk test; quality of life was assessed using the Kansas City Cardiomyopathy Questionnaire (KCCQ). Results: We did not find a significant difference in gender prevalence of ATTR amyloidosis in all of the population (p-value 0.79), but we found that cardiac amyloidosis significantly predominated in the male sex compared to patients with only neuropathy. In all patients, we found a slight improvement in functional capacity and quality of life. We did not find significant changes in left ventricular ejection fraction (LVEF), but we found a significant improvement in left ventricular global longitudinal strain (GLS), global work waste (GWW), and global work efficiency (GWE), especially in patients with cardiac amyloidosis; E/e’ average and left atrial stiffness also improved significantly in patients with cardiac amyloidosis. Conclusions: Our study confirms a positive effect of patisiran on cardiac function, particularly the absence of signs of subclinical deterioration as detected by very sensitive STE parameters such as GLS, MW, and atrial stiffness during follow up in patients treated with patisiran. Full article

Background: Patient reported outcomes (PROs) are important measures in acquired heart disease but have not been well defined in Adult Congenital Heart Disease (ACHD). Our aim was to explore the discriminatory capacity of PRO survey tools in Fontan circulatory failure (FCF). Methods: Consecutive adults were enrolled from our ambulatory clinics. Inclusion criteria were age ≥18 years, a Fontan circulation or a hemodynamically insignificant shunt lesion, and sufficient cognitive/language abilities to complete PROs. A comprehensive package of PRO measures, designed to assess perceived health-related quality of life (HRQOL) was administered (including the Kansas City Cardiomyopathy Questionnaire [KCCQ-12], EuroQol-5-dimension [EQ5D], Short Form Health Status Survey [SF-12], self-reported New York Heart Association [NYHA] Functional Class, and Specific Activity Scale [SAS]). Results: We compared 54 Fontan patients (35 ± 10 years) to 25 simple shunt lesion patients (34 ± 11 years). The KCCQ-12 score was lower in Fontan versus shunt lesion patients (87 [IQR 79, 95] versus 100 [IQR 97, 100], p-value < 0.001). The FCF subgroup was associated with lower KCCQ-12 scores as compared with the non-FCF subgroup (82 [IQR 56, 89] versus 93 [IQR 81, 98], p-value = 0.002). Although the KCCQ-12 had the best discriminatory capacity for determination of FCF of all PRO tools studied (c-statistic 0.75 [CI 0.62, 0.88]), superior FCF discrimination was achieved when the KCCQ-12 was combined with all PRO tools (c-statistic 0.82 [CI 0.71, 0.93]). Conclusions: The KCCQ-12 questionnaire demonstrated good discriminatory capacity for the identification of FCF, which was further improved through the addition of complementary PRO tools. Further research will establish the value of PRO tools to guide management strategies in ACHD. Full article

Background: This study aimed to evaluate the effect of treatment with tafamidis on clinical, laboratory, functional, and structural cardiovascular imaging parameters at the 12-month follow-up timepoint in patients with wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) and to assess the response to treatment in terms of disease progression. Methods: Patients with ATTRwt-CM undergoing treatment with tafamidis for >12 months were included. The patients underwent a comprehensive evaluation (including echocardiography, cardiac magnetic resonance imaging, six-minute walking test, assessment of quality of life, and laboratory tests) at baseline and the 12-month follow-up timepoint. Disease progression was assessed using a set of tools proposed by an international panel of experts, evaluating three main domains (clinical, biochemical, and structural). Results: The study cohort consisted of 25 patients (mean age of 75.9 ± 6.1 years, with 92% males). At the 12-month follow-up timepoint, an improvement in quality of life calculated with the KCCQ overall score (64 ± 20 vs. 75 ± 20, p = 0.002) and a reduction in pulmonary artery pressure (34 ± 10 mmHg vs. 30 ± 5 mmHg, p-value = 0.008) and in native T1 time were observed (1162 ± 66 ms vs. 1116 ± 52 ms, p-value = 0.001). Clinical, biochemical, and structural disease progression was observed in 6 (24%), 13 (52%), and 7 (28%) patients, respectively. Overall disease progression was observed in two patients (8%). Conclusions: This study described the impact of tafamidis treatment on clinical, laboratory, and functional parameters. Disease progression, assessed using a multiparametric tool recommended by a recent position paper of experts, was observed in a minority of patients. Full article