Background: The one-minute sit-to-stand-test (1-min STST) is a quick, space saving test to evaluate functional capacity. Exercise testing plays an important role in the long-term follow-up of pulmonary hypertension (PH) patients and is currently evaluated using the six-minute-walk-test (6MWT). The aim of the study was to assess the convergent validity of the 1-min STST in patients with PH and its association with markers of PH severity.Methods: We evaluated 106 PH patients with the 1-min-STST and 6MWT and measured cardiorespiratory parameters (heart rate, blood pressure, oxygen saturation) before and after test conduction. N-terminal pro brain-type natriuretic peptide (NT-proBNP), WHO functional class (WHO-FC) and mean pulmonary artery pressure (mPAP) were defined as markers of PH severity.Results: Strong correlation was found between performances of 1-min STST and 6MWT (r = .711,p< .001), indicating convergent validity. Both tests were inversely associated with NT-proBNP (STST: r = -.405,p< .001; 6MWT: r = -.358,p< .001), WHO-FC (STST: r = -.591,p< .001; 6MWT: r = -.643,p< .001) and mPAP (STST: r = -.280,p< .001; 6MWT: r = -.250,p< .001). Significant changes in cardiorespiratory parameters were observed in both tests (allp< 0.001). Further the post-exercise cardiorespiratory parameters correlated strongly between the 1-min STST and 6MWT (all r ≥ .651, allp< .001).Conclusion: The 1-min STST demonstrated good convergent validity with the 6MWT and was associated with markers of PH severity. Furthermore, both exercise tests caused similar cardiorespiratory responses.

Objectives: In cardiac amyloidosis (CA) protein misfolding and consecutive storage into the extracellular myocardial compartment causes left ventricular hypertrophy and, in later stages of the disease, heart failure. The aim of this study was to compare extracellular volume (ECV) measurements obtained from photon-counting CT (PCCT) to the imaging reference cardiac magnetic resonance imaging (CMR) and to evaluate coronary artery disease (CAD) in a CA cohort. Materials and Methods: Thirty CA patients (mean age 77.5 +/− 7.9 years) underwent clinically indicated coronary CT angiography (CCTA) for the evaluation of CAD on a first-generation PCCT including a late-phase scan for assessment of ECV. ECV in PCCT was derived using 2 different techniques: (I) a single-energy (SE) technique, based on attenuation changes between the precontrast calcium scoring scan and delayed CCTA in the equilibrium phase (II) a dual-energy (DE) technique, based on iodine density maps from the delayed scan. Both methods were compared with CMR-derived ECV. Statistical analysis included repeated-measures analysis of variance (RM-ANOVA) with Bonferroni-adjusted pairwise comparisons. Correlations between methods were assessed using Pearson's correlation coefficient, and agreement was evaluated using Bland-Altman analysis. Results: CMR exhibited the highest mean ECV value (42.93 ± 10.14), followed by the SE method (42.5 ± 9.1), while the DE method yielded the lowest ECV values (40.7 ± 9.2). When compared with CMR, ECV obtained via the DE method was significantly lower (M _Diff = −2.24, P = 0.04). In contrast, no significant difference was observed between CMR and the SE method (M _Diff = 0.43, P = 1.00). Differences between the DE and SE methods were significant (M _Diff = −1.82, P < 0.001). Despite these differences, all 3 methods demonstrated excellent positive correlations. The strongest correlation was observed between the DE and SE methods (r = 0.98, P < 0.001), indicating high consistency in their measurements. Comparatively, the correlation between CMR and DE (r = 0.892, P < 0.001) was slightly stronger than that between CMR and SE methods (r = 0.882, P < 0.001). CAD was present in 29 (97.0%) CA patients with a mean Agatston score of 1086 ± 1398 (range 0–6848.5). Despite this high mean plaque burden and 14 (47.6%) patients presenting with atrial fibrillation, image quality was preserved in 29 (97.0%) patients with 17 (57.6%) of the patients having nonobstructive CAD. Conclusions: Compared to the imaging reference standard CMR, ECV derived from the DE and SE methods via PCCT demonstrated excellent positive correlations with CMR. The DE method exhibited minor differences compared to CMR, which were clinically not relevant. CAD with an extensive burden of calcified plaque was highly prevalent in CA; however, 57.6% of patients presented with nonobstructive CAD. Therefore, PCCT is a valuable tool for imaging both the coronary arteries and myocardial structure in CA.

Cardiac amyloidosis (CA) is associated with several distinct electrocardiographic (ECG) changes. However, the impact of amyloid depositions on ECG parameters is not well investigated. We therefore aimed to assess the correlation of amyloid burden with ECG and test the prognostic power of ECG findings on outcomes in patients with CA. Consecutive CA patients underwent ECG assessment and cardiac magnetic resonance imaging (CMR), including the quantification of extracellular volume (ECV) with T1 mapping. Moreover, seven patients underwent additional amyloid quantification using immunohistochemistry staining of endomyocardial biopsies. A total of 105 CA patients (wild-type transthyretin: 74.3%, variant transthyretin: 8.6%, light chain: 17.1%) were analyzed for this study. We detected correlations of total QRS voltage with histologically quantified amyloid burden (r = −0.780, p = 0.039) and ECV (r = −0.266, p = 0.006). In patients above the ECV median (43.9%), PR intervals were significantly longer (p = 0.016) and left anterior fascicular blocks were more prevalent (p = 0.025). In our survival analysis, neither Kaplan–Meier curves (p = 0.996) nor Cox regression analysis detected associations of QRS voltage with adverse patient outcomes (hazard ratio: 0.995, p = 0.265). The present study demonstrated that an increased amyloid burden is associated with lower voltages in CA patients. However, baseline ECG findings, including QRS voltage, were not associated with adverse outcomes.

Growing interest has accrued in the co-existence of cardiac amyloidosis and valvular heart disease. Amyloid infiltration from either transthyretin (ATTR) or of light chain (AL) origin may affect any structure of the heart, including the valves. The recent literature has mainly focused on aortic stenosis and cardiac amyloidosis, improving our understanding of the epidemiology, diagnosis, treatment and prognosis of this dual pathology. Despite being of high clinical relevance, data on mitral/tricuspid regurgitation and cardiac amyloidosis are rather scarce and mostly limited to case reports and small cases series. It is the aim of this review article to summarize the current evidence of concomitant valvular heart disease and cardiac amyloidosis by including studies on epidemiology, diagnostic approaches, screening possibilities, therapeutic management, and prognostic implications.

Background: The pathophysiology of heart failure (HF) with preserved ejection fraction (HFpEF) is suggested to be influenced by inflammation. Leukocyte indices, including the neutrophil–lymphocyte ratio (NLR), the monocyte–lymphocyte ratio (MLR), and the pan-immune inflammation value (PIV), can be utilized as biomarkers of systemic inflammation. Their prognostic utility is yet to be fully understood. Methods: Between December 2010 and May 2023, patients presenting to a tertiary referral center for HFpEF were included into a prospective registry. The association of the NLR, MLR, and PIV with the composite endpoint of all-cause mortality and HF-related hospitalization was tested utilizing Cox regression analysis. Results: In total, 479 patients (median 74.3, interquartile range (IQR): 69.22–78.3 years, 27.8% male) were included. Patients were observed for 43 (IQR: 11–70) months, during which a total of 267 (55.7%) patients met the primary endpoint. In a univariate Cox regression analysis, an above-the-median NLR implied a hazard ratio (HR) of 1.76 (95%-confidence interval (CI): 1.38–2.24, p < 0.001), an MLR of 1.46 (95%-CI: 1.14–1.86, p = 0.003), and a PIV of 1.67, 95%-CI: 1.30–2.13, p < 0.001) for the composite endpoint. After adjustment in a step-wise model, the NLR (HR: 1.81, 95%-CI: 1.22–2.69, p = 0.003), the MLR (HR: 1.57, 95%-CI: 1.06–2.34, p = 0.026), and the PIV (HR: 1.64, 95%-CI: 1.10–2.46, p = 0.015) remained significantly associated with the combined endpoint. Conclusions: The NLR, the MLR, and the PIV are simple biomarkers independently associated with outcomes in patients with HFpEF.

Purpose: Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with severely impaired health-related quality of life (HRQL). HRQL is an independent predictor of outcome in heart failure (HF), but data on patients with ATTR-CM is scarce. This study therefore aims to evaluate the association of HRQL with outcome in ATTR-CM. Methods: Patients from our prospective ATTR-CM registry were assessed using the Kansas City cardiomyopathy questionnaire (KCCQ), the Minnesota living with HF questionnaire (MLHFQ), and the EuroQol five dimensions questionnaire (EQ-5D). Cox regression analysis was utilised to assess the impact of HRQL on all-cause mortality. Results: 167 patients [80 years; interquartile range (IQR): 76–84; 80.8% male] were followed for a median of 27.6 (IQR: 9.7–41.8) months. The primary endpoint of all-cause mortality was met by 43 (25.7%) patients after a median period of 16.2 (IQR: 9.1–28.1) months. In a univariate Cox regression for mortality, a 10-point change in the KCCQ implied a hazard ratio (HR) of 0.815 [95%-confidence interval (CI): 0.725–0.916; p = 0.001], in the EQ-5D VAS of 0.764 (95%-CI: 0.656–0.889; p < 0.001), and 1.163 (95%-CI: 1.114–1.433; p < 0.001) in the MLHFQ. After adjustment for established biomarkers of HF, all-cause mortality was predicted independently by the EQ-5D VAS (HR: 0.8; 95%-CI: 0.649–0.986; p = 0.037; per 10 points) and the MLHFQ (HR: 1.228; 95%-CI: 1.035–1.458; p = 0.019; per 10 points). Conclusion: HRQL is a predictor of outcome in ATTR-CM. The EQ-5D VAS and the MLHFQ predict survival independent of biomarkers of HF.