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.

Aims: Hypertrophic cardiomyopathy (HCM) is associated with functional limitations during exercise. We aimed to evaluate oxygen pulse (O2p) as a stroke volume (SV) surrogate and to propose a new HCM classification (RoMa) based on haemodynamic profiles during exercise: predicted peak O2p (O2pp) and peak heart rate (HRpp). Methods and results: This multicentre, prospective study included 90 clinically stable HCM patients who underwent cardiopulmonary exercise testing with simultaneous impedance cardiography (PhysioFlow®). We assessed the relationship between SV and O2p. Patients were stratified into four groups based on HRpp (≥80% predicted) and O2pp (≥100% predicted): RoMa I (high HRpp–high O2pp), RoMa II (high HRpp–low O2pp), RoMa III (low HRpp–high O2pp), and RoMa IV (low HRpp–low O2pp). Oxygen uptake (VO2), minute ventilation-to-carbon dioxide production (VE/VCO2) slope, SV, and mitral regurgitation (MR) were analysed. Patients (80% male, 53 [42–64] years) had preserved left ventricular ejection fraction (62 [58–68]%) and peakVO2 (23.1 ± 7.8 mL/min/kg = 81 ± 21% predicted). SV correlated with O2p (r = 0.48, P < 0.001; β = 3.59, P < 0.001). Resting moderate to severe MR was more prevalent in RoMa class IV (41%) vs. RoMa I (18%, P = 0.038). PeakVO2 declined across groups, from 29.7 ± 8.3 (RoMa I) to 16.2 ± 5.1 mL/min/kg (RoMa IV, P < 0.001). VE/VCO2 slope increased from 26.4 ± 4.5 (RoMa I) to 38.6 ± 6.0 (RoMa IV, P = 0.002). Peak SV decreased from 128.7 ± 24.8 (RoMa I) to 104.7 ± 28.0 mL (RoMa IV, P = 0.019), while rest to peak SV difference dropped from 38.6 (30.4–52.8) to 15.8 (8.2–27.9) mL (P = 0.002). Conclusion: SV and O2p are significantly related, and the RoMa classification effectively distinguished HCM patients. Key Findings: O2p is a reliable indicator of SV, helping to better understand exercise limitations in HCM patients. The newly introduced RoMa classification effectively distinguishes different levels of exercise impairment in HCM, linking poorer heart function to lower oxygen uptake and higher breathing inefficiency.

Background: Abdominal aortic aneurysm (AAA) is a multifactorial vascular disease associated with high morbidity and mortality. Currently, surgical intervention is the only treatment option, and there is no drug therapy available for AAA. Hence, surveillance of AAA until indication for surgery may impact patient quality of life (QoL). There is a paucity of high-quality observational data on health status and QoL, particularly among AAA patients participating in randomized controlled trials. The objective of this study was to compare the QoL scores of AAA patients on surveillance to those of AAA patients enrolled in the MetAAA trial. Material and methods: Overall, 54 MetAAA trial patients and 23 AAA patients under regular surveillance for small AAA (part of a longitudinal monitoring study) were asked to complete three established and validated (in total 561 longitudinally collected) QoL questionnaires: the 36-Item Short Form Health Survey (SF-36), the Aneurysm Symptom Rating Questionnaire (ASRQ), and the Aneurysm-Dependent Quality of Life questionnaire (ADQoL). Results: A superior health status and QoL was found in AAA patients participating in the MetAAA trial compared to AAA patients under regular surveillance. In detail, MetAAA trial patients showed superior general health perception (P=0.012), higher energy level (P=0.036) as well as enhanced emotional well-being (P=0.044) and fewer limitations due to general malaise (P=0.021), which was subsequently reflected in an overall superior current QoL score (P=0.039) compared to AAA patients under regular surveillance. Conclusion: AAA patients enrolled in the MetAAA trial showed superior health status and QoL compared to AAA patients under regular surveillance.