Background/Objectives: A comprehensive and up-to-date review on cardiovascular disease (CVD) risk in patients with COPD is needed. Therefore, we aimed to systematically review the risk of a range of CVD in patients with COPD. Methods: We searched three databases (Pubmed, Web of Science, SCOPUS) from inception to September 2023 using terms related to COPD and CVD. Observational studies were included if they (1) were conducted in adults with a diagnosis of COPD based on the GOLD criteria, spirometry, physician diagnosis, or review of electronic health records; (2) reported the risk of CVD, namely of myocardial infarction (MI), ischaemic heart disease (IHD), atrial fibrillation (AF), heart failure, cerebrovascular disease, pulmonary hypertension, and peripheral vascular disease, compared with a control population using a measure of risk. A narrative synthesis was used. Results: Twenty-four studies from 2015 to 2023, mainly from Europe (n = 17), were included. A total of 3,485,392 patients with COPD (43.5–76.0% male; 63.9–73.5 yrs) and 31,480,333 (40.0–55.4% male, 49.3–70.0 yrs) controls were included. A higher risk of CVD in patients with COPD was evident regarding overall CVD, MI, IHD, heart failure, and angina. Higher risks of arrhythmia and AF, stroke, sudden cardiac death/arrest, pulmonary embolism, pulmonary hypertension, and peripheral vascular disease were also found, although based on a small amount of evidence. Conclusions: Patients with COPD have a higher risk of CVD than the general population or matched controls. This review underscores the need for vigilant and close monitoring of cardiovascular risk in individuals with COPD to inform more precise preventive strategies and targeted interventions to enhance their overall management.

Background: Carpal tunnel syndrome (CTS) has emerged as an early indicator of cardiac amyloidosis (CA) caused by transthyretin-associated (ATTR) mutations, possibly linked to adverse cardiovascular outcomes. This case series examines the relationship between idiopathic CTS and CA imaging diagnosis. Methods: Twenty-two patients from the cross-sectional study CarPoS (NCT05409833) were included. These patients underwent physical evaluation, laboratory exams, electrocardiography, echocardiography, cardiac magnetic resonance (CMR) imaging, and scintigraphy with ^99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid. Results: Four of the twenty-two patients included had ATTR cardiomyopathy. These patients presented left-ventricle hypertrophy and signs of infiltrative cardiomyopathy in echocardiograms and late gadolinium enhancement in CMR images without having any cardiovascular symptoms. Conclusions: Our findings suggest a high prevalence of CA in patients with bilateral idiopathic CTS, highlighting the importance of screening for CA in patients with CTS. Early detection could significantly impact patient prognosis, underscoring the need for further research into diagnostic and therapeutic strategies.

Background/Objectives: Fabry disease (FD) is a rare X-linked disease caused by the deficient activity of the enzyme α-galactosidase A. Cardiac involvement is particularly critical, often determining the disease prognosis. Epidemiological data on FD in Portugal are limited and inconsistent, highlighting the need for targeted screening. The F-CHECK study aimed to determine the prevalence of FD through the systematic screening of a Portuguese cohort of patients with unexplained cardiomyopathies. Methods: This multicenter observational study (NCT05409846) assessed the prevalence and clinical characteristics of FD in a Portuguese cohort (n = 409) of patients from 10 central hospitals who presented with unexplained cardiomyopathies, including idiopathic hypertrophic cardiomyopathy (HCM), left ventricular hypertrophy, dilated-phase HCM, and dilated cardiomyopathy with late gadolinium enhancement in the inferolateral segment. Screening was performed using dried blood spot assays to measure α-galactosidase A activity and/or by GLA gene sequencing in whole-blood samples. Results: FD was diagnosed in 14 patients, corresponding to a prevalence of 3.4%. FD diagnosis was significantly associated with systemic manifestations such as acroparesthesias (p = 0.027) and angiokeratomas (p = 0.003), as well as an increased risk of prior arrhythmic events (p = 0.021) and cerebrovascular disease (p = 0.016). Most FD patients (57%) presented a non-founder mutation in the GLA gene; however, they were pathogenically relevant. Conclusions: The observed 3.4% prevalence highlights the importance of systematic FD screening among Portuguese patients with unexplained cardiomyopathy, extending beyond classic hypertrophic presentations to dilated forms. Specific clinical signs, electrocardiogram findings, and cardiac imaging features can serve as valuable indicators to guide targeted genetic testing for FD.

Fabry disease (FD) is characterized by hypertrophic cardiomyopathy and early diagnosis is essential, considering the response to enzyme replacement or chaperone therapies. Recently, a new clinical staging for Fabry cardiomyopathy suggested five stages (0A, 0B, IA, IB, II, III), where the imaging characterization of hypertrophy and fibrosis are critical key items. Our study aimed to evaluate the applicability of the recent proposal of Meucci et al. 2024 in patients with FD, considering the last imaging cardiac evaluations. This retrospective observational study collected clinical data from patients diagnosed with FD. Staging system followed a hierarchical flowchart, staging patients based on LVEF, LGE, LV wall thickness, and other cardiac findings (symptoms, ECG/Echo/CMR abnormalities). We included 53 FD patients. As cardiac magnetic resonance and echocardiogram parameters are essential to complete staging, the algorithm was fully applied to 44 patients. Among these patients, the majority (29,5%) were in the non-hypertrophic stage 0. In hypertrophic stage I, 27,2% of patients were classified according to the maximum wall thickness. The hypertrophic fibrotic stage II included 29,5% of the patients. Patients in stage III (13,6%) presented diffuse myocardial fibrosis ≥ 3 LV segments and/or impaired systolic function (LVEF < 50%). The staging scheme allows us to classify different stages of FD cardiomyopathy. Most patients were in early disease stages, probably related to the diagnosis in the context of familial screening. The stepwise algorithm proposal helped apply the staging in clinical practice; however, it must be validated in more extensive and prospective studies.

Myocardial fibrosis is a major pathological component of cardiac remodelling and contributes to ventricular stiffness, impaired myocardial function, heart failure progression, and arrhythmic vulnerability across a broad spectrum of cardiomyopathies. In hypertrophic cardiomyopathy (HCM), fibrotic remodelling encompasses both diffuse interstitial matrix expansion and focal replacement fibrosis and is closely associated with disease severity and adverse clinical outcomes. Cardiac magnetic resonance (CMR), using late gadolinium enhancement (LGE) and quantitative parametric mapping, is central to non-invasive myocardial tissue characterization. LGE primarily identifies focal areas of replacement fibrosis and scar, whereas native T1, T2, and extracellular volume (ECV) mapping provide complementary information on diffuse myocardial and interstitial tissue abnormalities, including extracellular matrix expansion. However, these techniques characterize the structural and tissue-level consequences of myocardial injury and remodelling rather than directly visualizing the cellular processes underlying fibroblast activation. Fibroblast activation protein inhibitor positron emission tomography (FAPI PET) has emerged as a promising molecular imaging approach for assessing active fibroblast-mediated tissue remodelling. By targeting fibroblast activation protein (FAP), a cell-surface serine protease upregulated in activated fibroblasts and myofibroblasts. FAPI radiotracers may provide a molecular readout of accessible FAP expression that complements structural and tissue characterization by CMR. This review examines the cellular and molecular mechanisms underlying active myocardial remodelling, with particular emphasis on transforming growth factor-β1 (TGF-β1)/Smad signalling, fibroblast-to-myofibroblast transition, FAP expression, and extracellular matrix turnover. We critically evaluate the current clinical evidence for FAPI PET/CT and hybrid PET/CMR across cardiomyopathies, focusing on HCM while also considering ischemic, inflammatory, infiltrative, and other non-ischemic forms of cardiac remodelling. Particular attention is given to the relationship between myocardial FAPI uptake, CMR tissue characterization, ventricular function, circulating biomarkers, and calculated arrhythmic risk. Although initial studies support the complementary value of FAPI PET and CMR for characterizing active and established myocardial remodelling, the available evidence remains limited by small, predominantly single-centre cohorts, heterogeneous radiotracers and acquisition protocols, and the absence of validated quantitative thresholds or prospective outcome data. Larger longitudinal studies are required to determine whether FAPI PET can provide incremental prognostic information or serve as a reproducible imaging biomarker for disease progression and therapeutic response.