Active clinical trials for "Cardiomyopathies"

Amyloidoses are systemic or acquired disorders characterized by the deposition in the extracellular spaces of amyloid fibers formed by proteins codified by mutated genes or non-mutated but misfolded proteins. Cardiac involvement in amyloidosis is an important determinant of the clinical presentation and can be found in patients with amyloid light-chain (AL) or transthyretin (ATTR) amyloidosis, the latter due to the deposition of normal proteins (formerly known as senile amyloidosis) or mutated proteins. Cardiac amyloidosis (CA) has a poor prognosis that further worsens if the diagnosis and treatment are delayed. Nuclear medicine techniques have emerged as important tools for the diagnosis and characterization of CA. It has been recently demonstrated that cardiac uptake of bone tracers allows to identify the deposition of transthyretin in the heart, while it is not useful for the diagnosis of AL-CA, which currently requires the histological demonstration of amyloid fibers in a tissue sample taken with invasive procedures such as an endomyocardial biopsy. Recently, some PET tracers developed to identify beta-amyloid deposits in the brain proved able to detect an uptake even in the heart; nonetheless their possible use to diagnose CA is still debated. One of those tracers is florbetaben labelled with 18F, which displays a high binding affinity with beta-amyloid in the brain, while the experience on its use to identify extracranial amyloid deposits is still limited. Three studies have reported a cardiac uptake of 18F-florbetaben in AL or ATTR amyloidosis. Tracer uptake could be detected starting from 15 minutes after tracer administration. In a case series of 60 patients (20 with AL-CA, 20 with ATTR-CA and 20 with CA suspected but excluded) we demonstrated that the evidence of a myocardial uptake in a late acquisition can effectively discriminate AL- from ATTR-CA or other conditions. Indeed, patients with AL-CA displayed an intense and persistent myocardial uptake in static acquisitions at all time points, while patients with ATTR-CA and those without CA displayed a rapid reduction of the uptake after the early acquisition. This study aims to compare the performance of PET/CT with 18F-florbetaben to diagnose AL-CA compared with the current diagnostic standard, which requires a tissue biopsy. Primary objective: To define the agreement (with its 95% confidence interval) between two diagnostic approaches to the diagnosis of AL-CA in patients with a monoclonal protein: the traditional invasive approach and a non-invasive approach using the visual assessment of 18F-florbetaben PET/TC. Secondary objectives: To define the diagnostic performance of PET/CT with 18F-florbetaben (visual evaluation) in terms of sensitivity, specificity, positive and negative predictive value; To define cut-offs from myocardial uptake quantification to confirm or discard AL-CA among patients with suspected CA and a monoclonal protein, compared to the standard diagnostic algorithm, from quantitative uptake values; To assess the changes in the degree of myocardial 18F-florbetaben uptake over 12 months in patients with AL-CA; To assess the safety and tolerability of PET/CT with 18F-florbetaben in patients evaluated for suspected CA.

Cardiac amyloidosis is a major cause of early treatment-related death and poor overall survival in individuals with systemic light chain amyloidosis. This project will develop a novel approach to visualize cardiac amyloid deposits using advanced imaging methods. The long-term goal of this work is to identify the mechanisms of cardiac dysfunction, in order to guide the development of novel life-saving treatments.

In this study, patients with endocardial myocardial biopsy were selected to observe the safety of the operation, and pathological examination was performed. If necessary, special tests such as viral examination, mass spectrometry and molecular biology were performed to confirm the diagnosis, and follow-up was performed.

In the current project, primary health care patients with mental illness such as anxiety, depression, fatigue or sleep disorders will be followed. The study includes both health conversations with the health curve as a systematic work with lifestyle habits, and the biochemical risk marker copeptin with a focus on improved lifestyle habits and the development of cardiovascular complications. Participants will be followed up at 12 and 24 months with renewed health interview including the health curve and blood sampling. National registries will be used for a, up to 20 year long follow-up regarding cardiovascular complications and mortality.

Purpose：1. Preliminary evaluation of the preventive effect of DH001 on doxorubicin-induced cardiotoxicity in cancer patients 2.To explore appropriate dosages to provide basis for dosages in subsequent confirmatory studies 3.To evaluate the effect of DH001 on the efficacy of doxorubicin treatment in cancer patients 4.To evaluate the safety of DH001 in cancer patients treated with doxorubicin

This study will evaluate whether cardiac MRI T1 and T2 mapping improves our ability to detect early abnormalities in the heart in patients with Fabry disease and identify patients at increase risk of adverse events.

Current guidelines advocate that ARVC patients, typically young and active individuals with a significant history of competitive endurance sports, cease endurance training in favour of activities with low cardiac burden such as bowling and golf. Empirically, it is often suggested that heart rate during exercise should not exceed 100-120 bpm in these patients, but these guidelines are arbitrary and not scientifically based. In practice, it is estimated that up to 50% of patients do not comply with these recommendations . Adequate quantification of the arrhythmogenic burden, defined as premature ventricular beats in proportion to all heart beats in each period of time, and cardiac load (defined as stroke volume for volume load and systolic blood pressure for pressure load) experienced by ARVC patients when performing different types of physical exercise would be a first step towards designing a safe and effective intervention so that these patients can profit from an active life style. This study therefore aims to quantify and describe the arrhythmogenic burden and cardiac load experienced by patients with ARVC while performing different physical exercise over a range of intensities - all strictly within the range currently recommended by different cardiological societies.

LEAP-CAR will evaluate the benefit of left bundle branch area pacing (LBBAP), comparing to conventional right ventricular pacing (RVP), in preventing pacing-induced cardiomyopathy (PICM) in patients undergoing pacemaker implant for advanced (2° or 3° degree) atrioventricular block, with baseline left ventricular ejection fraction (LVEF) >45%. LEAP-CAR is a randomized, prospective, double blind clinical trial.

Nonischemic dilated cardiomyopathy is a heterogeneous disease often associated with increased rates of sudden cardiac death. Although many algorithms have been proposed, risk stratification remains suboptimal, and implantable cardioverter-defibrillators are currently recommended only in patients with poor left ventricular function. However, most cases of sudden cardiac death occur at earlier stages, in patients with relatively preserved left ventricular function and exercise capacity, for which device-therapy is currently not indicated. Several noninvasive risk factors have been associated with increased arrhythmic risk, including clinical history (syncope), imaging (fibrosis on cardiac magnetic resonance imaging and left ventricular dimensions in echocardiography) and electrocardiographic parameters (ventricular arrhythmic burden, late potentials, heart rate variability and repolarization abnormalities). The investigators hypothesized that the encouraging findings of studies assessing more sophisticated stratification-algorithms in patients with ischemic heart disease could be extrapolated in patients with nonischemic dilated cardiomyopathy. Thus, combining noninvasive risk factors with programmed ventricular stimulation may risk-stratify such patients more accurately. In this regard, the prospective observational multicenter ReCONSIDER study aims to integrate several approaches to arrhythmic risk stratification in nonischemic dilated cardiomyopathy in a tiered, multifactorial, approach, in which noninvasive risk factors are combined with electrophysiologic studies. This approach may pave the way for a more comprehensive risk stratification algorithm in patients with nonischemic dilated cardiomyopathy, leading to more rational device-therapy, and, ultimately to lower mortality.

Anginal symptoms and signs of ischemia have been reported in some patients with cardiac amyloidosis (TTR) without obstructive epicardial coronary artery disease (CAD). It was found that coronary microvascular dysfunction was highly prevalent in subjects with cardiac amyloidosis, even in the absence of epicardial CAD. The investigators found lower stress and rest myocardial blood flow (MBF) and lower myocardial flow reserve (MFR) in their cardiac PET (Positron emission tomography) study (13N), including 21 patients. The advances in SPECT technology including cadmium zinc telluride (CZT) detectors allow to evaluate the MBF and MFR estimation by SPECT as shown in both experimental animal models and also in clinical studies with comparison to PET. SPECT is more widely available than cardiac PET. Thus, the investigators would like: to confirm the results of Dorbala et al using SPECT, and to go further with evaluation of the effect of Tafamidis on microvascular dysfunction.