Active clinical trials for "Cardiomyopathies"

In a hitherto ill-defined proportion of patients with inflammatory/familial cardiomyopathy, the phenotype dilative cardiomyopathy (DCM) is assumed to be the endstage of a multifactorial etiopathogenetic pathophysiology. Precipitating factors include enhanced autoimmunity, predisposition for viral infections, environmental factors in addition to a specific 'genetic background' of the individual patient. It is unresolved, whether the susceptibility to immunologically mediated myocardial damage reflects the presence of genetic risk factors shared by other autoimmune diseases, or is cardio-specific with individual predisposing factors. Aims of the project are the search for a genetic link or oredisposition to autoimmune diseases in patients with familial / inflammatory DCM.

The arrhythmogenic right ventricular cardiomyopathy, also known as arrhythmogenic right ventricular dysplasia (ARVC/D) is a rare myocardial disease with a prevalence estimated to range from 1 case on 5000 persons in the general population. It have a dominant genetic transmission characterized by alterations of desmosomial proteins and predominantly affects the right ventricle. The morphological alteration of the myocardium characterized by fibro-fatty substitution predisposes to arrhythmic events that can be fatal and cause death especially in young people and athletes. International guidelines provide a classification that includes clinical and histological criteria for diagnosis based on fibrous tissue substitution, percentage and right ventricle localization. Indeed, it is mainly affected the right ventricle but in some cases also the left ventricle result involved and the fatty tissue or fibro-fatty tissue with particular arrangement may affect the full-thickness wall are pathognomonic. Authors identified 10 cases of arrhythmogenic cardiomyopathy in forensic autopsy collocated in the 2003 to 2017, included 8 males and 2 females. Their age ranged from 16 to 45 years with an average age of 28.8±8.1 years. However, authors would like to demonstrate that with ARVC/D cannot be include only cases with increased fibrosis and exclusive localization to the right ventricle nor even cases with electrocardiographic alterations and other evident clinical criteria. Indeed, in the analyzed cases authors observed an amount of fibrosis often less than the percentage reported in the literature and an involvement not only of the right ventricle but also of the septum and associated sinister ventricular modifications. To this must be added that the cases of death did not have a sudden death of relatives in family history as described in guidelines and therefore this criterion is not reliable for the purpose of a classification.

The main purpose of this study is to determine the prevalence of ATTR Cardiomyopathy among patients admitted due to Left Ventricular Hypertrophy (LVH) >15mm of unknown etiology by using a 99mTc-tracer scintigraphy based protocol

The Physiologic Pacing Registry is a prospective, observational, multi-center registry performed to gain a broader understanding of 1) physiologic pacing implant and follow-up workflows, including pacing and sensing measurements and 2) the clinical utility in creating a 3-dimensional electro-anatomical map of cardiac structures prior to physiologic pacing device implants based on the clinical site's routine care.

The purpose of this observational study is to evaluate the performance of the Berlin Heart EXCOR Active driving unit while being used with the approved EXCOR ventricular assist device system.

Hypertrophic cardiomyopathy (HCM) is a frequent cardiac pathology with an estimated prevalence of 1/500 in France. The main risk factor for sudden death in this pathology is the presence and extent of left ventricular obstruction. To date, the only method allowing a reliable assessment of the extent of left ventricular obstruction is Doppler echocardiography. All patients with HCM should undergo cardiac magnetic resonance imaging (MRI) to confirm the diagnosis and for the detection of fibrosis, but conventional sequences cannot reliably assess the obstruction. 4D-flow MRI provides a complete coverage of an entire volume with the ability to simultaneously measure the outputs of all vessels within that volume in a single sequence and might be able to quantify left ventricular obstruction. The main objective of this study is to compare the quantification of left ventricular obstruction in hypertrophic cardiomyopathy by Doppler echocardiography and 4D flow MRI.

Approximately 30-40% of patients with non-ischaemic dilated cardiomyopathy (DCM) undergo significant left ventricular reverse remodelling in response to guideline-directed therapies. This is characterised by improvement in systolic dysfunction and regression of left ventricular dilatation. In some patients, extensive left ventricular reverse remodelling is accompanied by resolution of symptoms and normalisation of cardiac biomarkers, resulting in a state of clinical remission. The mechanistic drivers behind left ventricular reverse remodelling and clinical remission are poorly understood. Current techniques to predict ventricular remodelling trajectory and clinical remission in patients with recent-onset DCM are limited. The purpose of this study is to characterise predictors and markers of left ventricular reverse remodelling and clinical remission in patients with recent-onset DCM using molecular markers, genetics and advanced CMR imaging.

The aim of this clinical prospective study is to assess structural and functional myocardial changes in patients with liver cirrhosis after implantation of transjugular intrahepatic portosystemic shunt (TIPS).

Sudden cardiac death (SCD) risk stratification is a challenge in non-ischemic cardiomyopathy (NICM). The underlying mechanism of monomorphic ventricular tachycardia (VT) is mostly scar related. While electrophysiological mechanisms underlying ventricular arrhythmia are well known, late gadolinium enhanced (LGE) cardiac MRI-3D reconstructions are now able to guide VT ablation procedures. Such exam may help in identifying specific properties of scar at risk of malignant arrhythmia.

Left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by numerous excessively prominent left ventricular (LV) trabeculation and deep intertrabecular recesses communicating with the ventricular cavity and severely altering myocardial structure. Although most authors assume a developmental arrest in embryogenesis as the underlying pathology, the mechanisms of LVNC are not fully understood yet. Several gene mutations have been identified to be linked with LVNC and an autosomal dominant inheritance pattern is frequent To date the most commonly used imaging tool for diagnosing LVNC is echocardiography applying the criteria established by Jenni and coauthors However, qualitative parameters to differentiate normal compaction of the myocardium in healthy subjects from LVNC or from other cardiomyopathies like dilative cardiomyopathy (DCM) or hypertrophic cardiomyopathy (HCM) may fail due to highly variable LV trabeculation. Therefore, absolute quantification should be performed. Cardiac magnetic resonance (CMR) has been reported as a promising imaging modality to characterize patients with LVNC as it provides both a high spatial resolution and a good contrast between trabeculation and blood pool Jacquier et al. recently described a value of trabeculated LV myocardial mass above 20% of the global mass of the LV to be highly sensitive and specific for LVNC However, in their approach, a substantial degree of the LV cavity was included into calculated trabecular LV mass and led to systemic overestimation of the latter. Furthermore, the role and prognostic value of myocardial scarring as assessed by delayed enhancement (DE) CMR was not evaluated. The aim of the retrospective study was to establish revised and extended CMR criteria to distinguish LVNC from DCM, HCM and a group of healthy controls and to improve the assessment of trabeculated mass by excluding intertrabecular blood pool.