View clinical trials related to Cardiomyopathies.
Filter by:A Study on the Efficacy, Safety, and Tolerability of Perhexiline maleate in Subjects with Hypertrophic Cardiomyopathy and Moderate-To-Severe Heart Failure
The heart beat is controlled by electrical signals. Following a heart attack, part of the heart muscle dies and is later replaced by scar tissue. Within this area of scar, there often remain "channels" of surviving tissue still able to transmit electrical signals. However, it is well established that these "conduction channels" (CC) can form a short circuit around the scar, leading to electrical disturbances (arrhythmias) that are potentially life threatening. The commonest of these is ventricular tachycardia (VT), and is estimated to cause 300,000 deaths per year. One recognised treatment option of VT involves burning (ablation) these "conduction channels" (CC) within the scar. However, at present, the procedure is long and is far off 100% effective. Consequently, current best practice does not rely on treating the VT, but rather preventing it from causing sudden death - this is achieved with an Implantable Cardioverter Defibrillator (ICD), a device which can recognise when a patient is in VT and deliver an internal shock to restore the normal electrical conduction. Patients with defibrillators subsequently are subject to recurrent painful and debilitating shocks which, although lifesaving, significantly reduce their quality of life. The limitation with ablation at present is due to the difficulty in visualising these CC's. Investigators at Imperial College have created a novel electrogram visualisation program, Ripple Mapping (RM), which they have already found to be superior to currently used programmes in cases of arrhythmias in the upper chambers of the heart (the atria). During a retrospective study in patients with scar related VT following a heart attack, when ablation was delivered in areas associated with identified Ripple Mapping Conduction Channels, these patients remained free of VT recurrence for >2 year follow up interval. The study hypothesis is that Ripple Mapping can identify all conduction channels within scar tissue critical to the VT circuit, ablation of which will lead to long-term freedom from VT and ICD therapies. The investigators now aim to perform a prospective randomised study comparing Ripple Mapping guided VT ablation against conventional VT ablation.
This will be a cross-sectional, observational study. Null hypothesis: There is no difference in the amount of extracellular volume (ECV or scarring) in the hearts of patients with heart failure as compared to control subjects. Heart failure occurs when the heart muscle has become too weak to work properly. It is associated with an increase in the amount of connective tissue (collagen) which replaces dead heart muscle cells (scarring). Currently a biopsy of the muscle is the only way to measure the amount of scarring. This is invasive and rarely done in children. Because of this, it is difficult to measure the amount of scarring in a particular patient or disease process, which is important for improving our understanding and treatment of the disease. Cardiac magnetic resonance imaging (MRI) is a non-invasive imaging tool which is routinely used to look at areas of local scarring in heart muscle. Because the scarring is so widespread in paediatric patients, we have not been able to use this method previously. Now new imaging techniques allow us to look at widespread scarring but these have not yet been validated in children. We plan to use late gadolinium enhancement (T1 mapping) to measure the amount of scarring in patients with heart failure (we have evidence that their heart biopsies show increased amounts of scar tissue) and children having MRI scans for other reasons. We will use measures of function including echocardiography and 6 minute walk test to compare to the amount of scarring. This will help us to know whether the amount of scarring will be clinically useful. We will look at the amount of various proteins in the blood of patients and control subjects which are related to the scarring and cell death processes. We already use blood tests to monitor heart failure and these tests may help us to refine our testing and improve timing of treatment (e.g. transplantation). This study will help us to design further research in this field.
Presently, the left ventricular lead is placed in a similar position for all patients. It is not known whether placing this lead in different positions in the heart will make the heart pump better. In this study, the investigator will collect measurements of the heart's electrical activity during an Electrophysiology Study (EP study or EPS). The hope is that these measurements will provide the know how to develop an individualized left ventricular lead placement "prescription" for patients referred for left ventricular lead pacing.
Patients with dilated cardiomyopathy who present for indwelling right heart catheterization will be enrolled and randomized to either control or triheptanoin oil for five days. Hemodynamics will be assess serially.
The purpose of the study is to determine whether the ability of MEK162 to antagonize MEK activation in NS HCM patients, who usually have upstream mutations in the Ras-Raf-Mek-Erk pathway that lead to MEK activation, would be beneficial over a 6 month treatment period in hypertrophy regression.
A double blinded and placebo-controlled, dose escalation, single-center safety and preliminary efficacy study of cardiospheres delivered via NOGA MYOSTAR injection catheter in subjects with chronic ischemic cardiomyopathy. The objective is to achieve and document myocardial regeneration in patients with chronic scar.
Primary objective and endpoint is the analysis of the long-term course of lyso-Gb3 and its clinical correlation to the progression of the cardiomyopathy in N215S-Fabry patients.
The purpose of this study is to determine the genetic basis of cardiomyopathies and heart failure.
The purpose of this research study is to further establish the diagnostic use of magnetocardiography (MCG) in patients with hypertrophic cardiomyopathy (HCM). The use of MCG has not been extensively studied in these patients. This pilot study will serve to further characterize abnormalities found on MCG in comparison to patients without hypertrophic cardiomyopathy. Additionally, the study will be used to understand whether MCG has any additional diagnostic utility in offering clinicians insight on the patient's disease state, thereby aiding in the development of treatment plans. This research study is designed to test the effectiveness of the investigational use of magnetocardiography in patients with hypertrophic cardiomyopathy. The device itself has been approved by the U.S Food and Drug Administration (FDA).