View clinical trials related to Hypertrophic Cardiomyopathy.
Filter by:This research study has been designed to test whether a drug called trientine dihydrochloride (also called Cufence) reduces heart muscle thickening, improves exercise capacity, improves heart function and reduces abnormal heart rhythms in patients with hypertrophic cardiomyopathy (HCM). The study is also assessing how trientine works in HCM. Participants will be prescribed either trientine or placebo, for a period of 12 months.
The aim of this randomized trial is to compare the improvement in exercise capacity among patients with highly symptomatic hypertrophic obstructive cardiomyopathy despite optimal medical treatment who undergo alcohol septal ablation (ASA) or surgical septal myectomy (SSM).
Use lay language. Current guidelines regarding physical activity in patients with inherited arrhythmia and cardiomyopathy are mostly dedicated to adult patients, with a special focus on sports competition. Their application to the pediatric population has been scarcely evaluated. Physical activity is well known for its health benefits but may be dangerous in this population, which leads to confusion within the medical community and among patients. Actual physical activity of children with such inherited cardiac disorders is unknown. This study aimed to assess the level of physical activity in children with inherited arrhythmia and cardiomyopathy, and the adherence to the current European guidelines on the subject. Secondary objectives aimed to assess through a qualitative analysis the impact of the disease on physical activity and daily life in this population. The level of physical activity and adherence to current guidelines will be determined from interviews between the patient and the principal investigator. Each patient will be questioned in order to explore the experiences, motivations and feelings of participants regarding physical activity. The standardized questionnaire was created by the principal investigator and members of the clinical research team. The investigators believe that many children practice physical activity outside the current guidelines and hope to identify the main determinants of physical activity in this population.
This study is designed to quantify the ventricular stasis in patients with different forms of cardiomyopathy and at risk of stroke (ischemic, non-ischemic dilated cardiomyopathy and hypertrophic cardiomyopathy) by post-processing of 2D color Doppler echocardiography and phase contrast-magnetic resonance images in order to establish the relationship between quantitative variables of intraventricular stasis and the prevalence of silent embolic events and/or intraventricular mural thrombosis.
This study aims to identify and assess new CMR techniques that can improve current CMR protocols.
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.
Hypertrophic Cardiomyopathy (HCM) is the most common inherited heart muscle condition affecting up to 1 in 200 of the general population. It results from mutations in genes encoding components of the contractile apparatus in the heart muscle cell (myocyte). These mutations result in increased energy cost of force production for the myocyte which then cumulatively causes a myocardial energy deficit. This myocardial energy deficit is then thought to lead to cardiac hypertrophy ('left ventricular hypertrophy' or LVH) in HCM. LVH leads to impairments in heart muscle function, heart muscle oxygenation and microvascular blood flow and is the chief driver of patient symptoms in HCM. These symptoms consist of chest pain, shortness of breath, dizziness, fainting episodes or palpitations. Occasionally, the disease may cause sudden cardiac death (SCD). HCM is the most common cause of SCD in young people including competitive athletes. In addition, HCM has been found to result in significant global deterioration in health-related quality of life. Treatment of HCM has focused on relief of symptoms by drugs such as ß-blockers which slow the heart rate and improve heart function. However, symptom relief is often incomplete and there is no evidence on the benefit of ß-blockers or related medications to reverse LVH. Perhexiline, a potent carnitine palmitoyl transferase-1 (CPT-1) inhibitor shifts myocardial metabolism to more efficient glucose utilisation and rectifies impaired myocardial energetics. It is currently used to treat angina in patients with coronary artery disease. There is some preliminary evidence that Perhexiline may aid in the improvement of symptoms in patients with HCM. However, the effect of any form of therapy on potential regression of LVH in HCM remains unexplored. In this randomised double-blind placebo-controlled trial, the investigators will use state of the art cardiac imaging, principally advanced echocardiography and Cardiovascular Magnetic Resonance (CMR) to study the effects of perhexiline on LVH, cardiac function, and oxygenation in symptomatic patients with HCM. The investigators hypothesize that perhexiline will favourably reduce LVH and improve myocardial oxygenation by improving myocardial energetics, and that these putative morphological and functional changes can be accurately measured utilizing echocardiography and CMR. If this pilot study supports the hypothesis, then it will pave the way for a major randomised controlled trial to definitely determine the role of Perhexiline in HCM.
Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium and is most often caused by mutations in sarcomere genes. The structural and functional abnormalities cannot be explained by flow-limiting coronary artery disease or loading conditions. The disease affects at least 0,2% of the population worldwide and is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmia, but in most patients, however, HCM has a benign course. Therefore, it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation.
Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease. A relatively common subgroup of HCM patients have apical HCM - a type of heart muscle disease that causes abnormal muscle thickening towards the tip (apex) of the heart. This can impair the heart's own blood flow through the thickened heart muscle. We think this is one of the causes for symptoms such as shortness of breath and chest pain. If medications are ineffective at treating symptoms, there are few further options available, limited to invasive heart surgery. This study aims to determine if it is possible to improve the blood flow within by altering the settings of patients' permanent pacemakers, dynamic microvascular obstruction is an important cause of perfusion abnormalities in HCM and whether introducing localized dyssynchrony with ventricular pacing improves this. This phased study will include patients with apical HCM that already have implanted pacemaker devices to remove risks associated with device implantation. The study may provide insights into novel mechanisms for symptoms in HCM and provide new methods for treating a patient group in whom therapeutic options can be extremely limited.
Hypertrophic Cardiomyopathy (HCM) is an inherited heart condition. Most people who have it are unaware of any problems relating to it. Unfortunately, a small number of people with the condition can suddenly develop a dangerous fast heart beat that can lead to death. There is no cure, but implanting a cardioverter-defibrillator (ICD), which is like a pacemaker can save the life of affected individuals. However, ICD implantation has its own problems, so choosing who gets an ICD is a very important decision. The current approach for recommending people for an ICD has limitations and a better method is needed. Investigators have developed a new technique called the 'Ventricular Conduction Stability' (V-CoS). This involves wearing a special vest which records electrical signals from the heart, and then running on a treadmill. Investigators have used it to identify abnormalities in the hearts of people with (HCM) who have also survived a life-threatening event. This project aims to test new tool against current methods to ascertain which is better at identifying patients who should have an ICD.