View clinical trials related to Cardiomyopathy, Hypertrophic.
Filter by:RIKADA is a prospective study performing systematic family screening including clinical and genetic testing in pediatric patients with primary cardiomyopathy and their first-degree relatives with the aim to facilitate risk stratification.
Hypertrophic cardiomyopathy (HCM) is very common. The thickened heart muscle can disrupt the normal functioning of the heart's electrical system, resulting in fast or irregular heartbeats. Today, there are no reliable means to identify HCM patients at risk of sudden death. The CardioInsight™ has recently been developed by Medtronic for non-invasive advanced cardiac mapping system. The CardioInsight™ Noninvasive 3D Mapping System is a non-invasive mapping system that collects chest ECG signals and combines these signals with CT scan data to produce and display simultaneous, bi-atrial and biventricular, 3-D cardiac maps. It is a monocentric, prospective, comparative and analytical study. 20 HCM patients will be recruited in the National Reference Center for inherited cardiac diseases of Lyon. The 3D-propagation maps of 10 well phenotyped and genotyped HCM patients with previous VF (ventricular fibrillation) will be compared to 10 HCM patients without previous VF. After consent signed, Holter ECG, scanner and CardioInsightTM 3D_ECG mapping will be planned in the month following the inclusion. 12-lead ECG, 24h holter ECG, cardiac scanner will be performed as standard procedure of each center in the current health care of the patient. The widespread screening of HCM patients with 3D high-density noninvasive mapping should improve the risk-benefit ratio of implantable defibrillator therapy. It is expected that more patients at risk will benefit from this device.
Aims and objectives: The primary objective of this study is to assess whether a structured exercise program improves cardiac relaxing properties in patients with hypertrophic cardiomyopathy (HCM). Background: HCM is a hereditary disease in which the myocardium becomes thickened without an identifiable cause (other than genetic). It is the most common genetic cardiovascular disease with an estimated prevalence of 1/500 (i.e. 10.000 affected individuals in Denmark). The majority of patients with HCM suffers from shortness of breath and reduced exercise capacity due to increased left ventricular (LV) stiffness. Exercise training has been shown to improve exercise capacity and symptoms in patients with HCM, but the mechanisms responsible for this improvement are not known. Methods and materials: The study is a randomized, single blinded, prospective, controlled clinical trial. Eighty patients are recruited from outpatient clinics in the Capital Region of Denmark. Patients are randomized in a 1:1 ratio to 12 week of moderate-intensity exercise training or usual activity level. Assessments will include right heart catheterization, echocardiography, cardiopulmonary exercise testing, blood-samples, quality of life, and, in a subgroup of patients, cardiac magnetic resonance imaging. The primary end-point is change in LV filling pressure assessed as pulmonary capillary wedge pressure at 25 W workload. Expected outcome and perspectives: The investigators hypothesize that an exercise training program will reduce cardiac stiffness and improve symptoms in patients with HCM. Training of HCM patients has long been debated and the topic is poorly researched. The effects of exercise on hemodynamics in HCM patients are unknown and a better understanding of these mechanisms is pivotal for improving treatment.
Hypertrophic obstructive cardiomyopathy (HOCM) patients often develop disabling symptoms of heart failure. Current treatment strategies are predicated on the empirical use of long-standing drugs, such as beta-adrenergics, although with little evidence supporting their clinical benefit in this disease. Metoprolol is currently the most widely used beta-blocker in symptomatic HOCM patients, but a randomized, placebo-controlled trial, that looks at the effect in HOCM patients has never been conducted. No studies of HOCM combine invasive pressure measurement with exercise and echocardiography. All previous studies, both invasive and echocardiographic, have been conducted during rest, and not during exercise. Symptoms of HOCM patients are function-related, and exercise testing is essential to assess the condition and the effect of drugs.
This is a joint project by Sahlgrenska University Hospital: Sahlgrenska, Östra and Mölndal. Our objective is to diagnose and map patients with well phenotyped cardiomyopathies (CMP) including in depth clinical and molecular phenotyping to enable earlier and specific treatment. The project will serve as: 1. resource for diagnostic and therapeutic trials 2. common biomaterial bank 3. resource for detailed molecular analyses on patients' biomaterials and patient specific symptoms and examination results
This is a multicenter open-label study of the administration of mavacamten in participants with symptomatic obstructive HCM (oHCM) who previously participated in study MYK-461-004 (PIONEER-HCM).
This is a multicenter, international, double-blind study of the administration of mavacamten in participants with symptomatic obstructive HCM (oHCM). Approximately 220 participants will be randomized to receive placebo or mavacamten.
The main aim of this study is to assess the acute effects of a pacemaker on reducing abnormally high intracavity pressures in the hearts of patients with mid-cavity obstructive hypertrophic cardiomyopathy (HCM). During a 12-month period of double-blinded follow-up, descriptive data will be collected on patients symptomatic and physical performance during dichotomous pacemaker settings for 6-months each (active and back-up). The statistical information collected will be used to design a much larger research trial of patient benefit.
This is a multicenter, exploratory, randomized, double-blind study of the administration of mavacamten in 60 participants with symptomatic nHCM randomized to receive a 16-week course of mavacamten doses titrated to achieve 1 of 2 target drug concentrations.
This study evaluates predictors for the incidence of arrhythmias and sudden cardiac death as well as terminal heart failure in patients with Fabry disease.