View clinical trials related to Hypertrophic Cardiomyopathy.
Filter by:Background: Stress echocardiography (SE) has an established role in evidence-based guidelines, but recently the breadth and variety of applications has extended well beyond coronary artery disease (CAD). Purpose: To establish a prospective research study of SE applications, in and beyond CAD, also considering a variety of signs in addition to regional wall motion abnormalities. Methods: In a prospective, multicenter, international, observational study design, > 100 certified high-volume SE labs will be networked with an organized system of clinical, laboratory and imaging data collection at the time of physical or pharmacological SE, with structured follow-up information. The study is endorsed by the Italian Society of Echocardiography and organized in 10 subprojects focusing on: contractile reserve for prediction of cardiac resynchronization or medical therapy response; stress B-lines in heart failure; hypertrophic cardiomyopathy; heart failure with preserved ejection fraction; mitral regurgitation after either transcatheter or surgical aortic valve replacement; outdoor SE in extreme physiology; right ventricular contractile reserve in repaired tetralogy of Fallot; suspected or initial pulmonary arterial hypertension; coronary flow velocity, left ventricular elastance reserve and B-lines in known or suspected CAD; identification of subclinical familial disease in phenotype-negative healthy relatives of inherited disease (such as hypertrophic cardiomyopathy). Expected Results:To collect about 10,000 patients over a 5-year period (2016-2020), with sample sizes ranging from 5,000 for known or suspected CAD to around 250 for hypertrophic cardiomyopathy or repaired Fallot. This data base will allow to investigate technical questions such as feasibility and reproducibility of various SE parameters and to assess their prognostic value in different clinical scenarios. Conclusions: The study will create the cultural, informatic and scientific infrastructure connecting high-volume, accredited SE labs, to obtain original safety, feasibility, and outcome data in evidence-poor diagnostic fields, also outside the established core application of SE in CAD based on regional wall motion abnormalities. The study will standardize procedures, validate emerging signs, and integrate the new information with established knowledge, helping to build a next-generation SE lab without inner walls.
Fibrosis, myocardial deformation and biomarkers in hypertrophic cardiomyopathy (HCM)
Biobank is a program which collects biological samples, health information and imaging data from consented patients and stored them at the core facility. These information would be used to study the molecular, imaging and outcome studies of cardiovascular health and disease.
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disease characterized by unexplained hypertrophy of the left ventricle, often with predominant involvement of the interventricular septum, and characterized by myocyte disarray and fibrosis. HCM is the most common familial heart disease with strong genetic heterogeneity, demonstrated over the past 20 years. Mutations in 11 or more genes encoding proteins of the cardiac sarcomere are responsible for (or associated with) HCM. However, 30-40% of sporadic and familial cases of HCM are still genetically unlabelled. In addition, secondary HCM caused by Fabry's disease or amyloidosis, may mimic primary HCM and may be under diagnosed. This may result in a delay in accurate diagnosis and instauration of specific treatment, with possible clinical consequences for the patients. For these reasons, we decided to apply a new diagnostic strategy for patients with newly diagnosed HCM, including the whole exome sequencing (WES) technology. If correctly applied, this new technology has the potential to strongly reduce the diagnostic wavering leading to earlier diagnosis and genetic counseling in sarcomeric HCM and rarer forms of secondary HCM including Fabry's disease and amyloidosis, and also specific therapy set-up in secondary forms of HCM. It should also allow identifying new genes responsible for HCM.
The goal of this protocol is to obtain information from individuals with cardiomyopathy and from their families in order to elucidate the molecular genetics of this disorder. This will provide the basis for future genetic counseling as well as contribute to elucidating the biology of normal and abnormal cardiac function.
Hypertrophic cardiomyopathy (HCM) is a common inherited heart condition that causes breathlessness, chest pain and fatigue. There are few treatments available. The investigators have recently shown that a drug called perhexiline reduced symptoms and improved exercise capacity in patients with HCM. This change appears to be driven by alterations in myocardial energy metabolism. The aim of this trial is to test a similar drug, trimetazidine, in a group of symptomatic patients with non-obstructive HCM. HYPOTHESIS: trimetazidine will improve symptoms, peak oxygen consumption, cardiac function and arrhythmia burden in medically refractory symptomatic patients with non-obstructive HCM.
Exercise training (ET) is highly beneficial in heart failure patients and has been suggested to confer significant symptomatic and functional improvements in patients with diastolic dysfunction. Accordingly, the aim of this study was to examine the safety, feasibility of a structured ET program in symptomatic HCM patients.
Hypertrophic cardiomyopathy (Hypertrophic CardioMyopathy=HCM) remains a poorly understood disease with an assumption insufficiently codified. There is no data available in France on the profile of patients, diagnostic methods and assessment and therapeutic use. The purpose of this study is to establish a monitoring of patients with HCM (sarcomere of origin or not) in France (diagnosis, treatment)
The primary aim of this study will be to assess whether Biventricular pacing improves exercise capacity, and Quality of Life in patients with symptomatic drug resistant Non-Obstructive Hypertrophic Cardiomyopathy, using a Biventricular pacemaker.
Hypertrophic Cardiomyopathy is an inherited condition characterized by thickening (hypertrophy) of the heart muscle. Many patients who have this condition have a reduced ability to exercise because of breatlessness, which can in some cases be severe. This appears in most cases to be due to an impairment of the filling of the heart, especially on exercise this limits the amount of blood the heart is able to pump. Several factors may contribute to this slow filling of the heart, including (1) The heart contracts and relaxes in an incoordinate way (called 'dyssynchrony') which is inefficient, and (2) The filling of the main pumping chamber (the left ventricle) may be impeded by high pressure in the other ventricle(the right ventricle)- in other words the left ventricle is 'squashed' by the right ventricle. This is known as diastolic ventricular interaction. Although drugs can improve the filling of the heart and relieve symptoms, some patients remain very symptomatic despite these drugs. The mechanisms responsible for the filling abnormality in patients with Hypertrophic Cardiomyopathy are similar to those seen in the much more common condition known as Heart Failure. A special type of pacemaker technique called 'Biventricular Pacing' has been shown to markedly improve symtoms in patients with heart failure. This form of pacing has been shown to improve both 'dyssynchrony' ( incoordination) and 'ventricular interaction' (squashed left heart) in patients with Heart Failure. We propose that Biventricular pacing may similarly improve these abnormalities in patients with Hypertrophic Cardiomyopathy, resulting in an improvement of symptoms. The study will focus on patients with the condition who have severe symtoms despite being on optimal currently available drug therapy.