Active clinical trials for "Cardiomyopathy, Hypertrophic"

Heart failure (HF) is the common end-stage of different medical conditions. It is the only growing cardiovascular disease and its prognosis remains worse than that of many malignancies. The lack of evidence-based treatment for patients with diastolic HF (HFpEF) exemplifies that the current "one for all" therapy has to be advanced by an individualized approach. Inherited cardiomyopathies can serve as paradigmatic examples of different HF pathogenesis. Both gain- and loss-of-function mutations of the same gene cause disease, calling for disease-specific agonism or antagonism of this gene´s function. However, mutations alone do not predict the severity of cardiomyopathies nor therapy, because their impact on cardiac myocyte function is modified by numerous factors, including the genetic context. Today, patient-specific cardiac myocytes can be evaluated by the induced pluripotent stem cell (hiPSC) technology. Yet, unfolding the true potential of this technology requires robust, quantitative, high content assays. The researchers' recently developed method to generate 3D-engineered heart tissue (EHT) from hiPSC provides an automated, high content analysis of heart muscle function and the response to stressors in the dish. The aim of this project is to make the technology a clinically applicable test. Major steps are (i) in depths clinical phenotyping and genotyping of patients with cardiomyopathies or HFpEF, (ii) follow-up of the clinical course, (iii) generation of hiPSC lines (40 patients, 40 healthy controls), and (iv) quantitative assessment of hiPSC-EHT function under basal conditions and in response to pro-arrhythmic or cardio-active drugs and chronic afterload enhancement. The product of this study is an SOP-based assay with standard values for hiPSC-EHT function/stress responses from healthy volunteers and patients with different heart diseases. The project could change clinical practice and be a step towards individualized risk prediction and therapy of HF.

Cardiomyopathy is a disease of the heart muscle. It is rare, but it can be serious. Cardiomyopathy in children can result in death, disability, heart transplantation or serious heart rhythm disorders. Natural substances in the blood called cardiac biomarkers can be measured in the laboratory and could be a less invasive way (compared to echocardiograms or MRIs) to detect heart dysfunction in children with cardiomyopathy. Little is known about how useful and valid cardiac biomarkers are in the diagnosis and determination of the symptoms in children with cardiomyopathy. The long-term goal of this project is to study how helpful measuring cardiac biomarkers in children with cardiomyopathy is to their doctors in managing the care of these patients as well as improving their overall health. Measures of these cardiac biomarkers could help doctors in determining how best to care for a child with cardiomyopathy, including when to consider heart transplantation as a treatment option.

The primary hypothesis of the study is that treatment with AT1-R antagonist in patients with nonobstructive form of HCM will be first save, second will cause regression of myocardial hypertrophy.

The purpose of this study is to determine the genetic basis of cardiomyopathies and heart failure.

Fibrosis, myocardial deformation and biomarkers in hypertrophic cardiomyopathy (HCM)

This is a prospective, single-center study to assess clinical phenotype and prognosis of different pathogenic mutations in Chinese patients with hypertrophic cardiomyopathy. Patients with hypertrophic cardiomyopathy were consecutively recruited, and then DNA samples were extracted from peripheral blood. Targeted sequencing of 142 genes was performed to obtain variants associated with hypertrophic cardiomyopathy. Patients will undergo face-to-face interviews, phone calls, or/and chart reviews at 6 months, 12 months, 24 months, 36 months, 48 months and 60 months for data collection of clinical outcomes.

Perioperative management may have strong connections with postoperative complications (PCs). However, little is known about the perioperative risk factors of PCs after septal myectomy in hypertrophic obstructive cardiomyopathy (HOCM) patients. This study is designed to assess the in-hospital PCs rate of HOCM patients and to identify perioperative risk factors of PCs in patients who underwent septal myectomy. Retrospective chart review will identify adult HOCM patients who underwent septal myectomy from October 2013 to December 2018 in the investigators' hospital. Patients' data will be collected from electronic medical records. The multivariable logistic regression analysis will be used to determine independent predictors. The predictive ability of individual predictor and different combination of multiple risk factors on PCs will also be calculated.

The overall rationale is to reduce the risk of sudden cardiac death in individuals with hypertrophic cardiomyopathy (HCM). The novel approach of this study is to correlate Positron Emission Tomography (PET) findings to ventricular arrhythmias detected by the implantable cardioverter defibrillator (ICD). This could potentially lead to an improved risk stratification of HCM patients.

The long term health and cardiovascular benefits of a regular exercise program have been well-established. National guidelines recommend involvement in moderate aerobic fitness (i.e. walking, bicycling, light jogging, swimming) for patients with hypertrophic cardiomyopathy (HCM). However, data on potential benefits of recreational exercise, useful parameters for risk stratification, and methods of devising individual exercise prescriptions are completely lacking. The specific aims of this study are: 1) to devise a safe moderate intensity exercise training program in patients with HCM, and 2) to determine whether exercise training improves ability to perform activities and tasks, heart size and function, and quality of life in patients with HCM.

Chest pain and myocardial ischemia are prevailing features in patients with hypertrophic cardiomyopathy (HCM). Recently introduced single-photon emission computed tomography (SPECT) cameras with solid-state cadmium-zinc-telluride (CZT) detectors have been shown to decrease imaging time and improved the imaging quality of gated myocardial perfusion imaging (MPI). The investigators also correlate the MPI parameters with echocardiographic parameters. This study is to examine the spatial distribution of stress perfusion abnormalities and tissue injury in patients with HCM using a CZT SPECT camera.