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NCT07054073
This study aims to learn what might predict heart problems (like sudden death from a fast heart rhythm or heart failure) in people with a genetic condition called hypertrophic cardiomyopathy (HCM). HCM causes the heart muscle to become thick, which can make the heart stiff and harder to work properly. It can also affect the heart's electrical system. This study is looking to enroll patients that were previously part of a research project called "HCMR - Novel Predictors of Outcome in Hypertrophic Cardiomyopathy." The results of that study are still being reviewed, but they might show that people who had a substance called Gadolinium (MRI contrast or dye) collected in their heart muscle may have a higher risk for heart problems, including sudden cardiac death. This is called "late gadolinium enhancement" (LGE). This study is aiming to do follow-up imaging on those patients to better understand how LGE affects people with HCM.
NCT07344480
RASopathy-associated hypertrophic cardiomyopathy (RAS-CM) is a disease with high morbidity and high mortality if presenting during infancy. Targeted therapies have shown significant activity in preclinical models and case reports. Drugs that target the underlying cause of this disease are now developed in cancer patients. Conducting randomized trials is not possible in severely ill infants with RAS-CM. Existing historical controls from older eras are not sufficient as external controls to support drug development as they lack critical clinical and genetic information to allow comparison with the cohort planned for future clinical trials. The purpose of this investigator-initiated retrospective natural history study is to collect clinical information and genetic information in patients with RAS-CM. The first goal is to establish a data set that meets regulatory requirements for the use as external control data in a future clinical trial, composing non-randomized, single-arm, open-label study cohorts. The second goal is to obtain natural history information that supports the selection of secondary exploratory endpoints chosen in a clinical trial.
NCT07381894
Hypertrophic cardiomyopathy (HCM) is the most common inherited heart condition, affecting approximately 1 in 500 people. It causes the heart muscle to thicken, which can lead to blockages in blood flow (left ventricular outflow tract obstruction), shortness of breath, and an increased risk of heart failure or sudden cardiac arrest. While standard treatments exist and new targeted medications (cardiac myosin inhibitors) have recently been approved, doctors still need better data to predict which treatments will work best for each individual patient. This national registry based in the UK is a secure database that collects health information from HCM patients across multiple NHS hospital sites in the UK over several years. Participants in this study will have their routine health information collected from their medical records, including details from heart scans (echocardiograms and MRIs), blood tests, and genetic information. With this HCM registry, we aim to improve disease understanding and risk prediction, paving the way for more personalised treatment plans for the HCM community in the future
NCT07336290
This is a multi-center, observational study aiming to establish a precision diagnosis scheme and multi-dimensional risk prediction models for Hypertrophic Cardiomyopathy (HCM). The study plans to enroll 10000 adult HCM patients. It will integrate genetic testing, advanced cardiac imaging (echocardiography and cardiac magnetic resonance), and biomarker analysis to develop early diagnosis, differential diagnosis, and new risk prediction models for sudden cardiac death (SCD) and heart failure in HCM patients. Participants will undergo baseline assessments and be followed up every six months for up to 3 years to track clinical outcomes. This study is non-interventional and does not involve any investigational drugs or devices.
NCT07359690
The goal of this observational study is to pursue a multimodal approach to identify the molecular signatures and immune signalling molecules of various myocardial diseases and thereby contribute to improving diagnosis and therapy. The main aim is: -Identification of molecular profiles (e.g., proteome, lipidome, metabolome) and immune signalling profiles that are specifically associated with different myocardial diseases and the post-heart transplantation course. Participants already receiving an endomyocardial biopsy as part of their regular medical care will be enrolled. An additional biopsy sample will be taken for the above mentioned research.
NCT07354646
The goal of this observational study is to create a comprehensive real-world spectrum of T1 mapping measurements across different heart conditions. We aim to establish reference values for how heart tissue characteristics vary in various diseases, which will help doctors better interpret these advanced MRI measurements in clinical practice. The main questions it aims to answer are: What are the normal T1 mapping values for different heart diseases, and how do they compare to healthy hearts? Can we use the simpler "native T1" measurement (without contrast dye) instead of the more complex "ECV" measurement (which requires contrast dye) for diagnosis? Patients with various myocardial conditions will undergo CMR T1 mapping scans. We will analyze the MRI images and clinical records to establish disease-specific reference ranges for T1 mapping parameters, and validate the diagnostic accuracy of T1 mapping
NCT07263204
By harnessing artificial intelligence to decode the 12-lead electrocardiogram, the project will enable precise ECG-based phenotyping of hypertrophic cardiomyopathy-accurately classifying septal, apical, and other morphologic subtypes-while simultaneously differentiating HCM from hypertensive heart disease, aortic stenosis, and other phenocopy disorders.
NCT06775665
This study is a prospective cohort study aimed at exploring the baseline characteristics and treatment patterns of the Chinese population with hypertrophic cardiomyopathy (HCM) in real-world settings. The objective is to assess the real-world treatment approaches and longitudinal outcomes in this population.
NCT06683872
Hypertrophic cardiomyopathy (HCM) is a common autosomal dominant inherited heart disease, with previous data suggesting a prevalence of approximately 1 in 500 in the population. However, with the widespread use of genetic testing and high-sensitivity cardiac imaging in recent years, the estimated prevalence of HCM may be as high as 1 in 200. Approximately 75% of HCM patients will develop left ventricular outflow tract obstruction (LVOTO), leading to decreased exercise capacity, dyspnea, syncope, heart failure, and even sudden death, significantly impacting the patients\' quality of life and survival. The optimal treatment for hypertrophic obstructive cardiomyopathy (HOCM) is still a matter of controversy, despite surgical myectomy being considered the \"gold standard\" for HOCM treatment. Treatment options such as chemical (radiofrequency) ablation and medications (Mavacamten) are also challenging the status, and there is even debate over the surgical approach for myectomy. Currently, there is no consensus in the medical community about the pathophysiological mechanisms of LVOTO, and the exact mechanisms of its occurrence are not fully understood, which is also a major reason for the treatment controversy in HOCM patients. The focus of clinical decision-making is on the treatment based on the LVOTO pressure gradient, and it is generally recommended internationally to consider invasive treatment when the LVOTO pressure gradient is ≥50mmHg. However, there are still a small number of patients who do not have symptoms with a pressure gradient greater than 50mmHg or have significant symptoms with a pressure gradient less than 50mmHg, indicating that relying solely on the pressure gradient to assess the severity of HOCM and its prognosis may not be comprehensive enough. In recent years, with the development of medical engineering integration, the use of computational fluid dynamics (CFD) in the medical field, particularly in the field of cardiovascular diseases, has become increasingly widespread. CFD can construct cardiovascular geometric models based on specific clinical images of a patient and simulate ventricular wall motion and blood flow within the heart through computer calculations to obtain the required hemodynamic parameters. This enables the visualization and quantification of intraventricular blood flow. Compared to direct measurement techniques based on imaging, CFD has advantages such as non-invasiveness, comprehensiveness, and accuracy, leading to its increasing application in cardiovascular function research. In particular, in the field of cardiomyopathy, CFD can help deepen the understanding of the pathophysiological mechanisms of hypertrophic obstructive cardiomyopathy by reconstructing the anatomical configuration of the left ventricle and analyzing intraventricular blood flow and related hemodynamic parameters, which in turn can aid in clinical decision-making and the assessment of clinical prognosis. Therefore, we propose using three-dimensional (3D) computed tomography (CT) simulation technology to assess the hemodynamics of patients with hypertrophic obstructive cardiomyopathy, in order to guide clinical decision-making.
NCT06372457
COLLIGO-HCM is a global observational study that will conduct observational research of hypertrophic cardiomyopathy (HCM) treatment in real-world clinical practice.
NCT04189822
The Hearts in Rhythm Organization (HiRO) is a national network of Canadian researchers/clinicians, working towards a better understanding of the rare genetic causes of sudden cardiac death (SCD). Canadian adult and pediatric electrophysiology centres across Canada work together to gather data and bio sample in a national data registry and bio bank hoping to improve the detection and treatment of inherited heart rhythm disorders to prevent sudden death.
NCT01623245
Cardiac amyloidosis are related to the accumulation of fibrillar proteins in the extracellular leading to disruption of the cardiac tissue architecture. Amyloidosis in transthyretin (TTR) are the most common hereditary amyloidosis but remain poorly studied at heart. This is serious and deadly. The prevalence of TTR amyloidosis is probably underestimated in hypertrophic cardiomyopathy (HCM) often of unknown etiology because of the lack of systematic implementation of myocardial biopsy because of their side effects.