Extended ECG Monitoring in HCM Patients
Hypertrophic CardiomyopathyNonsustained Ventricular Tachycardia3 moreThe purpose of this study is to determine, among a large cohort of 300 consecutive patients with hypertrophic cardiomyopathy, if extended ambulatory monitoring using the iRhythm Technologies, Inc. Zio XT device results in identifying a greater burden of nonsustained ventricular tachyarrhythmia (nsVT) compared to current ACCF/AHA guideline recommended 48-hour monitoring.
Study of Myocardial Deformation Parameters in Patients With Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyThe purpose of the study is to investigate the potential correlations of global longitudinal strain and peak left atrial strain, measured by speckle tracking echocardiography with the severity of hypertrophic cardiomyopathy and the risk for arrhythmias. Specifically the investigational questions are: Is there a correlation between myocardial deformation parameters of the left heart chambers with other important ultrasound parameters (eg maximal wall thickness, presence of subaortic obstruction, etc.) in patients with hypertrophic cardiomyopathy? Is there a correlation between myocardial deformation parameters of the left heart chambers and the symptomatology or functional capacity of patients with hypertrophic cardiomyopathy? Is there a correlation between the myocardial deformation parameters of the left heart chambers and the exercise capacity, as evidenced by the cardiopulmonary exercise test, in patients with hypertrophic cardiomyopathy? Is there a correlation between myocardial deformation parameters of the left heart chambers with the risk of ventricular or supraventricular arrhythmias in patients with hypertrophic cardiomyopathy? Is there a correlation between myocardial deformation parameters of the left heart chambers and risk factors for sudden death?
Assessment of Wall Thickness in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyAssessment of wall thickness in hypertrophic cardiomyopathy (HCM) is of diagnostic and prognostic importance given its known association with sudden cardiac death. However, data regarding comparison of imaging modalities for this key measurement is lacking. This study seeks to compare assessment of maximum wall thickness between clinically indicated echocardiography (with and without contrast) and clinically indicated cardiac magnetic resonance imaging.
Belgian Screening Project for the Detection of Anderson-Fabry Disease in Hypertrophic Cardiomyopathy...
Left Ventricular HypertrophyThe purpose of this study is to determine the prevalence of Fabry mutations in patients with left ventricular hypertrophy (moderate to severe), as measured by echocardiography.This study is a screening study
Insertable Cardiac Monitors in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyHypertrophic cardiomyopathy (HCM) is associated with sudden cardiac death and an increased risk of atrial fibrillation and subsequent embolic event. An insertable cardiac monitor will provide data on cardiac rhythm over a period of 18 months. This will provide an extended monitoring far longer than 24-48 hours of Holter monitoring as is currently usual care. Therefore, detection of arrhythmias could be used in risk stratification and decision-making with regard to offer an implantable defibrillator and anticoagulants.
Patients' Long-Term Survival of Obstructive Hypertrophic Cardiomyopathy (HCM)
CardiomyopathyObstructive Hypertrophic CardiomyopathyBackground: HCM is a genetic heart disease. It can cause fatigue, chest pain, or even death. For more than 50 years, a surgery called septal myectomy has been used to help people with this disease. Dr. Andrew G. Morrow originated the surgery and performed it more than 200 times at NIH starting in 1960. Researchers want to learn the long-term success of this surgery. Objective: To determine long-term survival at least 35 years after surgical myectomy at NIH and examine data for people who are confirmed to be deceased or alive. Eligibility: People who had surgical myectomy by Dr. Morrow from 1960 to 1983. Design: This study uses images and data that were obtained in the past. Many of the participants are deceased. Most of the others are no longer being followed at the NIH. The medical records of people treated by Dr. Morrow were microfiched. These records can be accessed at the NIH. The records will be searched for keywords to find participants for this study. Participants clinical data, such as lab testing and imaging, will be used. Other data collected as part of the original study will also be used. Researchers will use participants name, date of birth, and Social Security number to learn if they are alive or deceased. If they are deceased, researchers will try to find the age of death. Online databases and search engines will also be used. Survival data will be compared to data from the general U.S. population for the same time period. Data will be stored in a database that is password protected. The study will last about 1 year.
Fabry Disease in High-risk Patients With Left Ventricular Hypertrophy: Prevalence and Implementation...
Fabry DiseaseFabry Disease4 moreThis study aims to evaluate the prevalence of Fabry Disease (FD) among a cohort of high risk patients with left ventricular hypertrophy (LVH) presenting at the University Hospital Würzburg over the last 20 years. Fabry disease is a rare disease that is known to be consistently underdiagnosed due to its largely variable symptoms. Considering that an early Fabry diagnosis is crucial for maximum benefit from therapies available, screening for Fabry patients can contribute to preventing development and worsening of symptoms in Fabry patients with LVH. In addition, a positive diagnosis in a family member opens the possibility to diagnose further family members in an earlier stage of the disease, therefore allowing treatment of symptoms and organ manifestations before they become irreversible.
Efficacy of Risk Assessment for Sudden Cardiac Death in Patients With Hypertrophic Cardiomyopathy...
Hypertrophic CardiomyopathyHypertrophic 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.
Residual or Recurrent Obstruction After Septal Myectomy
Hypertrophic Obstructive CardiomyopathyThe goal of this observational study is to report the outcomes after septal myectomy in young children and infants and identify the mechanisms of residual or recurrent obstruction after surgery. in The main question[s] it aims to answer are: What is the early and mid-term results of septal myectomy in young children and infants with severe and extensive obstructive hypertrophic cardiomyopathy (HCM)? What are the mechanisms of residual or recurrent obstruction? Echocardiography and clinical course of children and infants under the age of 14 who underwent septal myectomy for hypertrophic obstructive cardiomyopathy from January 2013 to December 2020 will be followed up.
Evaluating Microvascular Dysfunction in Symptomatic Patients With HypertroPhic CaRdiomyopathy
Hypertrophic CardiomyopathyPatients with hypertrophic cardiomyopathy are being compared to a control group. IMR will be assessed with a pressure wire. Clinical f/u at 3 months and 6 months and a 48 hour holter monitor.