Differentiation HHD From HCM (EARLY-MYO-HHD)
Hypertrophic CardiomyopathyHypertensive Heart DiseaseDifferentiating hypertrophic cardiomyopathy (HCM) from hypertensive heart disease (HHD) unavoidably encounters diagnostic challenges especially in patient of suspected HCM with history of hypertension. Diverse and overlapping forms of HCM can often lead to ambiguity when diagnosis is based on a single genetic or morphological index. The investigators have deduced a integrated formula based on cardiac magnetic resonance (CMR) imaging and established a differentiating flow-chart between HCM and HHD, the investigators aim to identify their method in the current multi-center trial.
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.
Cardiac Magnetic Resonance Features and Outcomes of Patients With Non-Compaction Cardiomyopathy...
Left Ventricle Non-compaction CardiomyopathyObjective: To evaluate clinical characteristics, cardiac magnetic resonance imaging features, and outcomes of patients with left ventricle non-compaction.
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.
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.
Genetics of Ischemic Cardiomyopathy
Ischemic CardiomyopathyBackground:Ischemic heart disease is one of the heaviest health-related burdens worldwide.We aimed to identify the common hub mRNA and pathways that are involved in pathological progression of ischemic cardiomyopathy. Methods: To explore potential differentially expressed genes (DEGs) of all ischemic heart disease stages, we used chipster and GEO2R tools to analyze of retrieved eight high throughput RNA datasets obtained from GEO database. Gene Ontology functional annotation and Pathways enrichment analyses were used to obtain the common functional enriched DEGs which were visualized in protein-protein interactions (PPI) network to explore the hub mRNA according to the interaction scores. Validation qRT-PCR was carried out for blood and cardiac biopsies compared with controls to validate the determined four hub mRNAs and subsequently reviewed inside comprehensive published meta-analysis database. The validated mRNAs were visualized in two interaction modules. Finally screening of approved drugs was applied.
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.
Genomic Sequencing in Patients With HCM Undergoing Septal Myectomy
Hypertrophic CardiomyopathyGenetic Disease1 moreInvestigators aim to use comparative exome and/or genome sequencing to discover causative molecular lesions for phenotypes hypothesized to be caused by somatic mutations. For this study, investigators have targeted hypertrophic cardiomyopathy.
Assessment of Appropriate ICD Implantation for Primary Prevention of Sudden Cardiac Death
CardiomyopathiesProphylactic implant of an ICD (implantable cardioverter defibrillator) for prevention of sudden cardiac death from a life-threatening heart rhythm abnormality is a standard recommendation for patients with ejection fractions (EFs) of 35% or less. The main purpose of the trial is to determine why patients who are receiving care at a community-based cardiology program and are known to have a low ejection fraction (EF) measurement of 35% or less per echocardiogram and/or cardiac nuclear scan testing are not being approached for ICD (implantable cardioverter defibrillator) implant. Additionally, other secondary purposes of the trial are: 1) to determine if these patients are or are not receiving recommendation from their provider to undergo prophylactic ICD implant 2) identify the reasons providers are not recommending ICD implant for their patients with reduced EFs 3) determine reasons patients recommended for ICD implant by their provider elect not to have the implant.