
Presentations of Hypertrophic Cardiomyopathy on Myocardial Perfusion Imaging
Hypertrophic CardiomyopathyMyocardial Perfusion ImagingChest 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.

Morbidity and Mortality in Patients With Hypertrophic Cardiomyopathy: a CALIBER Study
Hypertrophic CardiomyopathyThe aim of this project is to study the association of a number of demographic and cardiovascular risk factors with death, health care utilisation and systemic embolisation by examining the clinical evolution of hypertrophic cardiomyopathy in a large, community based cohort identified from linked electronic health records.

New Diagnostic Strategy in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyHypertrophic 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.

Extended Ambulatory Monitoring Improves Risk Stratification in Hypertrophic Cardiomyopathy
Hypertrophic CardiomyopathyEligible subjects will wear 4 consecutive external monitoring devices for a total of 28 days of monitoring.

Molecular Genetic Screening and Identification of Congenital Arrhythmogenic Diseases
Long QT SyndromeHypertrophic Cardiomyopathy1 moreThe aim of this study is the identification of familial congenital arrhythmogenic disorders and their clinical follow-up.

Comparison of Data Obtained by Echocardiography and Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy...
Hypertrophic CardiomyopathyCompare data (structural and functional) obtained by transthoracic rest echocardiography and magnetic resonance imaging (with gadolinium) in 30 patients with hypertrophic cardiomyopathy

Risk Stratification in Children and Adolescents With Primary Cardiomyopathy
Primary CardiomyopathyDilated Cardiomyopathy4 moreRIKADA is a prospective study performing systematic family screening including clinical and genetic testing in pediatric patients with primary cardiomyopathy and their first-degree relatives with the aim to facilitate risk stratification.

An Integrative-"Omics" Study of Cardiomyopathy Patients for Diagnosis and Prognosis in China
Dilated CardiomyopathyHypertrophic Cardiomyopathy3 moreThis is a multi-omics research of Chinese cardiomyopathies patients, aiming to determine genetic risk factor and serial biomarkers of cardiomyopathies in diagnosis and prognosis.