Hypertrophic Cardiomyopathy

2022 | Clinical Characteristics and Prognostic Importance of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy

150 150 sabrina.agostini@uhn.ca

Short Description: Left ventricular (LV) apical aneurysms in hypertrophic cardiomyopathy (HCM) are a recognized risk marker for adverse cardiovascular events. There is variable practice among clinicians and discordance between international guidelines regarding treatment recommendations and prognostication for this important phenotype. The authors sought to describe the morphology, clinical course, and risk of adverse events in a large single-center cohort of HCM patients with LV apical aneurysms.
Interest: Cardiac Imaging, Hypertrophic Cardiomyopathy, Medical Imaging
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2022 | Temporal Changes in Cardiac Morphology and Its Relationship with Clinical Characteristics and Outcomes in Patients with Hypertrophic Cardiomyopathy

150 150 sabrina.agostini@uhn.ca

Short Description:  In this study, we aimed to assess a large cohort of nonapical hypertrophic cardiomyopathy (HC) patients who have undergone 2 serial cardiac magnetic resonance studies to examine morphological dynamics and their correlation to patient characteristics and clinical outcomes. A total of 214 patients with nonapical HC were enrolled in this study, with 2 sequential cardiac magnetic resonance studies separated by a mean interval of 4.8 ± 2.1 years.
Interest: Hypertrophic Cardiomyopathy
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2022 | Importance of newer cardiac magnetic resonance–based risk markers for sudden death prevention in hypertrophic cardiomyopathy: An international multicenter study

150 150 sabrina.agostini@uhn.ca

Short Description: The sudden death (SD) risk stratification algorithm in hypertrophic cardiomyopathy (HCM) has evolved, underscored recently by novel cardiac magnetic resonance (CMR)–based risk markers (left ventricular apical aneurysm, extensive late gadolinium enhancement, and end-stage disease with systolic dysfunction) incorporated into the 2020 American Heart Association (AHA)/American College of Cardiology (ACC) HCM guidelines. The purpose of this study was to assess the specific impact of newer, predominantly CMR-based risk markers in a large multicenter HCM population that underwent primary prevention implantable cardioverter-defibrillator (ICD) implants.
Interest: Cardiac Imaging, Cardiac Magnetic Resonance Imaging, Hypertrophic Cardiomyopathy, Medical Imaging, Risk Stratification, Sudden Death
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2021 | Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy: Equivalent Detection by Magnetic Resonance Imaging and Contrast Echocardiography

150 150 sabrina.agostini@uhn.ca

Short Description: Left ventricular (LV) apical aneurysm is a unique morphological entity and novel adverse risk marker existing within the broad phenotypic spectrum of hypertrophic cardiomyopathy (HCM). Its true prevalence in the HCM population is likely underestimated because of inherent limitations of conventional noncontrast echocardiography. The authors hypothesized that contrast echocardiography is a reliable imaging technique compared with cardiovascular magnetic resonance (CMR) for the detection of apical aneurysms. The aim of this study was to assess the effectiveness of contrast echocardiography in the detection of LV apical aneurysms in patients with HCM in comparison with the gold standard, CMR.
Interest: Cardiac Imaging, Cardiac Magnetic Resonance Imaging, Hypertrophic Cardiomyopathy, Left Ventricular Apical Aneurysm, Medical Imaging
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2021 | Development and Validation of a Clinical Predictive Model for Identifying Hypertrophic Cardiomyopathy Patients at Risk for Atrial Fibrillation: The HCM-AF Score

150 150 sabrina.agostini@uhn.ca

Short Description: Atrial fibrillation (AF) is the most common sustained arrhythmia in hypertrophic cardiomyopathy (HCM), associated with impaired quality of life, risk for embolic stroke, and unpredictable onset. We sought to create a predictive model to identify risk for AF development in HCM.
Interest: Atrial Fibrillation, Hypertrophic Cardiomyopathy
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2021 | Progression of Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Cardiac Magnetic Resonance Study

150 150 sabrina.agostini@uhn.ca

Short Description: Myocardial fibrosis, as quantified by late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR), provides valuable prognostic information in patients with HCM. This study examined fibrosis progression in hypertrophic cardiomyopathy (HCM) patients, as well as its relationship to patient characteristics, clinical outcomes, and its effect on clinical decision making.
Interest: Cardiac Imaging, Cardiac Magnetic Resonance Imaging, Hypertrophic Cardiomyopathy, Medical Imaging
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2020 | Genetic Testing for Diagnosis of Hypertrophic Cardiomyopathy Mimics: Yield and Clinical Significance

150 150 sabrina.agostini@uhn.ca

Short Description: Genetic testing is helpful for diagnosis of hypertrophic cardiomyopathy (HCM) mimics. Little data are available regarding the yield of such testing and its clinical impact. The HCM genetic database at our center was used for identification of patients who underwent HCM-directed genetic testing including at least 1 gene associated with an HCM mimic (GLA, TTR, PRKAG2, LAMP2, PTPN11, RAF1, and DES). Charts were retrospectively reviewed and genetic and clinical data extracted.
Interest: Fabry Disease, Genetic Testing, Genetics, Hypertrophic Cardiomyopathy
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