Hypertrophic cardiomyopathy with concomitant left ventricular aneurysm is rare and has important clinical implications, including an increased risk of sudden cardiac death. Most patients with this rare combination have obstructive hypertrophic cardiomyopathy, but we treated a 26-year-old woman who had nonobstructive hypertrophic cardiomyopathy and a family history of probable sudden cardiac death. In our patient, coronary angiograms showed distal occlusion of the left anterior descending coronary artery. Late gadolinium-enhanced cardiac magnetic resonance images showed scattered fibrosis within and beyond the left ventricular aneurysm. Precautionary therapy with an implantable cardioverter-defibrillator yielded an uneventful outcome. Cardiac magnetic resonance has emerged as a promising method for diagnosing these aneurysms and detecting associated myocardial fibrosis, thereby enabling patient risk stratification and the determination of appropriate therapeutic options. We discuss the role of cardiac magnetic resonance imaging in the management of this rare clinical entity.
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Eur Heart J Cardiovasc Imaging
January 2025
Heart Institute. Department of Cardiology. Cardiovascular Imaging Unit. Hospital Universitari Germans Trias i Pujol, Barcelona, Spain.
Aims: How the underlying etiology and pathophysiology of left ventricular (LV) hypertrophy affects LA remodeling and function remains unexplored. The present study aims to investigate the influence of various hypertrophic phenotypes on LA remodeling and function.
Methods And Results: Patients with LV hypertrophy who underwent cardiac magnetic resonance (CMR) were compared to a control group.
CJC Open
January 2025
Genetics and Genome Biology, Research Institute, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, Ontario, Canada.
Sudden cardiac death is a leading cause of mortality in children with hypertrophic cardiomyopathy (HCM). The PRecIsion Medicine in CardiomYopathy consortium developed a validated tool (PRIMaCY) for sudden cardiac death risk prediction to help with implantable cardioverter defibrillator shared decision-making, as recommended by clinical practice guidelines. The mplemeting a udden Cardiac Dath isk Assessment ool in hildhood (INSERT-HCM) study aims to implement PRIMaCY into electronic health records (EHRs) and assess implementation determinants and outcomes.
View Article and Find Full Text PDFCPT Pharmacometrics Syst Pharmacol
January 2025
Huashan Hospital, Fudan University, Shanghai, China.
Mavacamten is a cardiac myosin inhibitor for adults with obstructive hypertrophic cardiomyopathy (HCM). Dose optimization is performed 4 weeks after starting mavacamten, guided by periodic echo measurements of Valsalva left ventricular outflow tract gradient (VLVOTg) and left ventricular ejection fraction (LVEF). Previously, a population pharmacokinetic (PPK) model was developed and exposure-response (E-R) of VLVOTg (efficacy) and LVEF (safety) was used to identify the mavacamten titration regimen with the optimal benefit/risk ratio, now included in the US prescribing information.
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