The electrocardiogram in the diagnosis and management of patients with hypertrophic cardiomyopathy

G Finocchiaro, N Sheikh, E Biagini, M Papadakis… - Heart Rhythm, 2020 - Elsevier
In an era of rapid technological development and evolving diagnostic possibilities, the
electrocardiogram (ECG) is living an authentic “renaissance” in myocardial diseases. To …

Targets for therapy in sarcomeric cardiomyopathies

JC Tardiff, L Carrier, DM Bers, C Poggesi… - Cardiovascular …, 2015 - academic.oup.com
To date, no compounds or interventions exist that treat or prevent sarcomeric
cardiomyopathies. Established therapies currently improve the outcome, but novel therapies …

Late sodium current inhibition reverses electromechanical dysfunction in human hypertrophic cardiomyopathy

R Coppini, C Ferrantini, L Yao, P Fan, M Del Lungo… - Circulation, 2013 - Am Heart Assoc
Background—Hypertrophic cardiomyopathy (HCM), the most common mendelian heart
disorder, remains an orphan of disease-specific pharmacological treatment because of the …

Disease modeling of a mutation in α‐actinin 2 guides clinical therapy in hypertrophic cardiomyopathy

M Prondzynski, MD Lemoine, ATL Zech… - EMBO Molecular …, 2019 - embopress.org
Hypertrophic cardiomyopathy (HCM) is a cardiac genetic disease accompanied by structural
and contractile alterations. We identified a rare c. 740C> T (p. T247M) mutation in ACTN 2 …

Clinical phenotype and outcome of hypertrophic cardiomyopathy associated with thin-filament gene mutations

R Coppini, CY Ho, E Ashley, S Day, C Ferrantini… - Journal of the American …, 2014 - jacc.org
Background: Mild hypertrophy but increased arrhythmic risk characterizes the stereotypic
phenotype proposed for hypertrophic cardiomyopathy (HCM) caused by thin-filament …

Altered Ca2+ and Na+ Homeostasis in Human Hypertrophic Cardiomyopathy: Implications for Arrhythmogenesis

R Coppini, C Ferrantini, A Mugelli, C Poggesi… - Frontiers in …, 2018 - frontiersin.org
Hypertrophic cardiomyopathy (HCM) is the most common mendelian heart disease, with a
prevalence of 1/500. HCM is a primary cause of sudden death, due to an heightened risk of …

Abnormalities in sodium current and calcium homoeostasis as drivers of arrhythmogenesis in hypertrophic cardiomyopathy

R Coppini, L Santini, I Olivotto… - Cardiovascular …, 2020 - academic.oup.com
Hypertrophic cardiomyopathy (HCM) is a common inherited monogenic disease with a
prevalence of 1/500 in the general population, representing an important cause of …

Genotype-dependent and-independent calcium signaling dysregulation in human hypertrophic cardiomyopathy

AS Helms, FJ Alvarado, J Yob, VT Tang, F Pagani… - Circulation, 2016 - Am Heart Assoc
Background: Aberrant calcium signaling may contribute to arrhythmias and adverse
remodeling in hypertrophic cardiomyopathy (HCM). Mutations in sarcomere genes may …

[HTML][HTML] Electrophysiological mechanisms underlying T wave pseudonormalisation on stress ECGs in hypertrophic cardiomyopathy

JA Coleman, R Doste, M Beltrami, R Coppini… - Computers in Biology …, 2024 - Elsevier
Background Pseudonormal T waves may be detected on stress electrocardiograms (ECGs)
in hypertrophic cardiomyopathy (HCM). Either myocardial ischaemia or purely exercise …

Identification and Functional Characterization of a Novel CACNA1C-Mediated Cardiac Disorder Characterized by Prolonged QT Intervals With Hypertrophic …

NJ Boczek, D Ye, F Jin, DJ Tester… - Circulation …, 2015 - Am Heart Assoc
Background—A portion of sudden cardiac deaths can be attributed to structural heart
diseases, such as hypertrophic cardiomyopathy (HCM) or cardiac channelopathies such as …