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Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy, characterized by abnormal cell adhesions, disrupted intercellular signaling, and fibrofatty replacement of the myocardium. These changes serve as a substrate for ventricular arrhythmias, placing patients at risk of sudden cardiac death, even in the early stages of the disease. Current echocardiographic criteria for diagnosing arrhythmogenic right ventricular cardiomyopathy lack sensitivity, but novel markers of cardiac deformation are not subject to the same technical limitations as current guideline-recommended measures. Measuring cardiac deformation using speckle tracking allows for meticulous quantification of global systolic function, regional function, and dyssynchronous contraction. Consequently, speckle tracking to quantify myocardial strain could potentially be useful in the diagnostic process for the determination of disease progression and to assist risk stratification for ventricular arrhythmias and sudden cardiac death. This narrative review provides an overview of the potential use of different myocardial right ventricular strain measures for characterizing right ventricular dysfunction in arrhythmogenic right ventricular cardiomyopathy and its utility in assessing the risk of ventricular arrhythmias.
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http://dx.doi.org/10.3390/jcm13030717 | DOI Listing |
Stem Cell Res
September 2025
Department of Cardiology, Affiliated Hospital of Jining Medical University, Shandong, China; Shandong Provincial Key Medical and Health Discipline of Cardiology Affiliated Hospital of Jining Medical University, Shandong, China; Key Laboratory of Cell and Biomedical Technology of Shandong Province, C
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary infiltrative cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium, which may extend to the left ventricle in the advanced stages. Clinically, the condition is commonly associated with right ventricular dilation, malignant arrhythmias, and an increased risk of sudden cardiac death. In this study, we successfully established induced pluripotent stem cell (iPSC) lines from peripheral blood mononuclear cells of ARVC patients carrying a heterozygous LMNA gene mutation (c.
View Article and Find Full Text PDFJACC Case Rep
September 2025
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Background: Cardiac sarcoidosis (CS) usually affects the left ventricle and presents with nonspecific features like conduction abnormalities and ventricular arrhythmias. However, right ventricle (RV)-dominant involvement has been increasingly reported, making diagnosis difficult.
Case Summary: A 55-year-old man presented with palpitations.
Cureus
August 2025
Department of Internal Medicine, Jersey City Medical Center, Jersey City, USA.
An electrical storm (ES) represents one of cardiology's most formidable and life-threatening crises, marked by relentless ventricular arrhythmias within a 24-hour period. While stimulant cardiotoxicity is an escalating concern, the devastating role of methamphetamine in triggering refractory ES and its deleterious outcomes in advanced cardiomyopathy, particularly within the critical care setting, remains profoundly underreported and poorly understood. We present the urgent case of a 44-year-old male with end-stage dilated cardiomyopathy and chronic, heavy methamphetamine abuse, who spiraled into incessant ventricular tachycardia (VT) storm following acute methamphetamine use.
View Article and Find Full Text PDFCirculation
September 2025
Cardiology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Italy (M.P.M).
Cardiac adipose tissue is normally present in the epicardium, but a variable amount can also be present in the myocardium, particularly in the subepicardial regions of the right ventricular anterolateral and apical regions. Pathological adipose tissue changes may occur in both ischemic (previous myocardial infarction) and nonischemic (previous myocarditis, arrhythmogenic cardiomyopathy, lipomatous hypertrophy of the interatrial septum, cardiac lipomas and liposarcomas) conditions, with or without extensive replacement-type myocardial fibrosis. Cardiac magnetic resonance is the gold standard imaging technique to characterize myocardial tissue changes and to distinguish between physiological and pathological cardiac fat deposits.
View Article and Find Full Text PDFFront Physiol
August 2025
Department of Electrophysiology, King Abdulaziz Cardiac Center, King Abdullah International Medical Research Center (KAIMRC), MNGHA, King Abdulaziz Medical City, Riyadh, Saudi Arabia.
Background: Mitral valve prolapse (MVP) is a common condition, typically benign, but in a small subset of patients, it may lead to life-threatening arrhythmias and sudden cardiac death (SCD). This arrhythmogenic MVP phenotype is often associated with bileaflet prolapse, mitral annular disjunction (MAD), and myocardial fibrosis identified via late gadolinium enhancement (LGE) on cardiac MRI.
Case Summary: Our patient is a 49-year-old man presented with monomorphic ventricular tachycardia and near-syncope.