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http://dx.doi.org/10.1161/CIRCEP.124.013144 | DOI Listing |
JACC Clin Electrophysiol
July 2025
Arrhythmogenic Cardiomyopathy Program, Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address:
Background: Diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is often made after arrhythmias are detected in the second or third decade but can also present later.
Objectives: The authors sought to compare the phenotypes and the long-term outcome between patients with early- vs late-onset ARVC.
Methods: Patients with a definite ARVC diagnosis fulfilling the 2010 Task Force criteria and symptomatic arrhythmias at initial presentation were candidates.
Zhonghua Xin Xue Guan Bing Za Zhi
July 2025
Magnetic Resonance Center, Fuwai Hospital, National Center for Cardiovascular Diseases of China, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
To explore the value of cardiac magnetic resonance imaging (CMR) derived left ventricular late gadolinium enhancement (LV LGE) for the primary prevention of malignant ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. This was a single-center retrospective study. Consecutive ARVC patients who underwent CMR at Fuwai Hospital between January 2016 and September 2020, with no history of malignant ventricular arrhythmias at diagnosis, were enrolled.
View Article and Find Full Text PDFJACC Case Rep
July 2025
Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, Alberta, Canada.
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) has late clinical manifestations of right heart dysfunction, chamber dilation, and ventricular dysrhythmias. Elevated right-sided pressure and cardiac remodeling can result in late presentation of right to left interatrial shunting.
Case Summary: A 70-year-old man presented with ventricular tachycardia (VT) storm and significant hypoxemia.
Circulation
August 2025
Division of Cardiology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD (S.A.M., B.A., A.G., R.T.C., C.T., B.M., S.L.Z., H.C., C.A.J.).
Background: Penetrance and risk of ventricular arrhythmias (VAs) in arrhythmogenic right ventricular cardiomyopathy (ARVC) are increasingly recognized as being genotype specific. Therefore, genotype-informed family screening protocols may lead to safer and more personalized recommendations than the current one-size-fits-all screening recommendations. We aimed to develop a safe, evidence-based plakophilin-2 ()-specific longitudinal screening algorithm.
View Article and Find Full Text PDFEur Heart J Open
May 2025
Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Aims: Catheter ablation is commonly performed in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) for management of ventricular arrhythmias (VAs). The impact of catheter ablation on cardiac function is unknown. The aim is to assess the impact of catheter ablation for VAs on cardiac function in patients with ARVC.
View Article and Find Full Text PDF