Advances in the Diagnosis and Management of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by ventricular arrhythmias, right ventricular dysfunction, and sudden cardiac death. Since the first description of ARVD/C in 1982, there have been major advances in the diagnosis and management of the disease. For instance, the discovery of desmosomal abnormalities as a genetic basis for ARVD/C; the importance of proband status and ventricular ectopy for risk stratification of patients at risk for sudden cardiac death; and the critical role that exercise plays in the development and progression of ARVD/C, just to name a few. From a treatment perspective, the placement of implantable cardioverter defibrillators in those at risk for sudden cardiac death and ablation techniques have also evolved over time. In 2010, an update of the 1994 Task Force Diagnostic criteria for ARVD/C was published with the hope to increase diagnostic sensitivity. This update incorporates new knowledge and technology to assess structural cardiac abnormalities and is the standard for diagnosis today.
Division of Cardiology, Johns Hopkins University, Baltimore, MD, USA.|Johns Hopkins ARVD/C Program, Blalock 545, 600 N. Wolfe Street, Baltimore, MD, 21287, USA.
Gabriela M
GM
Orgeron
Division of Cardiology, Johns Hopkins University, Baltimore, MD, USA. hcalkins@jhmi.edu.|Johns Hopkins ARVD/C Program, 1800 Orleans Street/Zayed Tower 7125R, Baltimore, MD, 21287, USA. hcalkins@jhmi.edu.
Hugh
H
Calkins
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D017115:Catheter Ablation / Q000379:methods*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017079:Exercise Tolerance; D000072696:High-Intensity Interval Training / Q000009:adverse effects*; D006801:Humans; D016896:Treatment Outcome
D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D000889:Anti-Arrhythmia Agents
Arrhythmogenic right ventricular dysplasia/cardiomyopathy; Implantable cardioverter defibrillator; Sudden cardiac death; Ventricular arrhythmia