BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy, characterized by right ventricular dysfunction and ventricular arrhythmias. Limited information is available concerning atrial arrhythmias in ARVD/C. OBJECTIVE: The purpose of this study was to characterize spontaneous atrial arrhythmias in a large registry population of ARVD/C patients. METHODS: Patients (n = 248) from the Johns Hopkins ARVD/C registry who met the diagnostic criteria and had undertaken genotype analysis were included. Medical records of each were reviewed to ascertain incidence and characteristics of atrial arrhythmia episodes. Detailed demographic, phenotypic, and structural information was obtained from registry data. RESULTS: Thirty-five patients with ARVD/C (14%) experienced one or more types of atrial arrhythmia during median follow-up of 5.78 (interquartile range 8.52) years. Atrial fibrillation was the most common atrial arrhythmia, occurring in 80% of ARVD/C patients with atrial arrhythmias. Patients developed atrial arrhythmias at a mean age of 43.0 ± 14.0 years. Atrial arrhythmia patients obtained a total of 22 inappropriate implantable cardioverter-defibrillator shocks during follow-up. Older age at last follow-up (P <.001) and male gender (P = .044) were associated with atrial arrhythmia development. Patients with atrial arrhythmias had a higher occurrence of death (P = .028), heart failure (P <.001), and left atrial enlargement on echocardiography (P = .004). CONCLUSION: Atrial arrhythmias are common in ARVD/C and present at a younger age than in the general population. They are associated with male gender, increasing age, and left atrial enlargement. Atrial arrhythmias are clinically important as they are associated with inappropriate implantable cardioverter-defibrillator shocks and increased risk of both death and heart failure.
BACKGROUND:Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy, characterized by right ventricular dysfunction and ventricular arrhythmias. Limited information is available concerning atrial arrhythmias in ARVD/C. OBJECTIVE: The purpose of this study was to characterize spontaneous atrial arrhythmias in a large registry population of ARVD/C patients. METHODS:Patients (n = 248) from the Johns Hopkins ARVD/C registry who met the diagnostic criteria and had undertaken genotype analysis were included. Medical records of each were reviewed to ascertain incidence and characteristics of atrial arrhythmia episodes. Detailed demographic, phenotypic, and structural information was obtained from registry data. RESULTS: Thirty-five patients with ARVD/C (14%) experienced one or more types of atrial arrhythmia during median follow-up of 5.78 (interquartile range 8.52) years. Atrial fibrillation was the most common atrial arrhythmia, occurring in 80% of ARVD/C patients with atrial arrhythmias. Patients developed atrial arrhythmias at a mean age of 43.0 ± 14.0 years. Atrial arrhythmiapatients obtained a total of 22 inappropriate implantable cardioverter-defibrillator shocks during follow-up. Older age at last follow-up (P <.001) and male gender (P = .044) were associated with atrial arrhythmia development. Patients with atrial arrhythmias had a higher occurrence of death (P = .028), heart failure (P <.001), and left atrial enlargement on echocardiography (P = .004). CONCLUSION:Atrial arrhythmias are common in ARVD/C and present at a younger age than in the general population. They are associated with male gender, increasing age, and left atrial enlargement. Atrial arrhythmias are clinically important as they are associated with inappropriate implantable cardioverter-defibrillator shocks and increased risk of both death and heart failure.
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Authors: Andreas Goette; Jonathan M Kalman; Luis Aguinaga; Joseph Akar; Jose Angel Cabrera; Shih Ann Chen; Sumeet S Chugh; Domenico Corradi; Andre D'Avila; Dobromir Dobrev; Guilherme Fenelon; Mario Gonzalez; Stephane N Hatem; Robert Helm; Gerhard Hindricks; Siew Yen Ho; Brian Hoit; Jose Jalife; Young-Hoon Kim; Gregory Y H Lip; Chang-Sheng Ma; Gregory M Marcus; Katherine Murray; Akihiko Nogami; Prashanthan Sanders; William Uribe; David R Van Wagoner; Stanley Nattel Journal: J Arrhythm Date: 2016-07-11
Authors: Larissa Fabritz; Eduard Guasch; Charalambos Antoniades; Isabel Bardinet; Gerlinde Benninger; Tim R Betts; Eva Brand; Günter Breithardt; Gabriela Bucklar-Suchankova; A John Camm; David Cartlidge; Barbara Casadei; Winnie W L Chua; Harry J G M Crijns; Jon Deeks; Stéphane Hatem; Françoise Hidden-Lucet; Stefan Kääb; Nikos Maniadakis; Stephan Martin; Lluis Mont; Holger Reinecke; Moritz F Sinner; Ulrich Schotten; Taunton Southwood; Monika Stoll; Panos Vardas; Reza Wakili; Andy West; André Ziegler; Paulus Kirchhof Journal: Nat Rev Cardiol Date: 2015-12-24 Impact factor: 32.419
Authors: Ka Hou Christien Li; George Bazoukis; Tong Liu; Guangping Li; William K K Wu; Sunny Hei Wong; Wing Tak Wong; Yat Sun Chan; Martin C S Wong; Katharina Wassilew; Vassilios S Vassiliou; Gary Tse Journal: J Arrhythm Date: 2017-12-21