BACKGROUND: In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG. OBJECTIVES: To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients. METHODS: We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (≥1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG. RESULTS: The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 ± 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed ≥1 TFC on Holter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor). CONCLUSIONS: Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.
BACKGROUND: In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG. OBJECTIVES: To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients. METHODS: We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (≥1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG. RESULTS: The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 ± 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed ≥1 TFC on Holter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor). CONCLUSIONS: Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.
Authors: Jonathan S Steinberg; Niraj Varma; Iwona Cygankiewicz; Peter Aziz; Paweł Balsam; Adrian Baranchuk; Daniel J Cantillon; Polychronis Dilaveris; Sergio J Dubner; Nabil El-Sherif; Jaroslaw Krol; Malgorzata Kurpesa; Maria Teresa La Rovere; Suave S Lobodzinski; Emanuela T Locati; Suneet Mittal; Brian Olshansky; Ewa Piotrowicz; Leslie Saxon; Peter H Stone; Larisa Tereshchenko; Mintu P Turakhia; Gioia Turitto; Neil J Wimmer; Richard L Verrier; Wojciech Zareba; Ryszard Piotrowicz Journal: Ann Noninvasive Electrocardiol Date: 2017-05 Impact factor: 1.468
Authors: Eric D Carruth; Wilson Young; Dominik Beer; Cynthia A James; Hugh Calkins; Linyuan Jing; Sushravya Raghunath; Dustin N Hartzel; Joseph B Leader; H Lester Kirchner; Diane T Smelser; David J Carey; Melissa A Kelly; Amy C Sturm; Amro Alsaid; Brandon K Fornwalt; Christopher M Haggerty Journal: Circ Genom Precis Med Date: 2019-10-22
Authors: Anneline S J M te Riele; Cynthia A James; Neda Rastegar; Aditya Bhonsale; Brittney Murray; Crystal Tichnell; Daniel P Judge; David A Bluemke; Stefan L Zimmerman; Ihab R Kamel; Hugh Calkins; Harikrishna Tandri Journal: J Am Coll Cardiol Date: 2014-07-22 Impact factor: 24.094