TY - JOUR
T1 - Malignant Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy with a normal 12-lead electrocardiogram
T2 - A rare but underrecognized clinical entity
AU - Te Riele, Anneline S.J.M.
AU - James, Cynthia A.
AU - Bhonsale, Aditya
AU - Groeneweg, Judith A.
AU - Camm, Christian F.
AU - Murray, Brittney
AU - Tichnell, Crystal
AU - Van Der Heijden, Jeroen F.
AU - Dooijes, Dennis
AU - Judge, Daniel P.
AU - Hauer, Richard N.W.
AU - Tandri, Harikrishna
AU - Calkins, Hugh
N1 - Funding Information:
This study was supported by the funding from the Alexandre Suerman Stipend (to Dr te Riele), the Interuniversity Cardiology Institute of the Netherlands (ICIN) (to Dr Groeneweg and Dr Hauer), the National Heart, Lung, and Blood Institute (grant K23HL093350 to Dr Tandri), the St Jude Medical Foundation, and Medtronic Inc. The Johns Hopkins ARVD/C Program (www.ARVD.com) is supported by the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, and Wilmerding Endowments, and the Dr. Francis P. Chiaramonte Private Foundation.
PY - 2013/10
Y1 - 2013/10
N2 - 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.
AB - 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.
KW - Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
KW - Cardiomyopathy
KW - Concealed
KW - Diagnosis
KW - Electrocardiogram
KW - Riskstratification
KW - Ventriculararrhythmias
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U2 - 10.1016/j.hrthm.2013.06.022
DO - 10.1016/j.hrthm.2013.06.022
M3 - Article
C2 - 23816439
AN - SCOPUS:84884669146
SN - 1547-5271
VL - 10
SP - 1484
EP - 1491
JO - Heart Rhythm
JF - Heart Rhythm
IS - 10
ER -