医学
四分位间距
内科学
植入式心律转复除颤器
心房颤动
心脏病学
致心律失常性右心室发育不良
QRS波群
旁道
心脏病
心肌病
心力衰竭
导管消融
作者
Douglas Darden,Jonathan C. Hsu,Wendy S. Tzou,Johannes C. von Alvensleben,Mary Brooks,Kurt S. Hoffmayer,Michela Brambatti,William H. Sauer,Gregory K. Feld,Eric Adler
出处
期刊:Heart Rhythm
[Elsevier]
日期:2021-07-01
卷期号:18 (7): 1194-1202
被引量:5
标识
DOI:10.1016/j.hrthm.2021.03.024
摘要
Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy.The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation.Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected.Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138-1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation.In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP.
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