Yanhui Li1,2,3, Yumei Xue4, Jianhua Yu5, Chenyang Jiang6, Zulu Wang7, Mackenzi Mbai2, Venkatakrishna Tholakanahalli2,3, David G Benditt3, Jian-Ming Li8,9,10. 1. School of Clinical Medicine, Tsinghua University, No. 1, Tsinghua Yuan, Handian District, Beijing, 100084, People's Republic of China. 2. Division of Cardiology, Minneapolis Veterans Affairs Medical Center, Minneapolis, MN, USA. 3. Cardiovascular Division, University of Minnesota Medical School, Minneapolis, MN, USA. 4. Guangdong Cardiovascular Institute, Guangdong Provincial Key Laboratory of Clinical Pharmacology, Guangdong Academy of Medical Sciences, Guangdong General Hospital, Guangzhou Shi, People's Republic of China. 5. Department of Cardiovascular Medicine, The Second Affiliated Hospital of Nanchang University, Nanchang, 330006, People's Republic of China. 6. Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China. 7. Department of Cardiology, the General Hospital of Shenyang Military Region, Shenyang, People's Republic of China. 8. Division of Cardiology, Minneapolis Veterans Affairs Medical Center, Minneapolis, MN, USA. Lixxx261@umn.edu. 9. Cardiovascular Division, University of Minnesota Medical School, Minneapolis, MN, USA. Lixxx261@umn.edu. 10. VA Medical Center, One Veterans Drive, Minneapolis, MN, 55417, USA. Lixxx261@umn.edu.
Abstract
PURPOSE: This study examined electrophysiological characteristics and outcomes of patients with sustained ventricular tachycardia (VT) in the setting of isolated ventricular noncompaction (IVNC). BACKGROUND: In patients with IVNC, VT has been associated with sudden cardiac death. However, the electrophysiological characteristics and optimal management of these VTs are only incompletely understood. METHODS: This retrospective cohort study assessed arrhythmia characteristics and outcomes in IVNC patients with sustained monomorphic VTs. Data were obtained from five academic centers covering the time period from January 1, 2006, to December 31, 2016, with a median follow-up of 40 months. RESULTS: Eighteen consecutive IVNC patients with sustained VTs (12 males [66%], mean age of 44.4 ± 16.9 years) were enrolled. Seven (39%) patients underwent VT ablation (five males, mean age of 43.3 ± 15.5 years) and nine (50%) patients received ICD therapy. Six of 18 patients (33%) died during a median follow-up of 40 months. Of these, three had ICDs, two had undergone VT ablation, and one had received only antiarrhythmic drugs. Among the seven patients with prior VT ablation, five VTs in the RV (three RVOT and one tricuspid annulus) and two LV VTs (one anterolateral papillary muscle and one inferolateral wall) were localized by 3-D mapping and successfully ablated. In six of seven ablation cases (85.7%), the VTs were distant from the noncompaction zone. VTs appeared to be focal in 57% (4/7) and macro-reentry in 43% (3/7) of patients based on 3-D mapping and entrainment studies. The success rate of VT ablation was 85.7% with one VT recurrence and two deaths during the mean follow-up of 54 (28-115) months. CONCLUSIONS: IVNC Patients with sustained VTs appear to have a poor prognosis despite receiving ICD or apparently successful VT ablation therapy. Further, most VTs appear to arise remote from the noncompaction zone. Whether these VTs were "idiopathic" or related to IVNC was uncertain.
PURPOSE: This study examined electrophysiological characteristics and outcomes of patients with sustained ventricular tachycardia (VT) in the setting of isolated ventricular noncompaction (IVNC). BACKGROUND: In patients with IVNC, VT has been associated with sudden cardiac death. However, the electrophysiological characteristics and optimal management of these VTs are only incompletely understood. METHODS: This retrospective cohort study assessed arrhythmia characteristics and outcomes in IVNC patients with sustained monomorphic VTs. Data were obtained from five academic centers covering the time period from January 1, 2006, to December 31, 2016, with a median follow-up of 40 months. RESULTS: Eighteen consecutive IVNC patients with sustained VTs (12 males [66%], mean age of 44.4 ± 16.9 years) were enrolled. Seven (39%) patients underwent VT ablation (five males, mean age of 43.3 ± 15.5 years) and nine (50%) patients received ICD therapy. Six of 18 patients (33%) died during a median follow-up of 40 months. Of these, three had ICDs, two had undergone VT ablation, and one had received only antiarrhythmic drugs. Among the seven patients with prior VT ablation, five VTs in the RV (three RVOT and one tricuspid annulus) and two LV VTs (one anterolateral papillary muscle and one inferolateral wall) were localized by 3-D mapping and successfully ablated. In six of seven ablation cases (85.7%), the VTs were distant from the noncompaction zone. VTs appeared to be focal in 57% (4/7) and macro-reentry in 43% (3/7) of patients based on 3-D mapping and entrainment studies. The success rate of VT ablation was 85.7% with one VT recurrence and two deaths during the mean follow-up of 54 (28-115) months. CONCLUSIONS: IVNC Patients with sustained VTs appear to have a poor prognosis despite receiving ICD or apparently successful VT ablation therapy. Further, most VTs appear to arise remote from the noncompaction zone. Whether these VTs were "idiopathic" or related to IVNC was uncertain.
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