Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients.
BACKGROUND
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of ventricular tachycardia (VT) and cardiac arrest in young patients. We hypothesized that treatment with implantable cardioverter/defibrillators (ICDs) is safe and improves the long-term prognosis of ARVC patients at high risk of sudden death.


RESULTS
Sixty patients with ARVC (aged 43+/-16 years) were treated with transvenous ICD systems. Despite a higher number of right ventricular sites tested for adequate lead positions (P<0.05), lower R-wave amplitudes (P<0.001) were achieved in ARVC patients compared with other entities. During follow-up of 80+/-43 months (396 patient-years), event-free survival was 49%, 30%, 26%, and 26% for appropriate ICD therapies and 79%, 64%, 59%, and 56% for potentially fatal VT (>240 bpm) after 1, 3, 5, and 7 years, respectively. Multivariate analysis identified extensive right ventricular dysfunction as an independent predictor of appropriate ICD discharge. Fifty-three adverse events occurred in 37 patients during the perioperative (n=10) or follow-up (n=43) period, mainly related to the leads (n=31 in 21 patients). No lead perforation was observed. Freedom from adverse events was 90%, 78%, 56%, and 42% and freedom from lead-related complications was 95%, 85%, 74%, and 63% after 1, 3, 5, and 7 years, respectively.


CONCLUSIONS
These results strongly suggest an improvement in long-term prognosis by ICD therapy in high-risk patients with ARVC. However, meticulous placement and long-term observation of transvenous lead performance with focus on sensing function are required for the prevention and/or early recognition of disease progression and lead-related morbidity during long-term follow-up of ICD therapy in ARVC.
Department of Cardiology and Angiology, University Hospital of Münster, Germany. wichtet@uni-muenster.de
Thomas
T
Wichter
Matthias
M
Paul
Christian
C
Wollmann
Tayfun
T
Acil
Petra
P
Gerdes
Obaidullah
O
Ashraf
Tonny D T
TD
Tjan
Rasijd
R
Soeparwata
Michael
M
Block
Martin
M
Borggrefe
Hans H
HH
Scheld
Günter
G
Breithardt
Dirk
D
Böcker
D000293:Adolescent; D000328:Adult; D000368:Aged; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D003131:Combined Modality Therapy; D017147:Defibrillators, Implantable / Q000009:adverse effects; D004554:Electric Countershock; D004562:Electrocardiography; D004868:Equipment Failure; D005260:Female; D005355:Fibrosis; D006801:Humans; D007430:Intraoperative Care; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011446:Prospective Studies; D013577:Syndrome; D009626:Terminology as Topic; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000209:etiology / Q000517:prevention & control
D000889:Anti-Arrhythmia Agents