Nixiao Zhang1, Yanyan Song2, Wei Hua3, Yiran Hu4, Liang Chen5, Minsi Cai4, Hongxia Niu4, Chi Cai4, Min Gu4, Shihua Zhao2, Shu Zhang4. 1. Cardiac Arrhythmia Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China; Department of Cardiology, Cardiovascular Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China. 2. Departments of CMR, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China. 3. Cardiac Arrhythmia Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China. Electronic address: drhuaweifw@sina.com. 4. Cardiac Arrhythmia Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China. 5. Department of Cardiac Surgery, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
Abstract
BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is characterized by a high incidence of ventricular tachyarrhythmia and sudden death. Implantable cardioverter-defibrillator (ICD) implantation is the cornerstone of management. OBJECTIVE: This study aims to reveal the prognostic value of the contrast-enhanced cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) amount in predicting varying lethal outcomes among ACM patients with ICDs. METHODS: The 88 patients with definite ACM who were all referred for contrast-enhanced CMR received an ICD and were followed up for a median of 4.0 years. RESULTS: Fifty-four patients had no left ventricular (LV) involvement and sixteen had an LV LGE amount > 15%. During the follow-up time, appropriate ICD therapy was seen in 57, electrical storm (ES) in 19, and cardiac death in 9 patients. Compared with those without LV involvement, patients with LV LGE amount > 15% had a higher risk of cardiac death (log-rank P = 0.021). LV LGE amount was associated with an increased risk of ICD therapy [adjusted hazard ratio (HR) 1.035, 95% confidence interval (CI) 1.008-1.062, P = 0.010], and cardiac death (adjusted HR 1.082, 95% 1.006-1.164, P = 0.034), independently of LV ejection fraction. LV LGE mass of >15% demonstrated an over 2-fold increase in ICD therapy (adjusted HR 2.180, 95%CI 1.058-4.488, P = 0.035) and an over 7-fold increase in cardiac death (unadjusted HR 7.198, 95%CI 1.399-37.043, P = 0.018) than those without LV involvement, respectively. CONCLUSIONS: The LV LGE-CMR in ACM shows a dose-dependent association with ICD therapy and cardiac death. And LV LGE amount of >15% is a strong predictor.
BACKGROUND:Arrhythmogenic cardiomyopathy (ACM) is characterized by a high incidence of ventricular tachyarrhythmia and sudden death. Implantable cardioverter-defibrillator (ICD) implantation is the cornerstone of management. OBJECTIVE: This study aims to reveal the prognostic value of the contrast-enhanced cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) amount in predicting varying lethal outcomes among ACM patients with ICDs. METHODS: The 88 patients with definite ACM who were all referred for contrast-enhanced CMR received an ICD and were followed up for a median of 4.0 years. RESULTS: Fifty-four patients had no left ventricular (LV) involvement and sixteen had an LV LGE amount > 15%. During the follow-up time, appropriate ICD therapy was seen in 57, electrical storm (ES) in 19, and cardiac death in 9 patients. Compared with those without LV involvement, patients with LV LGE amount > 15% had a higher risk of cardiac death (log-rank P = 0.021). LV LGE amount was associated with an increased risk of ICD therapy [adjusted hazard ratio (HR) 1.035, 95% confidence interval (CI) 1.008-1.062, P = 0.010], and cardiac death (adjusted HR 1.082, 95% 1.006-1.164, P = 0.034), independently of LV ejection fraction. LV LGE mass of >15% demonstrated an over 2-fold increase in ICD therapy (adjusted HR 2.180, 95%CI 1.058-4.488, P = 0.035) and an over 7-fold increase in cardiac death (unadjusted HR 7.198, 95%CI 1.399-37.043, P = 0.018) than those without LV involvement, respectively. CONCLUSIONS: The LV LGE-CMR in ACM shows a dose-dependent association with ICD therapy and cardiac death. And LV LGE amount of >15% is a strong predictor.