Abstract 18398: Malignant Ventricular Arrhythmias in Lamin A/C Cardiomyopathies: Prevalence, Clinical Course and Outcome
Introduction: Genetic mutations in the Lamin A/C (LMNA) genes have been recognized as a possible cause of familial cardiomyopathies (CMP). Ventricular Tachycardias (VT) are frequently associated with LMNA cardiomyopathy, but data is limited on time of occurrence, prevalence of and used
treatment options and their efficacy of these VTs.
The aim of this study is to describe ventricular arrhythmias in LMNA cardiomyopathy
Methods: This was a retrospective cohort study including data from all patients diagnosed with LMNA CMP within the Erasmus MC department of cardiology, a tertiary referral centre. Follow up continued until the patient either got a heart transplantation (HTX) or died.
Results: Twenty patients were identified with LMNA CMP. The follow up time ranged between 14 and 208 months with a median of 67. Malignant VTs were documented in 14 out of 20 (70%) patients. Five patients (25%) had documented non sustained (NS) VT before a diagnosis was made using genetic testing, whereas the remaining VTs occurred 45 ± 54 months during follow up. All patients with documented VT or frequent premature ventricular complexes showed at least 2 or more morphologies with variable coupling intervals. Pharmacological therapy was beta blockade for 80 % of the cohort. Amiodarone was used in 45% of the patients and was discontinued in another 20%. An ICD was implanted in 95% of the patients. Twelve of the 19 (63%) received appropriate therapy. Three patients (15%) underwent radio frequent catheter ablation (RFCA) for a recurrent VT. Two of whom had multiple morphologies. All three patients had VT recurrence showing different and multiple morphologies after 0, 2 and 33 months. Two of these patients died of electrical storm at 0 and 27 months after RFCA and the third received a HTX after 98 months. All patients of the total cohort that showed VT during monitoring had documented recurrences despite different treatment strategies. Five patients (25%) died after a mean of 9 ± 7 years. Three patients (15%) received a HTX.
Conclusions: Patients with LMNA CMP have an increased risk for malignant VT with very limited treatment options. Both medical regimes as well as the few ablations had 100% recurrences. Because the VTs are multiform in nature we would advise taking a reticent stand towards RFCA
Author Disclosures: S. Wijchers: None. F. Akca: None. K. Caliskan: None. A. Constantinescu: None. O. Manintveld: None. T. Szili-Torok: None.
- © 2014 by American Heart Association, Inc.