Abstract 2342: ICD Therapy in Noncompaction Cardiomyopathy: Data From the German Left Ventricular Noncompaction Registry (ALKK)
Noncompaction cardiomyopathy (NCCM) is considered to be a primary genetic cardiomyopathy with a high prevalence of heart failure, thromboembolic events, and arrhythmias. AHA/ACC/HRS guidelines 2008 recommend (Type C-level of evidence) ICD implantation as a reasonable strategy to reduce sudden cardiac death in patients (pts) with NCCM; however, it is not clear, which NCCM pts benefit from ICD therapy. To investigate this question we analysed the current data of the German left ventricular noncompaction registry (ALKK) which was initiated 2006.
Results: The registry consists of 88 pts with NCCM, 59 male, 29 female, age 53.9 yrs (20 –78 yrs). During a mean follow up period of 35.6 mths 6 pts died (1 sudden cardiac death, 4 cardiac death, 1 non cardiac death). 17 pts received ICD therapy, 4 pts as secondary prevention following sustained ventricular tachycardia (1 pt with severe LV dysfunction (EF<35%), 3 pts with normal or moderately reduced LV function). In 2 of the 4 pts antitachycardial pacing occurred during follow up. 13 pts received ICD implantation as primary prevention (9 pts with severely reduced LV function, 4 pts with normal or moderately reduced LV function). 2 of the 9 pts with severely reduced LV function exhibited adequate shock therapy whereas no shock delivery was observed in the pts with normal or moderately reduced LV function.
Conclusion: ICD implantation reduces sudden cardiac death in pts with NCCM and severely reduced LV function, whereas in pts with NCCM and normal or moderately reduced LV function ICD implantation as primary prevention should be considered with caution.