Ventricular arrhythmias and primary prevention of sudden cardiac death in Anderson-Fabry disease.

ABSTRACT

The Anderson-Fabry disease (AFD) is a X-linked lysosomal storage disorder due to the deficiency in the α-galactosidase A enzyme. Cardiovascular mortality is a major cause of death in patients with AFD and sudden cardiac death (SCD) is one of the main causes of death. The storage of glycosphingolipid along with ionic channel impairment, inflammation and fibrosis are involved in the arrhythmogenesis. Some risk factors have been associated with ventricular tachycardia (VT)/ventricular fibrillation (VF) and SCD. Left ventricular hypertrophy (LVH), cardiac fibrosis, non-sustained VTs seem to be the most important. Older age and male gender might be associated with higher risk of ventricular arrhythmias and SCD. Currently, the implantable cardioverter-defibrillator (ICD) is recommended in patients with AFD who have survived a cardiac arrest secondary to VT/VF or who experienced sustained VT causing syncope or hemodynamic compromise, and have a life expectancy >1 year. ICD implantation is also recommended in patients considered to be at high risk (e.g., patients with severe LVH or fibrosis). The present review sought to summarize the risk of ventricular arrythmias in AFD, the indications for ICD, focusing on pathophysiology and analyzing the role of possible predictors of arrhythmias in preventing SCD, especially as primary prevention.