Severe bradyarrhythmia linked to left atrial dysfunction in Fabry disease-A cross-sectional study.

BACKGROUND: Fabry disease (FD) is a lysosomal storage disorder caused by an enzymatic deficiency. Conduction abnormalities and bradyarrhythmias are common and can occur prior to the onset of left ventricular (LV) hypertrophy. We aimed to describe the clinical, electrocardiographic and echocardiographic, including left atrial (LA) function, determinants of bradyarrhythmic events in FD.

HYPOTHESIS: Bradyarrhythmic events are frequent in patients with FD and are associated with LA dysfunction.

METHODS: We designed a cross-sectional study that includes 53 FD patients (mean age, 45 years; 42% male). Clinical characteristics and electrocardiographic and echocardiographic data were collected. LA function was measured using biplane volumes and 2D speckle-tracking echocardiography. Bradyarrhythmic events were defined as pause of more than 2 seconds (sinus pause or atrioventricular block) recorded on Holter, severe bradycardia (≤ 40 bpm on ECG) or implantation of a permanent pacemaker.

RESULTS: Six (11%) patients had installation of a pacemaker, 4 (8%) patients had cardiac pause and 2 (4%) patients had an episode of severe bradycardia. Patients with bradyarrhythmic events were older and had a lower resting heart rate. On echocardiography, a significantly higher LV mass, a lower LV ejection fraction, and a more affected LA reservoir function were found in those with bradyarrhythmic events. Patients also experienced tachyarrhythmias frequently. Atrial fibrillation occurred in 11 (21%) patients and ventricular tachycardia in 4 (8%) patients.

CONCLUSIONS: Bradyarrhythmia are common manifestations of cardiac involvement in FD. Age, LV mass, LV ejection fraction and LA reservoir dysfunction can be useful markers associated with bradyarrhythmia.