PQ interval in patients with Fabry disease

Area of Science:

• Cardiology
• Genetics
• Biochemistry

Background:

• Fabry disease (FD) is an X-linked lysosomal storage disorder.
• Cardiac involvement, particularly left ventricular hypertrophy, is common in FD.
• Atrioventricular (AV) conduction abnormalities can also occur in FD.

Purpose of the Study:

• To analyze atrioventricular conduction abnormalities on baseline electrocardiograms in FD patients.
• To investigate the correlation between electrocardiographic findings and echocardiographic parameters in FD.

Main Methods:

• Electrocardiograms (ECGs) of 207 FD patients were analyzed.
• ECG findings were compared with echocardiographic data.
• Analysis included PQ interval, AV block, left ventricular hypertrophy, and atrial size.

Main Results:

• PQ-interval shortening was found in 14% of patients; first-degree AV block in 1.4%.
• No significant echocardiographic differences were observed between patients with or without PQ-interval shortening.
• No correlation was detected between PQ interval and echocardiographic parameters.

Conclusions:

• Shortening of the PQ interval is not a frequent electrocardiographic finding in newly diagnosed FD.
• Baseline ECG findings, specifically PQ interval, do not correlate with echocardiographic parameters or disease stage in FD.