Can glycogen storage disease cause tachycardia?

Can Glycogen Storage Disease Cause Tachycardia?

Yes, glycogen storage disease can cause tachycardia, particularly in Pompe disease (GSD Type II), where patients are at high risk of tachyarrhythmia and sudden death due to conduction abnormalities and hypertrophic cardiomyopathy. 1

Cardiac Manifestations by GSD Type

Pompe Disease (GSD Type II) - Highest Tachycardia Risk

Pompe disease presents the most significant risk for tachyarrhythmia among all glycogen storage diseases. 1

• Electrophysiological abnormalities include shortened PR interval on ECG due to glycogen accumulation in specialized conducting tissues 1
• Wolf-Parkinson-White syndrome has been reported in Pompe disease patients, creating additional arrhythmia risk 1
• Hypertrophic cardiomyopathy combined with conduction abnormalities places patients at high risk for tachyarrhythmia and sudden death 1
• Stress situations including infection, fever, dehydration, and anesthesia significantly increase tachyarrhythmia risk 1
• 24-hour Holter monitoring is essential in management to detect arrhythmias 1

GSD Type III (Debrancher Deficiency)

GSD Type IIIa involves cardiac muscle and can develop arrhythmias, though less commonly than Pompe disease. 1

• Cardiac involvement occurs in GSD IIIa (affecting both liver and muscle) but not in GSD IIIb (liver only) 1
• Ventricular hypertrophy can develop, particularly with age, though it doesn't always indicate clinically significant cardiomyopathy 1
• Serial 12-lead ECGs every 2 years are recommended for GSD IIIa patients to monitor for rhythm abnormalities 1
• Additional electrophysiologic monitoring is indicated for patients with palpitations, ECG abnormalities, or moderate-to-severe ventricular hypertrophy 1
• Beta blockers should be used with caution in patients with cardiomyopathy due to potential masking of hypoglycemia symptoms 1

GSD Types I, IV, VI, IX, and XI

These hepatic GSDs do not typically cause direct cardiac involvement or tachycardia. 1

• No primary cardiac manifestations are described in the differential diagnosis tables for these types 1
• Tachycardia in these patients would more likely be secondary to metabolic derangements (hypoglycemia, lactic acidosis) rather than direct cardiac glycogen accumulation 1

Clinical Pitfalls and Monitoring

The key pitfall is assuming all GSDs carry equal cardiac risk—only Pompe disease and GSD IIIa require routine cardiac surveillance for arrhythmias. 1

• For Pompe disease patients: Obtain baseline echocardiography and ECG at diagnosis, with regular follow-up and 24-hour Holter monitoring 1
• For GSD IIIa patients: Baseline echocardiography at diagnosis, repeat every 12-24 months, and serial ECGs every 2 years 1
• For GSD IIIb patients: Baseline echocardiography only, repeat every 5 years, as cardiac involvement is not expected 1
• Symptomatic tachycardia (palpitations) in any GSD patient warrants immediate ECG and potentially Holter monitoring 1