Glycogen storage disease type IV
ICD-10 E74.0 ICD-11 5C51.3.4

Treatment of Glycogen Storage Disease Type IV in Hypertrophic Cardiomyopathy (Without Dilated Cardiomyopathy)

This protocol addresses the management of Glycogen storage disease type IV (GSD IV) in patients presenting with hypertrophic cardiomyopathy in the absence of dilated cardiomyopathy — a clinically distinct cardiac phenotype that shapes the treatment approach.

Clinical Scenario

The patient has confirmed GSD IV with hypertrophic cardiomyopathy (HCM) and no concurrent dilated cardiomyopathy. This specific cardiac presentation defines the therapeutic context and influences the choice of intervention.

Treatment Approach

When cardiomyopathy in GSD IV proves refractory to medical management, a surgical intervention may be considered as the next step in care.

Full regimen, sequencing, and eligibility criteria available in the complete protocol →
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References

  1. For individuals with HCM, use of β1-selective β-blockers, such as metoprolol, is reasonable. DOI: 10.1016/j.ymgme.2023.107525
  2. For cases of GSD IV with cardiomyopathy refractory to medical management, surgical intervention, including mechanical support and cardiac transplantation, can be considered. DOI: 10.1016/j.ymgme.2023.107525
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