Cardiac Care

Infants often require frequent cardiac assessment and management of symptoms. These patients are at risk for cardiomyopathy, cardiomegaly, congestive heart failure, arrhythmias, and cardiac arrest during surgery. Pharmacologic treatment should be based on the stage of cardiomyopathy.[1]  

Caution with Anesthesia[1]

The need for any surgery in infants with Pompe disease should be carefully weighed against the significant risks of anesthesia in these patients. Therefore, anesthesia should be used only when absolutely necessary, and always overseen by experienced pediatric and/or cardiac anesthesiologists.

References

  1. Kishnani PS, Steiner RD, Bali D, et al. Pompe disease diagnosis and management guideline. Genet Med. 2006; 8 (5): 267-288

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Schedule of Assessments

The Pompe Registry has developed a recommended schedule of assessments to help in the care and monitoring of patients with Pompe disease.

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Pompe Registry

Find out about the Pompe Registry, an ongoing, observational database that tracks natural history and outcomes of patients with Pompe disease.

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