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Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease.
Genet Med 2006; 8(5):313-7GM

Abstract

PURPOSE

Infantile Pompe disease is caused by deficiency of lysosomal acid alpha-glucosidase. Trials with recombinant human acid alpha-glucosidase enzyme replacement therapy (ERT) show a decrease in left ventricular mass and improved function. We evaluated 24-hour ambulatory electrocardiograms (ECGs) at baseline and during ERT in patients with infantile Pompe disease.

METHODS

Thirty-two ambulatory ECGs were evaluated for 12 patients with infantile Pompe disease from 2003 to 2005. Patients had a median age of 7.4 months (2.9-37.8 months) at initiation of ERT. Ambulatory ECGs were obtained at determined intervals and analyzed.

RESULTS

Significant ectopy was present in 2 of 12 patients. Patient 1 had 211 and 229 premature ventricular contractions (0.2% of heart beats) at baseline and at 11.5 weeks of ERT, respectively. Patient 2 had 10,445 premature ventricular contractions (6.7% of heart beats) at 11 weeks of therapy.

CONCLUSION

Infantile Pompe disease may have preexisting ectopy; it may also develop during the course of ERT. Therefore, routinely monitoring patients using 24-hour ambulatory ECGs is useful. Periods of highest risk may be early in the course of ERT when there is a substantial decrease in left ventricular mass and an initial decrease in ejection fraction.

Authors+Show Affiliations

Division of Pediatric Cardiology, Duke University Medical Center, Durham, North Carolina 27710, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16702882

Citation

Cook, Amanda L., et al. "Ambulatory Electrocardiogram Analysis in Infants Treated With Recombinant Human Acid Alpha-glucosidase Enzyme Replacement Therapy for Pompe Disease." Genetics in Medicine : Official Journal of the American College of Medical Genetics, vol. 8, no. 5, 2006, pp. 313-7.
Cook AL, Kishnani PS, Carboni MP, et al. Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease. Genet Med. 2006;8(5):313-7.
Cook, A. L., Kishnani, P. S., Carboni, M. P., Kanter, R. J., Chen, Y. T., Ansong, A. K., ... Li, J. S. (2006). Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease. Genetics in Medicine : Official Journal of the American College of Medical Genetics, 8(5), pp. 313-7.
Cook AL, et al. Ambulatory Electrocardiogram Analysis in Infants Treated With Recombinant Human Acid Alpha-glucosidase Enzyme Replacement Therapy for Pompe Disease. Genet Med. 2006;8(5):313-7. PubMed PMID: 16702882.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Ambulatory electrocardiogram analysis in infants treated with recombinant human acid alpha-glucosidase enzyme replacement therapy for Pompe disease. AU - Cook,Amanda L, AU - Kishnani,Priya S, AU - Carboni,Michael P, AU - Kanter,Ronald J, AU - Chen,Y T, AU - Ansong,Annette K, AU - Kravitz,Richard M, AU - Rice,Henry, AU - Li,Jennifer S, PY - 2006/5/17/pubmed PY - 2006/7/11/medline PY - 2006/5/17/entrez SP - 313 EP - 7 JF - Genetics in medicine : official journal of the American College of Medical Genetics JO - Genet. Med. VL - 8 IS - 5 N2 - PURPOSE: Infantile Pompe disease is caused by deficiency of lysosomal acid alpha-glucosidase. Trials with recombinant human acid alpha-glucosidase enzyme replacement therapy (ERT) show a decrease in left ventricular mass and improved function. We evaluated 24-hour ambulatory electrocardiograms (ECGs) at baseline and during ERT in patients with infantile Pompe disease. METHODS: Thirty-two ambulatory ECGs were evaluated for 12 patients with infantile Pompe disease from 2003 to 2005. Patients had a median age of 7.4 months (2.9-37.8 months) at initiation of ERT. Ambulatory ECGs were obtained at determined intervals and analyzed. RESULTS: Significant ectopy was present in 2 of 12 patients. Patient 1 had 211 and 229 premature ventricular contractions (0.2% of heart beats) at baseline and at 11.5 weeks of ERT, respectively. Patient 2 had 10,445 premature ventricular contractions (6.7% of heart beats) at 11 weeks of therapy. CONCLUSION: Infantile Pompe disease may have preexisting ectopy; it may also develop during the course of ERT. Therefore, routinely monitoring patients using 24-hour ambulatory ECGs is useful. Periods of highest risk may be early in the course of ERT when there is a substantial decrease in left ventricular mass and an initial decrease in ejection fraction. SN - 1098-3600 UR - https://www.unboundmedicine.com/medline/citation/16702882/Ambulatory_electrocardiogram_analysis_in_infants_treated_with_recombinant_human_acid_alpha_glucosidase_enzyme_replacement_therapy_for_Pompe_disease_ L2 - http://dx.doi.org/10.1097/01.gim.0000217786.79173.a8 DB - PRIME DP - Unbound Medicine ER -