We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Article |

Electrophysiological Mechanism of the Short PR Interval in Pompe Disease

Paul C. Gillette, MD; Michael R. Nihill, MB, MS, MRCP; Don B. Singer, MD
Am J Dis Child. 1974;128(5):622-626. doi:10.1001/archpedi.1974.02110300032005.
Text Size: A A A
Published online


The pathophysiological cause of the short PR interval usually associated with glycogenosis of the heart (Pompe disease) has not been explained. In an effort to define this abnormality, intracardiac electrocardiography was performed in an 18-month-old child who had histologically confirmed Pompe disease. The low right atrium to His bundle interval (LRA-H), a reflection of atrioventricular nodal conduction time, measured only 40% of the average LRA-H for this age. Treatment of this patient with a glycolytic enzyme lowered the skeletal muscle glycogen level, decreased ventricular electrical forces, and slightly lengthened the PR interval. It is proposed that the excess glycogen caused both the accelerated conduction through the atrioventricular node and the increased QRS forces by a mechanical or chemical action on the myocardial cells.


Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?





Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.


Some tools below are only available to our subscribers or users with an online account.

0 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.