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Myotonic Dystrophy Type 1 | Electrocardiographic Findings Associated with Sudden Death

Purpose: To evaluate a patient with myotonic dystrophy type 1 for changes in the ECG associated with sudden death as reported by Groh et al.

Description

A patient with myotonic dystrophy Type 1 may be at risk for sudden death. Groh et al identified risk factors associated with sudden death in these patients. The authors are from Indiana University, Pennsylvania State University, University of Rochester, and SUNY Stonybrook.


 

Risk factors for sudden death:

(1) atrial tachyarrhythmia

(2) severe ECG abnormality

 

Severe ECG abnormalities included:

(1) rhythm other than sinus

(2) PR interval >= 240 msec

(3) QRS duration >= 120 msec

(4) second or third degree atrioventricular (AV) block

 

Interpretation:

• minimum number of risk factors present: 0

• maximum number of risk factors present: 2

• The risk for sudden death increases with the number of risk factors.

Number of Risk Factors

Risk of Sudden Death

0

very low

1

low to moderate

2

moderate to high

 


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Specialty

Objective

ICD-10