Daniel Guillen, MD, explains how DMD affects the heart muscle and why cardiac disease often progresses quietly over time. Learn why heart complications may go unnoticed in boys with Duchenne and why ongoing cardiac monitoring is essential.
Why is cardiac involvement often called the ‘silent threat’ in DMD?
Transcript
So we have to remember that the cardiac muscle is a muscle and there is dystrophin expression in it. And the problem with the boys and when they start developing their cardiac symptoms is that they are also starting to lose ambulation at that age.
So you may not notice as much typical cardiac symptoms like you do in adults. You go for a longer walk or you start running. You feel your chest pounding, you’re short of breath. You may have chest pains.
That is not something that necessarily boys with Duchenne are going to notice, because at the time they’re developing the cardiac disease, they are not running around as much. They are not going up and downstairs. They are not doing that level of physical activity.
The other concern is that that may be progressive over time. The cardiac muscle is still going to be contracting and relaxing all the time, regardless of whether we’re exercising or not.
And we do have progression of cardiac muscle disease over the years, with a number of patients even presenting subtle findings before 10 years of age. And I would ask a cardiac colleague this same question, but they tend to develop worsening cardiac diseases. Years go by, and sadly, this seems to be the leading cause of mortality once they become young adults.
