Petrified Heart

A disabling condition
Her height was a mere 154 cm. She was unable to even unbutton her blouse, crippled in a wheel chair. We had to lift her onto the bed and help her undress in order to perform an echo. Despite her disability she was cheerful and full of hope. Maria was diagnosed with systemic onset juvenile arthritis (Still´s disease) in her childhood. She was now 24 years old, and unable to live an independent life. Why did we perform an echo? Because she had chest pain. I was quite convinced her rather atypical pain had nothing to do with the heart. It was obviously associated with her spondylitis and osteoporosis, the latter caused by prolonged glucocorticoid therapy. Indeed, there were no regional wall motion abnormalities on the echo. What I found was something I had never seen before. Have you?
 
An unexpected finding
The aortic valve showed focal calcifications. You might say this is not unusual, but remember that the patient was a mere 24 years old.
 
The aortic valve is partly calcified.  
I initially thought this was related to a bicuspid valve, but as far as I could judge the aortic valve had three cusps. The opening of the valve was reduced. Looking at the gradients, it was quite obvious that the valve was stenotic.
 
Mild to moderate aortic stenosis with a mean gradient of
20 mmHg (maximum gradient, 35 mmHg).  
So far so good. But there was more: here is a parasternal short-axis view showing significant mitral annular calcification. A very unusual finding in a relatively young patient.
 
Mitral annular calcification.  
Mitral annular calcification – so what?
Let us take a closer look at the mitral annular calcification. Here is the apical long-axis view:  is this simple mitral annual calcification? Not really. See how it extends deep into the myocardium, even affecting parts of the basal posterior lateral wall. The acoustic shadow caused by the massive calcification is very evident.
 
Calcification extending into the myocardium  
Now look at the four-chamber view. Calcifications all over the place: on the lateral wall, in parts of the interventricular septum (at the base), at the apex, and extensively in the right ventricular free wall.
 
 
 
Calcification of the right ventricular free wall.
  A closer look at function
I would say this patient's heart is gradually turning into a rock. Like petrified wood. Does this affect myocardial function? She already has mild aortic stenosis, which at this point does not need any form of treatment. But what about myocardial function? I'm sure you'll agree that her ejection fraction is normal. However, is her left ventricular function really in good order? Here is the speckle tracking analysis: 
      The patient's global longitudinal strain is normal, but reduced
in the basal lateral posterolateral segment.  
The patient's global longitudinal strain (which represents the average strain of all segments) is normal. However, longitudinal function is reduced in specific regions.  These are the areas of lighter colors on the bull's eye view.  Which segments show impaired regional longitudinal function?  Exactly those that are most severely calcified: the basal posterior, lateral, and inferior wall.  While this finding will not alter the medical decision, and while the reliability of speckle tracking in the setting of massive calcifications is not entirely clear, the finding is certainly interesting in that it indicates the potential of speckle tracking imaging.
 
Why all this?
Do these intracardiac calcifications have anything to do with Maria's underlying problem? I believe they do. As mentioned earlier, patients with juvenile arthritis are at high risk of developing osteoporosis. Maria received high doses of corticosteroids. To treat her osteoporosis she also received rather high doses of calcium supplementation.
 
Calcium is no good
We have an increasing body of evidence to show that high-dosed calcium supplements not only lead to calcification of the coronary arteries (and increase the risk of coronary artery disease), but also affect the large vessels and the heart valves. Patients with secondary hyperparathyroidism, with disturbed calcium metabolism and renal failure, are known to develop severe mitral annular calcification. Besides, they are subject to the risk of developing aortic stenosis, with accelerated degeneration of bioprosthetic valves. While little is known about calcium supplementation and intracardiac calcifications, I assume this was the cause of Maria's cardiac abnormalities. Further workup of the patient revealed calcifications in the aorta as well. I guess we'll have to keep a close watch on her, check for relevant coronary artery disease, and monitor her valves.
 
A rare condition from which there is much to learn.  What can we learn from this case? 

1. Advances  in ultrasound imaging technology now allows us to look at the (myocardial)  tissue composition. I we can really visualize fibrosis calcification and determine the extent.
2. Systemic factors, drugs, and supplements can really affect the heart.
3. Finally, new technologies such as speckle tracking imaging might help to investigate such abnormalities.

 
Have you ever seen anything similar? What do you think about it? Share your thoughts with us and our growing community. Post your comments below ask us as at one of our Live events

Best,
Thomas Binder      
 
PS: If you want to see other interesting case stories, here's a selection you might like:
 
The Holy Grail – a critical view of left ventricular function
 
Elementary Watson – How blood pressure affects valvular function

Distrust – The natural course of severe mitral regurgitation

Peeling the heart – dealing with constrictive pericarditis