The Pretender

Here's a true story:
Helga, 34 years old and mother of three children, had always been a hard-working person, and had started to feel strange of late. It was very difficult to pinpoint her symptoms. She just didn't feel fit. Tired, dizzy, breathing problems… It took her some time to consult a doctor. The ECG, lab test and physical exam were normal. Her doctor sent her to a cardiologist, who performed an echo study. Her heart was “normal” as well. Conclusion: she was a healthy young woman and probably just overworked. Helga was sent home and asked to do more physical exercise.
Looking for alternatives
The problem was that she couldn’t do exercises. So she went to another doctor, and returned with an anxiolytic drug. However, as she didn't approve of pills, she started trying alternative therapies such as acupuncture, herbal medicine, magnet therapy and several diets.
Three years later
Helga was still not feeling better. She was now consulting a psychiatrist after being told that her complaints were purely imaginary.
A very diligent family doctor who examined her for her dizziness finally took action and sent her for a repeat echocardiogram.
Normal or not?

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4-chamber view: Anything that attracts your attention?


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Short-axis view: There is a very subtle abnormality here

The tricuspid regurgitant signal is too weak to measure maximal
velocity. No way you can determine systolic pulmonary pressure


Now take a look at the RVOT /PA signal. What do you conclude
from the shape of the signal? The acceleration time is short
(59 ms).

Pretender or not?
Those of you who have been following our courses and lectures on right heart disease probably realize what the problem is. Helga has pulmonary hypertension. The abnormalities are very subtle indeed but they are present. The right ventricle is rather large; compare it with the left ventricle. The septum shows very subtle systolic flattening. The TR signal does not provide information about pulmonary pressure, but the short acceleration time of the RVOT/PV signal is indicative of pulmonary hypertension.
Next steps:
Helga was put through the usual diagnostic work-up. The diagnosis was confirmed with a right heart catheter. Her pulmonary pressures were 75/30/47 mmHg with a pulmonary vascular resistance of 542 dyn/cm−5. After exclusion of other causes of pulmonary hypertension, her condition was classified as idiopathic PAH.
Can we help?
Yes, there is treatment, especially when the patient is a so-called responder to vasodilators. This is tested during right heart catheterization. Pulmonary pressures dropped after NO application. Helga was given an endothelin antagonist and improved. Her pulmonary pressures declined and her symptoms were clearly improved.
The key messages:

What are the key messages here? Quite a few. First: take “pretenders” (i.e., hypochondriacs) seriously. Look for subtle abnormalities on the echo to diagnose pulmonary hypertension. Second: there is treatment for these diseases, so don’t miss the diagnosis.
And don’t miss our lectures either. They will help you to diagnose conditions that others might have missed. So check out our Masterclass today an learn how you can take your echo skills to the next level.

yours Tommy Binder & the 123sonography team

PS: We have something VERY exciting lined up for you. We are just going to tell you this much - it has to do with echo in the emergency situation. We'll be sending out information about it in the next couple of days. So watch your inbox!!!