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Having shortness of breath and chest pressure. Am I at the risk of stenosis?

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Practicing since : 1981
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Hi, I have CCA (Beals Syndrome), closely related to Marfans Syndrome. I am having shortness of breath and chest pressure, usually as a result of exertion. Inter tracheal echo 13 years ago showed my aorta on the high side of normal. Due to chest pressure 4 years ago, I had a stress test done, but the symptoms didn't manifest during the test and they said I was fine. I work out 45 mins on the elliptical or bike 5 times a week, but went up a flight of stairs twice this morning and my heart acted up (pressure and strong beat) with severe shortness of breath. I don't have good insurance and am concerned the tests will be inconclusive again. I was told I do not have Mitral valve prolapse or potential aortic expansion/dissection? I have recalled a few more things - I frequently experience a "bounding beat" although it is not always present with chest tightness. I always experience chest pressure if symptoms arise, and usually have shortness of breath. I was gasping for air this morning, although it is not usually that bad. Pressure brought on by exertion is more pronounced. I have noticed that I will sometimes get pressure from caffeine or alcohol, but this is not typical and I never get shortness of breath or extremity tingling with it. I remembered one more thing. I had chest pressure (like my heart was being squeezed) while with my husband last month. There was a little shortness of breath too, but not enough for him to notice. My heart had a lot of difficulty during that time, and I almost had to stop what we were doing. This has not happened before.
Thanks for your consideration.
Posted Sat, 29 Dec 2012 in Hypertension and Heart Disease
Answered by Dr. Anil Grover 5 hours later
Thanks for writing in.
I am a qualified and certified cardiologist and I read your mail with diligence.
Symptoms and signs of Heals Syndrome are like that of Marfan only: both being variant of connective tissue (the tissue that cements the middle layer of vessels or walls). Four years ago you did not have third ventricle so probably you are referring to aorta is the only one which showed some abnormality. Therefore, your fear of aortic dissection or complication like leakage of aortic valve are legitimate. There are standard precautions which a cardiologist advises which are specific to a particular patient but in general these are:
1. Not to indulge in competitive sports for exertion there is beyond one's control.
2. Your blood pressure should always be on the lower side of normal say 110-120 mm Hg Systolic for this at times drugs are prescribed, which keep your heart rate also lower side of normal.
3. As such there is no bar in isotonic exercises like brisk walking even moderate treadmill but either with help of restraint with or without drugs your systolic blood pressure should not go to very high. Isometric exercises like against pressure for example weight lifting are to be avoided.
4. In case of breathlessness or chest pain on activities where it was not there should be reported to cardiologist. In any case an yearly follow up with a cardiologist and echocardiography is recommended.
5. I can reassure that your symptoms do not suggest any catastrophic event however I would recommend you ought to follow up regularly with the cardiologist.

I am not clear what you meant prolapse between second and third ventricle if you can just copy what the doctor has written along with aortic root diameter, ascending aorta diameter I would be wiser in advising you. Meanwhile, I have to urge to see a cardiology for setting up preventive program for you.
I hope that answers some of your questions. Good luck.
If you have any more query I will be most happy to answer it.
Dr Anil Grover,
Cardiologist & Internist
M.B.;B.S, M.D. (Internal Medicine) D.M(Cardiology)
http://www/ WWW.WWWW.WW
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