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relapsing polychondritis
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relapsing polychondritis

Posted By  CCF-CARDIO MD sc on September 09, 1997 at 17:00:18:

In Reply to: relapsing polychondritis posted by Ellen O. on September 01, 1997 at 15:37:00:

: I understand that relapsing polychondritis can have effects on the heart.
  What kinds of effects are there and how are they best assessed?  Also, can
  this be caused by a vascular injury, or is it always considered an autoimmune
  problem?  My situation is that I have only breathing problems, no outer ear
  or eye problems.  I do have a mild hearing loss, which could come under
  "cochlear dysfunction", one of the criteria for RP diagnosis.  Mainly my
  difficulty is respiratory chondritis.  As for my heart, it sure doesn't feel
  right, but that may be a result of not breathing well.
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Dear  Ellen
Sorry for the delay in answering your  very intriguing question. Polychondrites which most typically involves cartilaginous containing tissues may also involve noncartilagenous containing tissues such as the aorta and the sclera of the eye, organs which have high content of a substance called mucopolysaccharide. The heart appears to be involved in 6-12% of patients with this condition, with some reviews giving the incidence as high as 25%. The most significant manifestation of involvement of the heart in this condition includes aneurysms ( dilatation ) of the aorta ( which is the blood vessels that transports blood away from the heart ), and  leaking or regurgitation  of the aortic valve. The aortic valve lies between the heart at the aforementioned aorta.  The leaking of the valve which may occur in 6%  of patients may even be progressive,  occasionally requiring replacement of the valve itself. The mechanism by which this occurs is probably multifactorial and may be due to the loss of the supporting structures in the aorta, with dilatation of the aortic valve ring and secondary aortic regurgitation.
The systemic nature of the disease may also be associated with significant arterial inflammation with inflammatory aortic aneurysms and systemic vasculites.
Other cardiac conditions that may also occur include degeneration of the mitral valve ( the valve that lies between the upper and bottom chambers on the left side of the heart), pericardites ( inflammation of the lining of the heart) , myocardites ( inflammation of the muscle of the heart ), myocardial ischemia ( due to coronary arterial vasculites ) and rarely block of the conduction system of the heart.
Although this list is very impressive I would like to state that some of these conditions are extremely rare and all that is required on your part is to be seen by your doctor to have a careful clinical examination. If there any heart sounds that suggest leaking of the valves an Echocardiogram may be obtained for better definition of these structures. If you are taken care of by a physician who is well aware of your condition and who understands its possible complications you should not run into any major problems. The key here is to very aggressive in treating any evidence of cardiac involvement of this condition.
Before I end I would also like to alert you to the fact that there have been case reports of valve complications occurring in patients whose polychondrites was totally under control, this reinforces  the importance of careful follow up by  doctor with a high index of suspicion.
I hope you will find this information useful. If you have any further question or would like to be seen by one of our Consultant Rheumatologists and/or Cardiologist here at the Cleveland Clinic please feel free to call 1-800-CCF-CARE for an appointment.
Information provided in the Heart Forum is intended for general medical informational purposes only. Actual diagnosis and
treatment of any particular medical condition can only be made by your family physician(s).  

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