Hystiocytoid Cardiomyopathy

Posted By  CCF Cardio MD-SGM on February 02, 1998 at 18:10:47:

In Reply to: Hystiocytoid Cardiomyopathy posted by Gary Marshall on January 16, 1998 at 08:30:54:

: My niece suddenly died, 3 days off 6 months old of this disease, and nobody (even the doctors) seem to know a damn thing about it.
  I'm looking for as much info as possible about this disease (albeit very rare) to help my sister through this very difficult time.
  Apparently the last person to die of this was a little girl, years ago, in Greece.
  Please help.

[CCF Cardio MD-SGM]

Dear Gary,

Histiocytoid cardiomyopathy is a rare cardiac disorder of infancy and childhood that predominantly affects girls under the age of 2 years. The clinical picture is usually dominated by severe cardiac arrhythmias, and sudden death may occur.  Such instances may mimic sudden infant death syndrome (SIDS).  The abnormal cells appear to be transformed cardiac muscle cells that possess some features of the conducting system fibers.  Purkinje cells are now believed to be the origin of this problem.
You may with to visit your medical library and ask the librarian to help you locate the following review on the topic:   Am Heart J 1994 Nov;128(5):1009-21.   It describes the clinical and cardiac morphologic findings in 50 previously reported and 3 new patients with histiocytoid cardiomyopathy.  In addition to the cardiac findings, there was a high prevalence of anomalies involving the nervous system and eyes in this series of patients. The review postulates that this syndrome is likely caused by prenatal myocardial or systemic (viral?) injury. Reference is made to the possible benefit of  surgical excision of these histiocytoid nodules, sometimes resulting in clinical remission of life-threatening remission.  I want to caution you that this review is very technical, thus limiting its helpfulness.  Interestingly, there have been a small but significant number of cases of this disorder.  
I sympathize with your loss.  Hope this information has been helpful.
Information provided in the Medhelp Forum are for general purposes only.  Specific recommendations and therapy can only be provided by your physician.