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Dextrocardia , with Sitis Inversus Totalis
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Dextrocardia , with Sitis Inversus Totalis


     My son who is 2 1/2 years old was diagnosed with Dextrocardia, Sitis Inversus Totalis at the age of 9 months. He appears to be perfectly normal. he has been under the care of Pediatric Cardiologist at the Johns Hopkins Hospital in Baltimore, And University of Maryland Hospital. He has been cleared from having any heart defects. The only reoccurant problem is he has regular sinisitis, this is a indication of Kartageners Syndrome (Cilliary dysfunction). However his hearing is excellent according to the ENT dept. at Johns Hopkins. They feel it is unlikly he has Kartageners Syndrome. To be tested for this syndrome it requires anesthetic and a biopsy of the nose cilia.
     Yes, we have been to see excellent doctors. But none of them specialize in my son's condition. they also dont seem to be very familiar with the syndrome or condition . My Questions are as follows:
  1. Is there a leading authority on my son's condition?
  2. Should we be seeking out another opinion.
  3. Are there specialist in the Condition my son has?
  4. My son has been placed on antibiotics for a long peroid. (2months) is this safe?
  5. If we would determin my son has Kartageners syndrome, would we be treating him differently?
    Thank you very much for your time and response.
                                                   Jim Hovis
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Dear Jim, thank you for your question.  You ask a number of insightful questions that I will answer individually after some explanation.  As you know, Situs Inversus Totalis with dextrocardia can be associated with Kartagener's Syndrome which is characterized by chronic sinusitis, bronchiectasis, and situs inversus (rotation of the abdominal organs).  The cardiac structures usually are normal with this condition although the heart is rotated to the right side of the chest.  Johns Hopkins has a major cardiac center and I'm sure they have excellent pediatric cardiologists, but I will provide you with additional names here at Cleveland Clinic for resources.  Now, your questions:
  1. Is there a leading authority on my son's condition?
Since your situs inversus totalis is a rare condition, I'm not aware of any pediatric cardiologist who specializes in this disorder.  However, our pediatric cardiologists are specialists in all forms of pediatric cardiac disease.  I know they do care for some patients with situs inversus and that they are all excellent physicians.  Dr. Daniel Murphy would be the best pediatric cardiologist to speak with about your son's condition.  Dr. Murphy can be reached at 216-444-9393.
  2. Should we be seeking out another opinion.

I think you should speak with Dr. Murphy first and ask his thoughts about a second opinion. I'm sure he would be happy to evaluate your son, if needed.
  3. Are there specialist in the Condition my son has?
There are no pediatric cardiologists that specialize in situs inversus per se, although there may be researchers who have an interest in this condition.  Again, I think speaking with Dr. Murphy will be most helpful.
  4. My son has been placed on antibiotics for a long period. (2 months) is this safe?
Many children with recalcitrant ear infections take antibiotics for a prolonged period (> 1 month) and do not suffer any adverse consequences.  If your son is tolerating the antibiotics, then the only long-term consequence of 2 months of antibiotics is the theoretical risk that he could develop an infection resistant to the antibiotic being used.  If this were to happen, however, the antibiotics could always be changed.
  5. If we would determine my son has Kartageners syndrome, would we be treating him differently?
The main consequences of Kartagener's syndrome are chronic sinusitis and bronchiectasis (impaired mucociliary clearance of pulmonary secretions that causes chronic bronchitis).  I think it would be important to determine if your son has this condition because if he does, his physicians' may choose to treat him with chronic decongestants or mucolytic agents that help to loosen up nasal and pulmonary secretions.  Again, Dr. Murphy certainly would have an opinion on this subject.
I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies. Please feel free to write back with additional questions.
If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.   The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.





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