2: Redo of TGA and Generational frequency of Congenital defects

[Janine-Hampton]

  I have a marvelous 14 year old son, Kenny, who was born with a
   pretty straight forward case of TGA -
  Transposition of the Greater Vessels - in 1984. He had a Senning
  procedure at the age of three weeks, performed by Dr's Turley and Ebert
  at Moffit Hospital (UCSF) in San Franciso; the vessels were of too
  great a size differential to attempt our first choice, a switch (in
  those olden days the plumbing problems seemed to have played a much
   larger part in the possibilty of success. But you know that.)
  Mostly, he's done just fine - but -
   He's having a few rhythm
  anomolies and his cardiologist, Dr Culberson at Oakland's Children's
  Hospital has mentioned -sort of in passing -the fact that
  cardiologist redo these. Sometimes...
  First round of questions:
  How are kids/adults with shunt work (Mustard/Senning) faring as a
  general rule?
  I mean massive heart failure rates. Kenny could live, and live well,
  at the level of his quality of life now.(His little league
  team made all stars. Baseball, the sport of  heart patients and John Kruk.)
  What's the success rate with the redo?
  What are the criteria that determine when it is done?
  How does one tilt the windmills of managed health care if
  the procedure becomes a necessary evil?
  Next round of related but sufficiently different questions:
  Kenny's grandfather (my father) died of a massive coronary 30 years ago at
   the age of 42, even though he watched his cholesterol before it
  became fashionable, kept his weight down, and exercised . Let me add that
  he was a Dr. of Internal medicine and was playing tennis with a
  cardiologist at the time of his heart attack.
  Family legend is that he had an undetected congenital heart defect.
  I believe it was a thin or narrow vessel??(Aorta?)I don't know.
  Kenny's mom - that's me, Janine, - is a 41 year old woman. My cholesterol
  (Ignor my poor spelling. I do.)is obscene,
   as it is in all of my siblings, though I watch my diet and eschew
   meat...I'm active, but could lose 15 pounds and be happy about it.
  Okay, I could lose 25 and be exstatic.
  Should I worry, or do congenital defects skip a generation?
  Have I foolishly avoided tennis all this time for no reason?
  Grampa and kid's defects are not the same,and are probably just
   coincidental...right?
  Real question: how much pressure should I put on my reluctant primary
  doctor for a cardiac check-up if I am asymptomatic? (Let me add
  that my father also showed NO symptoms until he died. Hell of a way
  to find out)
  If you are feeling particularly like Sherlock Holmes or Kinsey Milhone,
  let me tell you that my cousin
  Dr. Diana Morledge, past ? present? participator in Cleveland's impressive
  Public Health buracuracy knew my father as her Uncle Stan Hampton.
   I believe she knows exactly what congenital defect he had.
   However, over the years I have lost touch with her,
   and found this great site while trying to locate her.
    
  I hope I have not gone on too long, but I adore my son (Usually.
  He IS a teenager.) and worry for myself as I reach that frightening age of
  42.
  Thank you.
  Janine Hampton
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Dear Janine
The arterial switch operation is now the procedure of choice. The Mustard and Senning procedures reroute the blood flow at the level of the atria. This results in some degree of disruption of the heart's normal electrical conduction system. This is why patients that have had the Mustard or Senning procedures have a high incidence of arrhythmias. This is avoided with the arterial switch. The other major advantage of the arterial switch is that the left ventricle is restored as the heart's main pumping chamber, which is what it was designed to do. Thus the major problem we see, somewhat commonly, with patients that had the Mustard or Senning is arrhythmias. These are often managed successfully with drugs; sometimes it is necessary to use catheters to try to eliminate the abnormal rhythms. Heart failure can be a problem if the right ventricle does not adapt to its new role as the heart's main pumping chamber; many times it can make the adjustment. As far as an arterial switch later in life, that depends on the presence of associated pulmonary vascular disease, other concomitant congenital heart defects, the status of the coronary arteries, and a number of other factors. You should ask your son's pediatric cardiologist about your son's specific situation.
It is impossible to say with certainty whether you do or don't have something wrong with your heart. Certainly, you should work on controlling your risk factors for coronary artery disease, as this is more likely a cause of sudden death in a middle-aged male who was feeling well. You should discuss your concerns with your doctor. It is unlikely that he would bar you from seeing a specialist if this were really indicated. If he does not refer you to a cardiologist, but you feel strongly about being evaluated, you can always make an appointment with a cardiologist outside of your HMO.
I hope this is useful. Feel free to write back. I wish you the best of luck.
If you would like to set up an appointment with one of our cardiologists here at the Cleveland Clinic please feel free to call 1-800-CCF-CARE.
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 physician(s).