Marfan syndrome follow-up question

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Submitted by Dr T on July 6, 2011 – 8:30am

Thank you very much for giving a real response. I am diagnosed with marfan syndrome. and I am also on Beta blockers. I have had my aorta measured a few times and it was around 4.6 cm. I read in your article that people often get surgery when it gets to 5cm. that scares me a little, as my doctor has not even mentioned this to me. Should I ask him about this?

Absolutely. There are a number of issues to consider:

  • Your Beta blocker therapy is insufficient if your heart rate increase to >100 BPM with minimal exercise;
  • At 4.6 cm your Aorta should be fixed now since with further enlargement the risk of rupture and/or dissection will only increase, especially when your present therapy is insufficient;
  • The procedure that is needed is called an Aortic root replacement. If your Aortic valve is normal, and functioning normally, a valve sparing procedure should be considered as the optimal treatment. This is best done in a major medical center by a surgeon experienced with these operations;
  • If you are in good shape, this is a low risk procedure now, vs. when it needs to be done as an emergency;
  • Remember, more than 90% of patients eventually need a cardiac procedure anyway!

Unfortunately, I am not familiar with the Indian health care systems, but I am sure there are several medical centers available where such operations are done with excellent results. It means you will need to do some homework! In the US, there are many hospitals where patients with Marfan syndrome are treated:

Hope this helps,
Dr T

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