Hi,
I was so sorry and concerned when I read about your husband's condition. You mentioned that he was evaluated by a renowned vascular surgeon. It is important that the surgeon specialize specificly in the Marfan's syndrome. Marfan's aortic surgery is very difficult and delicate, and the surgeon must have very specific skills and judgment.There are certain heart centers in the US that specialize in the Marfans syndrome. I would certainly get a second opinion from such a center immediately. The CCF has surgeons that specialize in Marfans surgery, Johns Hopkins, and other centers in the US also, depending on your location. One thing you no doubt have been told is that smoking is very bad for the aortic tissue, and stopping smoking is important. Also, I assume your husband is on a beta blocker of some type. I'm sure this is very difficult for you, and I hope that you can find additional help. Please feel free to contact me at ***@**** if I can help in any way.
My husband has been diagnosed with Marfan Syndrome. He is 52 years old and has clear and evident symptoms of the syndrome. He had an aortic disection Jan 97. Complete from the arch down to the biforcation into the Iliac artery. Its largest diameter
is just at the arch and measures 7cm. Renal, spinal, gastric arties are fed by the false lumen. We have been to a renound vascular surgen and have been told that there is no treatment relative to a graf stent or open surgery because the size of the disection and the Marfan syndrome complication. He has varying degrees of chest pain daily with no relief. He has developed kidney pain just in the last few weeks. I suspect that he has learned to live with substantial discomfort and only reacts when the pain is sharp and constant. I would like to know what to expect with regards to system failure and or the immediate potential of rupture. He is 6'5" tall weighs 255 and carries his weight in his mid-section. He smokes 2 packs of cigerets per day and is quite active. With little regard for his condition. He is leaving tomorrow with our children (all older) for a week on Lake Powell. Will the altitude and heat be a factor.
Any information will be greatly appreciated.
gr8wyfe.
elke,
Thanks for your question. If indeed your shortness of breath is caused by a congential aortic valve disorder, you most likely have a bicuspid aortic valve that is now narrowing (we call this aortic stenosis). Once patients begin to develop symptoms of shortness of breath from aortic stenosis, it is generally recommended that patients have the valve replaced. In large population studies, the average survival rate is approximately 2 years in patients who develop shortness of breath from aortic stenosis. Importantly, elderly age (> 80 yo) is not a reason to not have the valve replaced.
At the current time, there is no medical therapy for aortic stenosis.
If you are living a functional life and are otherwise healthy, you need to see a cardiologist and give strong consideration to having the valve replaced (assuming of course that the aortic stenosis is severe enough to be causing the shortness of breath). Consultation at a large heart center would be optimal.
Hope that helps, and good luck.