AORTIC ANEURYSM

  I'm a 47 year old male. I had open-heart surgery in 1971 to correct Tetrology of Fallot. I began having problems with ventricular tachycardia in 1984 because of scar tissue from surgery. (Only a few episodes in 13 years.) Nine months ago after an ablation procedure failed to correct the problem, I had a defibrillator implanted. During a routine defibrillator check-up my blood-pressure was high (I've never had high blood-pressure), so the cardiologist scheduled an echocardiogram. This showed an enlargement of the ascending aorta. A subsequent CT scan showed the aneurysm at 6.5 cm. I'm scheduled for a catheterization next week to determine what shape the valve is in. The Dr. is recommending surgery to prevent rupture. I have several questions:  1) Is there any connection between the aneurysm/high blood-pressure and the defibrillator? Could the ICD have caused the aneurysm?  2) Is there any way to determine what caused the aneurysm? (It's not Marfan's.) The cardiologist thinks it may be caused by a hereditary condition even though there

[CCF CARDIO MD APS]

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Dear Todd,
Yes, it would be in your best interest to seek out the most experienced surgeon, that is, experience in difficult operations.  I define you as a difficult surgery because it is certainly not straightforward since #1 your chest has already been opened once which means there will be scar tissue to go through and #2 depending on what was done in 1971 (tetralogy is highly variable in it's manifestations) the surgeon will not have the usual landmarks that s/he has when approaching an uncomplicated ascending aortic aneurysm repair.
As you may know the stakes are pretty high for anyone undergoing this surgery ( there is risk of paralysis and death), however given that hardly anyone survives a rupture of their aorta, surgical correction is the only option, i.e. there is no alternative for an ascending aortic aneurysm.  
So in choosing a surgeon, you not only want one that has done quite a few of there said repairs, you want one who has done plenty of the complicated patients, and there are only a few centers in the country that do enough cardiothoracic surgery to have such surgeons.  The Cleveland Clinic performs some 5000 open heart surgeries per year and being a referral center, a fair amount of those surgeries are complicated, redo operations.  
Just because it (the cause of your dilated aorta) is not Marfans does not mean it can not be inherited, since there are inherited weaknesses of the aortic wall that predispose patients to developing aneurysms.  Your sons can be screened a number of ways, and you should discuss this with their pediatrician as it can be done with an ultrasound and or an imaging procedure like x-ray and or CT scan or MRI.  
There is no connection of the aneurysm with the defibrillator, but there is a probable connection between the aneurysm and the Tetrallogy as well as the hypertension.
The defibrillator has no effect on your risk and or recovery from aneurysm repair, however your prior surgery does effect your risk of repair, but as implied above, the greater the experience of the surgeon performing your repair, the lower your risk.  
Good Luck and write back if you need anything further clarified, meanwhile I will attempt to seek out more US heart hospitals that have the kind of experienced surgeon that you will need.
I hope this information is useful. Information provided in the heart forum is for
general purposes only.  Only your physician can provided specific diagnoses and therapies.
Feel free to write back with further questions. Good luck!
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.