Reasonable schedule for serial scans of my aneurysms

[Shasari]

Hi there,

I'm 48 years old, and have been dealing with chronic dull aching pain in my chest for the past 3 years.  It was a night of pain on a 6-7 scale that sent me to the ER, ultimately resulting in the diagnosis of having two aneurysms - one on the ascending aorta of 4.2cm, and one on the pulmonary artery of 4.4cm.

I've done a lot of research, and recently had a follow-up echo after 2 years where my health provider was not scheduling or looking.  I think that's probably not the best course of action, given my condition, and the fact that I present with chronic pain behind my breastbone (sternum), and have mitral regurgitation, and dilation of the aortic root, also.

So, my question is, given my age and the size of the aneurysms (I realize they are on the low end of the risk scale at the present time), and the symptoms I experience (pain, frequent heavy PVC's, shortness of breath, dizziness), what would you advise for a proper and reasonable schedule for serial examinations, and what type is going to be the most accurately revealing - cost issues aside.  Echocardiogram, CT (though I'd rather not have repeated ones due to the xray exposure) or MRI?

How frequently should I be having them done?  every 6 months, every year or can it go longer with a good margin of safety?  I am not panicking, but I am concerned something will cause the aorta or pulmonary artery to enlarge sufficiently to be a serious issue in the intervening time, and I do admit it worries me.

Thanks for your time

[CCFHeart MD23]

Hi Shasari,

The first piece of the puzzle in your case is: What is the cause of the ascending aorta and pulmonary artery aneurysms? This is an important question because the underlying etiology can determine the rate at which your aneurysms enlarge and also the risk of complications. Aortic dissection is the main complication we want to avoid due to the risk of sudden cardiac death. Interestingly, pulmonary artery aneurysms are less prone to dissection/rupture and this is thought to relate to lower right sided heart pressures.

If you have not yet seen a doctor with expertise in "aortopathies" I would recommend that you do. Aortopathies encompasses a broad range of conditions including Marfan Syndrome, Loeys-Dietz Syndrome & Familial thoracic aneurysms among other things. The increasing recognition of underlying genetic predispositions to aortic aneurysms have meant that in addition to measuring aortic (& pulmonary artery) dimensions, many heart specialists are also referring patients to clinical geneticists to investigate this possibility.  

Moving on to your question regarding which test: Echo is useful because it is quick, non-invasive and not associated with radiation. However it only shows us the ascending aorta and we don't always see the pulmonary artery clearly for it to be useful tracking pulmonary artery aneurysms.

CT or MRI offer accurate assessments of the entire aorta and pulmonary artery and are the best modalities for following your aneurysms. You are correct that CT carries the risk of radiation exposure. However the risk of radiation-related malignancy is highest in children and adolescents exposed at a young age and over a long period of time. If the risk of radiation is a major concern (and that is reasonable) then MRI would be the best modality for serial imaging of your aorta and pulmonary arteries (in the absence of contraindications such as significant kidney disease / metal implants / pacemaker / claustrophobia).

Regarding frequency of studies: the 2010 Journal of American College of Cardiology Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease recommend annual imaging in patients with Marfan Syndrome who are at high risk for progressive dilation and aortic dissection. In patients without Marfan Syndrome but with newly diagnosed thoracic aortic (& pulmonary artery) aneurysms it would be reasonable to image annually for the first 2-3 years and then decide on frequency according to how stable the size of the aorta and pulmonary artery are. If you had a family history of aortic dissection or had future evidence of rapid increase in aneurysm size, repeat imaging may increase in frequency (i.e. every 6 months).

I am including a link to the Cleveland Clinic aortic disease management group in case you are interested in their approach to aortopathies:
http://my.clevelandclinic.org/heart/webchat/1452_aorta-disease-including-marfan-and-connective-tissue-disorders.aspx

I hope this information is helpful.

[Shasari]

Thank you for your very informative reply.  It's given me a lot to think about.  Trying to work with my current cardiologist and get tested for marfan's, but she seems reluctant to dig in and search for the root cause, so we shall see how that goes.  Might try to get an out of network referral to Stanford U.  Wish I was closer to the Cleveland Clinic, you folks have been far more responsive to my questions than my current provider.

[Shasari]

I also realize that just having the aneurysms is a serious issue in and of itself, so any advice you can give me for the above questions will be much appreciated.

Thank you for your time,

~David