Marfans and Dilation of Aorta to 5.5cm

[DimDon]

*The character limit is half what it is at the other forums so I will have to break this post up into two messages (one with history, one with questions).  I apologize for any inconvenience but the history is important for my questions.
I am a 6

[CCF-M.D.-RCJ]

DimDon,

Thanks for the post.

There is some controversy about exactly how much aortic root dilation demands surgery.  The Working Group on Valvular Heart Disease of the European Society of Cardiology released guidelines in 2002. The threshold for elective surgery for aortic root dilatation in patients with MFS was 50 mm, particularly if there has been a rapid increase in aortic root diameter or if a valve-sparing operation is possible.

However, the decision to operate needs to be made on a case by case basis, and should be made by a specialist in Marfan syndrome.

UF CT versus regular CT is important only if the particular institution is skilled at both.  For example, at the Cleveland Clinic, we often prefer CT over MRI because of the preference of one of our expert radiologists.  This decision is not true at other places.

I cannot say what the impact of the delay in medicines could have caused.

I would suggest that you seek expert opinion, either back at Johns Hopkins (Dr Thomas Traill would be great) or at the Cleveland Clinic.  You are pretty close to us, and we have the undisputed best aorta surgeons in the world.

Good luck.

[Avel]

Hi,
I just want to tell you both that you are doing so well in searching for information and asking questions. It is not easy to go through the diagnostic testing, assure oneself of the accuracy and meaning of the results, and understand what the next steps should be. It is so important to be on top of things, and be sure you are being taken seriously. Usually there are no symptoms as the aorta enlarges, and it is very easy to be in denial, especially when one is young and often so healthy in other ways. Aortic disease, whether caused by Marfans or some other condition, is serious and complex.
To Bob, please do ask where the "normal ranges" listed on your tests come from. In my husband's case, the range listed was just a range that was broad enough to include the aortic size of a small woman on one end of the scale and a large man on the other end. Even with his aorta beginning to grow, the range was so large that its size was in this span of numbers. However, his ascending aorta should not have been growing! His descending aorta is not enlarged, and it is a good indicator of what normal should be for him. My husband had bicuspid aortic disease - which affects the aortic valve but can also affect other areas, the most dangerous being the aorta. I have shared further details of his experience on the aneurysm support web site at
http://www.westga.edu/~wmaples/velebir_arlys.html
To Don, it is so very important that a Marfan's expert evaluates you - not only because of the timing of surgery, but also because when you do have surgery you want the techniques that are proven enough to assure you of the best long term durable result. Marfan's syndrome aortic tissue, as you know, is very delicate and must be handled with great care and skill.
I can be reached via email through the aneurysm support link mentioned in this post, or also at ***@****  Please feel free to contact me.

[Avel]

Hi Bob,
I was interested to see the "normal" ranges given to you for the size of the aorta. Do you know what these values were based on?  It is very important to determine what is normal for you individually, and not go by some general measurement range. The normal size of the aorta varies with each individual - some of the factors that affect it are age, gender, and body size. The sections of the aorta that are not enlarged are usually a good indicator of what is normal for an individual. It is also important that the measurement be done by an aortic expert - patients have been given different measurements from the same CT scan when it is "measured" by different people. Understanding how to measure the aorta is a very specialized skill, and it is very important to have an accurate measurement.
My husband's aorta had begun to enlarge, but we were never told because when an echo was done and showed 3.3 cm it was considered considered in a "normal" range. Actually normal for him is well below 3 cm! (We didn't see those test results until years later, and his aorta had continued to silently enlarge.)
If you check out the web site I mentioned, http://www.csmc.edu/aorta , there is information regarding this if you click on the aortic disease section and go to the thoracic aortic aneurysm area.
It is so important to get an accurate measurement of the aorta, and I wish you well in finding expertise that will be able to provide that to you.
Best wishes.

[DimDon]

Hi Folks,

Thanks all for your wishes and concerns.  I very much appreciate them. For the last 13 years I have not seen a change in the size of my aorta and I think, to some extent, I pushed the issue into the back of my mind and forgot about it (i.e. denial).  Now I am faced with an increase and tough decisions.  There seems to be controversy with respect to what I should do.  Some doctor's say stay the course with increased Atenelol and continued monitoring, others say I should have surgery NOW.  So it's all confusing and overwhelming.  

To Bob, I experience a very similar "loud" or "forceful" beating of the heart as you have described in your post.  It seems to come and go and at times can last for hours and can be very disconcerting.  It almost feels as if I am skipping a beat, pausing then resuming forcefully such that I can feel and almost hear it.  My cardiologist told me that I am not skipping beats, but rather in those beats where I feel a pause or skip,  the heart is not filling entirely which results in a smaller evacution (small and unnoticable beat) and the next beat fills normally and I feel the stronger beat.  Apperently this is not cause for concern and I have been told to live with it.

My goal at this point is to be evaluated by someone who specializes in Marfans.  As Avel mentioned (and thank you for the link) each person is different and the critical size of the aorta with respect to having surgery, varies based on the size of the individual and the relative size of the vasculature in general.  Since I am a big guy, I have been told that my threshold might be larger.  

It's not an easy decision by any means.  Medical advancements in Marfans related aortic surgery has pushed the mortality rate to well below 5% if the procedure is done electively and is not done as a result of a disection and an emergency procedure.

Five percent doesn't sound bad, but when you consider the bigger picture, that rate increases substantially.  At 5 years the mortality rate more then doubles to 12%. At 10 years it jumps to 19% and at 20 years it jumps to 25%.  These statistics have to be weighed of course against the liklihood of disection and subsequent rupture if surgery is not elected.  It's a hard choice and I hope that evaluation with an expert in Marfans will help better elucidate the right course of action.

Best,

-DimDon

[bob_sixsix]

Hello Avel,
Thank you for the information!

I went to the website you mentioned and I am still confused as to where the "normal ranges" comes from.
I have read quite a few postings on the MedHelp website about "normal ranges", but have not gotten an answer as to how the "normal ranges" are determined.
I can say one thing.........On my 2nd ECG they list a value for BSA (body surface area).  I assume this is where the normal ranges are determined.  The normal ranges I mentioned in my post were on my ECG & CT Scan reports alongside the actual measurements.  I am not 100% sure where they come from but..........I have a one year ECG follow up in two days (Thursday) and will definetly ask where these "normal ranges" come from.
These test were my first and my doctor doesn't really have any type of baseline to compare to except these "normal ranges".  That is why I am a little nervous about these tests Thursday.

What, if any, symptoms did your husband experience while his aorta "silently" dilated?   I am hoping this one year ECG follow up will not show any dilation.
How old was your husband during this?
Was your husband diagnosed with Marfan's?

I am going to Montefiore Medical Center in the Bronx for my tests.  This is where I went for my 2nd ECG in my initial post.

Thanks again for all of your help!

[DimDon]

This Post has the Questions listed Below:

I have several concerns:  Firstly is that I had not seen a change in the dilation of the aorta since I was diagnosed almost 13 years ago.  Would it be logical to assume that at least one viable explanation for the increase in dilation is due to the substantial increase in weight and moderate increase in blood pressure over the last several years?  

At this point, surgical correction has not been discussed as an option and the cardiologist I had seen for 13 years prior to moving to Pittsburgh, cited 6 cm as a point where surgery should be considered.  However, all of my research in the literature concerning aortic dilation associated with Marfans suggests 5 cm for surgical consideration.  I am concerned as to whether or not I should be looking seriously into this option.  Should I wait until I hit 6cm?  At this point, what is the risk of waiting and the general prognosis if I have the procedure?  

My current cardiologist also refused to order an UF and claimed that standard serial CT is just as accurate and that the UF version should only be undergone once every five years.  However, I cannot find any literature supporting this notion.  I could understand if a contrast agent was being used, but in my UF 1.5 years ago, there was none.  Everything I have researched suggests that UF is more accurate due to it being less prone to movement artifact and I am speculating that is why Dr. Curtis suggested that approach.  Should I demand an UF CT?  Is it a more accurate test?

A second major concern I have is that my chest CT showing an increase to 5.5 cm was performed on February 24th of this year, and my current cardiologist did not contact me or alert me to the results of the CT for 4 months.  I recently made a probe into the matter only to receive a letter with a new prescription increasing my Atenelol dosage to 100mg and a request to have a second CT to monitor for changes in the aortic dilation.  This is concerning to me since for the last 4 months I have been operating under the assumption that my dilation had not changed and I was taking the lesser dose of 50 mg and I am concerned that in that period of time I may have dilated even more.  I find this to be grossly negligent and am considering switching cardiologists.  What is your professional interpretation of any potential harm being at a lower dosage for 4 months may have caused with respect to an increased dilation?

I understand that answers to most if not all of these questions would be highly speculative and I do appreciate any insight into the matter you can offer.

Thank you in advance.

[Avel]

Hi,
I do want to want to encourage you to seek expert care from someone who will accurately assess your aorta, immediately deal effectively with your blood pressure, and go over all of the factors that should be considered in making the surgery decision.
When my husband was diagnosed with an aortic aneurysm, much of the information I found was based on Marfan's syndrome. Some of the older statistics mention 6 cm for aortic surgery. However, it is important to know that aortic surgery has progressed a great deal, especially in the last 15 years or so, and with the decline in risk, now surgery may be done sooner. Today there are expert centers where elective surgery is offered at less than 5 cm for those with Marfan's and other connective tissue disorders. While size is one of several factors when considering surgery, it is a very important factor. You need to be individually evaluated.
In addition to the other options mentioned to you, you might consider Dr. Randall Griepp at Mt. Sinai in New York. You may also be interested in a paper from Mt. Sinai entitled "Surgical treatment of the dilated ascending aorta: when and how" This was published in the Annals of Thoracic Surgery in June 1999.
The following web site may also be helpful, which has a section that discusses the timing of elective surgery for the thoracic aorta. Here is the URL http://www.csmc.edu/aorta
I sincerely hope that very soon you will be under care that brings your blood pressure under control and provides the expert treatment that you need.
Best wishes.

[bob_sixsix]

Hello Don.
I have recently undergone a few tests to "rule out" Marfan's Syndrome.
I am 35 years old, 6'6" tall and weigh 250lbs.  My blood pressure is 130/82.  I play basketball 2- 3 times per week.  No known family history of heart disease.
I have had two ECG's (one of which was a Stress ECG) and then a Chest CT With Contrast to correlate or confirm the ECG findings which demonstrated borderline aortic dilatation.

I thought you might be interested in the results from the ECG's compared to the Chest CT.

The first ECG impression:
1)Upper normal limits for size to mildly dilated left ventricle (End Diastole 5.65cm) with preserved systolic function.
2)Normal appearing mitral, aortic and tricuspid valves.
3)No left atrial dilatation (3.8cm).
4)Upper normal limits for size to mildly dilated aortic root(3.1cm)
5)Doppler color flow exam revealed trace to mild mitral and mild tricuspid regurgitation.
6)Ejection Fraction = 65%
Note:  patient was 6'6' in height and 250lbs.  A delayed relaxation left ventricular inflow pattern(?)was observed suggesting possible diastolic dysfunction.

I then went for a second opinion to a larger, well known medical center and got this ECG impression:
1)Aortic Root Diameter = 3.7cm
2)Left Atrium Internal Diameter Systole = 3.7cm
3)Left Ventricle Diastolic = 5.3cm
4)Left Ventricle Systolic = 3.4cm
Blood pressure started at 130/82 before Stress Test and elevated to 220/0(?) upon completion.  I was told this was normal because of the rapid/normal recovery of blood pressure back to 130's/80's.
No comments on the "delayed relaxation left ventricular inflow pattern".

The results from the Chest With Contrast CT:
The descending aorta is normal.  The ascending aorta at the level of pulmonary artery bifurcation measures 3.7cm(normal = 3.2cm +/- .5cm).  The ascending aorta at the level of the takeoff of the left coronary artery measures 3.68cm and the sinotubular junction measures 4.45cm (normal = 3.7cm +/- .3cm).  In addition, the aortic valve sinus is rather prominent.
There is no evidence of dissection or thrombus.
The hilar and mediastinal nodes are normal.  The lung windows are unremarkable and the upper abdomen is unrevealing.

Impression:
There is slight dilatation of the sinotubular junction of the root of the ascending aorta and prominent sinus of Valsalva(?).

These tests were all performed within one month of each other.
I am scheduled for a one year follow up this week to see if anything has changed.  They ruled out Marfan's for the time being but for he past year I have constantly had a strange feeling around my upper left chest area and also experience very "hard" or "loud" heart beats when I lay down in bed.  Not quite sure how to explain them but they have earned me a few trips to the ER and have been blamed on anxiety(?).  My heart doesn't race it just beats very "hard" until I fall asleep.