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Aortic Diseases&Marfan Syndrome (Drs Svensson Eagleton&Moran 03 24 14)

Monday, March 24, 2014 – Noon

Description

If you have aorta disease or Marfan Syndrome, it is crucial to know and understand the condition and the latest treatment options available. Cardiac Surgeon Lars Svensson, MD, PhD, Vascular Surgeon Matthew Eagleton, MD, and Clinical Geneticist Rocio Moran, MD answer your questions about aorta disease.

More Information


Aorta Disease

Cormorant: I am a 62 year old female that exercises regularly and often; 123 pounds, never been overweight, 5 foot 2.5 inches, never smoked and eats a healthy diet; normal blood pressure, taking statins. Yet I have three aneurysms in my aorta: ascending aorta 3.9 cm; descending aorta at same level 2.9 cm diameter; at diaphragmatic hiatus, 4.1 cm diameter; calcification in coronary arteries. Do I have an aortic disease that I might be unaware of? What should I DO or NOT do about these findings?

Lars_Svensson,_MD,_PhD: I presume you had a CT scan of most of your aorta. These sizes are not typically worrisome. But if you have a history of migraines, arthritis or fibro myalgia, it is possible you may have a type of aortitis, like giant cell arteritis. I would recommend yearly MRI follow ups - occasionally MRI may show wall thickening confirming aortitis.

Datadivasis: In an incidentally found small ascending aortic aneurysm (4.1, small to medium frame 64 y/o female) would you advise initial and/or serial follow-up by internist, cardiologist, or at CCF aortic clinic (assumption being likely five - ten years away from elective repair if typical growth pattern)? How much initial work-up would be warranted (CT, echo, genetic, etc.) absent any symptoms?

Lars_Svensson,_MD,_PhD: I would advise yearly MRI and echo studies - generally after the first CT scan we avoid using CT repeatedly because of the radiation exposure. However, CT is usually used for the decision when to confirm operation.

Rose99: Can BP spikes (200/165) that only last a few hours further weaken an aneurysm or is it only sustained high BP? What is optimal BP?

Lars_Svensson,_MD,_PhD: There is indirect evidence that sudden spikes in high blood pressure, for example with weight lifting or cocaine abuse, can cause aortic dissection.


Marfan Syndrome

robbockscc: My 14-year-old son has Marfan Syndrome, with mild mitral valve prolapse. His last two annual echocardiograms showed his aortic root hovering around 3.5cm. A recent MRA showed 4.5cm, which seems like a sudden dramatic change in under one year. Is rapid growth typical? Another echo is scheduled in April. If that echo shows the 3.5cm range, which measurement do I trust? 3.5cm is manageable but 4.5cm is approaching the threshold for surgery and changes our treatment strategy and lifestyle constraints. If aortic replacement surgery is imminent, I am very concerned about having an artificial valve requiring blood thinners. What are the pros and cons of valve-sparing surgery, and a mechanical versus a porcine valve?

Lars_Svensson,_MD,_PhD: I would recommend a CT scan to confirm the MRI changes. Depending on your son's height and whether this is a recent expansion he may need a root re-implatation operation. We have done about 450 root re-implantation operations with no deaths in patients with Marfan Syndrome and our data shows 95% durability of the repairs ten years after valve re-implantation operations. The likelihood of being able to keep his own aortic valve is usually in the 90-95% range for the operation but is dependent on whether there are holes in the leaflets that may be causing valve leakage.

sadiegrey: My 24 yr. old son died of dissecting aneurysm and autopsy suggested it was probably Marfans. Had never heard of it....then son at 40 started having bad symptoms of pain and went to hosp. and his aorta had huge aneurysm, so they took him by helicopter to a bigger hospital.....surgeon knowing brothers history took tissue samples to be diagnosed at laboratory in New Orleans, they were in Orlando, FL. My son now has artificial valve, on Coumadin for life and beta blockers, etc. Whole family has been tested for the gene and out of seven children four have, three don't. Out of 14 grandchildren four have, some not tested. I have and am 72 yrs old. Question, does the ascending, descending or root of aorta usually develop the problems where they get so large you need surgery? Thank you.

Lars_Svensson,_MD,_PhD: For most patients with Marfan Syndrome the most common site of aortic aneurysm formation is the aortic root. Secondly the abdominal aorta is said to be the most common site. However with aortic dissection the aneurysms of the descending aorta and thoraco-abdominal aorta are more common.


Ascending Aortic Aneurysm and Aortic Valve

xdwl: Hello, doctor. I know aneurysm and aortic valve regurgitation may be asymptomatic. But I do have symptoms, and no sure if they are related with my aorta problems? I started feeling chest discomfort (stuffy chest) indistinctly in Nov. 2012, with no clear reasons. Chest discomfort is not associated with exertion, and I do not feel chest pain. Chest discomfort usually happens when I sit quietly, e.g. working with computer, and it can lasts for hours. Coincidently, trace aortic valve regurgitation was detected in Nov. 2012, then mild AR in three months, and mid-moderate AR in 12 months. I am also with ascending aortic aneurysm. I would like to ask -- would a 50mm aneurysm OR mid-mod AR can be associated with my chest discomfort symptom? Thank you so much for taking the time to answer my questions!

Lars_Svensson,_MD,_PhD: Generally 5 cm ascending aortic aneurysms are not associated with symptoms. Occasionally they can cause mild heart rhythm problems or if the aorta is in abnormal position this can result in problems with swallowing or breathing. Unfortunately ascending aortic aneurysms typically do not cause warning symptoms before aortic dissection.

JJTech: IMPRESSION: Ascending thoracic aortic aneurysm measuring up to 5.2 cm. Bicuspid aortic valve with only mild reduction in aortic valve opening with doming and thickening of the aortic valve leaflets. No significant aortic stenosis. Trace aortic insufficiency. I have no outward signs and am physically fit. I am not eager to have any operation.
1. How can I tell what my chances are of dying any time in the next 20-30 years from this aneurysm which I understand is equally dilated?
2. I would think the "health" of the wall of the aorta would be crucial? How can one asses that without tearing open his chest?
3. I understand they want to cut with anything over 5 cm. How have they determined that number?
4. How do I submit my findings for an opinion from the CC?
Thank you, James W

Lars_Svensson,_MD,_PhD: You raise an important point: Mainly that currently with modern imaging techniques we have no reliable method of assessing either the tensile strength or the thickness of the aortic wall. It is true that as a general rule we like to address the aortic valve and the ascending aorta at the same time, reducing the possibility of requiring a second operation for the aortic valve. If there is evidence of growth of the aortic aneurysm, then you probably should be more pro-active about getting it fixed. We have a method to send in records for evaluation - see www.clevelandclinic.org/heartnurse.

kimberlee: Hi, I just found your website and am curious to see your opinion. I am a 49 yr. old woman that is active and healthy. I underwent AVR in 12/10 due to a defective aortic valve being bicuspid when born. My ascending aorta wall is slowly continuing to stretch. As of 12/13 it's 4.2cm. My cardiologist checks it with CT's and echos every six mos. When should I be seriously concerned with the size of the wall?

Lars_Svensson,_MD,_PhD: If you are of normal height, and your aortic valve is functioning well, I would typically wait until your aorta is about 5.2 - 5.3 cm. We would typically like to wait obviously, as long as possible, not only because of risks, but also in case you may need another valve procedure. Once again, based on our data for reoperations, the risk of death would be about one - two percent. I would recommend you continue with regular follow up, careful blood pressure control, and if you do smoke - stop smoking.

Rose99: My ascending aortic aneurysm is at the level of the pulmonary artery. Does that have any significance in how a repair would be done?

Lars_Svensson,_MD,_PhD: Typically echocardiographers report the size of the ascending aorta at the pulmonary artery. This is merely a marker of where they have measured it and has no consequence as far as surgery.


Dilated Aortic Root

PMKC21: Thank you for holding this online chat. I am a 40 yr. old male, recently diagnosed in October 2013 with a 4.9 cm dilated aortic root, moderate mitral valve prolapse, pre-hypertension (am 6'1" 195 lbs. and in otherwise good heath). I'm currently on beta-blockers and in a watch and wait period. I've accepted the fact that I will have elective surgery sometime in the near future. I've seen a cardiothoracic surgeon at Johns Hopkins who specializes in Marfan's and one look at me and knew I didn't have Marfan's. What are the benefits of the modified David's re-implantation technique vs. the regular aortic root graft technique? And is there a difference in the success rate or incision size?

Lars_Svensson,_MD,_PhD: The advantage of the re-implantation operation is that you would not need to be on a blood thinner for a mechanical valve and if you were to get a biological valve the durability of a re-implantation operation is better than a biological valve. We have also found that the risk of complications related to the valve is lower in patients who have re-implantations of the valve. In our patients with three leaflet aortic valve re-implantations the durability is the same as a biologic aortic valve replacement up to seven - eight years and thereafter the durability of re-implantation is considerably better. There are three centers that do re-implantation operations on a regular basis including Johns Hopkins and us.


Ascending Aortic Aneurysm: When should you operate?

marciapantin: Ten years ago, I had Aortic root replacement surgery and now I have another aneurysm. The CT scan report is as follows: "An extensive Thoracic-Abdominal Aortic Aneurysm with widespread intramural haematoma. The Thoracic aortic diameter measures 4.62cm, abdominal aortic aneurysm 4.06cm. The aneurysm extends to the renal artery". Other Info: Female, Age 48. I have been told (in Trinidad where I live) it is not frequent that this type of aneurysm occurs. I hardly see anything online about it. My surgeon here will do it but without reliable surgeons who have performed this procedure, what is the possible outcome of surgery? Additionally If it can be done, how much would it cost at Cleveland Clinic?

Lars_Svensson,_MD,_PhD: Unless there has been evidence of rapid growth, we would not recommend surgery at 4.6 cm. If it should get up to 5.5 cm, then I would recommend you see us. Assuming it involves the aortic arch, descending aorta or thoraco-abdominal aorta. We have a method to send in records for review when you are ready.

jkgrnc: My husband (76 yrs. old) was diagnosed with an aortic aneurysm (ascending) 5.6 cms. He had an aortic valve replacement (mechanical) in 1991, atrial fib (on Coumadin), CHF x three yrs., elevated creatine (1.9), on metformin (1000mg twice a day) for several years, but has cut down recently to 500mg. once a day. A1C was usually under 6. His wt. is around 220. -225. We had a consult with Dr. at Yale New Haven Hosp. and his suggestion was to see him again in two years for another echo. I am writing to you because we would like to get another opinion. How do we go about this? June G.

Lars_Svensson,_MD,_PhD: There has been a lot of recent debate about what size patients should be operated on: depending on whether they have connective tissue disorder like Marfans; bicuspid valve; or a degenerative aneurysm associated with for example older age. A lot of the decision making of when to operate on patients is predicated upon an individual institution outcomes with surgery. For example, at Cleveland Clinic for patients undergoing bicuspid aortic valve repair in a series of 728, the mortality rate was 0.4%; in addition for reoperations on the aortic valve for ascending aorta, we usually run a 1- 2% mortality rate depending on comorbidity and extent of repair. This is considerably lower than the national average, thus the decision to operate is dependent on the surgeon's experience, the team he has working with him, patient's comorbidity, and the extent of surgery required. We would be happy to see him for an evaluation.

xdwl: Hi, Dr. Svensson. I am a 56 yrs. female and was found ascending aorta 4.4cm in Jun. 2012. Recent MRI showed it is 5.0cm now. I would appreciate your advice on: 1) What size of an aneurysm indicating a surgery for me? I calculated my "Aorta Index", it is 3.02. I have no Marfan no BAV --- is "Aorta Index" also a useful indicator for me? 2) I have no family history of aneurysm. BP 90-100/60-70mmHg. But I have ten + year history of HCM. My LVOT gradient was 97mmHg, peak velocity 4.9m/s before I had septal myectomy in Sep. 2012. Can high peak velocity and high gradient in LVOT cause aneurysm? 3) I have another problem: aortic regurgitation. I never had it before septal myectomy. Trace AR was detected just in one week post-myectomy, then mild AR in three months, and mid-moderate AR in 12 months post-myectomy. Can AR be associated with myectomy? Thank you very much!

Lars_Svensson,_MD,_PhD: If you are of normal height then we generally do not recommend operation particularly since you have had a previous operation. We balance as indicated above the risks of the surgery vs. the benefits. At this stage, the risk of surgery would be about 1 - 2% and your risk of having a fatal aortic dissection is probably lower. But we don't have really good population natural history epidemiological studies. Myectomy can sometimes result in aortic valve regurgitation, partly from the scarring.


Ascending Aortic Aneurysm and Stent

1Grumpy: While the surgical team is reviewing my records, will they consider a stent procedure with my ascending aortic aneurysm? If able to stent it, what is approximate hospital stay and recovery time? If open heart surgery needed, what is approximate hospital stay and recovery time? What are the risks involved in both procedures?

Lars_Svensson,_MD,_PhD: Stenting for the ascending aortic aneurysm is very rarely done and it is a high risk procedure. We have done some through the groin or the apex of the left pumping chamber (ventricle); but this has been for inoperable patients. For most ascending aortic aneurysms the risk of death is 0.5% - 1 % and the risk of stroke 1% at the Cleveland Clinic; and the hospital stay five - seven days. We do these with minimally invasive keyhole operations without having to open up the whole chest.

dms76: What is a "keyhole" operation technique?

Lars_Svensson,_MD,_PhD: For aortic valve replacement, ascending aortic aneurysm and sometimes for mitral valve operations, we do this keyhole minimally invasive operation. The majority of aortic valves and ascending aortic aneurysms we do this way. What we do is make a little trap door at the top of the breast bone and then do the procedure. We call this a J-incision. We have shown that apart from the better cosmetic result, patients have less pain after surgery, have better breathing, and lose less blood and return to normal activities earlier. We tell patients they can start driving two weeks after surgery instead of the usual six weeks.


Dissection

smiley575: Post thoracic ascending aortic aneurysm repair two and 1/2 years ago (currently 43 yr. male) aortic dissection with dacron graft no valve repair needed, although RCA done needed from leg. Scan done every six months. What are the most important numbers to take from these CTA chest imaging reports, (5.5 cm) was mentioned earlier but what category in the report impressions are we talking about that we should use to compare previous scans regarding cost-risk of surgery. The doctor only called us and said they would not need to operate at this time. If surgery is needed at some time in future, they will have to open chest, not these less invasive options been hearing about.

Lars_Svensson,_MD,_PhD: At about the size of 5.5 cm - 6 cm we start considering reoperation in patients with post dissection aneurysms. Unfortunately this typically requires two operations that are substantial and so we do not rush into them. Typically a reoperation and replacement of the aortic arch - the elephant trunk procedure - carries a two - four percent risk of death and the second stage operation through the left chest carries a similar risk. Once again -dependent upon comorbidities and extent of surgery.


Bicuspid Aortic Valve (BAV)

MichelleCh: My grandmother and her sister, my uncle, my mother and I all have or had a Bicuspid Aortic Valve with associated heart disease. My grandmother, her sister and my uncle all died from it. I have two children, neither one has a BAV. Seems like it skipped a generation. What about their children (my grandchildren)? Would they be at risk of BAV due to the strong family genetic history of BAV and associated heart disease?

Lars_Svensson,_MD,_PhD: Generally the accepted risk of blood relatives of a patient with a bicuspid valve (BAV) also of having a bicuspid valve is 9%. However, in some families it can reach 70%. It can sometimes skip generations but remember that 1 - 2% of the general population have bicuspid valves. Unfortunately at this time there are no good generally applicable genetic blood tests to track the gene through families.

McCbon: I am 61 with BAV with borderline severe stenosis, slightly dilated aorta and in otherwise good health. I have always been active and at last check a year ago, was considered asymptomatic except for fatigue which was attributed to something other than BAV only no one knows what. At that check I was told I would need surgery in one-three years. Can you describe what a patient can expect to experience as the range of mild to operable symptoms? What will chest pain feel like, what constitutes "lightheadedness." and what is the range of shortness of breath or "windedness" one might expect and under what circumstances? Also, my last three echos have shown a mild but persistent pericardial effusion. Do you have any idea what might cause that? Is it typical and will it increase my risk of having a more serious effusion post-op? Would I experience any s/s from that, such as chest tightness when lying down? Thank you so much for your time, I will be seeing you in May.

Lars_Svensson,_MD,_PhD: As a general rule, the symptoms of aortic valve stenosis are fatigue; tiredness; lightheadedness; or chest pain. When these are associated with severe aortic valve stenosis, surgery is recommended. Additional aortic aneurysms of the ascending aorta are taken care of at the same time and we have shown that this does not increase the risk of surgery in patients of bicuspid aortic valves. I usually quote a 1% risk of death, however for isolated aortic valve replacements at Cleveland Clinic in 2011, the risk of death was 0.6%; 2012 0.4%; and 2013 0.7%. Generally we do about 2,600 aortic and mitral valve operations per year. The pericardial effusion is unusual and there are many potential reasons for it. If you have had any chest pain or recent increase in size of the aorta then you need to be seen as soon as possible.

Larry S: Would you ever recommend the Ross Procedure to replace the Aortic value in lieu to placing a mechanical or animal value for the Aortic valve?

Lars_Svensson,_MD,_PhD: No.

zman949: I have a bi-cuspid aortic valve that is going to need replaced very soon. I am 56 years old, so I am being told that a mechanical valve would probably be the best option for me. Are there any mechanical valves available, where you do not have to take coumadinor at least a lower dose of it? Also, if I am considered healthy, is a minimal invasive surgery possible?

Lars_Svensson,_MD,_PhD: The debate about when to use a mechanical valve vs. a biological valve is ongoing but we have some data that suggests biological valves may do a bit better in younger patients than we first thought. We just presented our research at the STS meeting . In addition for biological valves there may later be the option of putting a percutaneous valve within the old valve via the groin or chest wall. Most of us feel there is no convincing evidence that any two leaflet mechanical valve is better than others. And - we have seen thrombosis of all types of mechanical valves with inadequate anticoagulation.


Abdominal Aortic Aneurysm (AAA)

Labradork: My father died suddenly of some sort of cardiac episode at the age of 39. His sister had an AAA, but no autopsy was done when she died at 66. Is there a specific genetic test to tell if my aneurism is congenital?

Rocio_Moran,_MD: It is often challenging when family members pass away with limited information. Our genetics team works hard to obtain as many records as possible to help identify a cause. This includes any autopsy records if available. If you have an aneurysm with this family history, there is genetic testing available that can help identify the cause and provide specific medical management recommendations based on diagnosis. Depending on where you live, a consult can be obtained through a genetics department or many "Marfan Clinics".

lefty: Have an abdominal aortic aneurysm, diagnosed Jan 2013. Measuring 4.2 x 4 cm and 4.7 x 4.2 cm. Common iliac arteries are mildly ectatic measuring up to 1.8 cm on the right and 1.6 cm on the left. A recent Feb. 2014 UL shows that t has enlarged to 5.0 cm compared to 4.4 cm previously and measuring as best as can be estimated to true transverse diameter measures up to 5.8 cm although this may include some obliquity. Having a CT in May and meeting with vascular surgeon. May need open surgery because of location and position of what I assume to be the enlargement location. Can you provide any questions that I should be asking from my surgeon?

Matthew_Eagleton,_MD: It is not abnormal for abdominal aortic aneurysms to grow over time and the only way we can determine that is to follow them with an imaging study such as an ultrasound or a CT scan, as you have had. With an increase in growth, I would agree you should get a CT scan, which can be more accurate in determining the size of an aneurysm. This study will also provide the surgeon with information that is necessary to determine what is the best option for repair, and the two common types of repairs are conventional open surgery or endovascular repair (EVAR). When speaking with your surgeon you should ask if surgery is recommended, what type of surgery and why, what your other options are , how many procedures has the surgeon performed, what are the risks with the surgery?

Aorta: Can diet reduce the size of the abdominal aorta? Does an unusual high level of calcium in the body effect the ability to have a successful stent procedure?

Matthew_Eagleton,_MD: There is no data demonstrating that alterations in diet can reduce the size of an abdominal aortic aneurysm. In fact, there currently no medical therapy for this problem. There is an ongoing randomized, prospective trial evaluating the use of a medication (doxycycline) to potentially reduce the rate of growth of abdominal aortic aneurysms - but the trial is not yet complete so we do not know its effectiveness. In some situations, a very calcified aorta can make stent surgery difficult or even impossible, but this is not common.

RCross4774: What are the most recent advancements in the treatment of abdominal aortic aneurysms (AAA's)? Does the rate of growth of AAA's remain stable over time? What is now considered the size of an AAA that warrants surgery?

Matthew_Eagleton,_MD: The most recent advancement in treatment of AAA began almost two decades ago with the development of endovascular abdominal aortic aneurysm repair (EVAR). This is a less invasive fashion in which the aneurysm is repaired from the inside of the aorta through two small incision (or punctures) in the groins. The traditional way had been through an incision in the abdomen. While we began using this technique several years ago, there are many updates in the development of the types of grafts used in these procedures making them easier to perform and in more people. The rate of growth of AAA is difficult to predict. In some, aneurysms grow at a steady rate, others grow in a staccato type fashion (grow then stop, then grow again). This is why we follow aneurysms with an imaging tool (such as CT or ultrasound) to determine how it is growing and how large it is. Most of the evaluations trying to determine when to repair aneurysms used the cut-off of 5.5 cm in diameter. Some surgeons, however, will use 5.0 cm as the cutoff. What they are doing is balancing the risk/benefit ration - what is the risk of the aneurysm rupturing versus the risk of the surgery.

tiger148: Last July I had an AAA procedure. At the time my aneurysm was 5 cm. Obviously a stent was place in my abdominal aorta. The latest CT shows 5.8 cm. What does it mean? Thank you, C. Sharone.

Matthew_Eagleton,_MD: Without being able to actually see the CT images it can be difficult to determining exactly what is happening. One possibility is that the aneurysm is being measured differently between the two studies. The best way to assess this is to review the scans side by side. Alternately, there may still be pressurization of the aneurysm sac, despite the placement of an EVAR. This can happen for a variety of reasons and should be evaluated by your vascular surgeon.


Abdominal Aortic Aneurysm – when to have surgery

Dillard: What is the "average" size of a typical (AAA) when surgical intervention should be considered; and what are other factors? TIA.

Matthew_Eagleton,_MD: Most of the clinical evaluations that were used to determine the appropriate size of an AAA to perform repair on used 5.5 cm as the cutoff, and that is the current recommendation. Some surgeons will use 5.0 cm. These are general guidelines and surgeons need to balance the risks of the surgery with the risk of continued observation. This can be affected on a patient-specific fashion based on other medical problems (such as severe heart or lung disease).

jwsherman: I am a 71 year old man. I was diagnosed with a 3.9 centimeter abdominal aortic aneurism two months ago during an exploratory ultrasound order to rule out any liver or kidney causes of minor abnormal blood test results. The plan is now to have a follow up ultrasound in July to see how much if any increase there has been in the diameter of the aneurism. I have a relatively light daily exercise routine consisting of a 2.5 mile brisk walk (30 minutes), and an also light strength routine using 5 lb. weights. I have three questions: 1) Is there any danger in continuing these routines; 2) Is there any danger in continuing sexual activity with my wife; 3) Would there be an advantage to surgery reasonably soon while I am still relatively healthy rather than waiting until the risk of rupture is significantly greater than the risk of surgery. I am very grateful for the opportunity offered by Cleveland Clinic to learn more about this condition. Is quite wonderful. Thank you.

Matthew_Eagleton,_MD: Your aneurysm is considered a small AAA (abdominal aortic aneurysm). For an aneurysm this size, we typically following it with ultrasound on an annual basis to assess for growth. Currently, the recommendations for repair are when the aneurysm reaches approximately 5.5 cm in diameter, although some surgeons will reduce this to 5.0 cm. In addition, faster growing aneurysms may be repaired earlier. We have evaluated, in the era of endovascular therapy (EVAR) whether treating small aneurysms earlier would be of any benefit - and it is not. You should continue your current level of activity and there is no danger to continuing with sexual activity.


Kommerell Diverticulum

justmeAmy: I am a 38 y/o woman and have been diagnosed with a vascular ring (right sided aorta/aberrant left subclavian) and a Kommerell Diverticulum. Over the past year my shortness of breath has been increasing and I began having some difficulty swallowing pills in addition to having mild chest/back pain. I have just established care with a vascular surgeon and am awaiting a new CT scan (last was ten years ago) and have been advised to consider surgery. I feel my symptoms are not terrible yet and I'd prefer to avoid surgery, but was told that the symptoms will likely get worse over time as the arteries harden and the diverticulum enlarges. I have also read about the risk that the diverticulum could rupture or dissect or erode. Could you tell me the likelihood of a catastrophic event like this and whether or not surgery at my older age could actually be helpful? Also, I've read of several methods to repair this. What is recommended at your hospital? What would you advise?

Lars_Svensson,_MD,_PhD: Right sided arch with a Kommerell Diverticulum is quite uncommon. But we typically operate on probably a dozen a year. The reasons why we operate are because of compression of the airway by the aneurysm; difficulty in swallowing or because of aneurysm formation. For the operation we typically hook up the left arm artery to the carotid before replacing the aorta with a Kommerell Diverticulum with a new tube - this is done through the right chest. In our experience stenting has not been successful.


Abdominal Aortic Aneurysm – after Surgery

gm3: Five (5) months ago I had an Open Repair of Abdominal Aortic Aneurysm through retroperitoneal approach using a bifurcated poly graft with removal of a Excluder endovascular graft. Procedure required because Endoleak Two (2) could not be corrected. No problems with surgery; however, a bulge continues to exist along my upper surgical incision area. Will this bulge go down or am I possibly facing a Hernia Incision issue?

Matthew_Eagleton,_MD: A bulge along the left flank is not unusual after some retroperitoneal incisions such as ones used to treat aneurysms. The bulges can occur for two reasons. One is due to the presence of a hernia that develops. Unfortunately, patients that develop aneurysms are at higher risk for developing hernias - particularly at sites of incisions. In addition, incisions in this location can cause some bulging due to a laxity that develops in the muscle wall - likely from some denervation of a portion of the abdominal wall muscle from the incisions. Hernias are frequently repaired - but the decision to do so would need to be made by a surgeon that repairs hernias. The muscle laxity typically does not improve but does not cause any health risk.

Labradork: When answering another question you mentioned that people that have aneurisms have an increased risk of hernias, does it go the other way as well? My husband has had three hernias and AAA's run in his family, he has had a cat scan of his aorta and all is well so far. Do we need to make sure he has routine checks?

Matthew_Eagleton,_MD: No, it does not go the other way. Those patients with hernias are not generally at increased risk for AAA. A bigger risk for developing an aneurysm, however, is having a family history of them as it is familial in approximately 15% of the cases.

gm3: What are possible complications that can materialize after open surgery to repair a infra-renal abdominal aortic aneurysm? Does the Graft used for the repair have long term performance history?

Matthew_Eagleton,_MD: Abdominal aortic aneurysms have been repaired in an open fashion for decades - using graft material similar to what is available now. The main risks with this type of procedure occur in the immediate perioperative period and can include any of the complications seen with major surgery such as bleeding, infection, heart attack, stroke, etc. We know that these types of grafts perform well long-term. That is not to say that problems cannot develop. We do see a small incidence of long-term problems that can develop. These can include the development of a graft infection or failure of the location where the graft is sutured to the aorta. This may not be a failure of the graft, but continued degeneration of the aorta over time.


Aneurysm and Medications

asgalian: Aortic dissection runs in my mother's family - with four of her six siblings being affected, as well as several aunts, uncles and cousins. High blood pressure exacerbates the growth of the aneurysm as a result of some connective tissue disorder. I have been advised that losartan is a good medication for this condition as growth of aneurysms has been slowed in animal studies. Are there other specific medications recommended when high blood pressure exists and the family has a history of aortic aneurysms?

Rocio_Moran,_MD: The data on losartan is emerging and there is no consensus amongst cardiologists or surgeons regarding its use in patients with thoracic aortic disease or connective tissue disorders. With such a striking family history, genetic testing and genetic counseling should be considered to help identify the underlying etiology. A genetic diagnosis could refine medical recommendations, identify at risk relatives, and provide insight into your specific medication recommendations. Optimal blood pressure control is always our end goal in patients with aneurysms.

clifster: My husband has Marfan Syndrome and a Type B aortic dissection from his clavicle all the way into his left leg. The doctors here had some trouble managing his BP, and he has now been taking Exforge for just about a year, along with Atenolol. Exforge is extremely expensive, so I would like to know if there are any other medications that might do as well controlling his BP but are not so cost prohibitive.

Lars_Svensson,_MD,_PhD: This is a combination medication and you may be able to get each one of them separately at a cheaper price; and you may wish to ask your cardiologist about that option.


Reviewed: 03/14

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