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Marfan Syndrome&Aorta Disease (Dr Svensson 10 8 12)

Monday, October 8, 2012 – Noon

Description

It is crucial to know and understand the latest treatment options available if you have been diagnosed with a condition related to the aortic valve or aorta. Cardiovascular Surgeon, Dr. Svensson answers your questions about aorta and Marfan disease.

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Ascending Aorta – Thoracic Aorta

barbara: My father-in- law and sister-in-law both died from an ascending Aortic rupture (both smokers & both in their 60’s) Since then, my husband has been diagnosed with one also. It is in the same location and is 4.5 cm. He has a MRI ran every 6 months. My question is: What is the probability of another one from showing up in another location and is there any hope for stem cell treatment in the near future? My husband (a non-smoker) has been a runner for years until his knees went out. Since that time he has been riding a stationary bike. My husband is 67 now. Thank you, A worried wife

Dr__Svensson: To answer your questions:

  • What is the probability of another one from showing up in another location - It is very unlikely. 
  • Is there any hope for stem cell treatment in the near future? No - that is not an option. 
  • He should have genetic screening.

Swim343: I have an ascending aorta aneurysm size 4.6. I don't know what has caused it. My sister had ascending aorta dissection. Should I undergo genetic testing?

Dr__Svensson: Yes - you should undergo genetic testing. And - if this measurement is at your aortic root, you should be evaluated by an aorta surgeon.

Swim343: Thank you for this answer. My aneurysm is very close to the aortic valve, which is mildly dilated. I take Metoprolol (25 mg once a day) and Losartan (25 mg twice a day) and HCTZ 25 mg once a day) to control my BP, but sometimes it goes above the prescribed limits (up till 140/90). Do you think I should do more to control my BP? I am otherwise healthy, active and I have switched to a vegan diet.

Dr__Svensson: Yes. You should lower your blood pressure if you can tolerate it.

SaraEb: Hello, I'm Sara a 25 years old female from Chicago. I had an aortic arch repair surgery on Dec 27, 2011 and Dr. Lars Svensson was my surgeon. I'm feeling tired and sleepy most of the times, I have cold feet and I get hot flashes sometimes with palpitation. I was wondering if there's anything I should be concerned about?

Dr__Svensson: I would recommend you be seen by your internist and check for infection.

KareninBluffton: I was told I have an aortic aneurysm on my ascending thoracic aorta, diagnosed in March, 2011. Size was 4.4 cm on CT scan, 4.9 cm on Echo on April 15, 2011. Then In November 2011 I was told it decreased in size to 4.0 cm. My question: How can I be sure that all of my heart-echo interpretations are correct? Can the public expect standardized methods to interpret echos and prevent this important heart test from being mis-read? How do you suggest I find the best doctor and clinic to do these tests? I live in South Carolina.

Dr__Svensson: I would recommend you be seen at a major medical center that has accurate studies.

spinja187: Hi I am curious how the strength of the joints of an aortic graft like mine compare to a healthy aorta, and whether you would expect it to strengthen over time due to tissue infiltration, or deteriorate. Also, thanks again for your great work, I am trying to convince my wife to name our son after you; Sven Larson it would be.

Dr__Svensson: The joints are sewn and strengthened so they generally are very strong. It is very rare to get an aneurysm or leak unless infection occurs or glue was used. Thanks for your note.

spinja187:  I was told after you replaced my aorta and valve that my target INR was 2 to 3, if I remember correctly, but my general practitioner who monitors my INR one day didn't like that and wants it at 2.5 to 3.5 now. what is your recommendation currently for target INR for the St. Jude valve graft?

Dr__Svensson: That range is fine.

c0ral: I had an aortic dissection in 2006. It was successfully repaired with a graft. What is the risk of another dissection in a different place on the aorta? Is there any kind of time frame for this kind of thing?

Dr__Svensson: Patients should follow up with their doctor and have a follow up CT scan to check.

Thidwick50: Is it safe for someone with an enlarged aorta to train for and run in a half-marathon? This young man had surgery to repair coarctation of aorta at age 10 months. At age 22, he learned his aorta was enlarged to 4.3. It has stayed at that measurement for three years. He has low blood pressure, passes regular stress tests, and ordinarily runs 2 miles a day for exercise. He has participated in 5K runs. He wants to train for a half-marathon in January. I'm worried this is not safe. Your thoughts? I appreciate your help!

Dr__Svensson: He needs to see a cardiologist for an evaluation. There are cardiologists who specialize in patients who have had prior congenital heart surgery. If he would like to get evaluated at Cleveland Clinic – see the Center for Adult Congenital Heart Disease.

dnlczrt: Wow, the symptoms of the thoracic aortic aneurysm are VERY similar to my symptoms- what should I do?

Dr__Svensson: You should be evaluated by a cardiologist.

milessusan: My son had a thoracic aortic aneurysm (8.5cm) that was repaired last year at the Clinic. His surgeon said that it is likely that sometime over the course of his lifetime that he will have another aneurysm. He has been tested for Marfan Syndrome and other genetic disorders, but everything came back "negative". He does have other "symptoms" that would indicate he has some sort of connective tissue disorder. Do you think aneurysm recurrence is likely? Also, he still takes atenolol....will he have to remain on this med indefinitely?

Dr__Svensson: The risk of recurrence is dependent on blood pressures and whether dissection was present. If your son has specific questions about his condition, he should speak to his doctor. If he would like to contact his doctor, please either call his office or contact our Heart and Vascular Institute Resource Nurses for more information.


Aortic Root

jsandzzz: I am a 57 YO men with an aneurysm of the aortic root measuring 4.7cm. It has been stable since it was diagnosed 3 years ago. I have been taking toprol and losartan daily, 25mg each. Recently, a new cardiologist recommended that I stop taking one or the other since my BP is well-controlled. What are your thoughts on taking two medications that act differently to control BP? Thanks - Jack

Dr__Svensson: Aim for a blood pressure - systolic of 110 mm HG if tolerated.

meliz04: Dr. Svensson I had the good fortune to have you replace my mitral valve with a St. Jude mechanical valve as I had severe mitral regurgitation. I am followed at Brigham and Womens Marfan Clinic in Boston. I also have a dual chamber ICD placed because of ventricular tachycardia and fibrillation proven by EP study. My aortic root is 4.2cm, sinuses of Valsalva 4.4cm right and left, non-coronary 4.5cm, sinotubular junction 3.9cm, ascending aorta 3.5cm in July 2012 and it was only 2.9cm in Dec. 2011. I have syncope twice a week and have had three cardiac ablations and cannot have any more. My aorta is being watched every six months. Is aortic surgery a possibility in the near future? There is also mild dilatation of both pulmonary arteries. I only wish you were still at Lahey Clinic. Best wishes from a grateful patient you took very good care of me. With sincere gratitude MaryEliz

Dr__Svensson: It is very good to hear from you. I would advise follow up with echocardiograms and only have surgery if your aortic root is greater than 5.1 cm or so.

GARY: I had an expanding aorta replaced above the heart. What are the chances of an aneurism (sp) happening elsewhere along the aorta? GARY

Dr__Svensson: Gary, the chances are very small that you will have an aneurysm in another place on the aorta.

ecacooke: I'm a 27 y/o female - had my aortic root replaced 18 months ago. Diagnosed 6 months ago with aortic valve insufficiency. What is the tolerance for pregnancy with my situation? And once a new valve (tissue) is inserted, what's the tolerance then? Thanks.

Dr__Svensson: Tolerance will be dependent on the amount of regurgitation (leak). You should speak to your cardiologist about your specific condition.

sadiegrey: Can you explain the David procedure....is this for a first time only surgery on the aorta?

Dr__Svensson: The David's valve-sparing aortic root replacement method is a surgical treatment for aortic root aneurysms. There is a complete explanation of the David Procedure on our website at http://my.clevelandclinic.org/heart/disorders/aorta_marfan/davidreimplantation.aspx  and a video showing the actual surgery at http://my.clevelandclinic.org/heart/videos/aorta-disease-treatments.aspx

Shawnee: In 2004, I had surgery to replace my aortic root and ascending aorta replaced with a graft and remodel and reimplantation of my aortic valve (modified David procedure). What is the expected need for reoperation?

Dr__Svensson: At Cleveland Clinic, the freedom from reoperation at 10 years is 95 percent.

BillP: Background I am 56 years old, a road cyclist, ride about 2,000 miles each year, and exercise regularly. Weight is 186. Working out normally at 135 bpm, but can peak at 165-170 for 5 or 6 minute bursts. Never smoked. On statins since 2005. Echo Stress, confirmed by a CT Scan....enlarged Aortic Root. My understanding is 3.9=normal. I am at 4.5 Next CT Scan in 6 months. Questions: Does my exercise put me at risk of further stressing this? Any advice in the meantime?

Dr__Svensson: If you are straining, then yes, that does increase the risk.

Shawnee: Following my 2004 surgery to replace my aortic root and ascending aorta, I have remained very active. This includes bicycle racing (32 races this season). I often see max heart rates of 183-187, with averages of 168-174. My BP is well controlled w/o medications. Is there any data regarding high performance athletics following surgery? Also, I have been a bit frustrated with the lack of "exacting” post surgical care. Other than imaging, are there other suggestions, (meds etc.)?

Dr__Svensson: I have a couple NBA players doing well after David root repairs; so exercise is not an issue. Yearly echoes and MRI would be fine. You should speak to your cardiologist about your concerns or we would be happy to provide you with a second opinion at Cleveland Clinic.


Marfan Syndrome

nortan: Dear Dr. Svensson. A close relative underwent aortic valve/ root replacement at the Cleveland clinic more than two years ago with excellent results. We will always be thankful to the team of professionals at the Clinic. We have now moved to the United Kingdom and looking for specialist centers/ individuals who can follow up his Marfan Syndrome condition. I was wondering if, based on your international experience you can recommend any in the UK or Europe. Best regards.

Dr__Svensson: Look for cardiovascular surgeon, Dr. Tom Treasure in London. 
This article contains contact information - http://heart.bmj.com/content/early/2011/02/28/hrt.2010.217240.full 

bjsetter: If my Father had Marfans and two of his brothers had it am I more likely to have it. I have an enlarged aorta. I don't have the spider length arms, fingers, legs and such. I have the high pallet in my mouth and vein issues in my lower body. Have had a prolapsed uterus removed and a lot of foot trouble along with mallet toe and hammer toe issues.

Dr__Svensson: I would recommend genetic screening and an echocardiogram.

rei: Hello, I am 36 years and diagnosed with Marfan's syndrome in 2010 although doctors had asked my parents to consider the diagnosis when I was 13 but never went through with it; 2 of my 4 children have Marfans syndrome as well. At my last heart checkup, my aorta was measuring 4.2 cm; I enjoy hiking and running and my doctor instructed me to keep up the cardio exercise as it can strengthen the lining of the vessels; I'm currently on Lisinopril and amlodipine benzoate to reduce my blood pressure as it was on the verge of hypertension. Is the maintenance of moderate cardio exercise good advice? I haven't experienced any issues yet.

Dr__Svensson: Yes, moderate exercise is fine. Also – continue regular follow up with your cardiologist.

prussell: In July, 2011, I had an 18" aortic dissection of the first and second layers. They put a 10" endovascular graft in, but I have had problems since. I also have some fibrosis of the lung. What can I expect for the rest of my life? I have Marfan. My dad had it, and several members of my family. I have terrible back pain, get weak very easily and live with fear every day. I was told it could happen again anytime and if I had to be opened up I would have to come to you. You operated on my nephew, Rick P. Can you give me any insight?

Dr__Svensson: You are describing a complex question and issue. It depends on where the disease was and your aortic size.

prussell: They also had to take a lot of spinal fluid to put the graft in because it was so close. Would that be part of the reason for my back pain?

Dr__Svensson: Not sure. Unlikely.

sagaboy67: Hello doctor, what is the maximum dilated size an abdominal aneurysm can go to before surgical intervention in a Marfan patient?

Dr__Svensson: There is no defined size but 4.5 cm would probably be reasonable to fix.

jimmie_3: I am a 62 year old male with Marfan. I am facing a TURP to improve the flow of urine. With Marfan, is there any potential risk in this surgery?

Dr__Svensson: Bleeding may be increased.

kellbell: How do you suggest going about finding a doctor, cardiologist or even a PCP, when you are not from the area, but are far enough way from CC that it doesn't make it feasible to go to CC for care? What do you look for or where do you start to find a doctor that is qualified to handle a person with extensive cardiac issues and Marfan syndrome?

Dr__Svensson: A good idea is to go to the National Library of Medicine PubMed - http://www.ncbi.nlm.nih.gov/pubmed/  and do a search on Marfan plus the city you are looking for. You will find physicians who have performed research and published on the topic.


Children with Marfan Syndrome or Aorta Disease

susma: Dr.Svensson, My 6 year old son has Aortic Stenosis, bicuspid aortic valve and dilated ascending aorta. His recent measurement of the dilation was 5.5cms. His cardiologist warned us about a rare possibility of aortic dissection. We learn from many sources that an adult with a 5.5 or 6 cms of dilation needs to undergo surgery as soon as possible. We would like to know if that's the case for children also. My son does NOT have Marfan Syndrome. What is the first sign of aortic dissection we should look for and how fast does it develop. While he is at school, if he develops any back aches or neck pain while playing, will it be life threatening? Thank you for your answer. - Susma

Dr__Svensson: Your son should be evaluated by a cardiothoracic surgeon who treats aorta disease either locally or we would be happy to arrange a consultation here. If you are interested in a consultation, please contact our Heart and Vascular Institute Resource Nurses at www.clevelandclinic.org/heartnurse.

twinter: My 10 year old daughter was diagnosed with Marfan Syndrome last year. In working with a local Cardiologist, she has presented with a slight "bulge" in her aortic root. At her 3 month re-measurement of the bulge, it increased slightly and the doctor put her on 50 mg. of Atenolol. 3 months later she was re-checked and there was not any indication of growth. She has been checked once more since then without growth. The cardiologist has indicated that she should only be checked once a year from this point on. Do you recommend more frequent checks/visits to the cardiologist for a 10 year old with Marfans? Does her age and entering puberty indicate she should be seen more often?

Dr__Svensson: If she has a minor "bulge" then yearly is fine. If it grows - then she should be evaluated more often.

lloydJ: Dear Marfan chat person, My great-grand daughter, aged 3, was fathered by a person diagnosed with marfan's syndrome, The mother, who is raising the toddler, has not passed this info to the child's doctor. Should the child be having evaluations now to evaluate her aortal prospects? Best Regards, Lloyd J

Dr__Svensson: She should have yearly echocardiograms.

kbass: Who are the best pediatric surgeons in the country for Marfan related aortic surgery?

Dr__Svensson: Look for pediatric heart surgeons who have experience in this area. At Cleveland Clinic we have Dr. Pettersson and Dr. Stewart.


Pericardial Effusion

Jana: Hello, I am 17 years old, I am girl and I was on heart transthoracic echocardiography, according the echo I have idiopathic small pericardial effusion and tachycardia 120 bpm, tachycardia but was rather according my cardiologist caused by stress from exam. I had left ankle swelling which lasted few weeks, but pericardial effusion allegedly is not the cause of swelling according my doctor. Is this small pericardial effusion dangerous for me? Is needed any limitations in regular activities? What are treatment options? Heart surgery? According my cardiologist it may worsen at any time and then it says it is to solve. In addition, I can not be said to assess right heart valves and sections on the right side of the heart the echo test. Otherwise, on aortic valve and mitral valve is normal finding. Are there any other tests that can assess the right side of the heart? Thanks for advice.

Dr__Svensson: Typically effusions stop as scar tissue forms.


Abdominal Aortic Aneurysm

Gloria-T: Can an abdominal aneurism be seen or felt or even show bruising from the outside?

Dr__Svensson: Yes - bruising indicates leakage.

gatorfrog: My son was tested for Marfan Syndrome by a blood test to check for AM Cortisol. That came back ok, but I am still concerned as he has some of the signs of Marfan's. Should I pursue this with his primary and ask for a cardiology referral? Thank you!

Dr__Svensson: Have a geneticist see him if you are concerned.

dnlczrt: How would you begin to suspect an abdominal aortic aneurysm; what are the symptoms of this aneurysm?

Dr__Svensson: You need to have an abdominal ultrasound.

This information is provided by Cleveland Clinic as a convenience service only and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Please remember that this information, in the absence of a visit with a health care professional, must be considered as an educational service only and is not designed to replace a physician's independent judgment about the appropriateness or risks of a procedure for a given patient. The views and opinions expressed by an individual in this forum are not necessarily the views of  Cleveland Clinic institution or other Cleveland Clinic physicians.

Reviewed: 10/12

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