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5 year old son diagnosed with Mid-Aortic Syndrome

May 01, 2008 - 50 comments
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mid-aortic syndrome



After a complication with bleeding from a tonsillectomy, my 5 year old son was admitted in to the hospital for emergency surgery to repair the tonsil bleed.  While he was in there the staff notice that his blood pressure was very high...(150's/170's over 100's).  They asked me to follow up with his pediatrician.  When we followed up the pressure was still high.  She sent us to a pediatric cardiologist and they did an echocardiogram.  They found that the left side of his heart is enlarged.  This is due to having high blood pressure for a long time undetected.  After seeing his pressures, (now 200's over 100's) the cardiologist admitted him into the hospital for testing and to get his blood pressure down.  After 10 days in the hospital, and after many labs, cat scans and MRA's, he was diagnosed with Mid-aortic syndrome. This sydrome effects the abdominal aorta and arteries leading to the kidneys and intestines.  The arteries leading to his kidneys and one leading to his intestines are narrowed.  This causes very high blood pressure and has also caused one of his kidneys to be smaller than the other.  At the moment, he is on four blood pressure meds and potassium.  They will be operating on him shortly to try and fixed the narrowed arteries.  
The reason i am posting this is because i want to know if there is anyone else out there that has children effected by this syndrome.  When you go online to try and find info, it is very hard to find.  This is a very rare syndrome and i am really looking for some information on this.  I just hate that we have so many unanswered questions.  If anyone out there has info, or advice it would be so appreciated.  


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Avatar universal
by mickspix, May 14, 2008
Hey there...  I was searching for information on MAS after I ran into you last night and found your journal...  I am looking for information for you...  I am concerned about what you told me and want to make sure that your boy gets the best treatment.... I will call you tonight...
Michele

177505 tn?1220445858
by threesacharm, May 14, 2008
thanks michele!!

Avatar universal
by JB2, Jun 04, 2008
hi
my daugther had the same problem at birth (but I just found out the name of the condition she have). She had surgery at 24 days to try to repair the renal artery (she had only one kidney more affected) but they had to remove the kidney.  Her blood pressure went back to normal really quickly after the surgery and it caused her some complications.  However they manage to control it and she is ok now.  She just turned 3 yo and she is developing normally.
Try this for info:  www.orpha.net/data/patho/GB/uk-ACoA.pdf
JB

Avatar universal
by Mikkelson, Jul 26, 2008
Hello.  We have a daughter who was diagnosed with mid-aortic syndrome.  She is being followed by a nephrologist and vascular surgeon here in Georgia and when she needs by-pass surgery, we plan to have it done at Mayo Clinic in Rochester, MN.

LM

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by threesacharm, Jul 28, 2008
This is going to be a long post but i think you will benefit from reading it!
After posting my comments in May, my son had a major vascular surgery on May 28.  Because Tanner's blood pressure was so high, they wanted to operate right away.  He was on 4 blood pressure meds and was still only coming down into the 130's (top number).  They were worried because his heart was already enlarged and his kidneys were suffering.  The surgeon corrected both renal arteries and his main intestinal artery.  His abdominal aorta is narrowed but not enough to risk fixing right now.  The surgeon (Dr. Robert Atnip) did what is called "stretching" on his left renal artery and his intestinal artery.  What they do is cut the narrowed part out and sew the ends back together.  Because his right renal artery was so narrowed, they actually used his pelvic artery to bypass the right renal artery.  You can live with just one pelvic artery.  The surgery took 6 hours and he was in the hospital for 17 days.  At first his kidneys would not function properly because of the lack of blood flow during surgery.  His intestines also were injured in the surgery and they are still trying to come back to normal, although his kidneys came back in just a few days.  He just had a renal duplex scan and it showed that his arteries all have excellent blood flow.  He is still on meds, 4 blood pressure pills and a water pill.  His blood pressures are ranging anywhere from top numbers of 118-100.  That is great considering he was once in the 200's.  They will very slowly wean him off meds when they see his pressures going into the 90's.  I was frustrated because he is still on meds, and I kept thinking, I hope he didn't go through this for nothing.  They told me that it can take months for the artery muscles to finally relax after being clamped down for so long.  Once the arteries relax, the blood pressure will come down hopefully without meds or at least not so many meds.  He is amazing though.. you would never know by looking at him that he had such a major surgery.  He runs around and plays and jumps and makes me nervous!!!!  He had his surgery at Hershey Medical Center Childrens Hospital.  They were all amazing with him.  He is being followed by a nephrologist, vascular surgeon, cardiologist and a gastroenterolist.  They are all wonderful doctors.  What makes this so hard is that you really never get straight answers because it is such a rare syndrome.  There is not much info on it anywhere and it really is such and individual type of problem.  He may need more surgeries, he may not.  He may be off meds, he may not.  It is so hard for the doctors to say because it is different with every child.  So far, I am optimistic that he will live a long and fairly normal life.  As long as we teach him the right way to eat and keep up with his medical care, he will do good i think.  It is scary though, not knowing all the facts...  like i said though, he has wonderful doctors caring for him and they will make sure they do everything they can to keep him healthy.
How old is your daughter?  Was this found out through a routine blood pressure check?  When do they think she will be getting her surgery?

Thanks for your post....
jan

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by lLoribean, Aug 31, 2008
HI EVERYONE.   MY NAME IS LORI   MY SON WHO IS 3 MONTHS OLD NOW WAS DIAGNOSED WITH THIS MID AORTIC SYNDROME.  HOW THIS ALL CAME ABOUT WAS WE WENT TO THE DR FOR A ROUTINE VISIT AND THE DR HEARD A HEART MURMUR.  HE SENT US TO A PEDIATRIC CARDIOLOGIST TO RULE OUT VSD... ALL THE TESTS CAME BACK FINE. BUT THEN HE TOOK A BP ON MY SON.. HIS BP WAS 160/90   AT THIS TIME HE WAS ABOUT  1 1/2 MONTH OLD.   WE WERE THEN TOLD TO GO TO ALBANY MEDICAL CENTER FOR TESTING... THEY TOLD US THAT HE HAD NARROWING OF HIS ABDOMINAL AORTA AND RENAL STENOSIS., AND NARROWING OF SOME OTHER ARTERIES GOING TO HIS STOMACH... MY HUSBAND AND I WERE SO UPSET.  WE THEN WERE SENT TO BOSTON CHILDRENS HOSPITAL...THE  DOCTORS TOLD US THAT HE HAS THIS RARE CONDITION.  .. BY LOOKING AT MY SON HE LOOKS LIKE A HEALTHY 3 MONTH OLD NOW.   WHILE AT CHILDRENS HOSPITAL IN BOSTON  HE HAD A CARDIO CATH DONE TO TRY TO OPEN UP THE AORTA, AND THEN DID ONE RENAL ARTERY..  HIS BP IS STABLIZED RIGHT NOW WITH MEDICATIONS... HIS BP IS BETWEEN 100-130 TOP #  WE HAVE A VISITING  NURSE THAT COMES EVERYDAY TO DO BP CHECKS... WE GO BACK IN 6 WKS FOR TESTING AND ANGIOGRAM AND POSSIBLE ANOTHER CARDIO CATH .... WE ARE SO SCARED , AND FEEL SO HELPLESS THAT OUR LITTLE BABY HAS TO GO THROUGH THIS.  I IMMEDIATELY WENT ONLINE TO FIND INFO ABOUT THIS DISORDER AND THERE ISNT MUCH INFO..  THE DOCTORS WANT TO WAIT A WHILE TO DO ANY KIND OF A SURGERY.   THEY WANT HIM TO GROW AS MUCH AS HE CAN WITH OUT ANY COMPLICATIONS...I OFTEN THINK ABOUT WHAT KIND OF A LIFE HE WILL HAVE AND WILL HE BE OK..... BUT I AM LEARNING TO TAKE ONE DAY AT A TIME.  AND TO BE STRONG..

Avatar universal
by Mikkelson, Mar 24, 2009
Oh my goodness it has been a long time since I have written.  I'm sorry, I forgot about you guys!  In December, the vascular surgeon decided it was time for my daughter to have surgery.  She is 12 and she was diagnosed with Supravalvular aortic stenosis (SVAS) when she was 8, so we knew this was looming.  We took her to Mayo Clinic two years ago, and that is when they found the MAS.  So, we knew the surgery was coming, we just thought it would be to correct the heart problem.  She is now on 4 hypertensive meds and a multivitamin with iron.  She is anemic and that won't get better until she has surgery.  She does a good job of taking her pills.  She also has ADHD, so the meds have been a balancing act.  June 5, is the date reserved for her surgery.  We report to Mayo Clinic on June 1 for cardio tests like an echo, chest xray, CT angiogram and then we expect the vascular surgeon will want some tests done.  He makes the final call re: surgery and then the 5th is reserved for it.  They tell us she will be in the hospital 5-10 days and then we need to stay in the area for a week.  My husband and I have arranged to have the entire month of June off of work so we can be in MN as we live in GA.  They are going to do a graft.  The reason they said it was time for sugery is because she was up to 4 hypertension meds and she had reached 90% of her adult height.  This tube they are going to graft in will not grow with her, so we had to wait and wait.  They will put one end in her cardiac arch and another down near her kidneys.  Then, they will use a vein and an artery to either make new renal arteries in or a second set, I'm not sure.  Her descending aorta and mesenteric arteries are too narrow...all the way, not just here and there.  So, I think in the end, she will have a second descending aorta, a second mesenteric artery and maybe a second set of renal arteries.  They won't correct the SVAS because it has been progressing slowly and if that were her only problem, wouldn't need correcting yet.  I understand her surgery she will have in June will be an all day surgery as it is and correcting the SVAS would be too much for both patient and doctor.  It is so interesting to read what you both wrote.  I have to nephews, two sons of two sisters, who were also diagnosed with SVAS when they were babies.  One had emergency heart surgery when he was 3, the other was able to wait until he was 7 to have surgery.  My daughter's wasn't found until she was 8.  Anyway, the boys are in their mid-twenties now, doing fine.  They don't have MAS in addition to the SVAS, though, like my daughter has.  She is tired ALL of the time.  Our normal family weekends are too much for her, so we try to keep her home and resting, otherwise she can't make it a whole Monday in school.  If she gets a virus, she is just exhausted by it.  We worked it out with the school that she can sleep in if she needs to and not be counted as tardy, she can leave early without being counted late and she can lay down in the clinic after lunch for a few minutes, if she needs to, but she would rather not do that.  When they do surgery, they will need to put her on a bypass machine and have acces to her chest, abdomen and pelvis.  My husband and I are from MN and have a lot of family up there, so we will be well supported, but you can imagine the dread of us waiting these last couple months of school to finish before surgery.  We also have a 15 and a 6 year old.  I should also mention that because the SVAS is rare, we were in a genetic study.  I passed a mutated elastin gene to my daughter.  My 15 and 6 year olds are negative for the mutation.  They have linked SVAS to the elastin gene.  Later, after all that was over, the MAS was found, so I wonder if there is also a link to the elastin gene for MAS?  I guess we will never know, but I thought I would throw that out there in case you ever get genetic testing done, they can look at the elastin gene on chromosome #7.  
It is encouraging to me to read that your boys are doing well.  I wonder what the future will hold for our daughter and are we trading these problems for others after we do the surgery?  Like you said...one day at a time!
I'll try to look back here more often in the future.  I hope the two of you write again!

Avatar universal
by Leelee1970, Mar 27, 2009
My 8 1/2 month old little girl is going in for her third cardiac cath procedure at Children's in Boston this coming Wednesday.  She was diagnosed with MAS in October.  It's been such a hard road with the blood pressure meds and constantly checking it every 4 hours plus she does not feed very well.  Her kidneys have been affected too so there only so much we can do.  Do your children eat ok or is this common.  There is so little information out there that your mind goes crazy thinking about it.  Like you said though you'd never know by looking at this happy little baby what's going on inside and it just breaks your heart.

Avatar universal
by Mikkelson, Mar 30, 2009
Hello.  8 1/2 months!  I'm so sorry she has had to go through so much!  The school nurse checks our daughter's blood pressure 3 times a week.  Her kidney functioning is good, but she "has less reserves" than other people because her kidneys haven't grown in two years.  If you are breast feeding, I know my daughter didn't feed well either.  I tried for seven weeks, then gave up.  She would fall asleep at the breast right away.  They were telling me it was because I was too heavy on her chin, but now that I know what I know now, I wonder if it was just too exherting for her?  Also, we know my daughter's mesenteric artery is narrowed.  It hasn't been a problem until this past December.  She would mention stomach aches here and there, but we would let it go.  When the holidays came around, boom, she started complaining of stomach aches every time she ate.  It was like she hit the magic size so now she is ready for the graft and all her narrowed vessels won't keep up with her size.  My daughter is on 4 hypertensive meds, maxed out on 3 of them.  They told us that they were waiting for uncontrolled hypertension and our daughter reaching 90% of her adult size as the indications for surgery.  The tube they put in won't grow with her.  So, I didn't think our daughter's stomach aches were due to the MAS, but instead due to the narrowed mesenteric artery, or maybe it is all part of the same syndrome.  We received our packet from Mayo Clinic today and they are going to do a full body CT angiogram when we first get there.  I'm so glad.  That way they can see that mesenteric artery and include that in the plan.  She is also complaining that her right hip hurts.  It goes in spurts.  We thought maybe it had to do with the elastin gene mutation and her being less elastic, or too elastic and her growing.  I worry though about the blood flow and anything else I may not understand.  

What heart problem does your daughter have?  

Avatar universal
by Leelee1970, Mar 31, 2009
My daughters superior mesenteric artery was opened up with the last cardiac cath procedure, but I'm worried it has stenosed again since she's feeding poorly, but it could be something as simple as acid reflux which we know she has.  As far as I know this is part of the syndrome.  Basically, you never know what arteries you'll find that are narrowed when they go in.  Seems like they find something new everytime so we're hopeful that they will find something that will really give her a boost.  Keep your spirits up- it's amazing what modern medicine can do and the things they are always learning.  

Avatar universal
by dellie424, May 17, 2009
Hi, my daughter was diagnosed with long segment MAS at 6 weeks old during a paediatric checkup.  Her blood pressure was 120's/60's. At 9 weeks old she had ballooning and a stent inserted. She still required 2 medications as an area of stenosis could not be opened.  She is now 4 years and 5 months old and we are due to have aorta bypass surgery in 11 days at The Children's Hospital at Westmead, Sydney, Australia.  Her blood pressure has been 130's/60's even with 5 blood pressure medications.  Other than her condition she has developed normally and is a happy girl.  It has been frustrating that Doctors just don't seem to know much about the condition or can answer your questions.  We have an excellent relationship with her cardiologist in Adelaide, Australia and any information we have found on the internet we have passed onto him.  Her Doctors are optimistic about this surgery.  I will always remember her paediatrician saying she is lucky she was born when she was as they may not have been able to help her otherwise.  Although I have read many articles now from the 1970's where children have been helped surgically.  The only challenge with surgery is the child's continuing growth.  The whole experience has made us enjoy everyday with her.  There is always hope and children are just soooo resilient and determined.  
Mid aortic syndrome goes by many names e.g. abdominal coarctation, abdmonial stenosis

Avatar universal
by skully1983, Jul 08, 2010
Hallo, ich habe als 16 jähriges Mädchen, das MAS (Midaortic syndrom) diagnostiziert bekommen. Ich hatte immer so schwere Beine beim Laufen und ich dache, dass ich keine Konditon habe! Bei einer Untersuchung wurde eine viel zu hoher Blutdruck festgestellt! Werte über 200/120 und höher! Ich hatte ständig Kopfschmerzen! Es wurden dann einige Untersuchungen gemacht, ein Nierenultraschall, wobei man heraus gefunden hat, dass die rechte Niere viel kleiner ist als die linke! Grund dafür war die Nierarterienverengung rechts, die Niere wurde mangelhaft durchblutet und das war der Grund für den hohen Blutdruck! Ich wurde zunächst mit 4 verschieden Blutdruck-Medikamenten eingestellt! Danach folgten noch einige Untersuchungen wie die MRA - Magnetresonanz-Angiographie! Mein Blutdruck war damals sicher schon länger sehr stark erhöht, weil im EKG wurde eine Linksherzhypertrophie entdeckt.
Dabei wurde heraus gefunden, dass meine Bauchschlagader (Aortenisthmusstenose) verengt war und beide Nierenarterien rechts viel mehr als links! Die Darmschlagader (A. mesenterica superior) war auch verengt, das war auch der Grund dafür, dass ich nicht so viel essen konnte!
Zusätzlich war noch eine Abzweigung für Leber, Milz und Magenarterie verengt ( Truncus coeliacus) verengt. In einer großen Operation bekam ich einen Aortenersatz mittels einer Dacronprothese und einen Bypass auf die rechte Nierenarterie und die anderen Gefäße wurde in die Prothese implantiert!  Die Operation hat 12 Stunden gedauert.
Ich war 3 Wochen im Spital und konnte dann entlassen werden. Der Blutdruck war sicher schon lange vor meiner Diagnose hoch, deshalb hat sich der Blutdruck auch nach der Operation nicht gleich eingestellt und ich habe noch für ein Jahr lange Blutdruckmedikamente nehmen müssen! Jetzt muss ich Thrombo-*** ein Leben lang nehmen, dass ist ein Medikament für die Blutdverdünnung (ein leichtes).
Alle 2-3 Jahre wird ein Ultraschall der Gefäße bzw. eine Computertomographie oder Magnetresonanz Angiographie zur Kontrolle gemacht!
Meine Operation ist jetzt 9 Jahre her und es geht mir super! Ich habe keine Einschränkungen bzw. bin jetzt gesund! Da es ein sehr seltenes Syndrom ist, hoffe ich, dass meine Erklärungen aushelfen, falls es irgendwem anderen auf dieser Welt betrifft! Ich hatte das große Glück von einem der besten Gefäßchirurgen in Austria operiert worden zu sein!
Die Operation hat mir zu einem ganz normalen Leben verholfen, ohne der Operation würde ich wohl nicht mehr am Leben sein!
Mein Motto lautet seitdem genieße jeden Tag, als wäre es dein letzter!!!

Avatar universal
by Neue71, Oct 06, 2010
Hello

My 5 yr-old daughter Olivia had a seizure in the middle of the night this past Sept 11. We called 911 and immediately took her to our local ER in Brooklyn, NY. While there they took a CT scan of her brain and neck to rule out an aneurysm or a tumor. Luckily, her CT was clean.

The doctor in charge thought it was "benign" epilepsy and said she needed an EEG to confirm, but because it was Saturday, there was no one to do the EEG until later in the day. I, being a neurotic New Yorker, wanted the EEG done right away! So I asked for a transfer to New York Presbyterian in Manhattan. At NYP they did an EEG and ruled out epilepsy. it was then they noticed my daughter's BP was in the 170/110's. We ended up staying 7 days at this hospital. They did an MRI first then an echocardiogram and during the echo they saw a coarctation of the aorta at the level of the belly. After an MRA they confirmed Olivia had this rare condition called Middle Aortic Syndrome (MAS) with renal artery stenosis that causes high blood-pressure, which, during a dangerous spike, caused her seizure.

All in all, Olivia was fortunate to "only" have a seizure. We were told sometimes BPs get so be so high it causes malignant encephalopathy and children can then have a brain hemorrhage or irreversible mental retardation. She's currently on a low doses of medicine to keep her BP between 120 and 140 because they are concerned that their kidneys need the high BP to keep working properly.

Olivia's kidneys are fine, her heart just barely enlarged, her aorta has a contraction that the cardiologist thought didn't require fixing; she's well developed (has hight of tall 9yr old) and full of energy. The bad news is she needs a very delicate surgery to fix her arteries and only a handful of surgeons in the US can be called real experts in MAS surgery. The top surgeon is Dr. James Stanley at the university of Michigan in Ann Arbor. We asked at the Boston Children's Hospital for a second opinion, the Philadelphia Hospital, and at New York Presbyterian as well, and everyone said we should see Dr. Stanley. All the top children's hospitals in the country send their young children with MAS to him.

We have contacted Dr. Stanley and we were very impressed by his experience with MAS (has operated on 52 children in the last 2 years, which doesn't sound like much, but other surgeons maybe do it a handful of times in their careers). Olivia will undergo revascular surgery sometime in late October.

I'll keep you posted.

606463 tn?1297889964
by lLoribean, Feb 15, 2011
Hi everyone. its been a while since i been on here. My son has MAS, he was DX at 1 1/2 month of age.  He is 2 1/2 yrs of age and is stable and on bp meds..  we travel to childrens in boston for his care.. looking for families that have children with mas.

Avatar universal
by Mona1973, Oct 03, 2011
Hello, my son was diagnosed with severe MAS when he was 8 months old. He had an angiogram while in hospital at Westmead Children's hospital in Sydney and they found his aorta was closed approx 6cms in length. They put him on BP medications to try and get his BP down from 200+, it came down around the 170's. The Drs wanted to get him as big as they could before they did any surgery.  In may of this year, when he was 2, his BP spiked again to over 200+ and they put him on many more medications, he is on 6 at the moment.
His left side of heart is enlarged due to working so hard, his left kidney is smaller than the other, but kidney function is good. Developmentally he is a normal 2 1/2 year old.
In June of this, his cardio thoracic Dr decided it was time to do something, as he wasn't improving being on all of these meds, and his heart was working way to hard. At the end of June, they inserted a graft from his aortic arch down to his arteries in his legs, as his arteries there were'nt great either.  He came through the surgery really well. He is still on all of the meds, but his BP is down to 140's, which he has never been. His Dr says that time may bring his BP down again, as the arteries stretch with this new blood flow. We go for fortnightly visits for his BP check. They won't cut his meds down until he's down to 110-120's. But hopefully we are in the right direction. In a couple of years they may move his kidneys to give them better blood supply.
It is soooo hard going through this, as it is such the unknown! But I really feel that he is going to be ok.
Would love to hear from anyone that has been through such a situation

Avatar universal
by vslam, Oct 30, 2011
Hello,
My son was diagnosed with MAS earlier this year at 15 months old in March of 2011.  We discovered his condition after he had a hypertensive seizure and went into respiratory failure.  Thank God I was holding him when the seizure occured because at first glance it was difficult to see something was wrong, he just kind of turned into a zombie staring off into space.  My wife and I could have very easily put him down for bed and he likely would not have made it through the night.  Fortunately we got him to the emergency room in Denver right away and the doctors intubated him.  It wasn't until we transfered him to a nearby childrens pediatric ICU that they determined his systolic blood pressure was in the 180s.

The doctors put him on blood pressure medication and increased it slowly to bring his BP down.  After many imaging studies, it was determined my son had a severe narrowing of his abdominal aorta and a complete blockage of his left renal artery which had permanently damaged his left kidney.  My son underwent two angioplasty procedures, one on his aorta and another on his left renal artery.  Both procedures produced good initial results, but after a few months the narrowing recurred.

Even before the narrowing had recurred, my wife and I sought a second opinion.  Our new doctors immediately referred us to specialists at the University of Michigan and Childrens National in DC.  Initially we received very different opinions from two doctors who had substantial experience operating on children with MAS, but neither said they would have recommended angioplasty.  After many consultations and a repeat angiogram five months after the initial diagnoses, we have decided to wait on any operations until my son is older and the aortic narrowing becomes an issue.  The doctors have told us young kids can tolerate the aortic narrowing because they develop collateral blood vessels to compensate, but at some point my son will not get enough blood to his legs, likely between the age of four and six, and a bypass operation will need to happen and he'll need another one post adolescence.  

Currently my son is on two blood pressure medications and his systolic BP averages between 95 and 105, which is still high for a kiddo his age, but not dangerously high.  His left kidney is no good because his left renal artery had been occluded for too long and irreversibly damaged the left kidney.  His right kidney had two renal arteries and the largest one continues to exhibit great blood flow with no narrowing while the smaller one does have narrowing.  The plan is to wait until my son is older before operating, but if his right good kidneys becomes at risk we'll have to do something sooner.

I've learned a lot about MAS and would love to speak with anyone out there to share similar experiences.  I'm not sure what the protocol is on this website, but please feel free to email me directly at ***@****

I'm also going to send messages to those who have posted and hope to hear back.  Thank you.

Avatar universal
by mixali949, Jan 18, 2012
Hello, my son also was diagnosed with MAS 5 months ago. He has the right artery of the kidney closed and also the abdominal and another one artery are narrowing. The doctors told me that the best option for my son is Doctor James Stanley in Michican and i want some details for him please if anyone of you know something please let me know. thank you very much

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by margypops, Jan 18, 2012
I do not know as of this moment but I will do some research about him and see what I can come up with ..Have you put this into the cardiology forum here on Med Help ?

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by margypops, Jan 18, 2012
yes I have just put it into the search engine and a lot has come up..he is Dr James Stanley MD Michigan University he is a vascular surgeon  ..here is a link for you      www.vitals.com/doctors/Dr_James_C_Stanley.html  if you want other information put into the search engine   Dr James Stanley MD Vascular Surgeon.Michigan (note its G not C in Michigan)

Avatar universal
by Leelee1970, Feb 13, 2012
My daughter had a kidney transplant in October 2010 (she is now 3 1/2 and thriving), lower aorta, superior mesenteric artery and celiac transplant (all in one piece) and she is doing wonderful (learning to eat, going to pre school).  Her surgery was done at Children's Hospital Boston and more recently her surgeon performed the first esophagus transplant (along with 5 other organs) ever recorded (she is a little nine year old from maine).   here's the article http://www.necn.com/02/01/12/Nine-year-old-Maine-girl-has-six-organs-/landing_health.html?blockID=643000&feedID=4210 . There are so many people involved in our daughters care from beginning to now who have saved her life.  I believe they are looking to start a program there because they are seeing more cases of this rare disease and more agressive cases (like our daughter's case).

Avatar universal
by vslam, Feb 23, 2012
Dr. Heung Bae Kim at Childrens Hospital in Boston has developed an new procedure to address coarctation of the abdominal aorta that my very well be a better alternative to the standard bypass procedure other doctors recommend.  I would encourage anyone with a kiddo with MAS to contact him.  Much thanks to the post above for providing the information which enabled me to locate Dr. Kim and speak with him about my son's case.

Avatar universal
by lovingmotherof4, Apr 03, 2012
My 9 year old daughter was DX with Mid aortic syndrom, She also has Neurofibromatosis type 1. Which we were told by the Doctors in Johns Hopkins University in Balitmore Maryland can be a complication from her Neurofibromatosis. Her blood pressures have been running from 145/90 to 130/86.  She is on meds and is having all the tests. We just had a angiogram and are getting ready to have a test to check for Kidney damage. We have to go back and have another angiogram they think her intestines may be in danger now. They said bypass is a must but want to get these tests done so that if there is anything eles they have to repair they can do it all at the same time. I'm really scared right now because she is really under weight and short her age. This is also caused from the Neurofibromatosis as well. They said there was not enough blood flow getting to the kidneys but needed to check if there was any damage. I can usally follow the doctors and what they say but there is not enough info on the web for me to look and get addtional info. This has helped alot tho. I heard it was very rare and that scared me even more. I see one person said that thier kid was in the hospital for 17 days did everyone have a long stay. The hosptial my daughter will be having her surgery at is 2 1/2 to 3 hours away from where we live and I have 3 other kids and just want to get a idea of how long I will be away from home and will need extra family help.

Avatar universal
by Leelee1970, May 21, 2012
vslam,
Dr. Kim performed our daughter's surgery.  In my eyes he is nothing short of genius.  You will be in good hands with him and the team at Children's.  It is our jobs as parents to help the docs in any way with their research and I hope they are able to get this program off the ground because it's so scary when no one has any idea what is wrong with your child.  We got so lucky we live near Boston and that the staff at Tufts steered us toward Children's Hospital as they were feeling helpless after 4 months of our daughter being in the NICU and them trying tirelessly to come up with a diagnosis.  No egos they just wanted to make our baby better.

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by Leelee1970, Aug 29, 2012
Hi everyone just wanted to let you know that we have been informed that Children's Program has gotten off the ground!!!  
http://www.childrenshospital.org/clinicalservices/Site3353/mainpageS3353P0.html


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by gogirlgo, Sep 02, 2012
Hello, my 15yo daughter was diagnosed with MAS in May after developing edema and high blood pressure related to a protein losing enteropathy.  Her BP came down as we decreased her edema, so it's believed her high BP is due to the protein losing enteropathy and not the MAS.  We still have not found the exact reason for the protein loss through her GI system but everyone here feels it is connected to the narrowing of her mesenteric artery and celiac arch.  She is being seen at the University of Virginia and I have had the team at Boston go over her records, but they do not agree with her MAS affecting the PLE and agree she does not need surgery to correct her narrow aorta, but do not have a GI on their team. Another factor in her case is an enlarged liver with normal liver functions, which has not been explained.  Her doctors' are making plans to refer her on, probably to CHOP.  Has anyone else had PLE or significant liver/GI involvement?  I would like to hear about it.  

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by bigjackson, Aug 20, 2013
I am a pastor of a small church in rural Arizona. I have a young couple, in my church, whose son has been diagnose with MAS. We are all trying to get educated on this and are looking for more information and possible doctors and hospitals who will handle this challenge. Any suggestions?

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by Laurapg, Nov 05, 2013
My son was diagnosed with MAS 6 years ago, and there was so little information at that time. It is nice to see a community develop over this rare condition.  

Dr. James Stanley saved my son's life 6 years ago.  We live in PA but traveled to Michiganfor Dr. Stanley.  He may be retiring from doing surgeries soon, but I'm sure he will do consultations for years to come.  



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by Stacygrace, Nov 08, 2013
My son was diagnosed with MAS at 15 months old. His first renal artery reimplantation on the left kidney was performed at 18 months by dr Stanley and dr eliason at university of michigan Mott children's hospital. The secon surgery to repair the right artery was performed this past June. Both surgeries were a success and he no longer requires ANY bp meds. I highly recommend u of m 734 936 4000. My son is almost 5 and is doing great. Dr Stanley has two doctors that will be taking over, Dr. Eliason and Dr Coleman. Both extremely capable and experienced surgeons.  U of M is world renowned for treating this condition and they saved my sons life

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by Donna_Atkins, Nov 11, 2014
I was not as lucky as most of you.  I Thank God that your kids are doing great.  My youngest son 17 yr 32 days old woke me up at 12:30 at night with chest and back of the head pains.  I got up and took him to Thomas Hospital in Fairhope AL ER.  His heart rate was 54 (I was not told that at the time).  They did no CT or MRI scan even thought they have the lastest equipment there.  Because of his age they didn't check out his heart, even though I told them there was heart problems on both sides of the family.  He died at home 11 days later on March 25, 2007 instantly.  He had an Aortic Aneurysm behind his heart.  He had a small tear above the heart on the morning I took him to the hospital and a large tear behind his heart on Sunday March 25th.  Everyone down here seems to think that this only happens to  older people.  As a result, our children are dying.  I spoke with a man last year and he said his son 16 died in his sleep.  Later they found out he also had an Aortic Aneurysm.  I am trying to let people know that it does happen to children.  God Bless you all and kiss your kids for me.

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by jgrubb75, Dec 11, 2014
My 5 year old son was diagnosed in March of 2014.  He has severe headaches in the night and into the late morning hours.  I requested an MRI of the brain.  When they were prepping him for sedation they took his blood pressure and it was 180/140's.  He was put in the ICU for 9 days and then released home with uncontrolled B/P for the next two months while Dr's in our state tried to figure out who would manage his care.  I quit waiting around when I heard the diagnosis and started reading the medical journals.  I called Dr. Kim Bae Heung and Dr. Micheal Ferguson at Boston Children's Hospital asking for help.  They helped guide me through the medical maze and we finally had him med flighted to Boston.  He was the 4th child to have the TESLA procedure which worked but when it was time for his final surgery, his aorta ruptured and they could not save enough vessel.  He has an aortic bypass gortex graft and bilateral gortex renal arteries.  It saved his life.  I am encouraging pediatricians and parents to take blood pressures in during annual checkups and to not ignore abnormal readings, but to follow-up on them instead.  Cameron was sent home out of the ER one year prior to his diagnosis with a blood pressure reading of 169/120's.  No follow-up or any concern was given to this reading.  Boston doctors said they believe so few have been diagnosed because they die before the syndrome is found.  Boston Children's is the only hospital with an actual program...Midaortic syndrome Renovascular Hypertension Program, as of the last I knew (2014).  I can't say enough for the team.  I spent 3 months living with them in the hospital and the last 7 working very closely with them.  617-355-5226 is their number.  I wish you well and if you ever need to speak with someone who has been down the path, feel free to respond or contact me.  Jackie

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by jgrubb75, Dec 28, 2014
I was rereading my post and want to make a correction on Dr. Kim's name.  For some reason,  I transposed it and got it wrong.  It is Dr. Heung Bae Kim.  When I said that Boston Children's is the only program, I meant the only program that I found to actually be named specifically for the syndrome...the Mid Aortic Syndrome and Renovascular Hypertension Program.  The other hospital that sees the MAS kids is at the University of Michigan Cardiovascular Center and is called the Aortic Disease Program.  What I was told is that what makes my son's condition so rare is the location of the stenosis which is at the renal arteries in the middle of the Aorta, so there was no better way for us to go except to a program named directly for what he had...MAS and RVH.  I contacted both programs but Michigan never responded.  Boston doctors called me directly just like I was a good buddy and walked me through the entire process of getting him back into the ICU and transported within days to Boston where we began immediately to work to save a child that no one was concerned about.  I wish you and your child well and please don't ever let anyone act like this syndrome is not a life-threatening situation especially if the B/P is high.  Stand up for your child and reach out to others until someone pays attention.  Never ignore a high blood pressure.  Your child is at immediate risk of stroke, seizures, blindness, heart failure, kidney damage/failure and worst of all death.  It is not something that should be put off.  We were sent home for 2 months and they wanted to postpone things for another month.  I was told that he had probably had it all his life and so he was used to blood pressures that were high.  Problem is my son's blood pressures were EXTREME. I was told by our experts they were sitting on a ticking time bomb and playing with fire.  I think most of us know that to play with fire, you are bound to get burned and I was willing to play when my baby was involved.  You will be heard if you call the program at Boston.  I can't speak to the Michigan program because they never returned my call, even though I sent records...Nothing.  I really don't know what happened with that except that my son was meant to be in Boston and spirit intervened.  Go with your gut.  It usually never leads you astray.  Best wishes,  Jackie from Kansas.

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by NickiJp, Oct 06, 2015
Hi - I hope that someone might still be around to read this & reply.... my son is 21 & has MAS & RVH. We are in the UK ( where it is even more rare as we are such a small country). He has just had an 8 hour operation in which his kidneys were auto-transplanted, with one connected to his liver & the other to his spleen,,using a synthetic graft. He will probably still need medication for life to control his blood pressure, but his doctors have told us that by having this operation he has increased his life-expectancy by 20-30 years & hopefully avoided the need for dyalysis & a kidney transplant when he's in his 40's. He was diagnosed when he had a sub-arachnoid haemorrhage when he was 9. This was clipped & he has had no effects from that, he has led a normal life & is at university. The surgery was 6 weeks ago & he is back at university & looking after himself again. Don't worry too much about your kids, it's amazing what doctors can do these days!

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by abbiechap, May 28, 2016
My pediatrician sent us to a pediatric cardiologist to follow up my daughter's 10 y.o. well visit last July.  She had slightly elevated blood pressure and she detected a heart murmur.  My daughter, Reese was diagnosed with Mid Aortic Syndrome and was put on two blood pressure meds to help manage the symptoms.  The result has been leg pain when she runs or is too active.  We are scheduled to go to Michigan on Monday to have surgery at Mott's Children's Hospital in Ann Arbor, MI to correct the aortic coarctation and narrowing of her renal arteries with Dr. Dawn Coleman and Dr. John Eliason.  

I would love to connect with someone who has been through this same experience.  Not sure what to expect and very worried for her.  

Feeling very grateful for our pediatrician after reading these posts - that it was discovered so early and without any complications.  My heart goes out to all of you who have been through so much.  

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by lLoribean, Aug 12, 2016
Hi I posted a long time ago. My son is 8 now. He needs surgery for his MAS. I have a group on facebook called MAS KIDS.. we are a group of families from around the usa , uk, and Australia. Looking for more families..  

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by bentzy, Apr 26, 2017
Hi my daughter has MAS was diagnosed at 8 weeks and is now 22 months.

Currently stable on four blood pressure medications.

I would love to join the facebook group if it is still running.

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by Jkkay731, Jul 31, 2017
Hi, everyone I have read your stories and I have sympathy with you all, I was diagnosed with MAS when I was 9 years old in 1982  ,and I had 'to wait until 1986 for the operations , I had a graft bypass on the aorta, and my kidney was moved to my pelvis area , I have been well since surgery and had a child naturally, so that's been really good, but recently I have become quite unwell , with hypertension being raised 180/120  , tiredness and fatigue, I am going to the doctors, and they don't seem to know anything, because like yourselves, there is not much information out there , I am getting pushed from one doctor to another , with no answers, I will keep pushing to find more answers , to find out if we MAS sufferers can have a long normal life , I am 44 now ,and as a parent like yourselves, you naturally worry, I would like to live another 44 years, and get answers for  myself and your children's futures , I will keep you posted, keep well

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by alioconnor_123, Aug 18, 2017
Wow. Thanks for posting Jkkay731. I wish you all the best with your treatment. Keep us all updated please.
I am from Perth Western Australia. My daughter Lucy was diagnosed with MAS at 8 months. She's now almost 3 years old and no surgery yet. Her BP is around the 140's systolic. Her aorta is not even narrowed, but virtually split into two pieces, so all the blood flow to her kidneys is via collaterals. Her BP must be a little higher so her kidneys can get enough flow. At the moment she is on four meds; Prazosin, Minozodil, Propranolol and Hydrochlorothiazide. We are waiting as long as possible for her eventual aortic graft. I worry about her every day and what her future holds. It's relieving to see that I am not the only one here in the same boat.

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by lca33, Aug 24, 2017
Hi, is this post still active? We just received a MAS diagnosis today. My 3.5 yr old daughter was noted as having high blood pressure at a routine check-up this summer, which has led to a round of tests: urinalysis, blood work, doppler ultrasound of the kidneys, and finally a CT Angio. Everything was coming back as fine, and pointing to the likely possibility of Renal Artery Stenosis, so getting this diagnosis has been shocking and very scary. Her cardiologist is referring her case to Boston Children's, I have seen some of you have experience there. If anyone is still active on this chat, please let me know... really hoping to reach out to someone who has experience with this. Thanks, Laura.

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by bentzy, Nov 07, 2017
MAS is so uncommon that it seems no one checks back here regularly, myself included. I am leaving my email here so if anyone in the future has any questions or just wants to contact a parent of a child with MAS they can actually reach me.

Ben Raice
***@****

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by bentzy, Nov 07, 2017
MAS is so uncommon that it seems no one checks back here regularly, myself included. I am leaving my email here so if anyone in the future has any questions or just wants to contact a parent of a child with MAS they can actually reach me.

Ben Raice
Benraice @ gmail

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by windels, Nov 11, 2017
hi, my son is 7 year old now and was diagnosed MAS in 2015 (20 month) . He received surgery in 2015 in Paris (shunt aortic and his 2 kidney moved in front of abdomen). He received 2 stents in kidney arter after 2 month.  But now the bloodpressure is high with 2 treatments. I wait for medical opinion.with pleasure to read you...

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by Chiemsee, Nov 18, 2017
Hallo
Gibt es jemanden aus dem deutschsprachigen Raum mit Erfahrungen über MAS.
Meine Tochter erhielt 2012 die Diagnose und wurde 2013 operiert.

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by lca33, Nov 21, 2017
Hallo Chiemsee,

Ich bin eigentlich Amerikanerin, aber wohne in Berlin. Im August erhielt meine Tochter eine MAS-Diagnose. Ich würde mich sehr freuen zu hören, wo Ihre Tochter behandelt wurde und welche Art von Operation sie in Deutschland erhalten hat. Meine Tochter wird vom Boston Children's Hospital betreut, aber ich würde gerne etwas über Deutschland wissen. Ich hoffe, dass es Ihrer Tochter nach der Operation gut geht.

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by Chiemsee, Nov 22, 2017
Hallo lca33
ich freue mich ja so, endlich mit jemandem Kontakt zu haben mit dem man sich über MAS austauschen kann!
Meine Tochter erhielt im August 2012 mit 13 Jahren die Diagnose MAS ( hochgradige Stenose von Aorta abdominales ,hoch u. höchstgradige Stenosen A. hepatica, A.mesenterica superior, Aa. renales beidseitig, Truncus coeliacus.
Es bestehen einige Kollateralen zu verschiedenen Organen die deren Versorgung sichern.
Es war damals unglaublich schwer sich für eine passende OP Methode zu entscheiden.Meine Tochter wurde zuerst in München Rechts der Isar bei Prof. Eckstein vorgestellt. Dieser wollte einen große Bypassanlage machen mit Rohre auf alle Organe. Da wir (Eltern Tochter Familie) uns soviele Ersatzteile in einem so jungen Körper absolut nicht vorstellen konnten haben wir begonnen zu recherchieren. Ein Professor in Frankfurt (Name leider vergessen) wollte alles aufdehnen. Das erschien uns zu gefährlich und nicht ausreichend.
Prof. Sabine Däbritz (damals Herzklinik Duisburg) konnte uns in einem sehr ausführlichen Gespräch ( zu dem sie sich Prof. Kolvenbach aus Düsseldorf dazugeholt hatte) davon überzeugen nur die Aorta aufzupatchen und irgendwann später die linke Nierenarterie aufzudehnen ( Versorgung der anderen Organe ist durch Kollateralen gesichert).Im Januar 2013 wurde meine Tochter dann in Duisburg von Prof. Däbritz und Prof. Kolvenbach( der sich übrigens weltweit mit Kollegen über den Fall meiner Tochter ausgetauscht hat) operiert. Sie bekam einen 10 cm langen Goretex Patch in die Aorta genäht. Sie überstand  die OP den Umständen entsprechend gut. Sie war 5 Tage auf Intensiv undann noch 10 Tage auf Normalstation. Sie hatte einen hohen Blutverlust bekam aber aus Gründen die eventuell in der Zukunft liegen könnten ( Organtransplantation) keine Transfusion. Sie erholte sich nach und nach und konnte irgendwann im März wieder zur Schule. Auch der verdickte Herzmuskel bildete sich nach einem Jahr zurück.
2015 wurde dann in München von Prof. Berger eine PTA  der linken Nierenarterie durchgeführt, leider ohne Erfolg. da diese Niere aber nur eine Funktion von 17 % hat, wurde entschieden es erst mal so zu belassen. Die rechte Niere hat eine Funktion von 83%  und insgesamt sind ihre Nierenwerte gut.
Für ihren nach wie vor zu hohen Blutdruck nimmt sie zwei Medikamente Olmesartan und Amlodipin. Der Sollwert war wohl zu lange verstellt.
Mittlerweile ist sie 18 Jahre alt, es geht ihr gut, das ist das Wichtigste !!
Mich würde sehr interessieren wie es ihrem Kind geht und was geplant ist. Wird in Boston dieses neue Verfahren angewendet in dem eine Art Kugeln eingepflanzt wird um stenotisierte Arterien zu verlängern um  dann später  den verengten Teil entfernen zu können?
viele Grüße und alle guten Wünsche nach Berlin


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by lca33, Dec 09, 2017
Vielen Dank für das Schreiben eines so detaillierten Berichts über die medizinische Reise deine Tochter. Ich weiß, es muss so schrecklich für dich gewesen sein. Wir planen mit ihren Ärzten jetzt eine Operation im Februar, und natürlich habe ich unglaubliche Angst. Im Boston Children's Hospital haben sie eine sehr neue Operation, heisst TESLA, die ihre eigene Aorta über einen Zeitraum von zwei Monaten dehnt. Dann wird der schlechte Teil ihrer Aorta herausgenommen und die guten Teile zusammengenäht. Zu dieser Zeit werden auch ihre Nieren bewegt, weil die Nierenarterien zu klein sind. Wie konntest du die langen Operationen und ihre Genesung überleben? Ich bin so nervös wegen all dem - ich kann mir nicht vorstellen, wie ich meinem 4 Jahre alten Sohn helfen werde. Es ist schwer, weil sie wie ein ganz normales Kind aussieht und sich so verhält, dass die Operation an diesem Punkt verrückt ist, obwohl ich weiß, dass es absolut notwendig ist. Ich freue mich sehr, dass es deine Tochter an diesem Punkt gut geht. Hat sie ihren Lebensstil stark eingeschränkt? Planst du für weitere Operationen in der Zukunft? Danke noch einmal. MfG aus Berlin

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by lca33, Dec 09, 2017
Has anyone here had a child who had the TESLA procedure out of Boston Children's Hospital?

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by Chiemsee, Dec 10, 2017
Hallo Ica33
Über Tesla in Boston habe ich schon einiges gelesen. 2013 als die OP meiner Tochter Anstand steckte das aber noch in den Kinderschuhen. Aber diese Methode entspricht genau der Meinung der Chirurgen meiner Tochter- so nah an der Natur wie möglich. Das heißt eben KEINEN Bypass.Die 8 Stunden die die OP gedauert hat waren wirklich sehr hart. Aber Prof. Däbritz hat uns Eltern mit einer solchen Selbstverständlichkeit gesagt das unsere Tochter nicht sterben wird. Und wir haben ihr total vertraut. Meine Tochter hat jetzt eigentlich keine großen Einschränkungen. Sie muß halt Medikamente nehmen und ermüdet etwas leichter aber sonst führt sie ein ganz normales Leben.In der Zukunft ist vorerst noch nichts geplant. Dennoch sind ihre Nieren noch ein Problem. Wie gesagt hat sie links aufgrund der Minderdurchblutung nur 17 % Funktion und rechts 83% . Ich hoffe aber das die Zeit für uns arbeitet. Die Medizin macht so große Fortschritte. Es wird eine Lösung geben wenn es soweit ist. Halte mich doch bitte auf dem laufenden wie es deinem Kind geht. Und du musst immer daran glauben das alles gut wird. Ich wünsche dir ganz viel Kraft.
Alle guten Wünsche und ihr schafft das!

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by Don1cannes, Jul 25, 2018
Hi, my daughter has neurofibromatosis 1 and MAS, anyone can help me get some infos, she is 4 and we are so worried of course..thanks in advance, we are in france but i d like to come to Boston for instance to be checked, they seem to be specialised in MAS but she also has NF1, thank you all in advance, Donald

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by Chiemsee, Aug 11, 2018
Bonjour Donald
It's right that they are specialized in MAS in Boston. But i think " Tesla" is only for short-streched stenoses.
In Munich (Germany) Großhadern is a doctor with much expirience in MAS. His name ist Prof. Haas.
Maybe he can help You.

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by windels, Mar 27, 2019
Hi,
My son had high bloodpressure (MAS operated in 2014) and it's decided to put 2 new stents in his 2 renalearteries,in Necker Paris (juli 2018). He keep 3 antihypertensivetreatments.
Good luck vor anymore.


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