What Exactly is Portal Hypertension?
Portal hypertension is an increase in pressure within the portal vein. The portal vein is the main vein that extends from the digestive organ to the liver. The increased pressure is caused by a blockage (clot) in the blood flow from the digestive organs to the liver. The increased pressure in the portal vein causes large veins (varices) to develop across the esophagus and stomach to bypass the blockage. The varices become very fragile and can bleed easily. If the varices are discovered through a gastroscopy they are usually graded on a scale of 1 to 3, with 3 being the most dangerous to bleed. In the US, they are graded on a scale of 1 to 4. (Esophogeal varices will be discussed in greater detail on another post).
In my particular case, portal hypertension was caused by thrombosis or clotting of the portal vein due to an apparently unknown genetic defect, which was thought to be some kind of protein deficiency? This cause often termed non-cirrhotic is not the most common cause of this condition, as is cirrhosis of the liver.
Cirrhosis results from the healing of a liver injury caused by hepatitis, alcohol abuse, or other causes of liver damage. In cirrhosis, the scar tissue blocks the flow of blood through the liver and will slow it’s processing function.
{ 18 comments… read them below or add one }
Hi Clint. My name is Lynn and my grandson who is 6 y/o has a long hx of PVT secondary to pulmonary hypertension. He also has cirrhosis and 4 Grade 1 esophageal varices. He was in Ohio today, to see a specialist.We are from Alabama and he has been treated here for most of his life, but with no solution. The MD that he saw today has scheduled him for a rex shunt procedure and also to have his gallbladder removed. I am a nurse practitioner buy I have not heard of this procedure and I am trying to learn more about it. Any assitance would be appreciated (risk, side effects, what to expect in the coming years, etc). Thank you and hope to speak with you soon. Lynn
Hi Lynn,
Thanks for your comments!
I am always saddened to hear of children dealing with this illness, or for that matter anything that seems to be complicated. Thankfully we live in a day and age when we have seen medical advancements like never before. The Rex Shunt procedure, relatively rare as it is, is one of those advancements which has been a positive breakthrough for children with PVT.
On my blog there is a very high interest regarding the Rex Shunt Procedure for children, and fortunately I have been in touch with more than a few parent’s whose children have had this procedure. I am going to try to put you directly in touch with one or hopefully more of those parent’s so that you can possibly learn more of the side effects and risks concerning the operation. Can I have your permission to forward your name and email address to those people?
Thank you – I look forward to your response. Clint
My three year old son has suffered greatly from this… Today he saw a Hepatologist for the first time. Arizona didn’t have a peds Hep until a month ago. He wants to do a Rex or Porto systemic shunt depending on MRV results. We are at Phoenix Children’s Hospital. I’m scared that this isn’t a procedure that has been mastered, or maybe he will send us to Chicago. My baby is a bleeder and is very small. He sees genetics and they are stumped . I’m lost here. Many more symptoms and conditions sadly!
Hi Christine,
Thank you for your comments! I can relate to the emotions you are going through. There are many people who have contributed information to this blog regarding the results of the Rex Shunt procedure performed on their children – and most are positive outcomes. The following is a quote I found from an article I was reading earlier this evening:
“The Rex shunt has proven to be an effective method of resolving portal hypertension caused by EPVT including thrombosis after living donor transplantation. This shunt is preferable to other surgical procedures because it eliminates portal hypertension and its sequelae by restoring normal portal flow to the liver”.
Refer: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WKP-4F7S93F-5&_user=10&_coverDate=01%2F31%2F2000&_rdoc=1&_fmt=high&_orig=gateway&_origin=gateway&_sort=d&_docanchor=&view=c&_searchStrId=1670924732&_rerunOrigin=google&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=84e9926252aebfc61a5ced7a98b67ad6&searchtype=a
According to what I know so far, the Rex Shunt has been successfully performed on over 150 children at Children’s Memorial with many others now being performed in other places around the world. If you can ignore the “hype” as it were with the following article from Childrens Memorial – if anything this should be an encouraging article for you or anyone else to read: http://www.rbabyfoundation.org/medexchange/userfiles/s%20Memorial%20Hospital%20Stewardship%20Report.pdf
Genetically speaking, I was diagnosed with PVT in early 2002, and doctor’s have never pinpointed a cause. At that time there were 7 known genetic defects which could cause this kind of clot, however I have tested negative on all of them. have an underlying clotting condition, and the the complication is called non-cirrhotic portal hypertension. Don’t want to be negative or cause too much alarm for anyone, but the options for adults are slim except for what seems to be standard protocol of treating sufferers with medications to control the complications. See: http://www.portal-hypertension.com/the-management-and-prevention-of-portal-hypertension/
In summation I believe this procedure has been mastered, and any doctor’s without experience have the expertise of their collegues who no doubt would be in very close consultation. Of course you need to have your doctors advise you to what is best, so don’t solely take my word for it. There are many stories posted here and especially regarding the Rex Shunt – so have a look and see. Check under the header http://www.portal-hypertension.com/the-rex-shunt-procedure-for-portal-hypertension/
Hope this helps?
Clint
Hi Clint and everyone.
It looks like your web page turns into international. I want to share with you with my story – a 30 years old guy from Poland.
It all had started in NOV 1995, when I was 14 I had an appendectomy (latin: appendicitis acuta gangrenosa. Peri tonitis diffusa). I went to three different doctors to diagnose it… and finally the fourth one made a decision to do a surgery.
Three weeks after I was diagnosed with splenegomaly due to spleen vein thrombosis. After dozens of blood work, MRI, USG etc docs decided to removed my spleen. .
For next two years I didn’t have serious problems. In 1998 I was diagnosed with portal vein thrombosis. I was taken to the hospital for a treatment (accecumalor, heparin).
CT showed that around my liver and portal vein there was “new” blood circulation developed. Docs here calmed me, that everything is all right since the blood goes into the liver not to stomach, and there is no reason to wary.
But I hope everything will be fine.
For the next 10-12 years I was feeling good with now serious problems. Although USG showed problems with portal circulation, I wasn’t on any treatment.
Three years ago I did blood test which showed anemia. I was prescribed ferrum but after 3 months treatment results showed no effect. I was asked to do a gastroscopy to find out if there is any blood leak.
In October 08 a month before my wedding I was diagnosed with esophageal varices (medium, 2/3). Since then I have had DVL six or seven times. First time 10 bands was placed, month later 6 bands was placed. Another 6 bands was placed on February 09, etc.
Begining in October I was also prescribed Proplanolol, which I’m taking every day (2×40mg).
I have had a lot of tests and examinations. The liver functions seems to be ok. ALT, AST, Bilirubin and another tests for liver functions are well and in range. I have never had any of liver viruses (HCV, HBV, etc)
In February I was diagnosed with another thrombosis in portal system. I’m on treatment (heparin and acenocumalor).
Like most of you guys, I’m searching on-line to find new ways of treatments, new medicines, etc. Although my live is quite normal, for two and a half years the live conditions are a bit worse. A pain appears from time to time around my belly
If any of you have any questions, I’m happy to answer.
best
Bart
And sorry for possible grammar and spell mistakes. My English is far from perfect… yet.
No problem … I understood your post very well for the most part!
Hello Bart,
Thanks for your comments … this is so valuable to me and I am sure others! You certainly have had an interesting and scary journey these past 15 year’s.
You mention Proplanalol at 2 x 40mg per day. Although I fortunately have not had a bleed from my esophagus, and only my stomach – a beta blocker such as prescribed to you is standard treatment for the prevention of bleeding. I am on 140mg per day, which I understand is a very high dose and this maybe the reason I have not bled from my esophagus? If I can tolerate the associated fatigue of being on this dosage, my gastroentrologist may increase that dose another 20 mg to be on the safe side? My liver function is good also, and I think this is a definite plus for anyone who has portal hypertension.
It is amazing of just how many bleeds you have had, and the amount of banding that has been performed on you to date. You must live close to a good hospital? You also mention that you were on Ferrum to bring up your iron levels. It could be that you are losing iron from varices in your stomach from a slow bleed. This is what is happening to me so my Ferritin levels are monitored (bi-weekly) and at 4 month interval I have to go to the hospital for iron dextrin treatments to keep the levels high. Maybe this is something you could ask your doctor about? In my case my family doctor and my hematologist (blood specialist) monitor these levels. I have been going for iron infusion for almost 5 year’s now.
Well … I do hope this helps? I certainly know that what you have posted is valuable for anyone and I so appreciate you posting this information. Take care!
Hi Clint,
There is a miss understanding. Although I have 7 or 8 band ligation, I had never had a bleeding from esophagus. Maybe I have had a minor and long term one, since I have low iron level.
Anyway I will keep you posted about my condition. And here is what I found, and might be interesting form you http://www.springerlink.com/content/81215p4311667461/
bart
Thank you Bart!
Glad to hear you have not had a bleed! Doctor’s considered band litigation on me about 4 year’s ago, but decided against it as I am on a very high dosage of anti-coagulants. Because I am prone to further clotting I am on Fragmin injections twice per day, which from what I was told is extremely rare. I managed to survive a long time now without a bleed so I feel very blessed.
I looked at the article you sent and it is very interesting. I am not sure what the overall consensus is amongst medical specialist’s regarding what I could decifer on the page, but much of the latest research on Portal Hypertension originates from the University of Barcelona in Spain. There seems to be continuity on the overall treatment of PH though and that seems to be the use of Nadolol (beta blockers) to keep the blood pressure down in order to curb bleeding from the esophogas.
Thanks again for your input and please let me know if you hear of anything new in the area of treatment for this condition.
Take care,
Clint
Just caught up with this site and don’t know if it is still active. My story is a lot like Bart’s and would be happy to share it with any of you if you think it would help. Lived with PVT for 11 years now with 5 banding sessions following one episode of esophageal bleeding varices. Scarry stuff and am now on coumadin to help with pain – works really well most of the time but of course increases the risk if I should develop another bleed. Lots of decisions to make.
Hi Janet,
Thank you for your comments. Yes my site is still active – I just haven’t posted much on here lately and last night was the first in several weeks.
You have had a precarious journey with your portal hypertension. Most definitely any information you can offer is most helpful to others (myself included). I have had PH for 9 year’s with complications, but have yet to have any banding done. I had a bleed in the upper part of my stomach, but not the esophogas however I have columns of varices protruding into the area. For unknown genetic reasons I have a underlying clotting condition which caused PH and I am on Fragmin injections for a blood thinner as Coumadin does not work for me.
I would be curious to know what the cause of your PH was, and how you cope on a day to day basis. Your comments are always welcome – thanks again for contributing! Trust you will not experience any further complications!
Kindest regards,
Clint
Why can’t they just use clot buster to break up the clot?
From what I read you can get a liver transplant and they can sew the portal vein back on so why can’t they just cut the clot out and sew the vein back together?
Medicine has all these wonders for the heart and other things but they can’t remove a clot! Why? I read nearly everything on the this site and am feeling really bummed out. I can’t get transfusions so I feel my life is in jeopardy if I ever get a bleed that bad.
As I mentioned in the other post I am an athlete, is even possible to exercise or will this raise the risk of getting those varises in the esophagus and stomach?
Sorry recently diagnosed and am having a hard time mentally.
Any help or advise?
Thanks though Clint for all the good info here.
Please help with any answers to both my posts thanks.
Keith,
I completely understand your frustration over this as I myself and many others I know feel the same way. It would seem simple enough just to get a liver transplant, but because your liver is healthy it would be highly unlikely for that to happen. Secondly there is no clot busting method available but there is a procedure called the Rex Shunt which unfortunately works the best on children who are under the age of 5 years old. The reason for the success is the fact that there is more atrophy in this vein the older one is. Unfortunately the few attempts surgeons have tried on adults patients were unsuccessful, HOWEVER there is another bypass operation that might be a consideration for you. It is called the mesocaval shunt procedure: http://medical-dictionary.thefreedictionary.com/mesocaval+shunt This may be an option to discuss with one of the doctors I have listed in the post I created a few minutes ago.
My portal, splenic, and messeteric arteries are all clotted, but to see me you would never believe I had all that going on. I had a bleed in 2004 from the varices in my stomach, and I came close to having a blood transfusion but the bleeding stopped. I have also had clots to both my lungs – and that is very scary as that could also have taken my life. Because of the treatment I am on today, and my strong faith, I believe that has sustained me over these past 9 years. I say all this to tell you that it is not the end of the world if you have to be on thinners, because they will help in the end. I am also on a high dosage of Nadolol (beta blocker) to slow my heart rate down, and it makes me very tired – however I am still ticking so that is a blessing!
Please try not to get bummed out … PVT is serious enough, but you do not have esophogeal varices and that in my opinion puts one in a more precarious situation than without. If it is any consolation to you, I and many I know have survived a long time. I know some who are not as athletically inclined as you are, but they still exercise and live relatively normal lives. Of course I always tell people not to take my word for any of this, but always ask your doctor. I do hope you find a specialist somewhere that can help and advise you and you get answers soon.
Please hang in there and keep us posted as to what you find out.
Take care,
Clint
Hi Clint,
I am 52 years old and have just had the TIPS procedure due to cirrhosis of the liver leading to scarring and ascites. This is due to hard living when l was younger so it was self-inflicted and all my own doing.
What l’d like to know is if l can get a low salt diet on the net, l go to the Alfred Hospital in Melbourne, Australia which is excellent apart from the fact l can’t seem to get a diet that is ideal for me or a list of low salt foods that are the best for me. l spend a lot of time at the supermarket reading sodium quantities in food l buy but don’t fully understand what l should be eating.
l need a list of foods that are the best for me. l know processed foods are bad but sometimes there is no other choice.
sincerely, Dave Dawson
Hi Dave,
Thanks for your inquiry. Sorry to hear of your health situation. Sounds like you had a successful TIPS operation, and if you have a chance I would not mind hearing how long ago this took place and what kind of stabilizing meds you are on right now.
As far as a low sodium diet is concerned I have asked this question more than a few times in the last 10 years to see what may helpful to the specifics of portal hypertension. The last time I asked was at the beginning of this month (Jan. 2012) and was told exactly that there was nothing specific, because of the rarity of our condition. My problem with Portal Hypertension is all the related bowel issues due to the arteries in the lower part of the bowel being engorged with blood from the portal and spleenic vein clots. There are certain foods I abstain from, but as yet I have not been able to pinpoint what sets me off and causes severe pain at times. All being said, I know you have educated yourself in foods with low salt and at this point that is a good start until someone comes up with something specific to the condition. To help you and others who may be reading this I have done “some” research that may elighten you further (see links below). Before I do that though I want to point out that the DASH diet below is something that I have heard may be helpful to anyone (especially those with hypertension) who need to be on a low sodium diet.
http://heartfunction.ktp.utoronto.ca/modules/UHNsalt.pdf (More simple guidelines for what to eat)
http://dashdiet.org/ (The DASH diet)
http://www.ehow.com/how_5045419_create-low-sodium-diet-plan.html (Creating a low sodium diet plan)
Keep fighting,
Clint
Clint
I have been diagnosis with portal vein hypertension over 30 years ago and recently learned it was caused by thrombosis. I do not qualifiy for a liver transplant because my liver and liver function tests are fine. However, I have had 2 bandings and numerous bleeding episodes since first being diagnosis. I am currently on proponol and also take lasix for the ascetis. My hepatologist informed me that if the bleeding starts is just to keep having the bandings. I should explain that the bleeds are massive requiring 2-4 units of blood being transfused. Is there any other procedures are surgeries that you can suggest to help stop the bleeds? The bleeds are becomming more frequent at least 1time a year.
Hi Rachel,
Thanks for your post. I am truly sorry to hear of your bleeding episodes and my heart goes out to you. The Rex Shunt and a few other shunt procedures seem to be successful for children in particular as atrophy of the veins is an issue when one gets older. I had heard that over the age of 5 it is harder for doctors to do the Rex Shunt procedure, but one has to be the ideal candidate before consideration is made in all cases and if there are other complications of course this would play into the process. Now there is a procedure called the Sugiura procedure which was developed by the University of Tokyo and has showed some success to date. See: http://www.ncbi.nlm.nih.gov/pubmed/8042326 I have been meaning to write an article on this for my blog, but can’t seem to get information that is understandable for me to digest and re-write for the benefit of anyone needing a layperson’s view of it. The procedure was talked about in my case, and I would have easily forwarded my imaging to the doctor in Chicago who I believe has attempted a few of these, however my condition is considered to precarious to tamper with as I have to be so highly anti-coagulated for fear of further clotting. In your case it may be different so I am going to suggest that you contact Dr. Riccardo Superina at The Childrens Memorial Hospital in Chicago so maybe he will consider your case or refer you to another hospital/doctor who could at least review your situation and go from there. I would also suggest to contact Dr. Jean Emond at New York Presbyterian Hospital as I have been told by others that he is a good surgeon and may have some ideas for you? See contact information posted below. Please keep me informed if you hear of anything – hopefully this helps? Thanks again for your post! Regards, Clint
Dr.Riccardo Superina
2300 Children’s Plaza
(707 W. Fullerton Ave.)
Chicago, IL 60614-3363
Call 1.800.543.7362 (1.800.KIDS DOC)
Phone 773.880.4000
Dr. Jean Emond
NewYork-Presbyterian Hospital/Columbia
PH Room 14C
622 West 168th St
New York, NY 10032
Phone 212.305.9691