Teen with bicuspid aortic valve and dilated aortic root taking Losartan??

[MomofLenny]

Hello,

I'm the mom who has a son that was diagnosed by chance with a bicuspid aortic valve and dilated aortic root at the age of 13 years old.  He was diagnosed in May 2008.  Initially the ascending part of the aorta was 2.6 and has progressed steadily with his six month checkups to 4.0 as of Feb 15, 2010.  His root measured 3.8.  He has had all genetic testing (Marfan's, Loeys-Dietz, Famial Thoraic) and they were all negative.  He is now 15 - 5"11" and 145 lbs and still not done growing.  His Dr. is communicating with Dr. Dietz at Johns Hopkins in regards to Losartan.  Does anyone have any input on this drug with a teen?  Or does anyone have a child in this type of situation?  Any input would be comforting to me.

Thank You
Sue

[121212mom]

Any updateson this? I am in the same boat but with 2 sons

[Jpos]

I am 43 and have the same issue as your son.  I have enjoyed sports all my life and have been a competetive cyclist for the last 20 years riding and racing as many as 400 miles a week without any complications.  I was diagnosed approximately 3 years ago so this is all new to me now, but have continued to ride and even race occasionally. It's been disconcerting to have this floating in the back of my mind, but its' not the end of the world.

I'm going in for my annual checkup this week to see if there is any change from last year at 4.8.  Good luck.

[skydnsr]

I would follow up on this if it were my son.  I think this is a cutting-edge approach.  It does have research to back it up.  I also agree very much with what nickppatel says about going to a major heart center if and when your son does require surgery.  

You might want to check out the website www.bicuspidfoundation.com, if you have not already.  I think there are some "heart mom" organizations out there, too.  I can't give you a link for the heart moms, but try doing an internet search, or maybe the Bicuspid Foundation can point you in the right direction for that.

Good luck.  One lucky thing that has happened already is that your son was diagnosed.  Another lucky thing is that, as nickppatel says, you son's doctor has the good sense to seek out an expert.  I know it must feel just horrible to have your son going through this -- but with good care, he can live a long, normal, happy life.

[nickppatel]

Hi Sue,

Please take a moment to click on my screen name to view my many prior posts on this topic.

Also visit www. marfanlife .net/bb3 (remove the spaces; I put them in there so that this website would let me display the address) and look at the "Pharmacy" section for extensive discussion on this topic.

My understanding is that it would be OK for a teenager to be on this type of drug, especially one who is only 3 years from being an adult.  I do not know this for a fact, but I know that the clinical trials Dr. Dietz is conducting with losartan take patients with Marfan Syndrome who are far younger than your son.  So, I'd imagine it would be OK for your son to use.

This drug - and other ARBs - have shown great success in halting and reversing aortic root enlargement WHEN and ONLY WHEN TAKEN IN SUFFICIENT DOSAGES.  It is thought that losartan is not as good a choice as other ARBs like valsartan and telmisartan because losartan penetrates tissue less than those drugs and because losartan is a surmountable antagonist that could, theoretically, lose effectiveness over time.  This isn't a proven fact to my knowledge and Dr. Dietz for various reasons may or may not agree.  Regardless of the ARB taken, it must be taken in a sufficiently high dosage or it will do nothing, and your son still needs to be followed (imaged) frequently until we see some positive progress.

Genetic testing is worthless in my opinion because it often gives false negatives.  Your son very well may have MFS, LDS, etc. or he may not.  All that is besides the point - you need to watch the aorta.  If you are concerned about LDS, ask your cardiologist to look for other, classic, clinical signs, such as split uvula, tortuous aorta, etc.

I am glad your doctor has the sense to contact an expert in this groundbreaking research so that your son receives the best care possible.  Many, many other doctors would have dismissed your son.  You are on the right track.  The only thing I would be concerned about is the fact that your son has a bicuspid valve.  I am not sure of how successful ARBs have been or will be for people with aneurysms related to bicuspid aortic valves.  It may be the case that your son will need a surgery to replace the bicuspid valve and then supplement with ARBs to reduce aortic root size or perhaps resect the aortic root completely.  All this is guesswork, though.

You really need to be aggressive on this and get your son on these meds if your doctor/Dr Dietz approve.  MAKE SURE IT IS A HIGH ENOUGH DOSE.  Usually for someone your son's size that means at least 100mg /losartan/day (or 80mg/telmisartan/day or 320mg/valsartan/day), but I defer to Dr. Dietz (he will probably say the same thing as me).  After your son is on the meds, watch the aorta carefully to see what happens over the next few months.  A 6-mo. checkup is vital (if not sooner) to see how the aorta is progressing - is it shrinking, stabilizing, or growing?  If a high dose ARB does not work, surgery may be in the future, although you need not worry because the surgery - done at a high-volume center like Johns Hopkins, Cleveland Clinic or Texas Medical Center - will almost certainly give your son very excellent results.

He will be fine.

†
Nick