Pulmonary hypertension secondary to lupus

[loopey]

Hello and thank you for taking my question.  I am a 30 year old female who has pulmonary hypertension (mean pap in the upper 30's) and diastolic dysfunction secondary to lupus.  I've been told that I can't take the medications normally given for pulmonary hypertension because of the diastolic dysfunction. Also, I can't take beta blockers, they cause shortness of breath. My rhuematologist has told me that he wants me to use my oxygen on 2L 24 hours a day, regardless of the fact that my O2 sats are usually in the mid to upper 90's. The oxygen does help when I'm having palpitations and very strong/fast beats. He says this is to keep my heart from working harder than it needs to.  I thought using oxygen when you don't need it was dangerous.  

My questions are:
1. Is using oxygen to treat a heart condition, without having a low saturation, normal and safe?
2. Since my pulmonary hypertension can't be medicated, should I expect the lupus to continue to make my heart worse? (He has started me on plaquenil)

I know you can't make any specific statements to my case, but your thoughts on the subjects would be greatly appreciated.

[Forum-M.D.-bkj]

loopey,

Thanks for the post.

Lupus is a difficult disease to manage. The range of symptoms and damage to the different body systems it can cause is marked.  Pulmonary hypertension is also a difficult disease to manage.

Generally, I would recommend seeing a pulmonary hypertension specialist in order to treat your disease.  Im not sure why you are oxygen constantly, unless there is an issue with your oxygen saturation which could come from lupus lung disease.  Since you state this isnt the case, Im not sure why its being used.  There are multiple different medications for pulmonary hypertension. Your PA pressures are elevated, but not severely. Im not sure why they state they cant treat your with either beta or calcium channel blockers. These medications are often used to treat diastolic dysfucntion.  

There are several centers that are specialized to see patients with lupus. Johns Hopkins has a center, as do most tertiary centers. It might help to seek consultation at that type of center.

good luck

[EchoTech]

It is important to note that I'm not a doctor

1. As far as extra oxygen is concerned, I was under the impression that it really isn't going to help you.

2. As far as the lupus is concerned,  I would venture to say that it will continue to adversely affect your heart.  The pulmonary hypertension is something that just hardly ever gets better on it's own, but there are medicines that can help, but I can't remeber what they are.  I also beleive that sometimes it takes a few months to a half a year to get used to some medications, but since I don't write out prescriptions, I really can't judge how this would affect your situation.

Good night and good luck.