I wa diagnosed with PAH 3 years ago. Have been on Adempas, plus other BP medicines and diuretics. I also have numerous heart problems, triple bypass in 1993. I see a Cardio/Pulmonary doctor Who is also a PAH specialist. Since finding this community, I have learned so much from all of you. I love my doctor, but after reading so much here, I realize I have really no clue as to what type or severity PAH I have. I have had 2 right heart cath for pressure in the 3 years ( first one was to diagnose) I have checkups every 6 months, which generally involve a yearly echo, and a 6 minutes walk. I have an appointment next Thursday, and i am wondering exactly what questions I should ask him. You talk of "types , "stages" etc. He has never said any of this to me. I feel like I am fairly in the dark as to exactly where my PAH is, mostly because I did not know much before finding all of you. I have been reading and reading, but need advice on EXACTLY what I should ask him. Sorry this is so long, but I need to know what I should know. I am so grateful that I found this site, and I admire so many of you who are much worse off than I am. Thanks in advance if you can give me specific questions to ask him. See more
types of PH are listed in the "WHO Classification of PH" which you can find and download from the files page. I always keep a copy on me so that I can show doctors you disbelieve me (which they do often). As for stages, that is also listed in the files section as 'Functional classes' of PH - good idea to download that too. I would also ask your doctor what your mean pressures were from both of the RHC's and what your estimated mean pressures were from the last echo. They can only estimate the pressure in an echo as they can only get the systolic reading and therefore estimate what the diastolic reading could be before they work out your mean pressure. Therefore an echo is not reliable, but as long as the numbers are consistent they have not need to keep doing a RHC. If your doctor does not know enough about your condition then perhaps you need to get him to refer you to someone who is a ph specialist and who will keep you informed of your pressures, blood tests etc. It is important for you to become your own advocate
Also, remember that PAH is different from PH. PAH is Group 1 pulmonary hypertension and most of the newest medicines are for this group. Group 1 exhibits changes in the cellular structure of the linings of the pulmonary arteries.The other groups are caused by other problems in the body, not by changes in the pulmonary vasculature, and are generally treated by identifying and treating the underlying problems such as pulmonary embolisms, connective tissue diseases, or left heart disease.
Posted in Pulmonary Hypertension
Pulmonary hypertension is hypertension of the lung arteries that leads to narrowing of their lumen. Dyspnea is one of the most prominent symptoms, accompanied by fatigue, chest pressure or even syncope. Signs of right heart failure may also be present as a complication of pulmonary hypertension, which is the case of cor pulmonale.