Hi Johnann - I know exactly how you feel. I had to fight tooth and nail to have my PVR, which they finally got to December last year (7 weeks ago today!).
I didn't know what the "normal" RVEDV was (it seems all these tests are predominately set up to deal with the left side of the heart and so I only have "normal" ranges for the LVEDV), but in Jan 2007 my RVEDV was 189mls and by Jan 2008 was 239mls. Still they were telling me this was only moderate (although that's still quite a change if you ask me!).
I have a book on Tetralogy of Fallot (which is why I needed my PRV) and according to the experts in the field who wrote it, other indications it is time for a PVR are:
Fatigue, exercise intolerance unexplained by factors other than right ventricular abnormalities.
Greater than mild right ventricular enlargement.
Abnormal exercise test: low VO2 unexplained by other factors.
Mild to borderline decreased right ventricular systolic function.
Arrhythmia unexplained by factors other than right ventricular enlargement.
The final determination, in terms of when to intervene for pulmonary valve replacement despite all of the available studies, can be difficult. Those patients with moderate to severe RV enlargement and moderate to severe pulmonary regurgitation with symptoms, clearly need to have their valve replaced; if they have reached this stage, one has probably waited a bit too long. Because there is a good operation with low morbitity and mortality and the longevity for current biosprosthetic valves is reasonably good, earlier intervention is probably the proper choice. In most patientes with border-line decreases in ejection fraction, moderate or greater degrees of pulmonary regurgitation, and modest or greater degrees of exercise intolerance, repalcement of the pulmonary valve is indicated.
(The Adult with Tetralogy of Fallot: Michael A Gatzoulis, MD, PhD & Daniel J Murphy, Jr,. MD)
Now I don't know if you also have ToF, but since you're seeing an ACHD cardio, I'm guessing you probably do. Either way I would imagine the same parameters would apply.
In my own case, even when I was able to tick ALL the boxes above in regard to having severe pulmonary regurg, lowered EF, enlarged RV, exercise intolerance, thousands of PVCs a day, etc... they were still trying to tell me they didn't think my heart was the cause of my symptoms (I must have Chronic Fatigue Syndrome!!) and that they didn't want to go ahead with a PVR! I was lucky enough to move interstate again and see a new cardiologist who - while still doubful the PVR would help - was prepared to go ahead with it if that was what I wanted (YES!!!). I took all the above info with me to the meeting with my surgeon, but he agreed it was time, so I didn't have to try and convince him - but I wasn't going to leave his office unless he had agreed to the surgery!!
Anyway, I had the surgery and already I am seeing some fairly significant changes. I can talk on the phone and not get short of breath. I can walk at pace and barely get short of breath - even with me TALKING at the same time!!! My BF commented I hadn't been able to do that in years!! Another big difference is that going for a walk once again lifts my energy levels some, whereas in the last few years prior to the surgery, regular exercise and even walking was becoming increasingly difficult and tiring.
Even at my 2 week post-op Doppler, my cardio had to admit that even without having my last test to compare it to, he could see the improvement to my heart function.
Seems I was right after all!!
So, take it from me, get as much info as you can and push as hard as you need to, to get things done. Don't rely on waiting for them to get things sorted - you are only one of many patients they have and therefore not their highest priority. You have to MAKE yourself their highest priority!!
Like you I was also very scared that by waiting too long I wouldn't make the recovery I could if they acted in time and even with the improvements so far, I still don't know how much more I will recover. I'm hoping to get back to where I was before I became symptomatic, but at this stage it's still too soon to tell. Still - I HAVE made improvements and that is a good sign, so don't give up all hope yet.
Good luck!
Anna : )
