I thought that my increasing shortness of breath was from asthma but as it continued and didn't respond to asthma tx, my allergy and immunolgist encouraged me to see a cardiolgist. It took a couple of months but I was able to see one at Cleveland Clinic. He asked me to get an echo and a stress test. The echo showed pressures consistant with PH but I was told I needed the stress test before he could make any recommendations. My BP shot up too high and they couldn't put me on the treadmill. In the meantime, I got a new Primary at Metro health system (a Nurse Practiioner) and she got me referred for a nuclear stress test but that took another two months to get done.
When the stress test was normal, the cardioligist responded just to that on my patient online account. I had to push back with him and point out the previous findings reported on echo and ask what I should do. At that point he ordered spironolactone and told me to see a hypertension specialist if that didn't work.
The spironolactone has .brought my BP down but the shortness of breath is still bad. So my Nurse Prac put in a referral to "Pulmonary Hypertension" at Metro Health, I am scheduled for breathing tests and then to see a Pulmonolgist but not until almost the end of February (referral made Dec 8).
I have had pulomonolgists for 13 years for sleep apnea but, appartently, the sleep medicine is all they do.

I am also a Zebra for "rare diseases" as I have Common Variable Immune Deficiency that didn't get diagnosed for years despite seeing ENT and Allergy Specialist at Nicolet Clinic in Minnasota, an ENT at University of Kentucky Medical Center, an ENT specialist in Cinninati and an both an ENT and allergist at Cleveland Clinic. I finally was diagnosed by an immunologist with MetroHealth and then transferred to a local private practice where I get IV gammaglobulion therapy every 4 weeks.

I am on IV treprostnil (generic Remodulin). I have small patella syndrome, which is a heritable form of PAH. My doctor is a pulmonologist at a PH center, although some of the staff at the center are cardiologists and when my doctor isn’t available (on call), I have talked with the others.

Small patella syndrome is a mutation in a gene called TBX4. This gene controls when other genes are turned on and off. So, besides the PAH, the mutation can also cause problems with the hip, knee and feet development.

It’s a very rare disorder. It’s even more rare to develop the PAH from this disorder as an adult. I’ve learned that about 80% of patients who develop PAH from this mutation show symptoms as infants and toddlers. I had a better probability of winning the actual lottery, but I won the genetic lottery and I have all the symptoms, lucky me.

I will digress a minute for a shameless plug for rare diseases day. It’s Febuary 28th, 2023. Check out the National organization for Rare Disorders website: rarediseases.org

I’m fortunate I went to a PAH center that knew about it, Mayo Clinic Rochester. They also know how to best treat the PAH when caused by this rare genetic disorder.

Ironically, I’ve seen specialist at Mayo in the orthopedic department for years for my hip, knee and feet issues. However, small patella syndrome is so rare that the specialist in the orthopedic department at Mayo didn’t recognize is or prompt me to do predictive echocardiograms every year looking for PAH. I wish they had because maybe we could have caught it before the pressures were severe and I was in right side heart failure.

Again, lucky for me that the PH center doctors know what they are doing. The heart failure is reversing itself because the PAH is now better managed. The doctors believe I will never have normal pulmonary pressures, so the goal is to manage the pulmonary pressure to a point where my heart can handle it. At my last doctors appointment in December, they were happy with my progress. It seems triple therapy is achieving this goal.

Well that is more than asked and probably more than you wanted, but I’m long winded.

Hope all is going better for you.

I've had shortness of breath, chest pain, and heart palpitations since 2013. We used to travel fulltime in our RV. We were working a seasonal job at a high elevation when my symptoms started after about 6 weeks on the job. I found a cardiologist, did a full cardiac workup. Even had a heart cath. All was good and I was declared a low lander. The symptoms pretty much abated until 2017. The shortness of breath started again in 2017. I attributed it to being overweight and out of shape. I lost weight, began a regular exercise routine, and the symptoms resolved. In 2020 after a mystery illness to took me down big time I was diagnosed with Sjogren's. Within months of my Sjogren's diagnosis I began experiencing all the symptoms again. I was referred to a cardiologist. Another full workup revealed nothing. My PCP referred me to my current pulmonologist. She did a full workup. At that time there were no indications I had PAH. Based on my symptoms, she suspected I had allergic asthma and prescribed inhalers. After my annual high-resolution chest and PFTs she sent me for an annual cardiac echo. All previous echos had been normal. The most recent showed tricuspid valve regurgitation. Consequently, they were unable to calculate pressures. The pulmonologist insisted on a right and left heart catheterization. I was diagnosed with PAH October 2022, started Tadalafil last November, and just started Ambrisentan. I do indeed count myself as blessed! Thank goodness you were able to find a physician that recognized what was happening to you too.

You are so lucky. I was told I had Asthma, then it was Emphysema. I kept telling 5hem I had CREST, but the paid no attention. Almost cost me my life.

I have Sjogren's, a connective tissue disease in the same family as Systemic Scleroderma. I am quite fortunate to have an excellent pulmonologist that not only has an impressive knowledge of Sjogren's but is also a PAH expert. If not for her, I am not sure I would have been diagnosed as quickly as I was.