Are doctors often confused over a Pulmonary Hypertension diagnosis?

[Carl57]

My wife has had 2 echos done in the past 9 months. The 1st with RVSP of  35 to 42, second RVSP of 37 with mild to moderate tricuspid regurgitation and mild mitral regurgitation, no heart enlargement or other signs of heart problems.  EKG and chest Xray were both normal.

She was referred to a cardiologist, pulmonologist and ENT by her current Rheumatologist. The cardiologist 1st said it was pulmonary hypertension based on the results of the 2 echo's and her symptoms. He then decided to perform a heart cath and said it wasn't pulmonary hypertension based on PAP of 25/10 with a PCWP of ~12 with a wave to 15. Her cardiac output was 3.5 and 3.6 (by 2 different methods) with an index of 2.6.

A recent Spirometry test was normal. She had a lung angiogram performed a few days ago and they said the results were normal. They are going to refer her to Barnes Jewish Hospital in St. Louis for additional testing but it will be a couple of months before she can be seen.

She also has Relapsing Polychondritis (a rare autoimmune disease) which affects cartilage and can affect the trachea and airways.

Her symptoms are: She sounds hoarse quite often (for the last several months) She is short of breath on exertion and says her chest feels tight when she exerts herself (for example swimming one lap in a small pool). The SOB goes away very quickly after she stops the exertion. Her ankles swell sometimes (she has a very restricted diet due to stomach problems and eats no salt). Heart beat becomes rapid at high altitudes. Her hands, nose and ears get very cold sometimes even in warm temperatures.

Do you think this could be pulmonary hypertension since the RVSP on both echo’s  were above normal along with the valve regurgitation or does the heart cath PA of 25/10 rule out Pulmonary Hypertension as the cardiologist says?

[Jonathan D. Rich, MD]

Hello and welcome to the forum.

Straight to the point here and its good news:  Based on the information provided to me your wife does NOT have pulmonary hypertension...case closed, end of story, dont let anyone tell you otherwise.

It is indeed a scary world out there.  Echo in notoriously unreliable and should never be used to make the diagnosis of pulmonary hypertension.  The cath trumps everything and her pressures were as normal as they could possibly be.

I hope this provides you with some clarity as well as some relief that she does not have pulmonary hypertension.

Wishing you and your wife the best in health and happiness.

Best,

Dr. Rich

[Jonathan D. Rich, MD]

Dont even get me started on "exercise induced PH"...about as real as the tooth fairy  :)

Good luck.

Dr. Rich

[Carl57]

Dr. Rich,

Thank you so much for your quick response, as you can imagine knowing it is not PH is a great relief to us.
Based on her symptoms and echo results the pulmonologist seemed to think it might be something called exercise induced PH and that had us pretty scared.
I guess the next big dilemma is to determine what actually has been causing her symptoms and the SOB on exertion for the past 12 to 18 months.

Best Regards,

Carl