What is the progression of pulmonary hypertension?

[mlaurel]

My first heart cath in the late '80's showed mild pulmonary hypertension. However it was the only right/left cath I have had done. I have had several since but only right (?) side.  I have since developed NICM and have an ICD. Most recent cath showed 40 - 45% EF. The cath was done due to symptoms of chest pressure and SOB upon strenous exertion. The drs have told me that they do not believe my symptoms are heart related, but rather due to my lungs. I have been taking Imdur and it has helped with the chest pressure and pain a lot, but I still get very short of breath and have extreme exhaustion with activities such as mowing my yard, walking briskly, etc. I have been diagnosed with hypopnea with O2 in the 74% range a couple of years ago and have been using my CPAP faithfully.   My question is could the pulmonary hypertension just now beginning to progress? Thanks in advance.

[David Tinkelman, M.D.]

I am sorry to hear of your travails and hope to be of assistance.

Before commenting on your pulmonary hypertension, I would ask on what basis have your cardiologists concluded that your symptoms are lung related, and not heart related, especially with the evidence of NICM and a slightly reduced EF?  It would be fairly easy to resolve the question of impaired lung function with a Chest X-ray and or CT Scan of the lungs and complete Spirometry.  I would also wonder if a well-monitored exercise study, or perhaps a Stress-ECHO, would help your doctors to sort-out the relative contributions of heart and lungs to your severe dyspnea with exertion.   In that regard,  it is important to remember there is not always a good correlation between symptoms, including breathlessness, and the absolute level (40-45%) of the EF.  That is, LV function can be significantly deranged in the presence of a modest decrease in EF.

It is great that you have been using your CPAP “faithfully”, but it would be wise to seek reevaluation, probably to include a sleep study, of the effectiveness of the CPAP; i.e., is the CPAP actually preventing nocturnal hypoxemia, especially in a sustained fashion?

Depending somewhat on the cause or causes of your pulmonary hypertension (PH) I suspect that it could worsen any time, on the basis of one or more factors including but not limited to the effect of the cardiomyopathy on LV function,  possibly secondary to recurrent pulmonary emboli or to progressively worsening nocturnal hypoxemia secondary to apnea/hypopnea.

The best course of action might be a joint (cardiologist-pulmonologist) consultation.  Also, there is a steadily increasing number of Pulmonary Hypertension Clinics at many academic medical centers.  The one with which I am most familiar with is located at the University of Colorado in Denver; the Clinical Director of PH is Dr. David Badesch, whom I would highly recommend.  Should Denver not be a convenient site, I am sure you could contact Dr. Badesch for his recommendation of a PH specialist in your area.

Good luck

[mlaurel]

Thank you for your quick response. I assume the drs based their decision that my symptoms are pulmonary due to the fact that there was no blockages in my large vessels. The cardiologist's PA did recommend that I have pulmonary workup with Dr. Parker at WVU. Hopefully I will be able to get an appointment.  Thank you for recommending Dr. Badesch. I will contact his office for a recommendation to a PH specialist in the WV area. God bless.