I Have PH. Can I take any of the PH Medications?

By Cheryl Switzer

When you receive a diagnosis of Pulmonary Hypertension, it's easy to confuse PH (pulmonary hypertension) with PAH (pulmonary arterial hypertension).  For one thing, doctors sometimes speak of PH when they actually mean PAH.  Then too in support groups, chat rooms and on message boards, individuals rarely draw a distinction between the two terms.  But there is a difference and knowing the difference will help you to understand your treatment options and general prognosis.

How is PH different from PAH?
PH is a general term for any condition in which pressure is too high in the blood vessels of the lungs. There are many types of PH with many different causes. PAH is a subcategory of PH that is defined by abnormal changes in the walls of the arteries (a specific type of blood vessel) of the lungs. The treatment for one type of PH may differ from the treatment for another, so it is important for your doctor to know which type you have in order to prescribe the most effective treatment.

The WHO (World Health Organization) has created a table to classify the various forms of PH.  There are five logical groupings.  Review the following to find your place on this listing:

Group 1:  PAH - Pulmonary arterial hypertension
1.1 Idiopathic (IPAH) - Origin unknown
1.2 Familial (FPAH) - Inherited
1.3 Associated with (APAH):- One or more of the following
1.3.1 Connective tissue disorder
1.3.2 Congenital systemic-to-pulmonary shunts
1.3.3 Portal hypertension
1.3.4 HIV infection
1.3.5 Drugs and toxins
1.3.6 Other (thyroid disorders, glycogen storage disease, Gaucher's disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, chronic myeloproliferative disorders, splenectomy)
1.4 Associated with significant venous or capillary involvement
1.4.1 Pulmonary veno-occlusive disease (PVOD)
1.4.2 Pulmonary capillary hemangiomatosis (PCH)
1.5 Persistent pulmonary hypertension of the newborn

Group 2:  PH - Pulmonary hypertension with left heart disease
2.1 Left-sided atrial or ventricular heart disease
2.2 Left-sided valvular heart disease

Group 3:  PH - Pulmonary hypertension associated with lung diseases and/or hypoxemia
3.1 Chronic obstructive pulmonary disease
3.2 Interstitial lung disease
3.3 Sleep disordered breathing
3.4 Alveolar hypoventilation disorders
3.5 Chronic exposure to high altitude
3.6 Developmental abnormalities

Group 4:  PH - Pulmonary hypertension due to chronic thrombotic and/or embolic disease (CTEPH)
4.1 Thromboembolic obstruction of proximal pulmonary arteries
4.2 Thromboembolic obstruction of distal pulmonary arteries
4.3 Nonthrombotic pulmonary embolism (tumor, parasites, foreign material)

Group 5:  PH - Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)

Medications
All of the current approved medications (prostacyclin, ETRAs, PDE-5 Inhibitors) have been developed for the treatment of PAH which includes only patients falling within WHO Group 1.  If you have received a diagnosis of pulmonary hypertension (PH) not associated with any of the diseases listed for group 1, you may not be a candidate for any of these drugs or therapies.  In all probability they would not help you and might actually make you worse but this is something your doctor will judge, based on your individual case, the current research, guidelines and experience.

That does not mean there is nothing for you in the way of treatment.  Your underlying condition - the disease that has resulted in a secondary diagnosis of PH - should be the focus of your doctor visits.  For those with problems associated with the left side of the heart (WHO Group 2), you would want to work with a cardiologist.  For those with problems associated with the lungs, (WHO Group 3, you would choose a pulmonologist.  For anyone who falls outside of WHO Group 1, improving your underlying condition will possibly result in an improvement of your PH symptoms.

Sources:
Gilead Sciences - Insights on PAH 
Journal of Medical College of Cardiology -Revised WHO Classification of PH

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