Pulmonary hypertension (PH) is a complex and often misunderstood disease. PH means high blood pressure in the lungs. PH is different from regular hypertension. In regular hypertension (also known as high blood pressure), the arteries throughout a person’s body are constricted. In pulmonary hypertension, the blood vessels in the lungs become narrowed and the heart has to work harder to pump blood through them.

In many cases, no cause can be identified, and in these cases the disease is referred to as idiopathic pulmonary arterial hypertension (IPAH). Sometimes PH can develop due to another medical condition, including connective tissue disease, congenital heart disease, liver disease, HIV, and others. This type of PH is known as associated pulmonary arterial hypertension (APAH). Some families have a form of PH that can be inherited. This is known as heritable pulmonary arterial hypertension (HPAH).

PH can be difficult to diagnose in a routine medical exam because the most common symptoms of PH are also associated with many other conditions. To determine if you have PH, your medical team will schedule specialized tests, possibly to include a pulmonary function test and an echocardiogram. If the results of these tests point to PH, your doctor will schedule a cardiac catheterization, sometimes referred to as a “right-heart cath.” This test allows your doctor to directly measure the pressures in your heart and lungs. Right heart catheterization is the gold standard for diagnosing PH.

Yes, but heritable PAH (HPAH) is relatively uncommon. Of the small percentage of people who do carry the PH gene, only a small number of carriers will develop the disease. Genetic testing is available to find out if you carry the PH gene.

PH is a rare but serious disease, and many doctors, including cardiologists and pulmonologists, have little training or experience in diagnosing and treating PH. Patients are encouraged to see a cardiologist, pulmonologist or, in some cases, a rheumatologist who treats many PH patients. These doctors are familiar with PH medications and side-effects, and can determine which treatments or combination of therapies is right for you.

Once you’ve found a list of physicians in your area, interview them carefully to determine which centre will offer you the most specialized care.

Here are some questions to pose to doctors you’re considering:

• Are you part of a dedicated PH clinic that includes other PH-treating doctors?
• Do you have at least one nurse who works with patients on PH-specific medications?
• How many PH patients do you currently treat?
• Do you conduct clinical trials on PH medications?

Keep a list of your questions between appointments, and don’t be afraid to ask why your doctor is running a test, scheduling a procedure, or changing a medication. At your first appointment, you might consider asking: How often should I come to see you? How often do I need to have cardiac catheterization? What are the potential side-effects of my medication? If you wear a pump for your medication, you might want to ask: What do I do if the line leaks or comes out? How do I need to alter my daily routine to accommodate the pump? You may also want to discuss lifestyle changes, such as nutrition and exercise, with your PH team.

A number of FDA-approved medications are available for the treatment of PH. These medications are administered in several ways: directly into the vein (intravenously), beneath the skin (subcutaneously), orally, and by inhalation.

• Some PH medications work by mimicking prostaglandin, a substance that PH patients tend to be deficient in. These treatments are called are prostacyclins, and include epoprostenol (Flolan™), treprostinil (Remodulin™ and Tyvaso™), and iloprost (Ventavis™).
• Other PH medications work by reversing the effects of endothelin, a substance that PH patients tend to have in excess. These are called endothelin receptor antagonists, and include bosentan (Tracleer™) and ambrisentan (Letairis®).
• Still other PH treatments work by allowing the lungs to produce more of their own natural vasodilators. Called PDE 5 Inhibitors, this category of treatment includes sildenafil (Revatio™) and tadalafil (Adcirca™).
• Doctors may also prescribe the anticoagulant warfarin (Coumadin™) to prevent blood clots, diuretics to reduce fluid retention, and supplemental oxygen to help patients breathe.

Your doctor will take into consideration the severity of your illness (referred to as your “functional class”) and the results of your cardiac catheterization to help determine which medication is right for you. As your symptoms and pressures change, your doctor may want to adjust the type and dosage of your medication accordingly.

There’s no simple answer to this question. Every PH patient is different, and new research with the potential to improve the outlook for this disease is being conducted all the time. Your journey with PH depends on many factors, including the severity of your disease and how you respond to treatment. PH-specific therapies are available that can prolong and improve your quality of life. Once in the care of a PH specialist and on treatment, many PH patients live for many years.

While it can be difficult to imagine in the days and months following diagnosis, many patients develop strategies to cope with the physical and emotional aspects of living with PH and lead happy, fulfilling lives. Making small changes in daily activities can make a big impact on how you feel. Listen to your body and rest when you need to. Talk to your doctor about the possibility of supplemental oxygen if you haven’t already. With a bit of patience, planning, and flexibility, many patients find ways to work, travel, exercise, and do the things they loved before diagnosis. Visit the Living with PH section for more tips on living with PH.

While you’ll have time in the future to learn the ins and outs of PH, having a basic understanding of the illness and some common PH terms will be helpful for your first appointment. Read up on PH in the About PH section.

Call your new doctor’s office and ask if they can send you new patient paperwork by mail, fax or email. This will give you the chance to complete your medical history in the comfort of your own home, without feeling frazzled. Make two copies, one for your records and one to bring with you on the day of your appointment.

It is especially important for you to understand what cover you have for treatment in and out of hospital in order to plan and budget for possible co-payments.

Make sure you know the answers to the following questions before your appointment:

• Do you have prescription coverage?
• What is your co-pay structure?
• Do you need referrals and prior authorization and how do you obtain them? While you’re on the phone with the doctor’s office, find out whether the office accepts your insurance/medical aid and how to submit your co-pay.

Take it with you to your first appointment and every appointment thereafter. As you collect information from different doctors, store it in this file for future reference. Here are some ideas for things to include:

• A written list of all your doctors and their contact information
• A written list of all your medications. Include the following information on the list:
  • Name of medication
  • Dosage and frequency
  • Why you take the medication
  • When you began taking it
  • Which physician prescribed the medication
• A copy of all your medical records
• Test results and labs
• A note pad to take notes

Bring this list with you to your appointment and write down your doctor’s answers on the note pad in your file. If the doctor wants to order a test, procedure or new medication, you can add the following questions to your list:

• What is the test, procedure or medication?
• Why do I need it?
• What will be done during the test?
• How will the test results affect how you treat me?
• How often will I need to have it done?
• What are the potential complications and/or side effects?
• Don’t be afraid to tell your doctor if you don’t understand an answer or explanation.

Ask a family member or friend to accompany you to your appointment. Your loved one can provide support in the waiting room, confirm that you ran through all your questions and take notes on your doctor’s responses.

Refer your loved one to our caregivers section to for more information about providing care and support to a PH patient.

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You don’t want to get lost or feel hurried on the day of your appointment. Map the route and call the office for information about parking or if you have any questions.

Meeting a new doctor can be daunting, but remember that while your doctor is an expert in his or her field, you are the only one who knows exactly how you’re feeling. If you’d find it helpful, make a descriptive but concise list of your symptoms ahead of time so you have something to refer to when your doctor asks you questions. Remember – no one knows your body better than you.