Frequently Asked Questions For Newly Diagnosed Patients

What causes pulmonary hypertension?

Experts are still learning about the causes of PH. Sometimes it can develop due to another medical condition (including blood clots in the lungs, congenital heart disease, connective tissue disorders like scleroderma and lupus, as well as others). This type of PH is known as associated pulmonary arterial hypertension (APAH). In some cases, no cause for PH can be identified. This is referred to as idiopathic pulmonary arterial hypertension (IPAH).

How does my doctor know I have PH?

PH is a disease that can be difficult to diagnose because the most common symptoms associated with it are also common for many other conditions. To determine if you have PH, your medical team will include a variety of specialized tests, including a cardiac catheterization (sometimes called a ‘right-heart cath’), which will help the doctor to directly measure the pressures in your heart and lungs.

Could PH run in my family?

Yes, but this type of PH, known as familial PAH (FPAH) is very rare. It is caused by a mutation in the BMP-R2 gene, but only about 1 in 5 people who inherit the FPAH gene develop FPAH.

Why is it important to see a PH specialist?

Because PH is a relatively rare disease, most generalists have little or no training or experience in its diagnosis or treatment. Patients are encouraged to see a physician who has undergone extensive PH training, and who has experience treating patients with PH. A specialist is usually a cardiologist, pulmonologist or rheumatologist who has specialised in the treatment of PH. These doctors are familiar with diagnostic procedures, PH treatments and their side effects, and many of them participate in PH research so they keep current of the latest developments in the field of pulmonary hypertension. Some provinces require being followed by a specialist in order to receive coverage for PH specific medications.

How can I find a specialist in my area?

The best way to find a specialist is to look at the clinic directory.

What questions should I be asking my medical team?

During your day-to-day activities, you may come up with questions relating to your PH and your care. Keep a list of these questions to ask at your appointments. Don’t be afraid to ask the doctor why he/she is running a specific test, scheduling a procedure or changing a medication. Learn more about preparing for your first appointment. 

Are there medications available to treat PH?

Yes, a number of pulmonary hypertension specific treatments have been approved by Health Canada. Learn more about these treatment options.

How will my doctor determine which treatment is best for me?

Based on the tests that the doctor performed to diagnose your PH, he/she will be able to determine the severity of your illness (your functional class) and the pressures in your heart and lungs. Based on this, your doctor will work with you to determine which medication is best for you. As your symptoms and pressures change, the doctor may adjust the type and dose of the medication to better suit your needs.

How long do I have to live?

There is no true answer for this question. Each patient is different. Your personal experience with the disease may depend on many factors, including how severe your PH was at the time of diagnosis, and how you respond to medications. PH treatments can help to prolong and improve your quality of life, and many patients who are under the care of a specialist live for many years. Also, a lot of exciting research in pulmonary hypertension is ongoing, with potentially even better treatments constantly being tested.

How will PH affect my life?

Right after diagnosis, it is hard to imagine what life will be like. Over time, many patients develop strategies to cope with the difficulties associated with PH and lead happy, full lives. The key is to listen to your body and rest when needed, while making small adjustments in daily activities. Support from other patients who understand, who are living their lives and who are happy to share their tips and strategies is crucial. Connect with the Canadian PH community to start getting support and learning from others.