You were just diagnosed with PH. What’s next?

Learning that you have pulmonary hypertension can be overwhelming, but you’re not alone. Like the tens of thousands of people diagnosed with PH before you, you have a compassionate and inspiring community of people ready to help. We are here to provide you with information and support as you adjust to your new life with PH.

While there’s currently no cure for PH, there are PH-specific therapies available to manage your symptoms and improve your quality of life. We know patients on treatment who have been living with PH for more than 20 years and counting.


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What is

Pulmonary Hypertension?

Pulmonary Hypertension, or PH, is a disease affecting the arteries of the lungs. It can strike anyone regardless of age, gender, social or ethnic background. People affected with this disease suffer from continuous high blood pressure in the lungs which results in an enlargement of the heart, and can lead to heart failure.

The heart and circulatory system1

To understand what PH is, it first helps to understand a bit about the circulatory system. As shown in the figure, the heart pumps oxygen-poor blood to the lungs so the blood can take up oxygen. Blood that is oxygen-poor is normally shown on diagrams as blue, whereas oxygen-rich blood returning from the lungs is normally shown in red.

During periods of exercise, the heart beats more quickly to get more oxygen to the muscles. At the same time, the blood vessels carrying blood to the lungs (the pulmonary arteries) expand to allow more blood through. The pulmonary arteries do this by stretching slightly. In a person with PH, the walls of the pulmonary arteries are thicker, so are less able to stretch.

In a person with PH, because the pulmonary arteries are less able to stretch, the heart has to work harder to pump blood to the lungs. If the heart has to work harder than usual over a long period of time (months or years), it begins to work less effectively.


Pulmonary Artery

Artery stretches in response to increased heart rate

PH patient

Pulmonary Artery

Thickened artery cannot stretch enough in response to increased heart rate

Symptoms of

Pulmonary Hypertension

  • Breathlessness or shortness of breath, especially with activity
  • Feeling tired all the time
  • Light-headedness, especially when climbing stairs or standing up
  • Swollen ankles, legs, or abdomen
  • Chest pain, especially during physical activity
  • Fainting

References: 1. About Pulmonary Hypertension [Internet]. Available from: http// Accessed August 2012. 2. The heart and circulatory system [Internet]. Available from: http// Accessed August 2012. 3. Living with Pulmonary Hypertension. Patient Information [Internet]. Available from: Accessed August 2012.

FAQ's for

Newly Diagnosed Patients

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.

PHA is thankful to Shiela Bostelman, MSN, CPNP, CCTC, Pulmonary Hypertension Program, Children's National Heart Institute, for her input and medical review.

Plan for your

First Appointment

Once you’ve scheduled your first appointment with a pulmonary hypertension specialist, you’ll want to start preparing for it. Being prepared and having questions ready to ask your doctor will help you communicate effectively about your needs and expectations.

PH patients can receive excellent care from a team of specialists, but coordinating appointments and managing medications may take some getting used to. Before long, your physician and nurse specialists will feel like family. Here’s how to prepare for your first appointment.



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.

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