FAQs

Pulmonary hypertension (PH):

• Pulmonary = the lungs

• Hypertension = high blood pressure

Pulmonary hypertension is a disease of abnormally high blood pressure in the pulmonary arteries. In PH, the pulmonary arteries become narrowed and can be scarred to the point of being closed. Patients with PH develop symptoms such as shortness of breath, tiredness, and swelling of the feet and ankles. Pulmonary hypertension is a serious illness, and can be life-threatening.

Pulmonary hypertension can strike anyone. It typically affects people between 20 and 60 years of age, but PH can also affect children and the elderly. PH affects both men and women. However, certain types of pulmonary hypertension are more common in females:

• Idiopathic pulmonary arterial hypertension (IPAH, previously known as PPH)

• Pulmonary arterial hypertension associated with connective tissue disease like scleroderma.

Some people are more likely to develop pulmonary hypertension. Factors that increase the risk of a person developing PH include:

• A family history of a close relative being affected by pulmonary hypertension

• The use of appetite-suppressant medications for weight loss (for example, fenfluramine) or use of recreational drugs (for example, cocaine)

• A history of blood clots in the lungs (also known as pulmonary emboli)

• The presence of other medical conditions, including lung disease (for example, emphysema), heart disease (for example, heart failure), and general medical conditions (for example, connective tissue disease like scleroderma).

Yes, in some patients, pulmonary hypertension can be due to blood clots in the lungs (also known as pulmonary embolism). PH due to pulmonary embolism is called chronic thromboembolic pulmonary hypertension (CTEPH).

The risk of pulmonary hypertension after pulmonary embolism is quite small; in most patients with pulmonary embolism, the body is able to break up the clot, leaving no evidence of PH. However, pulmonary hypertension will develop within 2 years of pulmonary embolism in up to 4% of patients; the risk is highest in patients with multiple episodes of pulmonary embolism. Patients with pulmonary embolism who also develop pulmonary hypertension have a higher risk of dying.

In many other patients with pulmonary hypertension, blood clots in the lungs are not the cause of PH. However, even in these patients, pulmonary hypertension itself causes damage to the innermost lining cells (also known as endothelial cells) of the pulmonary arteries. Damage to endothelial cells can lead to blood clots forming (also known as thrombosis) in the pulmonary arteries. This thrombosis narrows the pulmonary arteries, further increasing the pulmonary artery pressure (blood pressure in the pulmonary arteries) and potentially leading to the worsening of pulmonary hypertension over time.

For several reasons, all patients diagnosed with pulmonary hypertension should be assessed for the presence of blood clots in the lungs:

• Pulmonary embolism is a common cause of pulmonary hypertension

• Treatment with blood thinners (also known as anti-coagulants) can greatly decrease the risk of recurrent pulmonary embolism.

Yes, in some patients, pulmonary hypertension can be due to blood clots in the lungs (also known as pulmonary embolism). PH due to pulmonary embolism is called chronic thromboembolic pulmonary hypertension (CTEPH).

The risk of pulmonary hypertension after pulmonary embolism is quite small; in most patients with pulmonary embolism, the body is able to break up the clot, leaving no evidence of PH. However, pulmonary hypertension will develop within 2 years of pulmonary embolism in up to 4% of patients; the risk is highest in patients with multiple episodes of pulmonary embolism. Patients with pulmonary embolism who also develop pulmonary hypertension have a higher risk of dying.

In many other patients with pulmonary hypertension, blood clots in the lungs are not the cause of PH. However, even in these patients, pulmonary hypertension itself causes damage to the innermost lining cells (also known as endothelial cells) of the pulmonary arteries. Damage to endothelial cells can lead to blood clots forming (also known as thrombosis) in the pulmonary arteries. This thrombosis narrows the pulmonary arteries, further increasing the pulmonary artery pressure (blood pressure in the pulmonary arteries) and potentially leading to the worsening of pulmonary hypertension over time.

For several reasons, all patients diagnosed with pulmonary hypertension should be assessed for the presence of blood clots in the lungs:

• Pulmonary embolism is a common cause of pulmonary hypertension

• Treatment with blood thinners (also known as anti-coagulants) can greatly decrease the risk of recurrent pulmonary embolism.

Yes, in many patients, pulmonary hypertension can be due to a disease of the lungs. PH is usually seen when the lung disease is severe. In patients with lung disease, low oxygen levels in the blood usually cause pulmonary hypertension. However, in most patients with lung disease, PH is usually mild. Diseases of the lungs that can cause pulmonary hypertension include:

• Emphysema of the lungs due to smoking

• Scarring (also known as fibrosis) of the lungs

• Recurrent or multiple blood clots in the lungs (also known as pulmonary emboli)

• Breathing problems at night that cause low oxygen levels in the blood, for example sleep apnea

Yes, in many patients, pulmonary hypertension can be due to a disease of the heart. In some types of heart disease, blood backs up into the lungs, causing PH:

• Heart failure, due to previous heart attacks, high blood pressure (also known as systemic hypertension), or diabetes

• Diseases of the valves of the left-side of the heart, specifically the mitral valve and aortic valve.

Another important type of heart disease is due to defects of the heart present at birth (also known as congenital heart disease), such as:

• atrial septal defect (ASD)

• ventricular septal defect (VSD).

Yes, pulmonary hypertension can be associated with other medical conditions:

• Connective tissue diseases, such as scleroderma (also known as CREST), or systemic lupus erythematosus (SLE)

• Infection with HIV

• Severe liver disease, such as cirrhosis

• Diseases of the blood, like sickle-cell disease and thalassemia.

Yes, some medications can cause pulmonary hypertension.

Patients who have taken weight loss medications (also known as appetite-suppressant medications or anorexigens) have a higher risk of pulmonary arterial hypertension. These medications include:

• Aminorex

• Fenfluramine (Ponderal, Pondamin)

• Dexfenfluramine (Redux)

• Phentermine (Ionamin)

• Mazindol

Patients who have used recreational drugs are at higher risk for pulmonary hypertension. These drugs include:

• Cocaine

• Methamphetamine

• Amphetamine

Some medications used to treat cancer (also known as chemotherapy) have been reported to cause pulmonary hypertension. These medications include:

• Bleomycin

• Busulfan

• Carmustine (BCNU)

• Interferon-alpha

• Interleukin-2

• Lomustine (CCNU)

• Mitomycin C

• Nitrosoureas

• Dasatinib

Other uncommon medications may sometimes cause pulmonary hypertension. These medications include:

• L-tryptophan

No, pregnancy is not believed to cause pulmonary hypertension. Pregnancy often causes some degree of difficulty breathing, fatigue, and swelling of the feet or ankles. This is often perfectly normal and does not necessarily mean you have pulmonary hypertension.

Pregnancy increases the risk of blood clots in the lung (also known as pulmonary embolism) which can lead to pulmonary hypertension.

People who already have certain types of pulmonary hypertension may first notice symptoms of PH during pregnancy. This includes:

• Idiopathic pulmonary arterial hypertension (IPAH, previously known as PPH)

• Pulmonary arterial hypertension associated with defects of the heart present at birth (also known as congenital heart disease)

• Pulmonary hypertension due to pulmonary embolism

Pregnancy can be extremely dangerous. Pulmonary hypertension often worsens during pregnancy and often leads to death. It is strongly recommended that pulmonary hypertension patients do not become pregnant.

Read the Pulmonary Hypertension and Contraception info sheet.

No, doctors and scientists don’t think pulmonary hypertension is a tumor or cancer.

However, there is scientific evidence that the innermost lining cells (also known as endothelial cells) of the pulmonary arteries as well as cells within the wall of pulmonary arteries (smooth-muscle cells) may behave like tumor cells in patients with idiopathic pulmonary arterial hypertension (IPAH). Indeed, these cells tend to intensely proliferate (multiply), just like cancer cells. This idea of tumor-like cells in IPAH is an area of ongoing research.

Some patients inherit pulmonary hypertension from their parents. Patients with inherited pulmonary arterial hypertension are said to have heritable pulmonary arterial hypertension (HPAH). HPAH should be suspected when more than one closely related person in a family (parent, child, sibling) has pulmonary arterial hypertension.

• In a family with known HPAH, the risk to individuals is still quite small. On average, only 1 in 10 will develop pulmonary arterial hypertension

• In patients thought to have idiopathic pulmonary arterial hypertension (IPAH), up to 1 in 5 may actually have heritable pulmonary arterial hypertension. Sometimes, a very detailed or careful medical history of a family can suggest HPAH.

Patients with heritable pulmonary arterial hypertension have a mutation (error) in the DNA code of a gene or a number of genes out of the roughly 30,000 genes on the human genome. These mutant genes are transmitted to a patient from their parents.

The first mutant gene that causes heritable pulmonary hypertension was discovered in 2000. This gene is called BMP-R2 (bone morphogenetic protein receptor 2). The normal function of the BMP-R2 gene is to allow cells to make a protein called BMP-R2 protein. BMP-R2 protein helps the body control the behaviour of cells in the walls of pulmonary arteries, including endothelial cells and smooth muscle cells. In these cells, BMP-R2 controls:

• How these cells grow and multiply (reproduce or proliferate)

• How these cells mature or change their function (differentiate)

• When these cells should normally die to make room for other cells.

Nevertheless, the mutation responsible for heritable pulmonary arterial hypertension is only found in about 80% of patients with HPAH. This is likely because other mutations causing HPAH have not been discovered yet, although less common mutations have been documented in recent years. Another mutant gene that causes heritable pulmonary arterial hypertension is ALK-1.

It is important to realize that in most patients with pulmonary hypertension (all types other than HPAH), there is no evidence that pulmonary hypertension is inherited.

Pulmonary hypertension is not contagious, and is not transmitted from one person to another. Thus, PH cannot be “caught” from another person or patient with PH.

However, infections can cause certain types of pulmonary hypertension. These infections themselves can spread between people (they are contagious). In a patient who is exposed to one of these infections and falls ill, there is a risk of also developing pulmonary hypertension. These infections include:

• Infection with HIV, the virus which causes AIDS. HIV infection can lead to pulmonary arterial hypertension

• Infection with hepatitis viruses B and C, which can lead to chronic liver disease, including chronic hepatitis and cirrhosis. Cirrhosis can lead to pulmonary arterial hypertension.

Pulmonary hypertension can be difficult to diagnose because it shares many of its symptoms with other more common conditions and requires specialized tests to be confirmed. When doctors suspect PH, they may order a series of preliminary tests to rule out other conditions before referring a patient to a specialist. These tests include an echocardiogram, blood tests, chest X-rays, an electrocardiogram, and pulmonary function tests. A specialist can confirm a pulmonary hypertension diagnosis after conducting one or more of these tests: exercise tolerance tests, CT scanning/imaging, ventilation/perfusion lung scan, and right heart catheterization.

This is a question that only your doctor can address specifically for you.

In a small number of patients, pulmonary hypertension can be inherited. In these patients, there is often a family history of PH in a close relative (for example, parents, siblings, children). It is likely that these patients inherited a defective (also known as mutant) gene that causes pulmonary hypertension. These patients are said to have familial pulmonary arterial hypertension (FPAH).

In a small number of patients, there is no clear cause for PH. In such patients, pulmonary hypertension is labeled idiopathic pulmonary arterial hypertension (IPAH; previously known as primary PH or PPH).

In many patients, pulmonary hypertension is the result of a condition of the heart or lungs or another medical condition that can cause PH. These include:

• Heart disease, such heart failure

• Lung disease, such as emphysema

• Blood clots in the lungs (also known as pulmonary embolism)

• Connective tissue diseases such as scleroderma (also known as CREST)

• Infection with HIV

• Severe liver disease, such as cirrhosis

• Use of certain medications, such as appetite-suppressant medications for weight loss (also known as anorexigens) or recreational drugs (like cocaine).

Some types of pulmonary hypertension can be prevented by early diagnosis and treatment of other medical conditions. For example:

• In patients with lung disease, diagnosis of low oxygen levels in the blood and appropriate treatment with oxygen may prevent pulmonary hypertension

• In patients born with defects of the heart (also known as congenital heart disease), early diagnosis of the defect, such as during childhood, and surgical repair of the defect may prevent pulmonary hypertension.

• In patients with pulmonary embolism, effective treatment with blood thinners (also known as anti-coagulants) may prevent pulmonary hypertension.

There is presently no way to prevent many other types of pulmonary hypertension. These types of PH include:

• Idiopathic pulmonary arterial hypertension (IPAH, previously known as PPH)

• Familial or inherited pulmonary arterial hypertension (FPAH or HPAH)

• Pulmonary arterial hypertension associated with connective tissue disease (such as scleroderma), HIV infection, or cirrhosis of the liver.

The symptoms of pulmonary hypertension include:

• Difficulty breathing or shortness of breath (also known as dyspnea)

• Fatigue or tiredness

• Swelling of feet and ankles (also known as edema), and possibly swelling of the belly or abdomen (also known as ascites)

• Chest pain, typically felt as a heaviness, pressure, or tightness in the center of the chest (also known as angina).This usually occurs upon activity or exertion

• Feelings of faintness or lightheadedness; feeling like passing out or actually losing consciousness (also known as syncope).This usually occurs upon activity or exertion, but may occur upon straining or coughing

• A rapid, forceful, or irregular heartbeat (also known as palpitations).

Other less common symptoms include cough, hoarseness of voice, and coughing up blood (also known as hemoptysis).

In most pulmonary hypertension patients, PH gets worse if left untreated. In some PH patients, pulmonary hypertension may remain stable over many months or years.

Whether pulmonary hypertension gets worse in an individual patient depends on many factors.

These include:

• The cause of pulmonary hypertension

• The severity of pulmonary hypertension

• The age of a patient

• The general health of a patient

• The presence of other conditions of the heart, lungs, or body

• What pulmonary hypertension treatment is available to a patient

• How a patient responds to pulmonary hypertension treatment.

There is no single answer to this question because everyone is different. Your experience with the disease depends on many factors, including how severe your pulmonary hypertension is at the time of diagnosis and how you respond to treatments.

PH is a progressive and potentially fatal disease, but thanks to available treatments, many patients are living longer and healthier lives. In the last twenty years, research has made great strides, and specialists are better equipped than ever to provide patients with the best care and improve their quality of life.

Medical alert bracelets communicate important information to healthcare providers in an emergency. However, not all people with pulmonary hypertension need to wear one. A medical alert bracelet can be useful if you are on advanced therapies for PH (such as intravenous medication) or anticoagulation medication. Speak to your medical team about whether or not you should wear a medical alert bracelet and what information it should provide.

Right after diagnosis it is hard to imagine what life with pulmonary hypertension will be like. Over time, many people develop strategies to cope with the challenges of living with PH and lead full, happy lives.

The key is to listen to your body, respect your limits, and rest when needed. Making small adjustments to your home, routine, and daily activities will allow you to conserve energy while remaining active. Support from other people who understand what it means to live with pulmonary hypertension and who have developed strategies to cope with the disease can also be crucial.

The tests performed to diagnose you will allow your doctor to determine which type of pulmonary hypertension you have and the severity of your illness. They will consider factors such as your functional class (a classification based on how pulmonary hypertension impacts your everyday life), the pressures in your lungs, and how well your heart is functioning to determine which treatment is best suited to your needs.

Your medical team will work with you to establish a personalized treatment plan and adjust it as your symptoms and lung pressures change.

Pulmonary arterial hypertension (PAH, Group 1 PH) is treated using medical therapies approved by Health Canada. While these treatments are not a cure for pulmonary hypertension, they can slow disease progression and alleviate symptoms.

There is a potential cure for chronic thromboembolic pulmonary hypertension (CTEPH, Group 4 PH) through a surgical procedure called pulmonary endarterectomy (PEA). Approved medical treatment may also slow disease progression and alleviate symptoms in patients not eligible for PEA.

Other types of pulmonary hypertension are primarily treated by treating the underlying condition or cause.

Pulmonary hypertension is a relatively rare disease; most physicians have little or no experience with its diagnosis and treatment. For this reason, referral to a centre specialized in treating PH is encouraged to confirm a diagnosis and ensure that patients receive the best care.

There are centres specializing in treating pulmonary hypertension (adult and pediatric) and chronic thromboembolic pulmonary hypertension (CTEPH) across Canada. Their experienced medical teams include PH nurses and specialists such as cardiologists, respirologists, and rheumatologists. Some provinces require patients to be followed by a specialist in order to receive coverage for pulmonary hypertension -specific medications.