FAQs

PH = Pulmonary Hypertension Pulmonary = the lungs Hypertension = high blood pressure PH 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. PH is a serious illness, and can be life-threatening.

PH 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 PH are more common in females: idiopathic PAH (IPAH, previously known as PPH) PAH associated with connective tissue disease like scleroderma Some people are more likely to develop PH. Factors that increase the risk of a person developing PH include: A family history of a close relative being affected by PH The use of appetite-suppressant medications for weight loss (for example, fenfluramine or abuse 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, PH can be due to infections in some patients. However, an infection is not the cause of PH in most patients. Specific types of infection can cause PH: Infection of the bowels and liver by the parasite, schistosomiasis, is a very common cause of PAH around the world, but is very rare in Canada Infection with HIV can cause PAH Infection with hepatitis viruses (Hepatitis B and C) causes chronic hepatitis and cirrhosis of the liver, which can lead to PAH

Yes, in some patients, PH can be due to blood clots in the lungs (also known as pulmonary embolism). After pulmonary embolism, up to 4% of patients may develop PH within 2 years. PH due to pulmonary embolism is called chronic thromboembolic PH (CTEPH). The risk of PH 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, PH will develop within 2 years of pulmonary embolism in a small number of patients (up to 4%); the risk is highest in patients with multiple episodes of pulmonary embolism Patients with pulmonary embolism who also develop PH have a higher risk of dying In many other patients with PH, blood clots in the lungs are not the cause of PH. However, even in these patients, PH itself causes damage to the innermost lining cells (also known as endothelial cells) of the pulmonary arteries. Damage to endothelial cellscan lead to blood clots forming (also known as thrombosis) in the pulmonary arteries. This thrombosis in the pulmonary arteries narrows the pulmonary arteries This thrombosis in the pulmonary arteries can further increase the blood pressure in the pulmonary arteries (pulmonary artery pressure) This thrombosis in the pulmonary arteries can lead to the worsening of PH over time All patients diagnosed with PH should be assessed for the presence of blood clots in the lungs. This is for several reasons: Pulmonary embolism is a common cause of PH Treatment with blood thinners (also known as anti-coagulants) can greatly decrease the risk of recurrent pulmonary embolism

Yes, in many patients, PH 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 PH. However, in most patients with lung disease, PH is usually mild. Diseases of the lungs that can cause PH 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, PH 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, in many patients, PH can be associated with a number of 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 PH. Patients who have taken weight loss medications (also known as appetite-suppressant medications or anorexigens) have a higher risk of PAH. These medications include: Aminorex Fenfluramine (Ponderal, Pondamin) Dexfenfluramine (Redux) Phentermine (Ionamin) Mazindol Patients who have abused recreational drugs are at higher risk for PH. These drugs include: Cocaine Methamphetamine Amphetamine Some medications used to treat cancer (also known as chemotherapy) have been reported to cause PH. These medications include: Bleomycin Busulfan Carmustine (BCNU) Interferon-alpha Interleukin-2 Lomustine (CCNU) Mitomycin C Nitrosoureas Dasatinib Many other uncommon medications may sometimes cause PH. These medications include: L-tryptophan

No, pregnancy is not believed to cause PH. In pregnancy, most healthy women will experience some degrees of difficulty breathing, fatigue, and swelling of feet or ankles. This is often perfectly normal and does not necessarily mean you have PH. Pregnancy increases the risk of blood clots in the lung (also known as pulmonary embolism) which can lead to PH. In women who already have certain types of PH, they may first notice symptoms of PH during pregnancy. This includes: IPAH (previously known as PPH) PAH associated with defects of the heart present at birth (also known as congenital heart disease) PH due to pulmonary embolism In women already diagnosed with PH, pregnancy can be extremely dangerous. PH often worsens during the pregnancy, and often leads to the death of the mother. It is strongly recommended that women with PH not become pregnant. For more information, read the info-sheet about PH and Contraception by clicking here.

No, doctors and scientists don’t think PH 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 idiopathicPAH) 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.

In some patients, PH can be inherited from their parents. Patients with inherited PAH are said to have heritable PAH (HPAH). HPAH should be suspected when more than one closely related person in a family (parent, child, sibling) has PAH In a family with known HPAH, the risk to individuals is still quite small. On average, only 1 in 10 will develop PAH In patients thought to have idiopathic PAH (IPAH), up to 1 in 5 may actually have HPAH. Sometimes, a very detailed or careful medical history of a family can suggest HPAH Patients with HPAH have a mutation (error) in the DNA code of 1 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 her/his parents. The first mutant gene that causes HPAH was discovered in 2000. This gene is called BMP-R2 (bone morphogenetic protein receptor 2). The normal function of the BMP-R2gene 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 (also known as reproduce or proliferate) How these cells mature or change their function (also known as differentiate) When these cells should normally die to make room for other cells Another mutant gene that causes HPAH is ALK-1 Nevertheless, the mutation responsible for HPAH 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. It is important to realize that in most patients with PH (all types other than HPAH), there is no evidence that PH is inherited.

PH 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 PH. These infections themselves can spread between people (also known as contagious). In a patient who is exposed to one of these infections and falls ill, there is a risk of also developing PH. These infections include: Infection with HIV, the virus which causes AIDS; HIV infection can lead to PAH Infection with hepatitis viruses B and C, which can lead to chronic liver disease, including chronic hepatitis and cirrhosis; cirrhosis can lead to PAH

PH 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 PH 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, PH 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 PH. These patients are said to have familial PAH (FPAH). In a small number of patients, there is no clear cause for PH. In such patients, PH is labeled idiopathic PAH (IPAH; previously known as primary PH = PPH). In many patients, PH is the result of a condition of the heart or lungs or another medical condition that can cause PH. These include: Heart disease, such a 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 PH 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 PH 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 PH in the future In patients with pulmonary embolism, effective treatment with blood thinners (also known as anti-coagulants) may prevent PH In many other types of PH, there is presently no way to prevent PH. These types of PH include: Idiopathic IPAH (previously known as PPH) Familial or inherited PAH (FPAH) PAH associated with connective tissue disease (such as scleroderma), HIV infection, or cirrhosis of the liver

The symptoms of PH 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 PH patients, PH gets worse if left untreated. In some PH patients, PH may remain stable over many months or years. Whether PH gets worse in an individual patient depends on many factors. These include: the cause of PH the severity of PH the age of a patient the general health of a patient the presence of other conditions of the heart, lungs, or body what PH treatment is available to a patient how a patient responds to PH 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 PH 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 PH 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 PH 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 PH 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 PH you have and the severity of your illness. They will consider factors such as your functional class (a classification based on how PH 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) is treated using medical therapies approved by Health Canada. While these treatments are not a cure for PH, they can slow disease progression and alleviate symptoms. There is a potential cure for CTEPH 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.

PH 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. Further, some provinces require patients to be followed by a specialist in order to receive coverage for PH-specific medications. Centres specializing in treating PH (adult and pediatric) and CTEPH are in Canada. Their experienced medical teams include PH nurses and specialists such as cardiologists, respirologists, and rheumatologists.