ABOUT PH
Glossary of Pulmonary Hypertension Terms
A
Activin signalling inhibitors
The pathway associated with BMPR2 signalling is important in controlling cell growth in pulmonary arteries. In patients with pulmonary arterial hypertension, this signal seems to be underactive, and a competing pathway known as the activin pathway stimulates too much cell growth. Activin signalling inhibitors bind the activins and inhibit abnormal cell proliferation in the pulmonary arteries.​
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Acute vasodilator challenge
A vasodilator challenge is used to help determine which medications may work best to treat pulmonary arterial hypertension. During a right heart catheterization, your doctor may test whether your pulmonary arteries can relax, thus decreasing the pulmonary artery blood pressure. Specific criteria determine if you are “vasoreactive.” Vasoreactive pulmonary arterial hypertension patients may respond to calcium channel blocker (CCB) therapy.
Adcirca®
Adcirca is the brand name for tadalafil, an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. Tadalafil is also sold as Cialis, which is used to treat erectile dysfunction. See also phosphodiesterase inhibitors | Read more about tadalafil
Ambrisentan
Ambrisentan is an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name for ambrisentan is Volibris. See also endothelin receptor antagonists | Read more about ambrisentan
Angina
Angina is chest pain or pressure that usually results from not enough blood or oxygen getting to the heart muscle. It typically occurs in patients with coronary artery disease, but it can affect patients with many different types of heart disease, including pulmonary arterial hypertension.
Angiogram
An angiogram is a scan that uses an X-ray, CT scan, or MRI in combination with dye injected into blood vessels to find narrow spots and blockages in blood flow.
Angiotensin Converting Enzyme inhibitors (ACE inhibitors)
ACE inhibitors are blood pressure medications that are prescribed to treat systemic (“regular”) hypertension and/or left-sided (“regular”) heart failure. Common ACE inhibitors include quinapril, lisinopril, and captopril. ACE inhibitors are commonly prescribed for pulmonary hypertension associated with left-sided heart disease (pulmonary hypertension Group 2). They are not generally used to treat pulmonary arterial hypertension (pulmonary hypertension Group 1).
Anticoagulant medications
Anticoagulant medications thin the blood and block some chemicals that make blood clot. These medications can be given by mouth, by injection, or by intravenous infusion. Some common blood thinners include warfarin (brand name Coumadin), heparin, and enoxaparin (brand name Lovenox). Acetylsalicylic acid (ASA, brand name Aspirin) is also a blood thinner that makes the platelets in blood less likely to clump together. Read more about conventional medical therapies
Apnea
Apnea is when breathing slows or stops, usually during sleep. It is always abnormal and needs immediate attention. See also obstructive sleep apnea
Arginine
Arginine is an essential amino acid (a protein building block in our bodies) that can be taken as a supplement. It works with a substance called nitric oxide, which helps open up blood vessels. It is generally considered to be safe to use, but of limited benefit when taken orally, and it can interact with other drugs taken for pulmonary hypertension.
Arterial blood gas (ABG) test
An arterial blood gas test measures oxygen, carbon dioxide, and acidity levels in the blood. It is used to see how well your lungs are working.
Associated pulmonary arterial hypertension
Many pulmonary hypertension patients are diagnosed with pulmonary hypertension in association with one or more other conditions. Other conditions commonly associated with pulmonary hypertension include scleroderma, sickle cell anemia, HIV, mixed connective-tissue disease, congenital heart disease, chronic obstructive pulmonary disease, hereditary hemorrhagic telangiectasia, sleep apnea, liver disease and lupus. Pulmonary hypertension has also been linked to the use of certain drugs, including fenfluramine, dexfenfluramine and methamphetamines. Read more about pulmonary hypertension associated with other conditions
Ascites
Ascites is a buildup of fluid (edema) in the abdomen. It can feel full or heavy around the waist, making clothing tighter. Water pills (diuretics) can treat ascites.
Atrial fibrillation
Atrial fibrillation is an irregular—often very fast—heartbeat, specifically of the atria. It typically occurs in left-sided heart disease but can also occur in pulmonary arterial hypertension. The rapid and irregular beating of the atria results in less blood flow to the ventricles of the heart and, therefore, to the rest of the body. This can cause palpitations, dizziness, lightheadedness, and fainting.
Atrial septal defects
Children can be born with an atrial septal defect, which is a hole in the wall (atrial septum) that separates the right and left upper chambers (atria) of the heart. The hole allows blood to flow between the atria. Small holes may not need treatment, while larger ones may need repairing. See congenital heart disease
Atrial septostomy
An atrial septostomy is a surgery that creates a small hole between the two upper chambers (atria) of the heart, allowing blood to flow between the atria. This can relieve pressure on the right side of the heart and improve blood flow to the lungs, but by allowing unoxygenated blood to bypass the lungs, it may also decrease oxygen levels in the blood pumped out to the body.
Atrium/atria
The heart is a pump with four chambers. The right and left atria (plural for atrium) sit above the right and left ventricles and pump blood into the ventricles through heart valves. Blood comes from the body into the right atrium, which pumps blood through the tricuspid valve into the right ventricle and out to the lungs. Blood comes back from the lungs into the left atrium, which pumps blood through the mitral valve into the left ventricle and out to the rest of the body.
B
Balloon angioplasty
A balloon angioplasty is a procedure in which a balloon catheter is inflated inside an artery (typically a coronary artery) to increase its diameter.
BiPAP machine
A bi-level positive airway pressure (BiPAP) machine uses two different levels of pressurized air to help people breathe properly. One pressure level assists with inhalation, while a second, lower pressure during exhalation keeps the airway open, making the next breath easier. It helps treat obstructive sleep apnea and other respiratory diseases. See also CPAP machine
Bosentan
Bosentan is an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name for bosentan is Tracleer. See also endothelin receptor antagonists | Read more about bosentan
BMPR2 gene
Sometimes called the “pulmonary hypertension gene,” this gene guides the production of bone morphogenetic protein receptor type 2 (BMPR2). Mutations in this gene have been found in families with more than one member with pulmonary arterial hypertension (heritable PAH) and in some patients with no known family history of the disease (idiopathic PAH).
Bradycardia
Bradycardia is a slow heartbeat, generally less than 55 to 60 beats per minute.
Bronchiolitis obliterans
Bronchiolitis obliterans, sometimes known as "popcorn lung," is a rare disease of the lung tissue. The smallest airways—the bronchial tubes and alveoli—become damaged, inflamed, or blocked. It can be caused by infection, breathing in toxins, or complications after lung transplantation.
C
Calcium channel blockers (CCBs)
Calcium channel blockers are blood pressure medicines for systemic hypertension (the normal kind of high blood pressure). In a small percentage of patients with pulmonary hypertension, CCBs decrease pulmonary artery pressure. During a right heart catheterization, a test may be done with a short-acting medication to assess whether a patient has a positive response. Only those with a positive response benefit from CCBs; in others, CCBs may cause harm.
Canada's Drug Agency (CDA-AMC)
Canada's Drug Agency is funded by the federal, provincial, and territorial governments to align drug policy across the country. Once Health Canada approves a drug for use in Canada, the CDA-AMC recommends whether or not it should be covered by provincial and territorial drug plans. Provinces may choose to follow their recommendation, or they may make a different decision.
Cardiac
Relating to the heart.
Cardiac catheterization
A right-heart catheterization is the definitive diagnostic test for pulmonary hypertension. Until a right-heart catheterization is performed, a formal diagnosis of pulmonary hypertension cannot be made.
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In a right-heart catheterization (RHC), a small catheter (tube) is inserted into a vein, usually in the neck or groin. The tube is advanced into the right side of the heart and then into the main pulmonary artery, where the pulmonary artery blood pressure can be measured. Next, advancing the catheter into a pulmonary artery branch allows doctors to estimate the pressure on the left side of the heart. This is called a pulmonary artery wedge pressure.
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In a left-heart catheterization (LHC), a catheter is placed into an artery, usually the femoral artery in the groin. The catheter is advanced into the left side of the heart to measure pressures in the aorta and the left ventricle, which may indicate problems with the structure and/or function of the left side of the heart. Left-sided heart disease can be a result of smoking, high cholesterol or high systemic blood pressure. It can also result from problems with the structure and function of the heart valves on the left side of the heart (mitral and aortic valves), or even from a problem with the heart muscle itself (cardiomyopathy). Left-sided catheterizations sometimes use contrast dye to look at the blood vessels supplying the heart (coronary angiogram).
Occasionally, your doctor may advise you to have both a right- and a left-heart catheterization to help make a diagnosis and to guide treatment.
Cardiac output
Cardiac output is the total amount of blood the heart pumps per minute. In a healthy person, this is about 5 to 6 litres per minute; in someone with severe PH, it can be 2 or 3 litres or even less. (A human has about 5 litres of blood inside their arteries at any one time.)
Cardiopulmonary exercise testing (CPET)
Cardiopulmonary exercise testing looks at your heart and lung function at the same time while you are exercising. This test helps determine your exercise tolerance.
Caripul®
Caripul is an intravenous treatment approved by Health Canada to treat pulmonary arterial hypertension. Caripul is the brand name for a form of epoprostenol that is stable at room temperature (meaning it can be used without ice packs for 24 hours). See also prostacyclin analogues/prostanoids | Read more about epoprostenol
Catheter
A catheter is a hollow, flexible tube that is inserted into a body cavity, duct or vessel to allow the passage of fluids. In pulmonary hypertension:
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A catheter that is placed in a central vein is called a central line. It may also be known as a Hickman catheter, Broviac catheter, or a PICC line. One end of a central line catheter is inserted into a vein and advanced to a larger (central) vein leading to your heart. In pulmonary arterial hypertension, this catheter is typically used to administer intravenous medications, including epoprostenol or treprostinil.
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A subcutaneous catheter is a small, flexible tube placed just under the skin in the body's fat tissue. It is used to deliver medications. These catheters are usually placed by the patient or caregiver.
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A diagnostic catheter is a thin, hollow tube used to measure pressures in the heart and blood vessels. See cardiac catheterization.
Chronic obstructive pulmonary disease (COPD)
COPD develops when lung damage leads to irritation and inflammation, reducing airflow and oxygen exchange and making it harder to breathe.
Chronic thromboembolic pulmonary hypertension (CTEPH)
CTEPH is WHO Group 4 pulmonary hypertension. In most patients with blood clots in the lungs (pulmonary embolisms, PE), the body can break them down. In a minority of patients, the pulmonary embolism does not resolve and can cause scarring in the pulmonary arteries, which leads to the development of CTEPH.
Cialis®
Cialis is the brand name for tadalafil, an oral treatment approved by Health Canada to treat erectile dysfunction. Tadalafil is also sold as Adcirca, which is used to treat pulmonary arterial hypertension. See also phosphodiesterase inhibitors | Read more about tadalafil
Cirrhosis
Cirrhosis is a buildup of fibrous tissue (scarring) in the liver, usually due to chronic liver disease.
Connective tissue diseases / Collagen vascular disease/autoimmune diseases
Connective tissue diseases affect tissues in the joints (muscles, bones, tendons, and cartilage) and solid organs of the body. These diseases can result from an autoimmune process (when your immune system turns against your body) or a genetic process (an inherited disease). Types associated with pulmonary hypertension include systemic lupus erythematosus (referred to as SLE or lupus), scleroderma, rheumatoid arthritis, and mixed connective tissue disease.
Congenital heart disease/defect (CHD)
A congenital heart defect is a heart problem that is present at birth. The defects can range from simple holes in the heart to much more complex problems.
Coronary artery
Coronary arteries transport oxygenated blood to the heart muscle itself.
Cor pulmonale
Pulmonary hypertension can cause cor pulmonale, a condition in which the right ventricle of the heart enlarges and weakens. It is usually treated by treating the underlying condition, in this case, pulmonary hypertension.
Coumadin®
Coumadin is the brand name for warfarin, a blood thinner approved by Health Canada. It is prescribed for patients who are at risk of developing blood clots (see CTEPH). It is also given to patients with pulmonary hypertension (typically IPAH patients), as there is some evidence that the blood vessels with high pulmonary pressures can be prone to microscopic clots in their pulmonary blood vessels. Warfarin can help prevent further clots and existing clots from getting bigger.
CPAP machine
A Continuous Positive Airway Pressure (CPAP) machine uses pressurized air to hold airways open and help people breathe properly. It helps treat obstructive sleep apnea and other respiratory diseases. It is a small machine for the treatment of obstructive sleep apnea and other respiratory diseases. See also BiPAP machine
CREST syndrome
CREST is an acronym for a form of scleroderma that involves:
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Calcinosis (calcium deposits in the skin)
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Raynaud’s syndrome
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Esophageal dysmotility (problems moving food and liquid from the mouth to the stomach)
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Sclerodactyly (swollen fingers with tight skin)
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Telangiectasia.
Patients with CREST syndrome are known to have a greater risk for developing pulmonary hypertension.
CT scan or CAT scan
CAT stands for cross axial tomography. CT scans/CAT scans are x-ray images of the inside of your body taken by narrow x-rays beamed at you from several angles and run through a computer to form an image with much greater detail than a single x-ray. It is excellent at evaluating solid organs, so it is commonly ordered to evaluate the heart and lungs in patients with pulmonary hypertension.
Cyanosis
Cyanosis is a bluish or purplish discoloration of the skin and mucus membranes caused by a lack of oxygen in the blood. It is often seen in the nails, face, lips and tongue.
D
Deep vein thrombosis (DVT)
A deep vein thrombosis is a blood clot in the veins deep in the body, most typically in the lower leg or thigh.
Diastolic
Diastolic pressure is the bottom, lower number in your blood pressure. For example, if your blood pressure is 120/80 mm Hg, the diastolic pressure is 80 mm Hg. This is the pressure in your arteries when your heart relaxes and fills with blood. See also systolic
Digoxin
Digoxin is a medication used to treat many different heart conditions. It helps improve the heart's strength and efficiency and helps regularize the heartbeat.
Dilate
To relax or expand.
Diuretic
Diuretics are substances that help you lose excess water by increasing urine production. Sometimes diuretics are referred to as a “water pill” or “fluid pill.”
Drug approval process
In Canada, new medications go through a multi-stage process:
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The manufacturer apply to Health Canada for approval of the drug. Because Canada is a relatively small country, manufacturers often do not consider it large enough to warrant the expense of the approval process.
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Health Canada approves the drug for use in Canada.
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Canada's Drug Agency (CDA-AMC) recommends whether or not it should be covered by provincial and territorial drug plans. In Québec, the Institut national d'excellence en santé et services sociaux (INESSS) plays a similar role.
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The Patented Medicine Prices Review Board (PMPRB) assesses whether a drug's price may be considered excessive.
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The pan-Canadian Pharmaceutical Alliance (pCPA), which includes all provinces and territories, decides whether to negotiate a price for the medication for provincial and territorial public drug plans, and then conducts that negotiation.
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Each province, territory, and private insurer decides whether to add the new medication to their drug plans.
Dyspnea
Shortness of breath or laboured breathing.
E
Echocardiogram (echo)
An echocardiogram is a test that uses moving images produced by ultrasonic waves (sound waves, or ultrasound) reflected off your heart to show its structures and function. The echocardiogram also estimates the pressures on the right side of the heart, which may help in the diagnosis and treatment of pulmonary arterial hypertension. The pictures are produced in the same way as a fetal ultrasound shows a baby in the womb.
Edema
Edema is swelling caused by fluid in your body’s tissues. It usually occurs in the feet, ankles, and legs, but it can affect your entire body. See also ascites
Eisenmenger syndrome
Eisenmenger syndrome results from a birth defect in which a hole in the heart causes red, oxygenated blood to flow from the left side of the heart to the right side and back to the lungs before it can be pumped to the body. Over time, this increased blood flow to the lungs damages the pulmonary arteries, leading to pulmonary arterial hypertension. Over time, as the resistance in the lung arteries increases due to this damage, deoxygenated blood begins to bypass the lungs and goes straight to the left side of the heart, where it is pumped back to the body. See congenital heart disease and cyanosis.
Electrocardiogram (ECG or EKG)
An electrocardiogram is a test that records the heart's electrical activity to help diagnose heart problems.
Embolus/emboli
An embolus is a clump of something (usually a blood clot) or a bubble that has plugged up a blood vessel. An embolism is the blockage of a vessel by an embolus. It’s usually a blood clot that has formed somewhere else and travelled to the lungs. A thrombus is a fibrinous blood clot that obstructs a blood vessel. If a thrombus forms in one place and moves to another, it is called a thrombotic embolus. Embolisms in the lungs (pulmonary embolisms) can result in pulmonary hypertension.
Emphysema
Emphysema is a type of chronic obstructive pulmonary disease (COPD) involving damage to the air sacs in the lungs.
Endothelin
Endothelin is a chemical made by the endothelium. It causes the smooth muscle in the blood vessel walls to contract (tighten). Pulmonary arterial hypertension patients have too much endothelin in their blood. See endothelium
Endothelin receptor antagonists
Bosentan (Tracleer®), ambrisentan (Volibris®), and macitentan (Opsumit®) are medications that block endothelin from attaching to endothelin receptors on the smooth muscle cells of the pulmonary arteries. This prevents the vessels from tightening (constricting) and helps lower pulmonary artery pressure. Read more about treatment options
Endothelium
The one-cell-thick lining of the blood vessels, lymph channels, and heart. Endothelial cells produce many chemical compounds that normally cause blood vessel walls to relax and dilate. Abnormalities with the endothelium that prevent blood vessels from relaxing and dilating are one possible cause of pulmonary arterial hypertension.
Enzymes
Enzymes are substances made by cells that can act inside or outside cells to control the rate of chemical reactions without the enzyme being used up (an enzyme can help the same process along over and over).
Epidemiology
Epidemiologists study the spread and control of illness in the population.
Epoprostenol
Epoprostenol is an intravenous treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand names for epoprostenol are Caripul and, less commonly, Flolan. See also prostacyclin analogues/prostanoids
Etiology
The cause of something, such as a disease.
F
Familial pulmonary arterial hypertension (FPAH)
The previous name for heritable (inherited) pulmonary arterial hypertension. See also heritable pulmonary arterial hypertension
Fibrin
Fibrin is the insoluble protein that is the end product of the blood coagulation process. It helps stop bleeding, playing a major role in blood clotting.
Fibrosis
Fibrosis is when too much fibrous connective tissue (scarring) builds up in response to inflammation, irritants, or an injury.
Flolan®
Flolan is an intravenous treatment approved by Health Canada to treat pulmonary arterial hypertension. Flolan is a brand name for epoprostenol. Caripul, a temperature-stable brand of epoprostenol, is more commonly used in Canada. See also prostacyclin analogues/prostanoids | Read more about epoprostenol
Functional class
A functional class defines how sick someone with pulmonary hypertension is based on how limited the patient's daily activities are. The World Health Organization defines four classes to describe how pulmonary hypertension affects someone's daily life:
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Class 1: No symptoms during everyday physical activity
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Class 2: Mild symptoms during activity, but no symptoms at rest
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Class 3: Noticeable symptoms with less activity, although the person is usually comfortable at rest
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Class 4: Symptoms occur even when resting, and any regular physical activity worsens symptoms. Fainting may occur.
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G
Gaucher’s disease
Gaucher's disease is an inherited disease in which a lack of an enzyme (glucocerebrosidase) leads to the accumulation of a fatty substance (glucocerebroside) in parts of the body. If the fatty cells accumulate in the lungs, it can lead to pulmonary hypertension.
Gene, pulmonary hypertension
A gene is a DNA sequence for a particular trait. See BMPR2 gene | Read more about heritable PH
H
Health Canada
Health Canada is responsible for approving drugs for use in Canada. Approval is the first step in making a new drug available. See also drug approval process
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Hemodynamics
Hemodynamic measurements describe how blood moves through your body. In pulmonary hypertension, hemodynamics typically refers to pulmonary blood pressures, cardiac output, and the calculation of pulmonary resistance, which is a function of both pulmonary artery pressure and cardiac output.
Heritable pulmonary arterial hypertension (HPAH)
This term is used when a gene associated with pulmonary hypertension (for example, the BMPR2 gene) has been identified and/or there is a family history of pulmonary hypertension. It is the new term for what was previously called familial pulmonary arterial hypertension (FPAH).
Hickman catheter
A Hickman catheter is an intravenous line that is used to administer medication. See also catheter
Human immunodeficiency virus (HIV)
HIV is an infection that causes the immune system to fail, making the body more prone to infections. For unknown reasons, patients with HIV develop pulmonary arterial hypertension at a rate higher than the general population.
Hypertension
Hypertension is abnormally high blood pressure.
Hypotension
Hypotension is abnormally low blood pressure.
Hypoxemia
Hypoxemia is a low concentration of oxygen in the blood. This can cause pulmonary vessels to contract and raise pulmonary artery pressure.
Hypoxia
Hypoxia is an inadequate oxygen supply to the body or part of the body.
I
Idiopathic
A medical term that is used to describe a disease that has no known underlying cause.
Idiopathic pulmonary arterial hypertension (IPAH)
Idiopathic pulmonary arterial hypertension was formerly called primary pulmonary hypertension (PPH). It describes pulmonary arterial hypertension where the cause is unknown. Pulmonary arterial hypertension is a rare disease, affecting 2,000-4,000 Canadians. Read more about types of PH
Iloprost
Iloprost is the brand name for an inhaled form of treprostinil. It is not approved in Canada. See also prostacyclin analogues/prostanoids | See also treprostinil
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Institut national d'excellence en santé et en services sociaux (INESSS)
In Québec, INESSS has a mandate to:
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Evaluate the clinical advantages and the costs of technology, medications and health care and social service interventions.
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Develop recommendations and guides for the optimal use of technologies, medications, and interventions, and update, publish, and promote their implementation.
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Promote and support the development of scientific evaluations regarding technology, medications, healthcare, and social service interventions.
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Formulate recommendations to the Minister as part of the updating of the List of Medications.
International normalized ratio (INR)
The international normalized ratio is a blood test used to determine how long it takes your blood to clot (how thin the blood is). It is used to determine an appropriate dose of blood-thinning medications.
Intravenous (IV)
Intravenous literally means “inside the vein.” It describes fluids or medications administered through a catheter or needle inserted into a vein. An intravenous administration is called an infusion.
Ischemia
Ischemia is a localized, temporary reduction in blood flow resulting in tissue or organ damage. In cardiac ischemia, blocked blood flow results in chest pain and can result in a heart attack. Ischemia can happen in any organ or tissue of the body.
K
Kaplan-Meier estimates of survival (Kaplan-Meier survival plot)
A statistical technique that allows researchers to estimate survival in research studies. A Kaplan-Meier survival plot describes the probability of surviving in a given length of time over many small time intervals. They are often used to compare treatments with each other and with placebo groups. In the sample chart below, you can see that 0.8, or 80%, of the treatment group (the blue line) survived for five years but only 0.4, or 40%, of the placebo group (the red line) did.
L
Left-heart catheterization
See cardiac catheterization
Lesion
An area of diseased or injured tissue.
Lupus
See systemic lupus erythematosus
M
Macitentan
Macitentan is an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name for macitentan is Opsumit. See also endothelin receptor antagonists | Read more about macitentan
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Mean pulmonary artery pressure (mean PAP, mPAP)
A measurement calculated using the upper (systolic) and lower (diastolic) numbers of the pulmonary artery pressure. Various formulas are used -- it is not the simple average of the two numbers. See also pulmonary artery pressure
Magnetic resonance imaging (MRI)
Magnetic resonance imaging is a technique that uses a strong magnetic field and low-energy radio waves to view the inside of the body, sometimes with the help of a radiopaque dye. MRI images usually provide great detail, allowing for easier diagnosis. In pulmonary hypertension, MRI captures better pictures of the heart, heart valves and heart functioning than an echocardiogram.
N
Nebulizer
A nebulizer is a machine that can change medications into small particles (aerosolize) so that they can be inhaled.
Nitric oxide (NO)
Nitric oxide is a potent vasodilator in gas form. It is not the same thing as nitrous oxide, which is “laughing gas” used during dental procedures and midwifery. Nitric oxide is delivered through a face mask or nasal cannula, like oxygen. It is used for newborn babies with persistent pulmonary hypertension and has various uses in adult pulmonary arterial hypertension.
O
Obstructive sleep apnea
Obstructive sleep apnea is a disorder in which the patient stops breathing for short periods during sleep because some part of the airway is blocked. Obstructive sleep apnea can result in pulmonary hypertension, but treating the apnea can help treat the pulmonary hypertension.
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Opsumit
Opsumit is the brand name for macitentan, an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. See also endothelin receptor antagonists| Read more about macitentan
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Oximeter/pulse oximeter
A pulse oximeter device measures the oxygen concentration in your blood by shining a specific wavelength of light through your skin. They usually clip onto your finger or earlobe. See also oxygen saturation
Oxygen (O2)
An essential element that is needed to sustain life. Extra oxygen is often given as an inhaled gas to patients when their blood oxygen level is low.
Oxygen saturation (O2 sat)
Your oxygen saturation level measures the amount of oxygen in your blood. Normal saturation is around 97 to 100 percent. Lung disease (pulmonary hypertension, COPD, pneumonia, etc.) can cause low oxygen saturation, as can low oxygen content in the air at high altitudes. In patients with lung disease, it is generally desirable to be above 90 percent “saturated.” Some patients with congenital heart disease have to live with saturations less than 90 percent since oxygen supplementation does not usually increase their saturation.
P
Palpitation
A sensation of rapid, skipped or pounding heartbeats. Sometimes palpitations are more noticeable when you are sitting quietly.
Pan-Canadian Pharmaceutical Alliance (pCPA)
The pan-Canadian Pharmaceutical Alliance brings all the provinces and territories together to negotiate a price for new drugs. The pCPA is one part of the overall Canadian drug reimbursement process. The pCPA negotiation process usually begins once Canada's Drug Agency (CDA-AMC) and/or l’Institut national d’excellence en santé et en services sociaux (INESSS) publishes a recommendation. pCPA uses the recommendations from the CDA-AMC and INESSS and other factors to determine whether or not it will enter into a negotiation for a drug.
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Patented Medicine Prices Review Board (PMPRB)
The Patented Medicine Prices Review Board (PMPRB) reviews patented medicine prices to protect Canadians from any patent abuse related to excessive prices. The PMPRB also reports on Canadian pharmaceutical trends. It is an independent quasi-judicial body that operates at arm’s length from the Federal Minister of Health.
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Pathogenesis
Pathogenesis is the process by which a disease originates and develops.
Persistent pulmonary hypertension of the newborn (PPHN)
Persistent pulmonary hypertension of the newborn occurs when the pulmonary arteries remain constricted after delivery, reducing blood flow to the lungs and leading to pulmonary hypertension. PPHN is treated with inhaled nitric oxide.
Phosphodiesterase (PDE-5) inhibitors
Patients with pulmonary arterial hypertension produce less nitric oxide (NO), a normal pulmonary vasodilator. Phosphodiesterase inhibitors (PDE-5 inhibitors) are medications that improve the action of patients' own nitric oxide by blocking the enzyme phosphodiesterase type 5 (PDE-5). PDE-5 breaks down a molecule called cyclic GMP (cGMP), a “messenger” of nitric oxide (NO) that causes vasodilation. Blocking the breakdown of cGMP thus leads to more nitric oxide effects. PDE-5 inhibitors are pulmonary vasodilators that can open up pulmonary arteries and reduce pulmonary arterial pressure, as well as improve right-side heart function. These medications, including sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis), were first used to treat erectile dysfunction. Read more about treatment options
Portopulmonary hypertension
Portopulmonary hypertension is a type of pulmonary arterial hypertension that develops due to high blood pressure in the liver. It is most often caused by cirrhosis (scarring) in the liver.
Primary pulmonary hypertension
Primary pulmonary hypertension is an old term for pulmonary arterial hypertension. See also idiopathic pulmonary arterial hypertension
Prostacyclin
Prostacyclin is a prostaglandin or prostanoid, one of a family of chemicals made in the body that help regulate blood vessel tone by dilating the blood vessels. Patients with pulmonary arterial hypertension produce less prostacyclin, so researchers correctly predicted that administering synthetic prostacyclin would cause vasodilation of the pulmonary arteries. In addition to being a vasodilator, prostacyclin may help treat pulmonary arterial hypertension by inhibiting the growth and thickening of the pulmonary vessel walls and by making platelets less “sticky”. Strange fact: The name prostacyclin comes from the prostate gland, because prostaglandins were first isolated from seminal fluid and were thought to have been made by the prostate! Read more about treatment options
Prostacyclin analogues / Prostanoids
Prostacyclin analogue (prostanoid) medications act similarly to the prostacyclin made by the body. Those used in Canada include epoprostenol (Flolan), treprostinil (Remodulin), and room-temperature stable epoprostenol (Caripul). These medications help many patients with pulmonary arterial hypertension by dilating blood vessels, reducing clotting, slowing the growth of smooth muscle cells, and improving cardiac output. Their toxicity, side effects and effectiveness may differ.
Pulmonary
Relating to the lungs.
Pulmonary angiogram
A pulmonary angiogram is an x-ray of the blood vessels of the lungs. They are injected with a special dye to highlight any blockages.
Pulmonary artery
The pulmonary arteries are the blood vessels that carry blood from the right ventricle of the heart to the lungs, where the blood is oxygenated. The main pulmonary artery branches into the right and left pulmonary arteries, which feed the right and left lungs. They continue to branch into smaller and smaller blood vessels, eventually forming capillaries that exchange oxygen (O2) and carbon dioxide (CO2) through the alveoli (air sacs).
Pulmonary artery pressure (PAP)
A measurement of the blood pressure in the pulmonary artery. Like a traditional blood pressure measurement, it has a systolic (upper) and diastolic (lower) component, for example, 25/9 mmHg. See also mean pulmonary artery pressure.
Pulmonary arterial hypertension (PAH)
Pulmonary arterial hypertension is a type of pulmonary hypertension caused by the narrowing of the pulmonary arteries (blood vessels) that carry blood to the lungs. In pulmonary arterial hypertension, the pulmonary arteries narrow due to the proliferation and growth of cells in their walls, as well as the formation of scar tissue. Read more about types of pulmonary hypertension
Pulmonary artery wedge pressure
A measurement obtained during right heart catheterization. A balloon on the tip of the right heart catheter is inflated (wedged) in a pulmonary artery, where it can estimate pressures.
Pulmonary function tests (PFTs)
Pulmonary function tests find out how well your lungs work: how much air your lungs can hold, how well they move air in and out, and how well they exchange oxygen. You breathe into a device called a spirometer, or flow meter.
Pulmonary hypertension (PH)
Pulmonary hypertension is a general term used to describe high pressure in the pulmonary arteries from any cause. Read more about types of pulmonary hypertension
Pulmonary hypertension gene
See BMPR2 gene. Other genes related to pulmonary hypertension likely exist but are yet to be discovered.
Pulmonary thromboendarterectomy (PEA or PTE)
Pulmonary thromboendarterectomy is a major surgery that removes clots and scar tissue from the blood vessels of the lungs of people with chronic thromboembolic pulmonary hypertension (CTEPH). See also CTEPH
Pulmonary vascular resistance (PVR)
Pulmonary vascular resistance is a measure of how difficult it is for the heart to pump blood through the lungs. PVR is calculated during cardiac catheterization and is often very high in patients with pulmonary hypertension.
Pulmonary vein
The pulmonary vein returns oxygenated blood from the lungs to the left side of the heart.
Pulmonary venous hypertension
In pulmonary venous hypertension, which is part of Group 2 pulmonary hypertension, the left side of the heart has difficulty carrying blood away from the lungs. This causes blood to back up in the pulmonary veins, causing higher pulmonary vein pressure and pulmonary artery pressure. The cause of this disease is not in the pulmonary arteries, so it is not usually treated with drugs designed to treat pulmonary arterial hypertension. See also WHO pulmonary hypertension groups
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Raynaud’s phenomenon
In Raynaud's phenomenon, strong emotions or cold temperatures cause small blood vessels in the fingers (and sometimes toes) to spasm. This can be painful. The spasms block blood flow, causing the fingers to appear white or blue. Pulmonary arterial hypertension and autoimmune disorders are associated with Raynaud's phenomenon.
Remodulin
Remodulin is the brand name for the intravenous and subcutaneous forms of treprostinil, a treatment approved by Health Canada to treat pulmonary arterial hypertension. See also treprostinil | See also prostacyclin analogues | Read more about treprostinil
Revatio
Revatio is the brand name for sildenafil, an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. See also sildenafil | See also phosphodiesterase inhibitors | Read more about sildenafil
Right atrial pressure
The pressure in the right atrium of the heart. Usually, the right atrial pressure is less than 5 mmHg. Elevated right atrial pressure is a sign of right-heart failure and too much fluid in the body. See also atrium
Right-heart catheterization
See cardiac catheterization
Right ventricle
The chamber of the heart that pumps un-oxygenated blood through the pulmonary arteries into the lungs. In patients with pulmonary hypertension, this chamber is often enlarged, its walls are thickened, and its ability to contract is reduced. See also ventricle
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Sarcoidosis
A disease in which swelling (inflammation) occurs in the lymph nodes, lungs, liver, eyes, skin or other tissues. Lumps of inflamed immune cells (granulomas) and scarring (fibrosis) in the lungs can cause pulmonary hypertension.
Scleroderma
Scleroderma is a progressive and potentially fatal disease in which the body’s tissues slowly harden. In some people, only the skin is affected. In others, even the internal organs harden, including the lungs. It is not known what causes it, but there is a genetic component. It is more common in women than in men. Scleroderma is a common cause of pulmonary hypertension and sometimes pulmonary arterial hypertension.
Secondary pulmonary hypertension (SPH)
An older term for pulmonary hypertension caused by another disease or condition. See also associated pulmonary arterial hypertension
Sickle cell anemia
Sickle cell anemia is a genetic disease in which the usually round and flexible red blood cells of the body are sickle-shaped and rigid. Because abnormally shaped red blood cells cannot flow easily through the arteries and veins, severe joint pain, chest pain, pulmonary hypertension, and other complications may result. Patients with sickle cell anemia require frequent blood transfusions and pain medications.
Sildenafil
Sildenafil is an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name for sildenafil is Revatio (when sold for pulmonary arterial hypertension) and Viagra (when sold for erectile dysfunction). See also phosphodiesterase inhibitors | Read more about sildenafil
Six-minute walk test (6MWT)/Six-minute walk distance (6MWD)
A measurement of how far you can walk in six minutes, including your oxygenation level and how short of breath you are at completion. Six-minute walk tests can measure whether your pulmonary hypertension is improving or worsening.
Sleep apnea
Sleep apnea is when breathing slows or stops during sleep. It is always abnormal and needs immediate attention. See also obstructive sleep apnea
Sotatercept
Sotatercept is an injectable treatment approved by Health Canada to treat pulmonary arterial hypertension. See also activin signalling inhibitors | Read more about sotatercept
Subcutaneous
Under all the layers of skin. Some medications for pulmonary arterial hypertension are given subcutaneously using an infusion pump.
Syncope
Fainting or passing out. This happens when there is a sudden drop in blood flow to the brain.
Systemic
Something that affects the body generally rather than just affecting one of the body’s parts.
Systemic lupus erythematosus (SLE)
Systemic lupus erythematosus, often known as lupus, is a progressive disease in which the body attacks its own healthy tissue. The disease can involve many parts of the body. People with lupus typically have joint pain, and a skin rash often spreads across the bridge of the nose and cheeks in a butterfly wing pattern. Patients with lupus are much more likely to get pulmonary arterial hypertension than people in the general population.
Systolic
Systolic pressure is the top, higher number in your blood pressure. For example, if your blood pressure is 120/80 mm Hg, the systolic pressure is 120 mm Hg. This is the pressure in your arteries when your heart contracts and squeezes blood out. See also diastolic
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Tadalafil
Tadalafil is an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name for tadalafil is Adcirca (when sold for pulmonary arterial hypertension) and Cialis (when sold for erectile dysfunction). See also phosphodiesterase inhibitors | Read more about tadalafil
Telangiectasia
Telangiectasias are reddened blotches of widened, very small blood vessels on the skin.
Teratogenic
A teratogenic substance is something that is capable of interfering with the development of a fetus, causing developmental problems or birth defects.
Tracleer
Tracleer is the brand name for bosentan, an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. See also endothelin receptor antagonists| Read more about bosentan
Treprostinil
Treprostinil is an intravenous or subcutaneous treatment approved by Health Canada to treat pulmonary arterial hypertension. The brand name in Canada for treprostinil is Remodulin. Inhaled forms of treprostinil exist but are not approved in Canada. See also prostacyclin analogues/prostanoids | Read more about treprostinil
Tyvaso
Tyvaso is the brand name for an inhaled form of treprostinil. It is not approved in Canada. See also prostacyclin analogues/prostanoids | See also treprostinil
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Vasoconstriction and vasodilation
There is a balance between constriction (tightening) and dilation (opening) in blood vessels so that blood flow can be regulated. Constriction of a blood vessel is called vasoconstriction, and dilation of a blood vessel is called vasodilation.
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Vasoconstrictor
Anything that narrows the blood vessels, thereby increasing blood pressure.
Vasodilator
Anything that relaxes and widens blood vessels, thereby decreasing blood pressure.
Viagra
Viagra is the brand name for sildenafil, an oral treatment approved by Health Canada to treat erectile dysfunction. Sildenafil is also sold as Revatio, which is used to treat pulmonary arterial hypertension. See also sildenafil
Ventavis
Ventavis is the brand name for an inhaled form of iloprost. It is not approved in Canada. See also prostacyclin analogues/prostanoids | See also iloprost
Ventilation/perfusion (V/Q) scan
A ventilation/perfusion scan is a two-part medical imaging scan. The ventilation scan shows where air flows in the lungs, and the perfusion scan shows where blood flows in the lungs. (Why Q and not P? In early days, the Q apparently referred to the "quantity" of blood flow, and the abbreviation stuck.)
Ventricle
A ventricle is a cavity or chamber in the body. In pulmonary hypertension, ventricle generally refers to either of the heart's two lower chambers. The heart is a pump that consists of four chambers. The right and left atria (plural for atrium) sit above the right and left ventricles and pump blood into the ventricles through heart valves. Blood comes from the body into the right atrium, which pumps blood through the tricuspid valve into the right ventricle and then out to the lungs. Blood comes back from the lungs into the left atrium, which pumps blood through the mitral valve into the left ventricle and then out to the rest of the body. | See also right ventricle
Ventricular septal defect (VSD)
A ventricular septal defect is a hole in the ventricular septum, the wall separating the heart's right and left lower chambers (ventricles). The hole allows blood to flow between these two chambers. See also congenital heart disease |See also Eisenmenger syndrome | Read more about VSD
VO2
A measure of the amount of oxygen (O2) that the body takes in and uses. Usually, doctors measure the peak or maximum VO2 (VO2 max) you can achieve during exercise. The higher the number, the better your heart and lungs can deliver oxygen to your body. It is measured in litres of oxygen per minute, or sometimes millilitres of oxygen per kilogram of body mass per minute.
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Volibris
Volibris is the brand name for ambrisentan, an oral treatment approved by Health Canada to treat pulmonary arterial hypertension. See also endothelin receptor antagonists | Read more about ambrisentan
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Warfarin
Warfarin is an anticoagulant medication approved by Health Canada. The brand name for warfarin is Coumadin. See also anticoagulant medications
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WHO pulmonary hypertension groups
The World Health Organization divides pulmonary hypertension into five groups:
Group 1 - Pulmonary arterial hypertension
Group 2 - Pulmonary hypertension due to left-side heart disease
Group 3 - Pulmonary hypertension due to lung disease
Group 4 - Chronic thromboembolic pulmonary hypertension
Group 5 - Pulmonary hypertension due to other conditions
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Winrevair
Winrevair is the brand name for sotatercept, an injectable treatment approved by Health Canada to treat pulmonary arterial hypertension. See also activin signalling inhibitors | Read more about sotatercept
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Yutrepia
Yutrepia is the brand name for an inhaled form of treprostinil. It is not approved in Canada. See also prostacyclin analogues/prostanoids | See also treprostinil
Thank you
Adapted with permission from the Pulmonary Hypertension Association in the USA. Thank you!