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 (PH Group 2). They are not generally used to treat pulmonary arterial hypertension (PH 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 PH 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 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.
B
Balloon angioplasty
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 amount of 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 “PH gene,” this gene guides the production of bone morphogenetic protein receptor type 2 (BMPR2). Mutations of this gene have been found in families that have more than one member with pulmonary arterial hypertension (heritable PAH) and in some patients with no known family history of the disease (idiopathic PAH). Read more about heritable PAH
Bradycardia
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)
Blood pressure medicines for systemic hypertension. CCBs have been found to decrease pulmonary artery pressures in a small percentage of PH patients. CCBs that may help include nifedipine (Procardia XL®, Adalat®), diltiazem (Cardizem®), and amlodipine (Norvasc®). Be aware that only certain PAH patients benefit from CCBs; in others, CCBs can do a lot of harm. Read more about conventional medical therapies
Canada's Drug Agency (CDA)
Cardiac
Relating to the heart
Cardiac catheterization
The definitive final diagnostic test performed to determine if a patient has pulmonary hypertension. Until a right-heart catheterization is performed, a diagnosis of pulmonary arterial hypertension cannot be made. Read more about diagnositic tests
Right-heart catheterization (RHC) is performed by placing a small catheter (tube) into a vein, usually a vein in the neck or groin. The tube is advanced in the direction of blood flow into the right side of the heart and then into the main pulmonary artery. Once the catheter is in the pulmonary artery, measurements of the blood pressure in the pulmonary artery can be made. 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 capillary wedge pressure. Read more about common tests in PH
Left-heart catheterization (LHC) is performed in a similar manner except that the 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 make measurements of pressures in the aorta and left ventricle. These measurements may indicate the presence of a disease related to 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, which predispose to heart attacks.Or it can be a result of problems related to the structure and function of the heart valves in the left side of the heart (mitral and aortic valves), or even just 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 left-side heart catheterization to help make a diagnosis and to guide treatment. Read more about common tests in PH
Cardiac output
The total amount of blood the heart pumps per minute. In a healthy person this is about 5 to 6 liters per minute; in someone with severe PH it can be as low as 2 to 3 liters or less. (A human has about 5 liters [roughly 5.3 quarts] of blood inside of their arteries at any one time.)
Cardiopulmonary exercise testing (CPET)
A way of determining cardiac and pulmonary function by measuring lung gas (oxygen and carbon dioxide) exchange efficiency. Read more about common tests for PH
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 Caripul
Catheter
A hollow flexible tube that is inserted into a body cavity, duct or vessel to allow the passage of fluids. There are many types of “catheters.” Below are descriptions of three specific to PH:
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. A surgeon may use different areas to place one end of a central line catheter into a vein. It is then advanced it to a larger (central) vein leading to your heart. In PAH, the catheter it is typically used to administer intravenous medications, including epoprostenol or treprostinil.
A subcutaneous catheter is a smaller flexible tube that is placed just under the skin in the fat tissue of the body. It is used to deliver medications. These catheters are usually placed by the patient or caregiver.
A diagnostic catheter, a thin, hollow tube that is used to measure pressures in the heart and blood vessels. See cardiac catheterization.
Chronic obstructive pulmonary disease (COPD)
A chronic disease characterized by difficulty in breathing due to over inflation of the lung sacs and/or constriction of the tubes leading to the air sacs. Learn more about COPD
Chronic thromboembolic pulmonary hypertension (CTEPH)
A form of pulmonary hypertension that is secondary to chronic (long-time) blood clots in the lungs.
Cialis®
An FDA-approved oral treatment of erectile dysfunction. Cialis® is the brand name for tadalafil (the same chemical used in the PAH drug, Adcirca®). See also tadalafil
Cirrhosis
buildup of fiber-like tissue (scarring) in the liver, usually due to chronic liver disease.
Connective tissue disease (a.k.a. Collagen vascular disease/autoimmune disease)
Disorders affecting joints (muscles, bones, tendons, cartilage), and solid organs of the body. Can be the result of an autoimmune (immune system in your body turns against itself) or genetic process (inherited disease). Types associated with PH include SLE (lupus), scleroderma, rheumatoid arthritis, and mixed connective tissue disease. Read more about scleroderma and PH
Congenital heart disease (CHD)
A defect of the heart present at birth. The defects can be simple holes in the heart or much more complex defects. Learn more about CHD
Coronary artery
Cor pulmonale
Enlargement of the right ventricle of the heart, which can be caused by pulmonary hypertension. Consists of symptoms of right side heart failure and fluid overload.
Coumadin®
The brand name for warfarin. It is a blood thinner prescribed for patients that 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. Coumadin® can help prevent further clots and existing clots from getting bigger. Read more about conventional medical therapies
CPAP machine
Defined as Continuous Positive Airway Pressure. It is a small machine for the treatment of obstructive sleep apnea and other respiratory diseases. In obstructive sleep apnea, the airway is “obstructed” due to a patient’s size or changes in the airway anatomy that cause collapse during sleep. The CPAP air pressure helps hold open the airways. See also BiPAP machine
CREST syndrome
CREST is an acronym for calcinosis, Raynaud’s syndrome, esophageal dysmotility, sclerodactyly and telangiectasia. CREST is a form of scleroderma that results in calcium deposits in the skin, Raynaud’s phenomenon, esophageal involvement, swollen fingers with tight skin, and reddened/discolored skin from by blood vessels. Patients with CREST syndrome are known to have a greater risk for developing pulmonary hypertension. Learn more about CREST syndrome
CT or CAT scan
An x-ray image 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 and therefore is a common x-ray ordered for evaluation of the heart and lungs in patients with pulmonary hypertension. CAT stands for cross axial tomography. Read more about common tests in PH
Cyanosis
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)
Formation a blood clot in the veins deep in the body, most typically in the lower leg or thigh. Learn more about DVT
Diastolic
Systolic 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
A medication used to treat many different heart conditions. It may help a weak heart muscle squeeze better. Read more about conventional medical therapies
Dilate
To relax, expand.
Diuretic
A chemical that helps you lose water by increasing the amount of urine. Sometimes called a “water pill” or “fluid pill.” Read more about conventional medical therapies
Dyspnea
Labored breathing; shortness of breath.
E
Echocardiogram (echo)
A test that uses moving pictures produced by ultrasonic waves (sound waves, or ultrasound) bounced off your heart to show its structures and functioning. The echocardiogram also estimates the pressures on the right side of the heart, which may help in the diagnosis and treatment of PAH. The pictures are produced in the same way a fetal ultrasound shows a baby in the mother’s womb. Read more about diagnositic tests
Edema
Swelling caused by fluid in your body’s tissues. It usually occurs in the feet, ankles and legs, but it can occur in your entire body. See also ascites
Eisenmenger syndrome
A syndrome that results from a birth defect where a hole in the heart that originally caused red, oxygenated blood to go from the left side of the heart to the right side of the heart and back to the lungs before it had a chance to be pumped to the body. After some time, this extra blood flow to the lungs damages the pulmonary arteries and PAH occurs. Over time, as the resistance in the lung arteries increases due to this damage, blue blood begins to skip going to 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. Learn more about Eisenmenger syndrome
Electrocardiogram (ECG or EKG)
An image produced by a device that records the electrical activity of the heart. Read more about diagnositic tests
Embolus (plural, emboli)
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 traveled 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. These are all known as blood clots, and can result in PH.
Emphysema
A type of chronic obstructive pulmonary disease (COPD) involving damage to the air sacs in the lungs. Learn more about emphysema
Endothelin
A chemical made by the endothelium (see below). It causes the smooth muscles in the blood vessel’s walls to constrict (tighten up). PAH patients have too much endothelin in their blood.
Endothelin receptor antagonists
Bosentan (Tracleer®) and ambrisentan (Letairis®) are medications that block endothelin from attaching to the endothelium receptors located in the smooth muscle cells of the pulmonary arteries. This prevents the vessels from tightening up/constricting and helps lower the pulmonary artery pressures. Read more about treatment options
Endothelium
The one-cell thick lining of the blood, lymph channels and the heart. Endothelial cells produce lots of chemical compounds that normally make blood vessel walls relax and dilate. In PAH there is an abnormality in the endothelium, which can result in the blood vessels’ inability to relax and dilate. Abnormalities with the endothelium are one possible cause of pulmonary arterial hypertension.
Enzymes
Organic substances made by cells that can act inside or outside of cells to control the rate of chemical reactions without the substance being changed themselves (an enzyme can help the same process along over and over).
Epidemiology
The study of illness in the population.
Epoprostenol sodium
An FDA-approved intravenous treatment for PAH. It is delivered intravenously through a central line catheter and with a small pump. Epoprostenol sodium is the chemical name for Flolan® and Veletri®. See also prostanoids
Etiology
The cause of something, such as a disease.
F
Familial pulmonary arterial hypertension (FPAH)
Old name for heritable (inherited) pulmonary arterial hypertension. See also heritable pulmonary arterial hypertension | Read more about heritable PH
Fibrin
The insoluble protein end product of blood coagulation, formed from fibrinogen by the action of thrombin in the presence of calcium ions. Thrombin acts on fibrinogen to form this insoluble protein called fibrin. (Part of the blood coagulation process.)
Fibrosis
Occurs when inflammation or other irritants cause a build-up of fibre-like tissue (scarring).
Flolan®
An FDA-approved intravenous treatment for PAH. Flolan® is the brand name for epoprostenol. See also epoprostenol sodium | Read more about Flolan®
Functional classifications for PH
A scale designed to define how sick someone with PH is based on how limited the patient is in his/her daily activities. There are two different scales that doctors use to define limitations:
New York Heart Association (NYHA) scale for all cardiac patients. The scale is from one to four: Class I having no symptoms to Class IV in which symptoms occur at rest. Most PAH patients are diagnosed when they have Class II or III symptoms. Learn more about the NYHA scale
World Health Organization (WHO) classifications are a modification the New York Heart Association (NYHA) categories for generic heart failure to make them specific to PH patients. The scale is also from one to four. The WHO and NYHA classes are very similar. One area where they differ is that if you are prone to fainting, you automatically go into Class IV under the WHO class. Learn more about WHO classifications
G
Gaucher’s disease
An inherited disease where the inherited lack of an enzyme leads to the accumulation of a fatty substance. If the fatty cells accumulate in the lungs, it can lead to secondary PH.
Gene, PH
See BMPR2 gene | Read more about heritable PH
H
Health Canada
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Hemodynamics
The pressure measurements obtained during the right and left heart catheterization. Typically, in PH, hemodynamics refers to pulmonary blood pressures, cardiac output and the calculation of pulmonary resistance, which is a combination of both the pulmonary artery pressure and the cardiac output.
Heritable pulmonary arterial hypertension (HPAH)
This term is used when the BMPR2 gene has been identified and/or there is a family history of pulmonary hypertension. It is the new term for what used to be called familial pulmonary arterial hypertension (FPAH). Read more about heritable PH
Hickman catheter
An intravenous line used to administer medication. See also catheter
Human immunodeficiency virus (HIV)
An infection that causes the immune system to fail and makes the body prone to infectious organisms that typically would not cause infections. Patients with HIV infection can develop PAH at a rate higher than the general population. The reason is unknown. Read more about HIV and PH
Hypertension
Abnormally high blood pressure.
Hypotension
Abnormally low blood pressure.
Hypoxemia
A low concentration of oxygen in the blood. This condition can make the pulmonary vessels contract and raise pulmonary artery pressure.
Hypoxia
A low concentration of oxygen in the air that is breathed, such as occurs aboard an airliner or high on a mountain.
I
Idiopathic
A medical term that is used to describe a disease that has no known underlying cause.
Idiopathic pulmonary arterial hypertension (IPAH)
Formerly called primary pulmonary hypertension (PPH). It means pulmonary arterial hypertension where the cause is unknown. It is very rare, with an incidence of approximately 2 to 5 per million worldwide. Read more about types of PH
Iloprost
An FDA-approved inhaled treatment for PAH. Iloprost is the chemical name for Ventavis®. See also prostanoids | Read more about iloprost
International normalized range (INR) level
A blood test used to determine how long it takes your blood to clot (how thin the blood is). It is used to determine how much Coumadin®/warfarin to prescribe to a patient to help keep the blood thin.
Intravenous (IV)
Literally “inside the vein.” Used to designate fluids or medications administered via a catheter or needle placed into the vein. An intravenous administration is called an infusion.
Ischemia
A localized, temporary reduction in blood flow resulting in tissue/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/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.
Letairis®
An FDA-approved oral treatment for PAH. Letairis® is the brand name for ambrisentan. See also ambrisentan |Read more about Letairis®
Lupus
See systemic lupus erythematosus
M
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)
An imaging technique that uses a strong magnetic field and low-energy radio waves to view the inside of the body, sometimes with the help of radiopaque dye. MRI images usually have great detail, allowing easier diagnosis. In pulmonary hypertension, MRI captures better pictures of the heart, heart valves and heart functioning than an echocardiogram. Read more about common tests in PH
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 pulm.
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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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. Read more about conventional medical therapies
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 (PAH, 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 more noticeable when sitting quietly.
Patented Drug Price Review Board (PMPRB)
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Pathogenesis
Pathogenesis is the process by which a disease originates and develops.
Persistent pulmonary hypertension of the newborn (PPHN)
Occurs when a newborn’s arteries to the lungs remain constricted after delivery, cutting down on blood flow to the lungs and resulting in PH. PPHN is treated with inhaled nitric oxide. Learn more about PPHN
Phosphodiesterase (PDE-5) inhibitors
A type of medication that inhibits (blocks) the enzyme phosphodiesterase (PDE-5). PDE-5 breaks down a molecule called cyclic GMP (cGMP). cGMP is the “messenger” of nitric oxide (NO) that causes vasodilation (opening up of the blood vessels). Blocking the breakdown of cGMP then leads to more NO effects. These medications, including sildenafil (Viagra®), tadalafil (Cialis®) and vardenafil (Levitra®), were first used to treat erectile dysfunction. Brand name drugs Revatio® (sildenafil) and Adcirca® (tadalafil) are the only PDE-5 mediations specifically approved for the treatment of PAH. Read more about treatment options
Portopulmonary hypertension
PH that develops due to high blood pressure in the liver. Usually caused by cirrhosis (scarring). Read more about PH and liver disease
Primary pulmonary hypertension
Old classification for PH without a known cause. See also idiopathic pulmonary arterial hypertension
Prostacyclin
A substance made by our bodies that helps regulate blood vessel “tone” by dilating the blood vessels. It is particularly active in the blood vessels of the lungs and is found to be in very low amounts in patients with PH. It is because of this fact that scientists first thought that the addition of man-made prostacyclin (epoprostenol) to the blood by infusion or inhalation could result in vasodilation of the pulmonary arteries. They were correct! The study of epoprostenol infusion for PH in the 1980s and early 1990s proved helpful to PH patients and led to its approval in 1995. As it turns out, in addition to being a vasodilator, prostacyclin may inhibit the growth and thickening of the pulmonary vessel walls, and make platelets less “sticky,” both very good things for treating PH. You may not know … The name prostacyclin comes from the prostate gland because prostaglandin was first isolated from seminal fluid and was thought to have been made by the prostate! Read a brief note about blood vessels
Prostacyclin analogues / Prostanoids
Prostanoids are a family of chemicals normally made in the body that help regulate blood vessel tone (see above). Members of the prostanoid family of drugs are chemically very similar to one another (they are analogs). Prostacyclin is a type of prostanoid. Present prostacyclin analogs (commonly referred to as prostanoids) include epoprostenol sodium (Flolan®), treprostinil sodium (Remodulin®), generic epoprostenol, room-temperature stable epoprostenol (Veletri®), iloprost (Ventavis®), and inhaled treprostinil (Tyvaso®). These prostacyclin analogs help many PH patients by dilating blood vessels, reducing clotting, slowing down the growth of smooth muscle cells, and improving cardiac output. Although they are all in the same class of drugs, chemically they are not identical, and their toxicity, side effects and effectiveness may differ. Read a brief note about blood vessels
Pulmonary
Relating to the lungs.
Pulmonary angiogram
An x-ray of the blood vessels injected with dye that highlights any blockages. Read more about common tests in PH
Pulmonary artery
The blood vessels carrying blood from the right ventricle of the heart to the lungs, where the blood is oxygenated. It starts out as the main pulmonary artery, then branches into the right and left pulmonary arteries feeding the right and left lungs, respectively, then continues to branch into smaller and smaller blood vessels, eventually becoming capillaries that exchange oxygen (O2) and carbon dioxide (CO2)with the alveoli (air sacs).
Pulmonary artery pressure (PAP)
A measurement of the blood pressure in the pulmonary artery. It has a systolic (upper) and diastolic (lower) component. This is written like a traditional blood pressure measurement. An example for PH is a pulmonary artery pressure 90/40. See mean pulmonary artery pressure. See also mean pulmonary artery pressure
Pulmonary arterial hypertension (PAH)
Pulmonary arterial hypertension (PAH) is more specific than the general term “pulmonary hypertension” (see pulmonary hypertension below). PAH is high blood pressure in the pulmonary arteries from disease of the medium- and small-sized vessels of the lung, causing them to narrow and decrease blood flow. Read more about types of PH
Pulmonary artery wedge pressure
A measurement obtained during right heart catheterization (see also cardiac catheterization). A balloon on the tip of the right heart catheter is inflated (wedged) in the pulmonary artery where it can also estimate pulmonary vein pressures.
Pulmonary function tests (PFTs)
A series of tests to find out 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. The tests can help diagnose some conditions that cause PH. Read more about diagnostic tests
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 PH
Pulmonary hypertension gene: See BMPR2 gene. Others are yet be discovered.
Pulmonary thromboendarterectomy (PTE)
A surgical procedure to remove a blood clot (or clots) in the pulmonary arteries in the lungs.
Pulmonary vascular resistance (PVR)
A measure of how difficult it is for the heart to pump blood through the lungs. PVR is a calculation obtained during cardiac catheterization. The PVR is often very high in patients with pulmonary hypertension.
Pulmonary vein
The blood vessel returning oxygenated blood from the lungs back to the left side of the heart.
Pulmonary venous hypertension
A condition in which blood flow through the left side of the heart is decreased, causing a backup of blood in the pulmonary veins and therefore causing higher pulmonary vein and pulmonary artery pressure. This cause of this disease is not in the pulmonary arteries, and therefore is not usually treated with drugs designed to treat PAH.
R
Raynaud’s phenomenon
When fingers get blue and cold easily, and sometimes painful because of blood vessel spasms. Persons with PAH can also have Raynaud’s phenomenon. It is also associated with autoimmune disorders. Learn more about Raynaud’s phenomenon
Remodulin®
An FDA-approved intravenous or subcutaneous treatment for PAH. Remodulin® is the brand name for the intravenous and subcutaneous forms of treprostinil. See also treprostinil | Read more about Remodulin®
Revatio®
An FDA-approved oral treatment for PAH. Revatio® is the brand name for sildenafil. See also sildenafil | Read more about Revatio®
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.
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 PH patients, this chamber is often enlarged, its walls are thickened, and its ability to contract is reduced.
S
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. Read more about scleroderma and PH
Secondary pulmonary hypertension (SPH)
The old classification for pulmonary hypertension due to 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 the abnormally-shaped red blood cells cannot flow through the arteries and veins easily, severe joint pain, chest pain, pulmonary hypertension, and other complications may result. Patients with sickle cell anemia require frequent blood transfusions and pain medications. Read more about sickle cell anemia and pulmonary hypertension
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. Read more about common tests in PH
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 for treating 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
T
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
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
V
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
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.
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 congenital heart disease and Eisenmenger syndrome. Learn 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.
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.)
W
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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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
Y
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