Definitions

Allergic reaction: An inappropriate overreaction of the body’s immune system.

Ambrisentan (Volibris): A drug used to treat some forms of PH. Ambrisentan is an endothelin receptor antagonist (ERA).

Anaemia: A condition where there are too few red blood cells (the cells that carry oxygen) in the bloodstream. This reduces the amount of oxygen that can be carried by the blood.

Angina: Chest pain that starts in the heart due to poor blood supply to the heart itself. This normally feels like an oppressive, heavy, crushing pain or a constricting feeling in the centre of the chest. It can spread to the arms, more commonly the left arm. It can sometimes be felt in the throat, jaw, or more rarely, between the shoulder blades.

Anticoagulant: A drug used to reduce the ability of the blood to clot. Warfarin is an anticoagulant that is used to treat some people with PH.

Aorta: A large blood vessel (artery) running directly from the heart. The aorta delivers oxygen-rich blood from the left ventricle of the heart to the body.

Arrhythmia: An arrhythmia is a disturbance in the normal rhythm of the heart. The heart normally beats between sixty and eighty times a minute. Arrhythmia is when the heart rate becomes abnormally rapid, slow and/or irregular.

Arterial blood gas test: A test that measures the amount of oxygen and carbon dioxide in the blood in the arteries. This blood sample is normally taken from an artery in the wrist.

Artery: A blood vessel that carries blood away from the heart. Apart from the pulmonary artery, arteries carry oxygen-rich blood to the cells, tissues and organs of the body.

Asthma: A common condition causing inflammation of the airways. Symptoms include wheezing, coughing, chest tightness and shortness of breath. Many people with PH are initially incorrectly diagnosed with asthma.

Atria: The upper chambers in the heart. Atria is the plural of atrium.

Atrial fibrillation: Rapid, irregular contractions of the atria.

Atrium: One of the two upper chambers of the heart that receives blood. The left atrium receives oxygen-rich blood from the lungs. The right atrium receives oxygen-poor blood from the body.

Blood clots: Lumps of blood that stick together (like a scab) inside the blood vessels. In some people, these can move through the circulatory system and stick in the blood vessels of the lungs, causing partial blockages.

Blood pressure: The pressure that the blood is under as it is pumped around the body. Blood pressure can be different in different parts of the body.

Blood pressure monitor: A device that checks your blood pressure.

Blood vessel: Something that blood passes through, such as a vein or artery.

Bosentan (Tracleer): A drug used to treat some forms of PH. Bosentan is an endothelin receptor antagonist (ERA).

Brand names: Names for drugs that are used by drug companies. For example, the common painkiller Nurofen is a brand name for a product containing the drug ibuprofen. In PH, many drugs have a generic name (eg bosentan), and a brand name (eg Tracleer).

Calcium channel blocker (CCB): A type of drug that is sometimes used in PH. CCBs are helpful for about 10% of people with PH.

Cardiac output: The total amount of blood being pumped by the heart over a particular period of time.

Catheter/Cardiac catheter: A thin, flexible tube. In PH, catheters can be guided through the arteries to take measurements of the heart and pulmonary artery.

Catheterisation: Use of a catheter to study how the heart and lungs are working. Catheterisation allows pictures, blood samples and pressures to be recorded.

Chronic blood clots: Blood clots that do not dissolve by themselves or with drug therapy.

Chronic obstructive pulmonary disease (COPD): A collection of lung diseases that occur together and prevent the lungs working properly.

Circulatory system: The system of blood vessels and the heart that together moves blood throughout the body.

Claustrophobia: A fear of being in enclosed spaces.

Clinical trials: Medical studies that assess how effective specific treatments are. Also called clinical studies.

Combined pill: A contraceptive pill containing two hormones, one of which is normally oestrogen. Oestrogen can make PH worse, so these pills should be avoided in women with PH.

Congenital heart disease: A heart condition that is present at birth. Sometimes, congenital heart disease will not be found out until later in a child’s life, sometimes at the time of being diagnosed with PH.

Connective tissue disease: Any of many conditions where the connective tissues are attacked. Connective tissues are the framework that link together things like bone, organs and skin. Many connective tissue diseases involve an abnormal immune response.

Constrict: To tighten or narrow. Normally used to describe blood vessels.

Continuous infusion: A method of administering a drug directly into the bloodstream. In a continuous infusion, small amounts of the drug are given constantly over time via a catheter.

CT/CAT scan: A CT (computerised tomography) or CAT (computed axial tomography) scan is a special type of X-ray. It can give very clear pictures of the inside of your body, especially ‘soft’ tissues like the lungs, which do not show on ordinary X-ray pictures.

Cyanosis: A bluish colour in the skin caused by lack of oxygen in the blood. This is often seen in the lips and fingernail beds of people with PH. It can become more noticeable after exercise.

Diagnosis: Identifying a disease or condition, such as PH.

Diastolic pressure: The lowest pressure to which blood pressure falls between contractions of the ventricles (the lower chambers in the heart).

Dilate: To relax or expand. Normally used to describe blood vessels.

Diuretic: A drug that increases the amount of water that you pass out from your kidneys. They are sometimes called ‘water tablets’ as they cause an increase in urine.

Down’s syndrome: A genetic condition causing mental retardation and characteristic flat facial features. It is also associated with other conditions such as heart defects.

Dyspnoea: Difficulty in breathing, or shortness of breath, either while resting or when doing exercise.

Echocardiogram (Echo): An ultrasound scan of the heart. An ‘echo’ can give accurate pictures of the heart muscle, the heart chambers and structures within the heart such as the valves.

Eisenmenger syndrome: A condition caused by an opening in the central wall of the heart that divides the right side from the left. This is a possible consequence of the congenital heart disorder known as ‘hole in the heart’.

Electrocardiogram (ECG): A simple test used in the diagnosis of PH that records electrical activity of the heart.

Emphysema: A disorder where the tiny air sacs in the lungs become damaged. People with emphysema have a reduced ability to get oxygen from the air, so often feel breathless.

Endothelin: A chemical produced naturally by the body. In normal amounts, it plays an important role in controlling blood flow. In conditions such as PH, too much endothelin is produced, which tightens and narrows blood vessels and affects the blood pressure in the lungs.

Endothelin receptor antagonist (ERA): A class of drug that blocks endothelin receptors and therefore limits the harmful effects of excess endothelin in people with PH.

Enzyme: A type of molecule in the body, which has a specific function. Some enzymes involved in controlling blood vessel constriction (such as PDE5) can be targeted by drugs used to treat PH.

Epoprostenol (Flolan): A prostaglandin treatment for PH. Epoprostenol is often given as a continuous infusion via a Hickman line by a small battery-powered pump.

Fatigue: Tiredness or lethargy. This can be extremely hard to define, since everybody has their own idea of what being tired means.

Fibrosis: Scarring of tissue, such as in the lungs. Fibrosis makes tissues stiffer and less functional than normal tissue.

Fluid retention: A failure to get rid of excess water from the body.

Generic name: The name for a drug that is not the brand name. For example, ibuprofen is the generic name of the drug whose brand name is Nurofen. In PH, many drugs have a generic name (eg bosentan), and a brand name (eg Tracleer). However, some drugs only have a generic name (eg, warfarin).

Groshong line: A type of catheter sometimes used instead of a Hickman line. Both catheters are very similar.

Heart failure: A condition where the heart loses its ability to pump blood efficiently throughout the body.

Heart rate: The speed at which the heart beats. This is normally expressed as a number per minute.

Hickman line: A catheter that is inserted directly into a blood vessel in order to supply a drug over a long period of time. In PH this drug is either epoprostenol or iloprost. The end of the Hickman line is placed such that its tip lies within the right atrium, the superior vena cava or at the junction between them.

HIV (human immunodeficiency virus): The virus that causes AIDS. HIV infection increases the risk of a person developing PH.

Hormonal contraception: Female contraceptives that rely on hormones (such as oestrogen) to work. These are normally pills.

Hypertension (systemic): Abnormally high blood pressure in the body, not the lungs. Hypertension occurs when blood is forced through the arteries at an increased pressure. Around 10 million people in the UK have high blood pressure – that’s 1 in 5 of us.

Hypotension (systemic): Abnormally low blood pressure. Low blood pressure tends only to be a problem if it causes symptoms such as fainting, light-headedness and fatigue, or if it relates to an underlying condition that needs monitoring and treatment. It is not uncommon for healthy people to have low blood pressure without any related problems as blood pressure varies enormously from person to person.

Hypoxia: Inadequate amounts of oxygen in the blood.

Idiopathic pulmonary hypertension (IPH): PH with no known cause. Until recently this was called primary pulmonary hypertension.

Iloprost (Ventavis): A prostaglandin treatment for PH. Iloprost is often inhaled using a nebuliser. It can also be given as a continuous infusion by a small battery powered pump.

Immune response: The body’s way of fighting disease. In some disorders, an immune response can be triggered inappropriately against part of the body that is healthy.

Immune system: The system that is normally involved in fighting disease. The immune system recognises things that are not part of the body. This leads to those things (such as bacteria and viruses) being attacked by the molecules that make up the immune system.

Immunosuppression: Suppression of the body’s immune system and its ability to fight infections or disease. Immunosuppression may be deliberately induced with drugs in preparation for bone marrow or other organ transplantation. This is to prevent its rejection by the recipient’s immune system.

International normalised ratio (INR): A standard measure of blood clotting time. Individuals on warfarin or other forms anticoagulants will have this blood test regularly to check the INR is within a desirable range.

Intravenous: A way to administer a drug by injecting it directly into a vein.

Jaundice: Yellow-coloured tinge to the skin and whites of the eyes.

Kidney function test: A blood test to check that the kidneys are working properly. It may be done as part of a general health assessment, or before and after starting treatment with certain medicines. If you have any degree of kidney failure, your medicines and/or doses may need altering.

Liver function tests: A blood test to check the liver is working properly. Some drugs that are used to treat PH can affect the function of the liver, as can PH itself.

Liver toxicity: Damage to the liver. This is a side effect of some drugs used to treat PH.

Lung fibrosis: A disease (also called idiopathic pulmonary fibrosis) that is caused by the reaction of the body to foreign elements. The air sacs in the lungs become inflamed and scars develop on the lung tissue as it attempts to repair itself.

Lung volume: The amount of air the lungs can hold.

Malaise: The feeling of being generally unwell, run down or out-of-sorts.

Mean: An average. For example, the mean blood pressure is the average blood pressure from a number of assessments.

Mitral regurgitation: Leakage of blood back through the mitral valve in the heart. This is caused by the valve not closing properly. Mitral regurgitation increases the pressure in the left atrium and in the blood vessels coming from the lungs. This may lead to various problems and symptoms, depending on the severity of the leak.

Mitral valve: Valve located in the heart between the left atrium and left ventricle that regulates blood flow between these chambers.

MRI scan: MRI stands for magnetic resonance imaging. An MRI scan uses a strong magnetic field and radio waves to create computer images of tissues, organs and structures inside your body. An MRI scan is a safe and painless test.

Nasopharyngitis: Inflammation of the airways in and around the nose.

Nebuliser: A device used to administer medication into the airways in a liquid mist form. It is very commonly used in lung diseases.

O₂: The chemical symbol for oxygen.

Oedema: Swelling caused by excess fluid in the body (often in the ankles and lower legs).

Oestrogen: A female sex hormone that is often used in contraceptive pills. Oestrogen can make PH worse, so should be avoided in women with PH.

Off-label: Use of a drug when it has not been formally licensed, or when it is intended for another medical condition. Unlike with many other conditions, because PH is rare, some drugs can be used off-label if your PH team think you will benefit from them.

Organ rejection: The process where the body’s immune system attacks an organ transplanted from someone else. If organ rejection is not properly managed with drugs, this can lead to the organ stopping working.

Oximeter: A device for monitoring the amount of oxygen in the blood.

Oxygen-poor blood: Blood that is returning to the heart and has had most of the oxygen removed. Oxygen is removed by other organs and muscles throughout the body to be used to make energy.

Oxygen-rich blood: Blood that has a lot of oxygen dissolved in it. This is normally blood returning from the lungs and being pumped to the rest of the body by the heart.

Oxygen saturation: The amount of oxygen in the blood.

Oxygen therapy: Supplementary oxygen often given to PH patients who don’t have enough oxygen in their blood. It is obtained from compressed oxygen cylinders or from an oxygen concentrator machine, which extracts oxygen from the air.

Palpitation: Palpitations are an awareness of your own heartbeat, usually because the heartbeat seems irregular, unusually rapid or in some other way different to normal. An abnormal heart rhythm is referred to as an arrhythmia.

Perfusion: Flow of liquid through a network of blood vessels or tissue.

Placebo: A dummy drug containing no active medicine.

Portal hypertension: High blood pressure in the vein that carries blood to the liver. This is often caused by liver disorders.

Progesterone: A female sex hormone.

Prognosis: A prediction of the likely outcome of a condition.

Prostacyclin: A molecule involved in controlling whether blood vessels dilate or constrict. Synthetic (man-made) forms can be used to treat PH. These include epoprostenol, iloprost and treprostinil.

Pulmonary: Medical term for anything relating to the lungs.

Pulmonary angiography: A test used to see whether there are blood clots in the lungs.

Pulmonary arterial hypertension (PAH): The most common form of PH. The term includes many subtypes of PH. These includes PH with no known cause (idiopathic PH), and PH that is passed from generation to generation.

Pulmonary artery: The blood vessel delivering blood to the lungs from the right side of the heart.

Pulmonary artery pressure: The blood pressure in the pulmonary artery.

Pulmonary emboli: Blood clots that move through the bloodstream and get stuck in the blood vessels of the lungs.

Pulmonary endarterectomy (PEA): An operation to remove old blood clots from the pulmonary arteries in the lungs.

Pulmonary function test: A group of tests to measure how well the lungs are working. This includes the lungs’ capacity to hold air as well as their ability to move air in and out, and to exchange oxygen and carbon dioxide.

Pulmonary hypertension (PH): A rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels. At the same time as the pressure rises, the walls of the blood vessels become thicker. PH can occur with or without an identifiable or known cause.

Pulse oximeter: A device that attaches to your fingertip, used for measuring the amount of oxygen in the blood.

Quality of life: The ability to enjoy a normal life. This can be hard to define, and is different for different people.

Radiation: Something given off from radioactive substances. In high doses, radiation can be dangerous. In low doses, radiation is routinely used in several medical tests, including X-rays.

Radioactive: Something that gives off radiation. In some medical tests, radioactive dyes are injected into parts of the body. This is to allow doctors to look at internal structures, such as the blood vessels in the lungs.

Radiographer: A medically trained person who performs scans such as MRI scans, CT scans and X-rays.

Radiography: Using X-rays to see things inside the body, such as the heart and lungs.

Radiologist: A doctor who specialises in tests such as MRI scans, CT scans and X-rays.

Raynaud’s phenomenon: A disorder affecting the small arteries supplying the fingers and toes with blood. Exposure to cold causes them to constrict more than normal, so blood-flow into the fingers and toes is restricted.

Right heart catheterisation (RHC): Use of a catheter to study heart and lung function. Pictures, blood samples and pressures are recorded. Considered the ‘gold standard’ for diagnosing PH.

6-minute walk test: A variation of the shuttle walk test. This test, assesses how far you can walk in 6 minutes. It is used to see how much your PH prevents you doing normal exercise.

Shuttle walk test: A test to see how much your PH is stopping you being able to exercise. Normally this is done by seeing how many times you can walk up and down a corridor in a certain amount of time.

Sickle cell disease: A disease where the red blood cells (the cells that carry oxygen) are defective. Sickle cell disease is an inherited disease almost exclusively found in black people of African descent. People with sickle cell disease are more likely to get PH.

Sildenafil (Viagra/Revatio): A drug used to treat some forms of PH. Sildenafil is a phosphodiesterase type-5 (PDE5) inhibitor.

Sleep apnoea: A condition in which the individual’s breathing stops for a short time during sleep. In obstructive sleep apnoea (OSA), the throat is sucked closed during sleep. OSA occurs in 1 in 25 middle-aged people.

Subcutaneous infusion: A drug delivery method that administers the drug under the skin using a small pump. Treprostinil is delivered in this manner.

Symptoms: The noticeable effects of a disease or condition. For example, symptoms of PH include breathlessness and tiredness.

Syncope: Fainting or losing consciousness for a moment. People normally recover straightaway.

Systemic: Relating to the circulation system (the blood system). Affecting all of the body.

Systemic lupus: A disease where a person’s immune system attacks parts of their body. This can affect the skin, joints and internal organs. People with systemic lupus are more likely to get PH.

Systemic sclerosis: A disease involving thickening of the skin and tissues in and around internal organs. More than 1 in 10 people with systemic sclerosis develop PH.

Systolic pressure: The highest blood pressure achieved with the pumping of the heart.

Tadalafil (Adcirca): A drug used to treat some forms of PH. Tadalafil is a phosphodiesterase type-5 (PDE5) inhibitor.

Transducer: A device that is used to do an echocardiogram. It emits and detects ultrasound to allow moving images of internal organs to be seen.

Transplantation: The process of moving organ(s) from one person’s body to another. For organs such as lungs and hearts, the donor is always a person who has recently died.

Treprostinil (Remodulin): A drug used to treat PH. Treprostinil is a synthetic (man-made) form of prostaglandin. It is administered under the skin (subcutaneous infusion) via a very small battery-operated pump.

Ultrasound: A very high frequency sound that you cannot hear. It is used to do an echocardiogram.

Vasoconstrictor: Something that causes the blood vessels to narrow or tighten.

Vasodilator: Something that causes the blood vessels to widen or relax.

Vein: A blood vessel that carries oxygen-poor blood back to the heart.

Vena cava: Either of two large veins that return oxygen-poor blood from the body into the right atrium of the heart.

Ventilation-perfusion (V/Q) scanning: A test often used to see if there are blood clots in the lungs. Chronic blood clots in the lungs may be a cause of PH.

Ventricle: A pumping chamber of the heart. There are two ventricles in the heart. The right ventricle pumps oxygen-poor blood to the lungs. The left ventricle pumps oxygen-rich blood to the body.

Warfarin: A drug that makes the blood less likely to clot (an anticoagulant). This is taken by people who are at risk of developing blood clots.

X-ray: High-energy radiation that is used to produce images of certain parts of the body. X-ray images allow doctors to see dense parts of the body, such as bones and the heart.

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No definitions