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Common Terms and Acronyms Used with PH
Angina
Chest pain that originates in the heart.
Aorta
Blood vessel that delivers oxygen rich blood from the left ventricle to the body; it is the largest blood vessel in the body.
Arterial blood gas - ABG
A blood test that measures the concentrations of oxygen and carbon dioxide in the arterial blood. Usually, the blood sample is taken from the radial artery at the wrist.
Atrial fibrillation & a extremely rapidly, thus causing the ventricles to contract faster and less efficientltrial flutter
Atrial fibrillation and atrial flutter are very fast electrical discharge patterns that make the atrial chamber of the heart contracty than normal. In fibrillation, the atrial rhythm is irregular, so the ventricular rhythm is also irregular; in flutter, the atrial and ventricular rhythms usually are regular.
Atrium
One of the two receiving chambers of the heart. The left atrium receives oxygen-rich blood from the lungs. The right atrium receives oxygen-depleted blood from the body.
Calcium Channel Blocker - CCB
A treatment for PH; tablets.
Cardiac output
Total amount of blood being pumped by the heart over a particular period of time.
Catheter
Thin flexible tube.
Catheterization
Use of catheter to study heart and lung function. Pictures, blood samples, and pressures are recorded.
Central line catheter
A surgically implanted line that goes directly into the heart. The end, which remains outside the body, is connected to IV medication lines.
Clinical trials
Medical studies of patients that evaluate the effectiveness of treatments.
Constrict
Tighten; narrow.
CREST syndrome
A less severe form of scleroderma, named for its symptoms: calcium deposits in the skin and throughout the body, Raynaud's phenomenon, esophageal dysfunction, sclerodactylly (skin damage on the fingers) and telanggieectasia (spider veins). People who have CREST syndrome can develop pulmonary hypertension.
Cyanosis
A bluish color in the skin because of lack of adequate oxygen.
Diastolic pressure
The lowest pressure to which blood pressure falls between contractions of the ventricles.
Dilate
Relax; expand.
Diluent
A sterile solution used for reconstituting Flolan powder.
Dual ERA
An endothelin receptor antagonist (ERA) that blocks both the ET-A and ET- B endothelin receptors
Dyspnea
Difficulty breathing, or shortness of breath.
Echocardiogram
A non-invasive diagnostic procedure using ultrasound waves to study the heart. It is used to assess disorders of cardiac muscle function or valve function, or other abnormalities such as elevated pulmonary pressure.
Endothelial cells
Lining of organs of blood flow.
Endothelin
A chemical produced naturally by the body. In healthy amounts, it plays an important role in regulating blood flow. In conditions such as Pulmonary Arterial Hypertension (PAH), excess endothelin is produced, tightening and narrowing blood vessels and affecting the blood pressure in the lungs.
Endothelin receptor antagonist (ERA)
A new class of drug that blocks endothelin receptors and limits the harmful effects of excess endothelin in PAH. There are two main kinds of ERAs: selective and dual ERAs.
Endothelin receptors
In order to have an effect, endothelin must connect with an endothelin receptor. There are two main endothelin receptors: ET-A and ET-B. Endothelin receptor antagonists (ERAs) work by blocking one or both of the endothelin receptors. It seems clear that ET-A tightens and narrows blood vessels, but the role of ET-B is a matter of debate among researchers.
Endothelium
The thin layer of cells that lines blood vessels. It is the primary producer
of endothelin.
Edema
Swelling due to too much fluid.
FDA
U.S. Food and Drug Administration; the regulatory agency which approves new drugs for use.
Fibrosis
Scarring of tissue, making it stiffer and not as functional as normal tissue.
Flolan
A treatment for PH; continuous infusion of prostacyclin via battery operated pump.
Heartbeat
One pump of the heart.
Hyperactive
Excessive reaction of tissue.
Hypertension
Abnormally high pressure.
Hypotension
Abnormally low pressure.
Hypoxia
Inadequate amounts of available oxygen in the blood
INR
International Normalization Ratio value. A standardized measure of blood clotting time/ratio. Prothrombin time blood test results are reported in seconds and can vary depending upon the reagent used; the INR result is independent of the reagents used and is therefore a more uniform test result. Therapeutic INR is usually considered to be 2.0 to 3.5 in most institutions.
Lung volume
The amount of air the lungs hold.
Mean blood pressure
The average blood pressure.
NYHA Class I - IV
New York Heart Association functional classification. An assessment of physical limitations and symptoms of patients with heart disease. This classification system has been modified slightly by the WHO World Symposium on PH to describe pulmonary hypertension patients. Briefly stated:
- Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.
- Class II: slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.
- Class III: marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.
- Class IV: inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.
Nuclear testing
Use of radioactive medication to trace the blood flow- usually done with very low level of radiation exposure (equal to chest x ray).
O2
Oxygen
Oximeter
A device for measuring or monitoring oxygen concentration in the blood using an electrode placed on a finger or an earlobe--a procedure called oximetry.
Oxygen saturation
The amount of available oxygen in the blood. The percentage of oxygen concentration is referred to as O2 Sats.
PCP
Primary Care Physician
PH Specialist
A physician, usually a cardiologist or pulmonologist, who has diagnosed and treated a large number of PH patients. A PH specialist is familiar with current diagnostic methods and treatments for PH.
Palpitation
The sensation of rapid heartbeats.
Perfusion
Flow of liquid through a network of vessels or tissue.
Primary pulmonary hypertension - PPH
Unexplained cases with no known cause (idiopathic).
Prostacyclin
A synthetic form of prostaglandin. It is also known as epoprostenol. Flolan and Remodulin are two forms of prostacyclin.
Prostaglandin
A steroid that is produced naturally in the body in a normally healthy person. It causes blood vessels in the lungs to relax and allow blood to flow through them more easily.
Pulmonary
Medical term for the lungs.
Pulmonary arterial hypertension - PAH
Pulmonary arterial hypertension (PAH): One of five categories of pulmonary hypertension; this category includes PH of unknown origin. Most kinds of pulmonary hypertension are PAH. PAH constricts or contracts the arterioles of the lungs, which are small, muscular branches of arteries within the lungs. When they are constricted, they increase resistance to blood flow, and blood pressure in the pulmonary artery increases.
Pulmonary artery - PA
Blood vessel delivering blood to the lung from the right side of the heart.
Pulmonary Artery Pressure – PAP
Blood pressure in the artery carrying blood from the heart to the lungs.
Pulmonary Edema
Fluid in the lung(s)
Pulmonary Function Test – PFT
A group of tests to measure 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)
Abnormally high blood pressure in the lungs. The small vessels that supply blood to the lungs constrict, making it harder for blood to get to the lungs and forcing the heart to work harder. This term includes PAH and all other kinds of pulmonary hypertension, such as PH associated with disorders of the respiratory system, due to chronic thrombotic or embolic disease, or due to disorders directly affecting the pulmonary blood vessels.
Pulse oximeter
A fingertip device for measuring oxygen concentration in the blood.
Remodulin
A synthetic, stable form of prostacyclin that is administered under the skin rather than into the bloodstream.
Right Heart Catheterizations - RHC
Use of catheter to study heart and lung function. Pictures, blood samples, and pressures are recorded. Considered the "gold standard" for diagnosis of pulmonary hypertension.
Secondary pulmonary hypertension - SPH
Cases that result from another disease that elevates the pressure in the arteries of the lungs.
Selective ERA
An endothelin receptor antagonist (ERA) that blocks only the ET-A endothelin receptor. ET-A plays a significant role in the constricting of blood vessels.
Sitaxsentan
An oral endothelin receptor antagonist (ERA) currently in clinical trials. It is a selective ERA.
Sleep apnea
A condition in which the patient has short periods of not breathing during sleep.
Smooth muscle
Usually the middle layer of an artery, which contains elastic and performs automatic tasks, such as tightening blood vessels.
SOB
Short of Breath.
Specialty pharmacy distributor
Providers of pharmaceutical services for patients with chronic diseases or genetic disorders that require high-cost, complex therapies.
Subcutaneous infusion
A drug treatment delivery method that infuses the drug under the skin by means of a small pump. Remodulin is delivered in this manner.
Syncope
Fainting, temporary loss of wakefulness.
Systemic
Affects all of the body.
Systolic pressure
The highest pressure to which blood pressure rises with pumping of the heart.
Tachycardia
An abnormally rapid and uneven heartbeat
Targeted ERAs
An endothelin receptor antagonist (ERA) that blocks only the ET-A endothelin receptor. ET-A plays a significant role in the constricting of blood vessels.
Thoracentesis
A procedure in which pleural effusion (fluid that has collected abnormally in the space around the lungs) is removed with a needle and syringe, so it can be analyzed, and also to relieve shortness of breath caused by lung tissue compression.
Tracleer (formerly Bosentan)
The first oral endothelin receptor antagonist (ERA) approved for use in PAH patients with WHO Class III or IV symptoms. It is a dual ERA.
Trismus
Spasms of the jaw muscles; difficulty opening the mouth.
UT-15
The name of Remodulin while it was in clinical trials.
Vasoconstrictor
Something (drug or action) that narrows and tightens the blood vessels.
Vasodilator
Something (drug or action) that widens and relaxes the blood vessels.
Ventricle
One of the two pumping chambers of the heart. The right ventricle receives oxygen -poor blood from the right atrium and pumps it to the body through the aorta.
Viagra (Sildenafil)
An oral drug treatment for PH in clinical trials in Sydney, Australia. It is hoped that Viagra will increase the capacity to produce nitric oxide, which decreases the elevated pulmonary artery pressure minimizing the symptoms associated with PAH.
WHO Classification I - IV
See NYHA Class I-IV.
This glossary has been compiled from information provided by the National Heart Lung and Blood Institute, Mosby's Diagnostic and Laboratory Test Reference, Barron's Dictionary of Medical Terms and Merck Manual Home Edition. - Lynn Z. Schaeffer
The information in this page was last updated 09/09/2002
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