Medical Issues



PH Glossary

Common Terms and Acronyms Used with PH

Adcirca® (Tadalafil)
A once-a-day oral medication for the treatment of Pulmonary Arterial Hypertension (PAH).

Ambrisentan (Letaris™)
An endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH).

Ampule

A small, sealed vial commonly made of glass or plastic that contains a drug.

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.

Arrhythmia
A term for any of a large and heterogenous group of conditions in which there is abnormal electrical activity in the heart.

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 & atrial flutter
Atrial fibrillation and atrial flutter are very fast electrical discharge patterns that make the atrial chamber of the heart contract extremely rapidly, thus causing the ventricles to contract faster and less efficiently 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.

Atrial Septal Defect (ASD)
A heart defect in which The wall between the two entry chambers of the heart (the atria) does not finish forming.

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.

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BiPAP - Bi-level Positive Air Pressure
This is a non-invasive ventilator for people with sleep disorders that make it difficult for them to maintain oxygen saturation levels during sleep. Often prescribed for those with central sleep apnea or muscle weakness.

C-PAP - Continous Positive Air Pressure
This is a non-invasive ventilator for people with obstructive sleep apnea. The C-PAP forces their airways to remain open during sleep by providing a constant flow of forced air into the lungs.

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.

Central Sleep Apnea
A sleep disorder in which the brain does not tell the lungs to breathe regularly during sleep. Patients with CSA may be prescribed a BiPAP ventilator.

Clinical trials
Medical studies of patients that evaluate the effectiveness of treatments.

Constrict
Tighten; narrow.

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COPD
Chronic Obstructive Pulmonary Disease

CREST syndrome
A less severe form of scleroderma, named for it's 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.

Edema
Swelling due to too much fluid.

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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 (ETRA)
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.

EPAP (exhalation positive air pressure)
A pressure setting on a BiPAP for breath exhalation.

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.

Gas Exchange
The exchange of co2 for oxygen that takes place in the lungs. In people with PH and others with diseases that cause vascular changeds in the lungs, this may be diminished over time.

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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.

IPAP (inhalation positive air pressure)
A pressure setting on a BiPAP for breath inhalation.

Lung volume
The amount of air the lungs hold.

Mean blood pressure
The average blood pressure.

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).

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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.

O2
Oxygen

Obstructive Sleep Apnea
A sleep disorder in which fatty tissue in the neck blocks the flow of air into the lungs during sleep. People with OSA may be prescribed a CPAP ventilator.

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.

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.

Palpitation
The sensation of rapid heartbeats.

Palpitation
The sensation of rapid heartbeats.

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.

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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
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 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.

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Remodulin™

A synthetic, stable form of prostacyclin that is administered under the skin rather than into the bloodstream.

Revatio (Sildenafil)
An oral drug used in the treatment for PH. Often prescribed under the trademark name Revatio&tm;.

RHC - Right Heart Catheterizations
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.

Scleroderma
An autoimmune disease sometimes associated with PH.

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.

Sildenafil (Revatio™, Viagra™)
An oral drug often used in the treatment for PH. Often prescribed under the trademark name Revatio™.

Sitaxsentan

An oral endothelin receptor antagonist (ERA) currently in clinical trials. It is a selective ERA.

Sleep Apnea
A condition in which the patient stops breathing for short periods during sleep. May be either Obstructive or Central.

Sleep Mask
A device used with either the BiPAP or the CPAP to deliver oxygen through the nose

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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.

Tadalafil (Adcirca®)
A once-a-day oral medication for the treatment of Pulmonary Arterial Hypertension (PAH).

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.

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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.

Treprostinil

The generic name for a synthetic, stable form of prostacyclin produced by United Therapeutics, Inc.

UT-15
The name of Remodulin while it was in clinical trials.

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.

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.

Ventricular Septal Defect (VSD)
A heart defect in which the wall between the two largest chambers of the heart (the ventricles) does not finish forming properly.

Viagra™ (Sildenafil)
An oral drug often used in the treatment for PH. Often prescribed under the trademark name Revatio™.

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