For more information about this disease go to:
You will find a wealth of information about PH as well as
input from patients and caregivers.
Another good source for PH information
To insure good health: Eat lightly, breathe deeply, live moderately, cultivate cheerfulness, and maintain an interest in life.
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Always ask your doctor if you are concerned about or don't understand the testing or numbers of your reports.
This is information only and should be used only
as a guide.
For Articles on Research and Bio Med interests Dr. Don an informative web site. (He posted my poem)
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FYI~The average total lung capacity is about 5.8 liters (5800 cm3), however it varies from one person to the next and is dependent upon factors such as weight, sex, age and activity.
Women tend to have 20 - 25% lower capacity than males. Tall people tend to have a greater total lung capacity than shorter people. Heavy smokers generally have a drastically lower total lung capacity than non-smokers. To find out if your lung capacity is "good" or "bad", you should talk to your doctor - preferably a pulmonologist.
Pharmaceutical Companies and Providers
(what they provide beyond the meds)
TEVA go to-Epoprostenol - Generic Flolan
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What do your ph pressures mean
PAP SYSTOLIC (Pulmonary Artery Pressure Systolic) represents the rapid blood flow from the right ventricle into the pulmonary artery. Occurs with the opening of the pulmonic valve. Normal: 15-30mmHg
PAP DIASTOLIC (Pulmonary Artery Pressure Diastolic)represents the diminished blood flow from the right ventricle into the pulmonary artery. Occurs with the closure of the pulmonic valve. Normal: 5-15mmHg
MPAP (Mean Pulmonary Artery Pressure) is calculated as follows:
(DPAP x 2) + SPAP
What is Pulmonary Hypertension ~ also known as Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension (PPH) <- or no known cause - PH or PAH (Pulmonary Arterial Hypertension). I will refer to it as PH. Often misunderstood; the severity of PH is still not recognized by many doctors nor insurance companies, employers or others who associate PH with common high blood pressure instead of a distinct and grave illness. Someone with high blood pressure can live 30 or 40 more years. Someone with pulmonary hypertension, which is similar but in the lungs, can live 2.8 years (??) That is without treatment. It is very doable with early diagnosis and treatment. Symptoms of PH may include: Shortness of breath with minimal exertion, chest pain, unusual fatigue, a dry cough, edema, heart palpitations, fainting and dizzy spells. The disease, described as "progressive and fatal", causes blood vessels in the lungs to thicken, restricting blood flow therefore making the right side of the heart overwork and lead to heart failure. It can be a silent killer but can also be treatable and therefore prolong your life. Studies show that most patients experience a one to two year delay between the onset of symptoms and a confirmed diagnosis of PH.
Symptoms range in severity and a given patient may not have all of the symptoms. PH may be secondary to other contributing diseases or there can be no known cause. Untreated, however, PH can have a worse prognosis than many forms of cancer. Did you know lung disease is the fourth leading cause of death in the U.S., responsible for one of every seven deaths?
Because of the rarity of this disease; most doctors do not know what it is. It can be present in all ages and does not discriminate with being a male or female or ethnic background but is most common in women of child bearing years.
People with PH before realizing what they have, often go to a doctor complaining of shortness of breath and unusual fatigue. If they are overweight or getting a bit older, their doctors may tell them to lose some weight and get more exercise. That is why it is often misdiagnosed or overlooked as a lung disorder or as a complication of a large number of respiratory or cardiac disease disorders. Remember, with proper treatment you can maintain a somewhat normal life and many have been known to go back to their regular routine. It is scary but it is also doable. It is a lung / heart disease so we need to take care of both. Various testing will be performed to eliminate other disorders and those are listed on the right (-->) BUT the gold standard of all tests is the right heart cathertization (RHC) this will let the doctor know your pulmonary pressures and what would be the best treatment for you. Some patients are on single therapy treatment some are on multiple medications. We are each unique in our treatment and we need to listen to what our bodies are telling us and in turn, relate that to our doctor. Some who are currently on medication can look the picture of health but inside they can come apart with worry. They worry about where their next breath will come from. They worry if they can maintain enough strength to live a proper and somewhat normal life. (Remember doable with treatment) If there should be some form of an emergency or accident, they worry will this doctor or nurse know what I have and how to treat it properly. Many PH patients cannot follow standard procedure for normal/standard ER care.
For years lung transplants were the only treatment for pulmonary arterial hypertension and just a few short years ago there was only one, then there were only three medications available for treatment. Today through research there are several available treatments and a number are still in trial. Those include:
Conventional Medical Treatments
-- ACE inhibitors - widen blood vessels. They do this by blocking the chemicals that cause blood vessel constriction.
--Beta Blockers - will block the effect of adrenaline so the heart then slows down, resulting in lower blood pressure.
-- Calcium Channel Blockers (CCBs)• widen blood vessels by slowing down the movement of calcium ions into cells. This relaxes the blood vessels and the heart muscle.
-- Digoxin - useful in treating atrial fibrillation, atrial flutter, and atrial tachycardia and symptoms of shortness of breath.
-- Diuretics - Water pill - helps the body get rid of extra water. Less fluid in the blood vessels decreases pressure.
-- Oxygen Therapy
-- Warfarin (Coumadin®) - blood thinner
Also and specific to PAH ~ a history
1996 - Epoprostenol (Flolan®) IV - a prostacyclin; widens the blood vessels in the lungs; lowers the blood pressure in the lungs (known as vasidilation action) ice packs needed 2002 - Bosetan (Tracleer®) oral - endothelin receptor antigonist ~ to improve exercise ability and decrease the rate of clinical worsening 2005 - Treprostinil (Remodulin®) Sub Cu - a potent pulmonary and systemic vasodilator; inhibits platelet aggregation and used to diminish symptoms associated with exercise -- to diminish the rate of clinical deterioration
2006 - Treprostinil (Remodulin®) IV - no ice needed - see above
2006 - Iloprost (Ventavis®) inhaled - shown to decrease pulmonary arterial hypertension signs including lowering high blood pressure and resistance in the pulmonary artery; to allow the heart to pump more. 2006 - Sildenafil (Revatio®) PDE5 inhibitor oral - has vasodilatory properties, – to improve exercise ability, help lessen symptoms, and slow down worsening changes in your physical condition 2007 - Ambrisentan (Letaris™) oral - endothelin reception antagonist, a vasodilating agent
2008 - Thelin (Sitaxsetin) not available in USA
2009 - Tadalafil (Adcirca®) PDE5 inhibator oral - phosphodiesterase type 5 (PDE-5) inhibitor 2009 - Treprostinil (Tyvaso) inhaled - a synthetic form of prostacyclin (known as a prostacyclin analogue); reduces the ability of your blood to clot, aids in easier breathing 2010 - Epoprostenol (Veletri) IV - no ice- a prostacyclin can help slow the thickening of blood vessels in the lungs, preventing further constriction of the vessels
2012 - Sildenafil -- Oral - PDE5 inhibitor –vasodilator to decrease pressure in pulmonary artery (Generic)
2013 - Opsumit – Macitentan – oral endothelin receptor antagonist; to delay the disease progression 2013 - Adempas - Riociguat – oral, approved drug therapy for persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH chronic thromboembolic pulmonary hypertension (blood clots) 2013 - Orenitram - Treprostinil - oral extended-release tablets
2015 - Uptravi (selexipag) oral prostacyclin
Pulmonary Hypertension ~ or some of the 'other' causes of PH (formerly SPH or secondary; now APAH or associated) If there is a pre-existing disease it may trigger PH and then PH would be secondary to that primary problem/disease and can be called SPH. Pulmonary hypertension secondary to another disease is relatively common but is still under-diagnosed. Reliable estimates of this condition are difficult to obtain because of the diversity of identifiable causes.
To name a few:
Air travels to the lungs though a series of tubes and airways. The two branches of the trachea, called bronchi, subdivide within the lobes into smaller and smaller air vessels. They terminate in alveoli, tiny air sacs surrounded by capillaries. When the alveoli inflate with inhaled air, oxygen diffuses into the blood in the capillaries to be pumped by the heart to the tissues of the body, and carbon dioxide diffuses out of the blood into the lungs, where it is exhaled.
The smallest subdivisions of the bronchial tubes are called BRONCHIOLES, at the end of which are the air sacs or alveoli (plural of alveolus).
The ALVEOLI are the very small air sacs that are the destination of air breathed in. The CAPILLARIES are blood vessels that are imbedded in the walls of the alveoli. Blood passes through the capillaries, brought to them by the PULMONARY ARTERY and taken away by the PULMONARY VEIN. While in the capillaries the blood gives off carbon dioxide through the capillary wall into the alveoli and takes up oxygen from the air in the alveoli.
New York Heart Association Classification
I. PH patients in this category have no symptoms during ordinary physical activity, their hearts function normally.
II. Although these patients are comfortable at rest, ordinary physical activity is somewhat limited by undue breathlessness, chest pain, fatigue, or near fainting.
III. These PH patients usually have no symptoms at rest, but their physical activity is greatly limited by breathlessness, chest pain, fatigue, or near fainting while doing routine things.
IV. These PH patients are often breathless and tired even while resting and can't do any physical activity without symptoms. They show signs of right-heart failure. Under the WHO system anyone who is prone to fainting goes into this class.
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Though the right lung has three lobes, the left lung, with a cleft to accommodate the heart, has only two. The two branches of the trachea, called bronchi, subdivide within the lobes into smaller and smaller air vessels. They terminate in alveoli, tiny air sacs surrounded by capillaries. When the alveoli inflate with inhaled air, oxygen diffuses into the blood in the capillaries to be pumped by the heart to the tissues of the body, and carbon dioxide diffuses out of the blood into the lungs, where it is exhaled
2003 Classification by WHO – World Health Organization
There were 5 classifications given for defining different kinds of PH.
1. PAH – IPAH, occurs without any apparent cause – Familial – APAH (Associated with PAH) similar to IPAH but exposure to toxins, or the effects of other diseases (scleroderma, lupus), congenital heart disease, chronic liver disease, HIV, diet drugs etc
2. PVH – caused by diseases of the left side of the heart
3. PH associated w/ disorders of the respiratory system – COPD, Sleep Apnea, developmental abnormalities etc
4. PH due to chronic thrombotic and/or embolic disease – obstructions (clots) etc
5. Miscellaneous (unclear) – Sarcoidosis, tumor, fibrosing, blood disorders, thyroid etc
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Pulmonary Artery Pressures another view:
Systolic Pressures: (ECHOs show Systolic Pressure.)
Mild PH: 40-55 mm hg
Moderate: 55-75 mm hg
Severe: above 75 mm hg
Mean Pulmonary Pressures:
Mild PH: 26-35 mm hg
Moderate: 36-45 mm hg
Severe: above 45 mm hg
15 is considered a normal mean pulmonary artery pressure and anything over 25 is considered PH.
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2013 -- UPDATED CLINICAL CLASSIFICATIONS OF PULMONARY HYPERTENSION – WHO
The term pulmonary hypertension refers to high blood pressure in the lungs. While in regular hypertension (also known as high blood pressure), the arteries throughout the body are constricted, PH affects only the blood vessels in the lungs and the right side of the heart.
PH and PAH: What’s the Difference? Pulmonary Hypertension, or PH, is the general term used to describe high blood pressure in the pulmonary arteries from any cause. Pulmonary Arterial Hypertension, or PAH, is high blood pressure in the pulmonary arteries from diseases that affect the small vessels of the lung, causing them to narrow and impede blood flow. It is a chronic and currently incurable disease that causes the walls of the arteries of the lungs to tighten and stiffen. In someone with PAH, the right side of the heart has to work harder to push blood through narrowed arteries in the lungs. Eventually, the extra stress causes the heart to enlarge and become less flexible, compromising the heart's ability to push blood out of the heart, through the lungs, and into the rest of the body. In associated pulmonary arterial hypertension, the PAH is associated with another disease or condition. PAH is commonly associated with connective tissue diseases (such as scleroderma, CREST syndrome and lupus), congenital heart disease, chronic liver disease, HIV, drugs and toxins, and more.
1. Group 1: Pulmonary arterial hypertension (PAH)
1-1 Idiopathic (IPAH) -- no known cause
1-2.1 Bone morphogenetic protein receptor type 2 (BMPR2)
1-2.2 Activin receptor-like kinase type 1 (ALK1), endoglin (with or without hereditary hemorrhagic telangiectasia)
1-3 Drug and toxin-induced1-2.3 Unknown
1-4 Associated with (APAH)
1-4.1 Connective tissue diseases
1-4.2 Human immunodeficiency virus (HIV) infection
1-4.3 Portal hypertension
1-4.4 Congenital heart diseases
1-4.6 Chronic hermolytic anemia
1-5 Persistent pulmonary hypertension of the newborn (PPPH)
1’ Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemanglomatosis (PCH)
2. Group 2: Pulmonary hypertension owing to left heart disease
2-1 Systolic dysfunction
2-2 Diastolic dysfunction
2-3 Valvular disease
2-4 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies
3. Group 3: Pulmonary hypertension owing to lung diseases and/or hypoxia
3-1 Chronic obstructive pulmonary disease (COPD)
3-2 Interstitial lung disease (ILD - a forming of scar tissue in the lungs)
3-3 Other pulmonary diseases with mixed restrictive and obstructive pattern
3-4 Sleep-disordered breathing (ie: sleep apnea)
3-5 Alveolar hypoventilation disorders
3-6 Chronic exposure to high altitude
3-7 Developmental abnormalities
4. Group: 4 Chronic thromboembolic pulmonary hypertension (CTEPH) (Blood clots)
5. Group 5: Pulmonary hypertension with unclear multifactorial mechanisms
5-1 Hermatologic (bloodborne) disorders: chronic hendytiv anemia, myeloproliferative disorders, splenectomy
neurofibromatosis, vasculitis5-2 Systemic (affects the whole body) disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis,
5-3 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
5-4 Others: tumoral obstructions, fibrosing mediastinithis, chronic renal failure on dialysis
Regular Blood pressure readings are measured in millimeters of mercury (mmHg) and usually given as 2 numbers. For example, 110 over 70 (written as 110/70).
* The top number is the systolic blood pressure reading. It represents the maximum pressure exerted when the heart contracts. * The bottom number is the diastolic blood pressure reading. It represents the minimum pressure in the arteries when the heart is at rest.
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The pulmonary artery carries blood from the heart’s right lower chamber (ventricle) to the lungs, where it is loaded up with oxygen. From the lungs, the blood returns to the heart’s left ventricle and is pumped out through the aorta to the body and all is fine. If those arteries are constricted... then symptoms occur and we will need to call our doctor.
Ever wonder what those letters and numbers mean on all those fancy tests: Click here to find out.
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