What is Pulmonary Hypertension? What is Pulmonary Arterial Hypertension?

You’ve probably heard of high blood pressure (hypertension), a common condition that affects the way your blood flows through the arteries in your body from the left side of your heart.

A less common type of high blood pressure, called pulmonary arterial hypertension, affects only the arteries in the lungs and the right side of your heart.

Pulmonary Hypertension (PH) begins when tiny arteries in your lungs, called pulmonary arteries and capillaries, become narrowed, blocked or destroyed.

This makes it harder for blood to flow through your lungs, which raises pressure within the pulmonary arteries.

As the pressure builds, your heart’s lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and sometimes fail completely.

Having this disease is a lifetime commitment for a patient and his or her doctor. But, with proper diagnosis and treatment, it is a manageable disease for a time.

Because of the rarity of this disease, there are still many doctors who do not know or understand what it is. It can be present in all ages from infant to senior citizen and does not discriminate between male or female, or ethnic backgrounds, but is most common in women of child bearing years.

The key note to remember: with proper diagnosis and treatment this disease is manageable.


Pulmonary hypertension is a serious illness that becomes progressively worse and can be fatal. Although it is not curable at this time, treatments are available that can help lessen symptoms and improve your quality of life.

Symptoms may include: shortness of breath with minimal exertion; chest pain; edema; fainting; unusual fatigue; dry cough; and/or heart palpitations.

Symptoms range in severity and a given patient may not have all of the symptoms. As mentioned, PH (specifically PAH) may be secondary to other contributing diseases or there can be no known cause.

Medical conditions that may lead to secondary pulmonary hypertension include:

  • AIDS – HIV
  • Blood clots in the lungs (pulmonary emboli) CTEPH
  • Chronic exposure to high altitude
  • Chronic liver disease (cirrhosis)
  • Chronic obstructive pulmonary diseases, (COPD) such as emphysema
  • Connective tissue disorders, such as scleroderma, lupus, rheumatoid Arthritis
  • Congenital heart defects (i.e. ASD / VSD and other heart defects)
  • Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs’ air sacs (interstitium)
  • Left-sided heart failure (PVH)
  • PH with unclear multifactorial mechanisms
  • Raynaud’s phenomenon – affected skin area turns white or blue – usually toes and fingers
  • Sarcoidosis – can affect any organ in your body-usually starts in lungs or skin
  • Sickle cell anemia
  • Sleep apnea

Symptoms of pulmonary hypertension do not usually occur until the condition has started to progress. The first symptom of pulmonary hypertension is usually shortness of breath with minimal exertion, you may also feel extreme fatigue, have dizziness or fainting spells, heart palpitations, a dry cough; all these can be symptoms. Edema or swelling in the ankles, the legs or the abdomen can occur; bluish lips and skin and chest pain may occur as strain on the heart increases. Again, the blood vessels in the lungs thicken, restricting blood flow which makes the right side of the heart overwork and can lead to heart failure. It can be a silent killer. Untreated, PH has a worse prognosis than many forms of cancer.

REMEMBER – with proper treatment the progression can be delayed.


It is often misdiagnosed or overlooked as a lung disorder or as a complication of a large number of respiratory or cardiac disease disorders, see above.

With proper treatment you can maintain a somewhat normal life and many have been known to go back to their regular routine.

Various testing will be performed to determine if you have PAH i.e.: EKG’s, pulmonary function tests, an Echocardiogram – to name a few. These would be conducted to eliminate other disorders.

The gold standard of all tests is the right heart catheterization (RHC). This will let the doctor know your pulmonary pressures and what would be the best treatment for you and in short, if you have the disease.


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, relay that to our doctor.

For years, lung transplants were the only treatment for pulmonary arterial hypertension. Just a few short years ago, there were only three FDA-approved medications available for treatment. Today, through research, there are over a dozen available treatments and a number are still in trial. Those include:

  • Some 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 – help 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, here is a history of approved PAH treatment options meant to improve exercise ability and slow the worsening of PAH.

  • 1996 Flolan – Epoprostenol – IV (ice needed) – a naturally occurring prostacyclin with potent vasodilatory activity and inhibitory activity of platelet aggregation.
  • 2002 Tracleer – Bosetan – Oral – reverses (reduces) the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow
  • 2005 Remodulin – Treprostinil – SC – Prostacyclin medications work by opening the blood vessels and may reduce the buildup of cells around the blood vessel walls
  • 2006 Ventavis – Iloprost – Inhaled – it goes directly to the lungs to open narrowed blood vessels dilates systemic and pulmonary arterial vascular beds. It also affects platelet aggregation
  • 2006 Revatio – Sildenafil – Oral – PDE5 inhibitor – vasodilator (Viagra) to decrease pressure in pulmonary artery
  • 2006 Remodulin – Treprostinil – IV – a potent pulmonary and systemic vasodilator; inhibits platelet aggregation.
  • 2007 Letaris – Ambrisentan – Oral – can help slow down the worsening of your symptoms.
  • 2008 Thelin – Sitaxsentan – not available in USA is available in Canada, Europe and Australia
  • 2009 Adcirca – Tadalafil – Oral – PDE5 inhibitor – can cause blood vessels to widen (Cialis)
  • 2009 Tyvaso – Treprostinil – Inhaled – a synthetic form of prostacyclin which patient bodies produce too little of
  • 2010 Veletri – Epoprostenol – IV – can help slow the thickening of blood vessels in the lungs, preventing further constriction of the vessels making blood flow easier
  • 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) Extended-Release Tablets for oral administration
  • 2015 Uptravi – (selexipag) oral prostacyclin

Most PH medications lower your blood pressure, so be warned about being given nitroglycerin. It can cause your heart to bottom out.

Not everything that happens is because of the PAH, so don’t think you are necessarily doing worse just because you are having a bad day; even those who don’t have PH have bad days.

Also, try to think of yourself as healthy – approach life with that attitude; it can’t hurt and it may help.

If you are being symptomatic make sure something isn’t going on in your life to cause this: stress, tension, have you gained weight are you retaining fluid? Being symptomatic does not always mean you need an increase or change in your PH medication. Talk with your doctor to rule these things out.

What do your 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: SPAP + (DPAP x 2).

Pulmonary Artery Pressures – another view – (ECHO’s show Systolic Pressure).
Systolic Pressures:
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.

RHC – Conditions that can also be diagnosed or evaluated with Swan-Ganz catheterization include pulmonary hypertension, cardiac tamponade, and restrictive cardiomyopathy.

Normal Results

  • Cardiac index is 2.4 to 4.2 liters per minute per square meter (of body surface area)
  • Cardiac output (CO) average range 4.0 – 8.0 L/minute (5.6 L/min male & 4.9 L/min female)
  • Pulmonary artery systolic pressure is 15 to 30 millimeters of mercury (mmHg)
  • Pulmonary artery mean pressure is 9 to 17 mmHg
  • Pulmonary diastolic pressure is 0 to 8 mmHg
  • Pulmonary capillary wedge pressure is 5 to 15 mmHg
  • Right atrial pressure is 0 to 8 mmHg

What Abnormal Results Mean

Abnormal results may indicate heart valve disease, circulatory flow problems such as heart failure or shock, or another lung disease.


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 (syncope).
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.

WHO (World Health Organization) Functional Class
Class 1. No limitation of physical activity; no symptoms with ordinary physical activity.
Class 2. Slight limitation of physical activity; ordinary physical activity causes PAH symptoms
Class 3. Marked limitation of physical activity; less than ordinary physical activity causes PAH symptoms
Class 4. PAH symptoms with any physical activity and even at rest; discomfort with any physical activity, signs of right heart failure.

1. Group 1: Pulmonary arterial hypertension (PAH)
1.1 Idiopathic (IPAH) — no known cause
1-2 Heritable
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
1-4.5 Schistosomiasis
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

Researching for Improved Treatments or a Cure

It is important that continued funding be made available for research – to find a cure for this disease.

For donations visit either:
Team Phemonemal Hope – https://www.teamphenomenalhope.org/contribute/ways-to-give/ or
Pulmonary Hypertension Association www.phassociation.org

Information compliments of Merle Reeseman – a phenomenal patient, member of the Team PH Board of Directors, and Support Group Leader