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Understanding Pulmonary Hypertension...

​​What is the difference between Pulmonary Hypertension (PH) and Pulmonary Arterial Hypertension (PAH)?  There is an important difference between PH & PAH. Pulmonary hypertension is a general term and simply means high blood pressure in the lung. The high blood pressure can be caused by many medical conditions such as diseases of the left heart, chronic lung diseases, sleep apnoea, and blood clots in the lungs. PAH is a specific cause of Pulmonary Hypertension where the arteries of the lungs are directly diseased and become narrowed. It is very important that the exact cause of PH is determined since treatment must be directed at the underlying condition.
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What is Pulmonary Arterial Hypertension (PAH)? Pulmonary Arterial Hypertension (PAH) is the medical term for a progressive disease caused by narrowing or tightening (constriction) of the pulmonary arteries that connect the right side of the heart to the lungs. PAH is a serious condition that affects the blood vessels (pulmonary arteries) that carry blood from the heart to the lungs. PAH is the medical term for a progressive disease caused by narrowing or tightening (constriction) of the pulmonary arteries that connect the right side of the heart to the lungs. ​
PAH gradually restricts the flow of blood through the lungs. As a result, the pressure in the pulmonary arteries increases. The heart must pump against this increased pressure to maintain blood flow in the lungs and to the rest of the body. Over time, this can affect the heart's ability to work effectively and may eventually lead to heart failure. 

​Although PAH is still a rare condition, it is a disease that can affect males and females of all ages and ethnic backgrounds. Very rarely, PAH occurs in childhood. This makes early diagnosis very important as treatment can alleviate many of the symptoms associated with pulmonary arterial hypertension, improving people's quality of life.

​Treatment and ongoing management by specialist pulmonary hypertension doctors, nurses, and their healthcare team is vital. People with certain existing medical conditions can also be at a higher risk of developing pulmonary hypertension CLICK (for more info). Currently their is no cure for this serious progressive disease but every year new and improved medications and treatments are being developed which have prolonged and improved the lives ​of those living with this disease.  ​
There  are a great many new and exciting developments in the world of Pulmonary Hypertension. ​With more effective treatments being available people with pulmonary hypertension have the opportunity to manage their disease better, and achieve a higher quality of physical, emotional, and psychological health and well being.  

PHNA have gathered together the most relevant & up-to-date information for people living with pulmonary hypertension, either written and produced directly through us (see our PH Patient Pack & Resources) or shared with you and/or adapted from other respected organisations. We have endeavoured to present information in an easy to navigate format. We have offered other links to our sister groups, where necessary, and relevant, to further expand your information base.

​(Part of this explanation for PH/PAH was shared with you from the Lung Foundations Better Living Series PAH A Patients Guide. For your own complete guide contact PHNA or LFA). ​​

​Is there a difference between pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH)?

Yes, there is a difference between pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH).
Pulmonary arterial hypertension (PAH) is a specific subgroup of pulmonary hypertension (PH). The definition of PH is more inclusive than the definition of PAH, meaning that the specific hemodynamic measurements used to define PAH do not apply to PH. Pulmonary hypertension (PH) is simply an elevation in the pressure in the arteries of the lungs. Many diseases such as obstructive sleep apnea (OSA), lung diseases, diastolic heart failure, and diseases of the left heart can cause the pressures in the pulmonary arteries to rise but this does not mean you have pulmonary arterial hypertension (PAH). Pulmonary arterial hypertension is a disease of the blood vessels of the lungs meaning these vessels have changed causing the elevation in pressure. In pulmonary hypertension, the elevation of the pressures is caused by another disease, the blood vessels themselves are not the problem.
How is this type of hypertension different?
It is important to remember that the pulmonary blood vessels are a low pressure system much different than the rest of the blood vessels throughout your body. The left side of the heart is designed to be the work horse, large and muscular, generating enough pressure to propel blood flow throughout your entire body. The right side of the heart is the smaller gentler side of the heart, supplying a gentler blood flow to the blood vessels in the lungs. In pulmonary arterial hypertension (PAH), the right side of the heart has to work harder and harder to pump against the increased pressure in the pulmonary arteries. Just like any muscle that is constantly worked, it grows bigger. Unfortunately, when the right side of the heart grows bigger it starts to take over space the left heart needs to effectively pump. As the size of the right atrium and ventricle increases, the ability for the chambers to efficiently pump blood diminishes, eventually leading to right heart failure.
How does pulmonary hypertension affect my body?
The pulmonary arteries in PH patients are being assaulted by three main problems. Vasoconstriction, meaning the blood vessels are tightening, and cell proliferation, meaning the cells on the inside of the blood vessels are multiplying. This causes the vessel to become thicker and stiffer. Lastly, blood clots may form inside the blood vessels of the lungs. This further obstructs blood flow. Healthy vessels start out like open pipes that the blood can easily travel through and as the disease progresses the pipes become more obstructed. As the vessels become narrower the pressure inside the vessels becomes higher much like when you block the end of a garden hose with your thumb and the water squirts out at a higher pressure. The increased pressure of the blood flow does additional damage to the diseased arteries. Blood thinners are often used as part of the treatment plan for pulmonary hypertension patients to help prevent small blood clots from forming inside the diseased blood vessels.
Pulmonary hypertension hemodynamic definition
The hemodynamic definition of pulmonary arterial hypertension (PAH) is a mean pulmonary artery pressure at rest greater than or equal to 25 mmHg in the presence of a pulmonary capillary wedge pressure less than or equal to 15 mmHg. A normal mean pulmonary artery pressure for a healthy patient is 12-16 mmHg and a normal wedge pressure is 6-12 mmHg. Basically, the pressures in the right side of the heart and the pulmonary arteries are elevated while the pressures in the left side of the heart are normal. These specific pressures can only be measured accurately via right heart catheterisation.
​Understanding Pulmonary Hypertension PAHuman Actelion 

Pulmonary hypertension is an extremely serious and rare condition that can affect anyone, at any age, and either gender.

In this video from Discover Actelion, you can learn more about PH, how Actelion has been supporting the patients’ community throughout the years, how they have been helping to raise awareness and working together with those communities to better understand and improve the management of pulmonary arterial hypertension, a sub type of pulmonary hypertension.

Watch the video to understand this condition a little more and also to witness how the knowledge of PH has been evolving.
Defining Pulmonary Hypertension Causes, Symptoms & Diagnosis 

​Pulmonary hypertension (PH) is a severe lung condition that causes shortness of breath, chest pain, fatigue, and dizziness among other symptoms. It’s a disease that can affect anyone, at any age, although women are more prone to developing the condition.

Since pulmonary hypertension is a rare disease and its symptoms are very similar to other respiratory and lung diseases, patients are often misdiagnosed many times and doctors can take several years to get an accurate diagnosis of PH. Asthma, sleep apnea or COPD are some of the diseases that are usually diagnosed instead of pulmonary hypertension.
In this video from 2012 by UT Health Northeast, we find the renowned University of Texas Health Science Center pulmonologist Dr. Julie Philley briefly explaining pulmonary hypertension, defining PH, pointing to causes, symptoms, how to diagnose, and general differences between pulmonary hypertension and “regular” high blood pressure.  ​She also acknowledges the existence of prior defects or illnesses that lead up to pulmonary hypertension and how properly treating those defects earlier may make PH “disappear”.  ​

​But how is pulmonary hypertension diagnosed? Learn more about it here.  ​Find out who is more at risk of developing pulmonary hypertension in this article.