What is Pulmonary Hypertension (PH)?

Simply put, Pulmonary Hypertension is abnormally high blood pressure (hypertension) within the lung's (pulmonary) blood vessels. That sounds pretty straightforward but Pulmonary Hypertension is not a simple disease. It is a complex set of disorders of the delicately balanced circulatory system that encompasses the heart and lungs, the job of which is to gather oxygen and send it to the rest of the body. When abnormally high pressures are present, it results in a strain being placed upon a person's cardio-pulmonary (heart and lungs) system that in turn causes the various symptoms of Pulmonary Hypertension.
 

How is Pulmonary Hypertension different from "regular" hypertension (high blood pressure)?

Whereas the typical hypertension that people hear about every day involves an increase in pumping pressure throughout a person's body that can be measured with a blood pressure cuff, Pulmonary Hypertension is confined to the area that involves the lungs and heart and is caused by an entirely different process. Both disorders result in a form of "hypertension" and both are serious. It is the area that Pulmonary Hypertension affects that makes it different and unusual. And yes, a person may have Pulmonary Hypertension and high blood pressure at the same time.
 

Anatomy

The heart is a muscle that acts as the pump for moving blood throughout the body. The two chambers in the right side of the heart are responsible for collecting the blood from the body that has been depleted of it's oxygen supply and returning it to the lungs through the pulmonary artery. The blood then travels through the lung's vessels to pick up more oxygen. The two chambers in the left side of the heart then collect the re-oxygenated blood from the lungs and pumps it out to the rest of the body to deliver the oxygen to the various organs and tissues. These two actions comprise a person's circulatory system. In effect, a person's body has two separate yet intertwined circulation systems - one specifically designed to pump blood through the lungs and the other to pump the blood throughout the rest of the body.
 

What is abnormal mean pulmonary-artery pressure?

Normal mean pulmonary-artery pressure (PAP) is approximately 14 mmHg at rest. In the patient with Pulmonary Hypertension, the mean blood pressure, measured by special tests at the main pulmonary artery within the heart, is greater than 25 mmHg at rest or 30 mmHg during exercise. This abnormally high pressure is associated with changes in the small arteries or veins in the lungs, resulting in an increased resistance or pressure for the blood trying to flow through the vessels.
 

What does having higher than normal pulmonary artery pressures do?

The elevation in pulmonary artery pressure and resulting damage of these important vessels causes the right side of the heart to work harder than it normally would. The right side of the heart was not designed to handle the amount of stress that is placed on it by the high pumping pressure required of it to overcome the failing circulation system within the lungs. Over time, the lungs become so damaged and the heart so weakened, the heart will eventually fail. This is why early diagnosis and treatment is so important in the prognosis of Pulmonary Hypertension.
 

Why does it take so long and seem so difficult to diagnose?

People with undiagnosed Pulmonary Hypertension often go to their doctors complaining of shortness-of-breath and unusual fatigue. These are vague symptoms that are associated with many different disorders, both mental and physical. If a person is overweight or getting a bit older, their doctors may tell them to lose some weight and get more exercise. Their symptoms may also be confused with asthma, depression or a myriad of other disorders. Pulmonary Hypertension is a fairly rare disorder that is often not suspected until all other possible causes for the symptoms have been ruled out. It often is not until the actual red-flag warning symptoms of heart failure, such as edema (water buildup in the legs and/or the rest of the body), severe shortness-of-breath, fainting and/or actual damage to the heart become readily apparent that the diagnosis is even considered. For many, Pulmonary Hypertension may be present for years before it is properly diagnosed.
 

Where to find proper treatment

Once a person has been diagnosed with Pulmonary Hypertension, we encourage patients to seek out the care of a specialist in the field of Pulmonary Hypertension rather than simply a local cardiologist or pulmonologist who may not be up to date on the latest treatments and clinical trials. A PH specialist will also be either a cardiologist or a pulmonologist but will have more experience and special training in treating this complicated disease. You will find a listing of PH specialists on this website under the "Medical" tab at the top of each page. A link is also prominently displayed on the home page or you may click here.
 

Severity and Prognosis

Pulmonary hypertension may be mild in some patients and severe in others. The severity and prognosis will depend on the underlying cause and the stage at which a diagnosis is made. This is not a disease that you want to take a "wait and see" approach to. It is a progressive disorder that will not improve over time without treatment. Once you have a firm diagnosis, you will want to seek treatment as soon as possible.
 

Where in the past, doctors had little to offer in terms of treatment options, this has changed dramatically in recent years with the introduction of several new medications and treatments. Although currently there remains no cure for this disease, it can often be well managed for many years through the use of medications. There is much ongoing research into the causes and treatments of Pulmonary Hypertension and the outlook is much brighter than it has been in the past.
 

The learning experience

We at PHCentral would like you to join us in the most important aspect in your journey with Pulmonary Hypertension - which is the process of self-education in the handling and management of your disorder. One of the most important aspects of dealing with a chronic disorder is in understanding and learning as much as you can about it so that you may have the tools for which to control it. We hope that we can help you along on this path. Please feel free to browse the rest of our website for more information. If you have any questions, please feel free to join our forums to discuss your issues with others or contact us and we will try to help.

 

November 2011 - Page Reviewed and Updated.


Other Resources

Journal of the American College of Cardiology
Updated Classification of Pulmonary Hypertension 

Mayo Clinic, Rochester, Minnesota
An Introduction to Pulmonary Hypertension

Pulmonary Hypertension Association of Canada
Learn about the disease and the science of pulmonary hypertension.

National Heart Lung and Blood Institute
What is Pulmonary Hypertension?

American Lung Association
Pulmonary Arterial Hypertension (PAH)

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