PPH Primary Pulmonary Hypertension Pain

What’s happening to me?: Defining PPH Pain 

Pulmonary hypertension is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening.  When pulmonary hypertension occurs in the absence of a known cause, it is referred to as primary pulmonary hypertension (PPH).

PPH starts when the small vessels that supply blood to the lungs constrict, or tighten up. It is more difficult for blood to get through to the lungs, and the heart must pump harder to overcome the resistance. As time passes, scarring (or fibrosis) of the vessels makes them stiffer and thicker, and some may be completely blocked. The extra stress causes the heart to enlarge and become less flexible. Less and less blood is able to flow out of the heart, through the lungs, and into the body, and more and more symptoms start to show.

Below is some background on PPH pain and its management from a medical perspective, so that you can be better informed as you battle your illness. For more on emotional and other support, click here.

Physical Pain: What will I be feeling?

The symptoms of PPH include chronic fatigue, shortness of breath (dyspnea), chest pain (angina), fainting, swollen ankles and legs (edema), and fluid in the abdomen (ascites).

An increase in the pressures within the pulmonary arteries places an increased workload on the right side of the heart. That is, the right ventricle and the right atrium are forced to work harder to overcome the increased blood-flow resistance created by the abnormally high pressure in the pulmonary arteries. As a result, the right side of the heart becomes enlarged - oftentimes, as the disease progresses, leading to congestive heart failure. 

What are some of the clinical effects of PPH?

Increased pressure in the pulmonary arteries results from a progressive narrowing of the pulmonary arterioles - the smaller branches of the main pulmonary arteries. One direct effect of these abnormally elevated pressures is blood leakage from the pulmonary vessels. A blood-producing cough is oftentimes an indicator of leakage from the pulmonary vessels.

The increased pressures in the pulmonary arteries and arterioles can trigger a cyclical chain of adverse events beginning with a decrease in the blood's oxygen content. The body then responds to this low oxygen content by increasing its production of red blood cells. This is known as polycythemia. By doing so, the body attempts to compensate for the blood's low oxygen content by increasing the number of oxygen carriers, i.e. the red blood cells. As the red blood cell count increases, the blood thickens, making the heart (already under increased stress from the elevated pressures) work that much harder to pump the thicker blood throughout the body. This further increases arterial pressures and can lead to congestive heart failure.

Emotional Pain: How will this affect my quality of life?

An often overlooked but important concern is the emotional well being of chronically ill patients and their caregivers. To address these concerns, we have provided links to support groups and other resources around the Internet to help you cope with the emotional effects of PPH, as well as the medical resources to give patients and caretakers the peace of mind that comes with being well informed.

Many people, when told they have PPH, feel helpless. But there are many things you and your family can do.

Understanding Your PPH Illness and Its Treatment

• Talk to someone who really knows about PPH.
   

• Call us at 1-800-780-2686 or email us for a list of PPH doctors, clinical trials, and support groups.
   

• Ask about sources of information and support when you go to the hospital. You may not be told what is available unless you do.
   

• See our section on communicating with your health care provider.