Primary Pulmonary Hypertension (PPHP) is a condition involving increased blood pressure in the pulmonary arteries due to an unknown cause. (It is identified as “primary” because the cause is unknown, unlike Pulmonary Arterial Hypertension where a cause has been identified involving an underlying condition of the heart or lungs.) The pulmonary arteries bring blood from the heart to the lungs to gather oxygen to deliver to the rest of the body.
With the increased blood pressure, at least one of three conditions affects the arteries: 1) The arterial walls were stiff from birth or became stiff due to cell overgrowth. 2) There is a tightening of the artery walls or 3) Blood clots form in the arteries.
With any of these three problems, the heart has a hard time pumping blood into the lungs. This strain in the heart leads to heart failure. The right ventricle (the chamber of the heart on the lower right side) is the part of the heart that pumps the blood to the pulmonary arteries. With heart failure, the strain that this part of the heart endures can cause an enlarging of the heart.
The heart becomes weaker with all the strain and can’t get enough blood to the lungs. The condition will worsen gradually over time, making activity harder and harder until everyday tasks are affected or stopped. It can eventually lead to a heart attack and death.
The severity of cases varies as a lot depends on the patient’s overall health and fitness, eating habits, lifestyle, age at diagnosis, and the severity of the condition at the time of original diagnosis. When people are diagnosed at age 40 or older or do not suffer from heart failure, they will live longer than those who develop the condition earlier in life or who already suffer from congestive heart failure.
PPHP is divided into four classes of severity, based in large part on the affect the condition is having on the daily activities of the patient. It is important to follow a physician’s advice and maintain treatment for maximum benefit and longer life.
Symptoms and Treatments for Primary Pulmonary Hypertension
Primary Pulmonary Hypertension (PPH) is a condition that involves high blood pressure in the pulmonary arteries. It can develop slowly over time, for years not manifesting anything more than mild symptoms. Some of the early symptoms involve chest pain, fatigue, and a racing heartbeat. These may be passed off as other issues for a time, until further symptoms develop.
As the disease progresses, there can be swelling of the ankles and legs, lightheadedness with climbing stairs and other light daily physical activity, shortness of breath with exertion, bluish skin or lips, and even fainting. The further along the disease progresses, the worse the symptoms become.
The increasing levels of severity are ranked in classes. In Class I the patient is not limited at all in physical activity. Class II involves only mild limitations on physical activity, but no issue when at rest. With Class III, there is a significant limitation on physical activity and symptoms are noticeable even with minimal activity. Finally, Class IV entails symptoms even at rest and the inability to perform any physical activity without troublesome symptoms.
The patient is diagnosed with a physical exam, a full medical and family history, and various tests and procedures such as an EKG, a chest x-ray, pulmonary function tests, exercise testing, lung scan, right heart catheterization, overnight oximetry, and an echocardiogram.
While there is no cure for PPH, treatments can help slow the progression of the condition and can alleviate some of the symptoms. Blood thinners prevent blood clots and reduce the blood pressure overall. Diuretics reduce fluid buildup, which helps with the swelling. And the medication Digoxin strengthens the heartbeat and thereby increases blood flow. Oxygen therapy is often prescribed and can increase oxygenation throughout the body. There are also surgical procedures and lifestyle recommendations that can improve PPH.
Information on this page is for reference and educational purposes only. For more information about primary pulmonary hypertension, talk to your doctor or primary care provider.