PAH Center

Q.What is pulmonary hypertension (PH)?

A. Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs (pulmonary arteries) become constricted or narrowed. This in turn hinders the flow of blood through the vessels, causing blood pressure to rise above normal levels. As a result, the right ventricle of the heart is dangerously strained and becomes enlarged and weakened. This deterioration of the ventricle can eventually lead to heart failure.

Q. What are some alternative names for PH?      

A. PH can be referred to by a variety of different names, including pulmonary arterial hypertension (PAH). Primary pulmonary hypertension(PPH)orprimary pulmonary arterial hypertension(PPAH) is used to describe cases of PH caused by unknown factors, although some medical experts believe it may be inherited. When pulmonary hypertension is known to have been caused by a particular medical condition, it is often referred to as secondary pulmonary arterial hypertension (SPAH).

Pulmonary hypertension may also be called idiopathic pulmonary arterial hypertension,  sporadic primary pulmonary hypertension, familial primary pulmonary hypertension, or primary obliterative pulmonary vascular disease.

Q. What are some of the symptoms of PPH / PAH?

A. Initially, the symptoms of PPH / PAH are often minor in severity. They may include:

• Shortness of breath following physical work or exercise
• Angina (chest pain)
• Extreme tiredness
• Fainting spells, dizziness or weakness
• Ankle or leg swelling
• Bluish skin and lips
• Fast pulse
• Tachycardia (rapid heartbeat)

In patients with PPH / PAH, the heart weakens and physical energy decreases over time. Eventually, an individual with this condition may be able to do little activity and may experience symptoms even while resting.

Q. What are the causes of PPH / PAH?

A. Certain medical conditions seem to precede the development of PAH. These include:

• HIV/AIDS
• Liver disease, such as cirrhosis
• Connective tissue diseases, such as scleroderma or lupus erythematosus
• Chronic obstructive pulmonary disease (COPD), such as emphysema
• Sleep apnea
• Blood clots in the pulmonary artery
• Sickle cell anemia
• Left-sided heart failure

In the case of primary pulmonary hypertension (PPH), the underlying cause is unknown or can't be found. In some people with PPH, researchers have discovered a genetic defect that can lead to the kinds of changes in the pulmonary artery that are exhibited by the condition.  Scientists are hopeful that further genetic research into PPH may result in prevention or improved treatments.

Other factors that appear to increase the risk of developing PPH / PAH include the use of cocaine or amphetamines as well as certain medications used for weight loss.  

Q. What is the nature of the link between diet drugs and PPH / PAH?

A. Recent evidence has come to light which suggests a link between certain appetite suppressants and PPH / PAH. These include fenfluramine (marketed as Pondimin), dexfenfluramine (marketed as Redux), and the fenfluramine-phentermine drug combination, known as Fen-Phen. Studies estimate that treatment with these drugs increases a patient's risk of developing PPH / PAH from about 1 to 28 cases per million person-years (one person-year represents a patient treated for one year). Learn more about diet drugs and PPH / PAH.

Q. How is PPH / PAH diagnosed?

A. The procedures used to diagnose PPH / PAH include a complete physical examination, blood tests, chest X-rays, echocardiograms and various pulmonary tests. It is important to note, however, that

accurately diagnosing PPH / PAH is very difficult for a number of reasons. First, there can be a single or multiple causes. Secondly, even if the disease has progressed, symptoms may be incorrectly attributed to other heart or lung conditions. Lastly, since PPH / PAH is not a common disorder, learning more about the disease continues to be a challenge. Therefore, if a patient's symptoms suggest PPH / PAH, a thorough examination at specialized clinic is usually recommended.

Q. Is there a cure for PPH / PAH?

A. There is currently no known cure for PPH / PAH. However, recent advances in treatments for the disease have enabled patients to live longer and more comfortably.

Q. How is PPH / PAH treated?

A. PPH / PAH is treated with medications, oxygen therapy and lung transplantation, or a combination of any of these. The objectives in treating PPH / PAH are to address the underlying cause, minimize symptoms, slow the development of smooth muscle cells and blood clots, and potentially increase blood flow to the heart.  Learn more about the latest treatments for PPH / PAH.