What is PH?

 

High blood pressure in the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). PAH is a chronic and life-changing disease that can lead to right heart failure if left untreated.  - Pulmonary Hypertension Association

There are now 14 FDA approved medications to treat PH, 3 of which were approved in 2013, but NO medications approved to treat PH children. There is currently NO CURE. As the often terminal disease progresses, most patients face a double lung (and sometimes heart) transplant.


PH Symptoms

The symptoms for all types of PH may be similar and symptoms are usually more severe as the disease progresses. Symptoms of PH may include:

  • Breathlessness
  • Chest pain (also called angina pectoris)
  • Dizziness
  • Fainting (also called syncope)
  • Loss of energy
  • Swelling of the arms, legs, ankles or abdomen (also called edema)
  • Dry cough
  • Raynaud's phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)
 

In advanced stages of PH, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest.

Sometimes these symptoms mean you have another condition, but sometimes, these symptoms mean you have PH.


Risk Factors

 

People of all ages, races and ethnic backgrounds are diagnosed with PH. Even though anyone can be diagnosed with PH, certain risk factors make some people more likely to get the disease:

Family History -- If two or more members of your family have PH or if a family member in your lineage is known to have a PH-causing gene mutation, the risk of getting PH is more likely. Genetic counseling is available to discuss these issues. Learn what genetics can teach us about PH.

Obesity & Obstructive Sleep Apnea -- In isolation, obesity is not a risk factor. However, if obesity is combined with OSA, mild PH may occur.

Gender -- Idiopathic PAH and heritable PAH (also known as familial PAH) are at least two-and-a-half times more common in women than in men. Females of childbearing age are also more susceptible.

Pregnancy -- Pregnancy is a possible risk factor suggested by registries and expert opinion. Women who already have PH and become pregnant have a much higher risk of mortality. Read more about pregnancy and PH.

Altitude -- Living at a high altitude for years can make you more predisposed to PH. When traveling to high altitudes, PH symptoms can be aggravated by the altitude.

Other Diseases -- Other diseases, including congenital heart disease, lung disease, liver disease and connective tissue disorders like scleroderma and lupus can lead to the development of pulmonary hypertension. Read more about PH and associated diseases.

Drugs & Toxins -- Certain drugs, such as methamphetamine and the diet drug fen-phen, are known to cause PH.