Pulmonary hypertension (PH) is a progressive, life threatening type of high blood pressure. Medications and surgery can help you manage this rare condition.
Pulmonary hypertension (PH) is a serious health condition that results when the arteries carrying blood from the right side of the heart to the lungs are constricted, disrupting the flow of blood.
Blood must travel through the lungs for air exchange in order to pick up the oxygen that it delivers to the organs, muscles, and tissues. When the arteries between the heart and lungs become narrowed and flow is constricted, the heart has to work extra hard to pump blood to the lungs.
Over time, the heart can grow weak, and proper circulation can diminish throughout the body.
Treatments are available that can help delay the progression of PH.
There are five types, or groups, of PH, as defined by the World Health Organization (WHO).
All types of PH require medical attention. Treating the cause of PH can help slow its progression.
Group 1 PH
Group 1 PH is also known as group 1 pulmonary arterial hypertension or group 1 PAH. It can have a variety of causes, including:
- connective tissue diseases, including certain autoimmune diseases
- liver disease
- congenital heart disease
- sickle cell anemia
- schistosomiasis, a type of parasitic infection
- certain drugs or toxins, including recreational drugs (such as methamphetamine and cocaine) and some diet medications
Group 2 PH
Group 3 PH
Group 3 PH is associated with certain lung and breathing conditions, including:
- chronic obstructive pulmonary disease (COPD)
- interstitial lung diseases (such as pulmonary fibrosis), which can cause scarring of lung tissue
- sleep apnea
- hypoxia, or low levels of oxygen in your tissues
Group 2 PH and group 3 PH are the most common types of PH.
Group 4 PH
Group 5 PH
Group 5 PH is caused by other various conditions, including:
- blood disorders such as polycythemia vera and thrombocythemia
- systemic disorders such as sarcoidosis and vasculitis
- metabolic disorders such as thyroid disease and glycogen storage disease
- other conditions such as kidney disease and tumors compressing the pulmonary arteries
Group 5 PH may have either multiple causes or an unknown cause.
At the moment, there’s no readily available cure for PH.
It’s a progressive disease, which means it can advance over time, sometimes much faster for some people than others.
For many people, PH can lead to significant heart failure, and their overall health can be in great danger. However, some people can effectively manage their condition by making lifestyle changes and monitoring their health.
Medications and lifestyle changes such as getting more exercise are intended to slow the progression of the disease.
Life expectancy will depend on the type of PH you have and the cause of your PH.
The median survival time after diagnosis for people with group 1 PAH is more than 5 years. It’s noticeably improved
The other groups all have varied mortality rates.
According to a 2022 study on trends in the United States, mortality rates for group 2 PH and group 3 PH are higher than those for group 1 PH. From 1999 to 2019, the mortality rate for group 2 PH increased by almost twice as much as the mortality rate for PH overall.
In addition, when adjusted for age, mortality rates were higher than average for women and non-Hispanic Black people with PH.
One 2019 study examined the outlooks of people in a referral center in France. The researchers found that people with group 2 PH and group 3 PH had lower 5-year survival rates than those of people in the other three groups. They were 70% and 62%, respectively, whereas the others ranged from 75% to 85%.
Over the years, in comparison with the other groups of PH, the mortality rates for people with group 2 PH and group 3 PH doubled and tripled, respectively.
Treatments for PH vary depending on the type of PH you have and the cause of your condition.
Medications for improved blood flow
In many cases, a doctor or healthcare professional will prescribe medications that help relax certain blood vessels. These include calcium channel blockers, which are also used to help treat traditional high blood pressure.
Other drugs that help improve blood flow are phosphodiesterase 5 (PDE5) inhibitors such as sildenafil (Revatio, Viagra). These drugs help improve blood flow by relaxing the smooth muscle inside the pulmonary arteries, causing the arteries to dilate. This lowers the heart’s burden of working overtime to pump enough blood to the lungs.
Some drugs are administered orally. Others may be administered continuously via a pump that puts the drug into your veins.
Other medications and treatments
Other medications used to treat PH include digoxin (Lanoxin), which helps the heart pump stronger. Digoxin is also sometimes used to treat heart failure or other heart diseases.
If you have PH, excess fluid can build up in your feet and ankles. You may be prescribed diuretics to help bring your fluid levels back to normal.
Oxygen therapy may also be required to help increase oxygen levels in your blood.
A healthy lifestyle plan, which includes exercise and a heart-healthy diet, is also essential to long-term PH treatment. It can be tailored to your specific needs with a doctor’s guidance.
If you have severe mitral valve disease, surgery to repair or replace your mitral valve may help improve your PH.
Lung or heart-lung transplantation is used for the most serious cases of PH.
A lung transplant is performed on people who have severe PH and lung disease but with heart function that is deemed adequate. A heart-lung transplant may be necessary if both the heart and lungs can no longer function well enough to keep you alive.
Healthy organs can be implanted, but transplant surgery has its risks. It’s a very complex operation that can come with associated risks, and there’s always a waiting list for healthy organs.
If you have PH, talk with a healthcare professional about support groups in your area.
Because PH can restrict your physical activities, it often leads to emotional complications, too. You may want to talk with a mental health professional to deal with these issues.
And remember: Your outlook with PH can be improved if you receive a diagnosis and get treatment as early as possible.
With proper and early treatment, can I still live a normal life span with pulmonary hypertension (PH)?A Healthline reader
People who get care early in the disease process can go on to live normal lives, in both quality and quantity, with the continued guidance and management of a healthcare professional and the team they work with.
There are several options for medical management depending on what World Health Organization (WHO) group classification a person falls into, and these can be initiated under the guidance of a cardiologist or pulmonary doctor.Nick Villalobos, MDAnswers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.