Pulmonary hypertension (PH) is a serious health condition that results when the arteries carrying blood to the lungs are constricted, disrupting blood flow. Blood must travel through the lungs to pick up oxygen that it delivers to all the organs, muscles, and tissue in the body.
When the arteries between the heart and lungs become narrowed or flow is constricted, the heart has to work overtime to pump blood to the lungs. Over time, the heart can grow weak and circulation will diminish throughout the body.
Types of PH
There are five types of PH:
Group 1 pulmonary arterial hypertension (PAH)
Group 1 PAH can have a variety of causes including:
- connective tissue diseases
- liver disease
- congenital heart disease
- sickle cell disease
- schistosomiasis (parasitic infection)
- certain drugs or toxins, including some street drugs and diet medications
- conditions that affect the veins and small blood vessels of the lungs
PAH can also be inherited from your parents. Occasionally, PAH appears with no known cause.
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, which can cause scarring on lung tissue
- sleep apnea
Group 4 PH
Blood clots in the lungs and clotting disorders are associated with Group 4 PH.
Group 5 PH
Group 5 PH is caused by various other conditions, which include:
- 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 tumors and kidney disease
All types of PH require medical attention. Treating the cause of PH can often help slow the disease’s progression.
Survival rates and prognosis
There is currently no cure for PH. It’s a progressive disease, which means it can advance quickly. If left untreated, the disease can be life threatening within a couple of years.
However, the disease can be managed. Some patients can make lifestyle changes and monitor their health. But for many patients, PH can lead to heart failure and their overall health can be in great danger. Medications and lifestyle modifications are intended to slow the progression of the disease.
In cases where PH goes untreated, the long-term outlook is grim. There is a 68 percent chance of survival after one year. The survival odds drop to 34 percent after five years, according to the Ohio State University Lung Center.
If you have PH and scleroderma, a skin disease that also affects the small arteries, your two-year survival odds are 40 percent, according to a 2008 study published in Annals of Thoracic Medicine. Survival rates with PH depend on the cause of the condition.
There’s currently no cure for PH. But treatments are available that can delay its progression. Treatments for PH vary depending on the cause of the condition. If you have severe mitral valve disease, surgery to repair or replace your mitral valve may help improve your PH.
In many cases, medications that help relax the blood vessels are prescribed. These include calcium-channel blockers, which are also used to treat traditional hypertension.
Other drugs that help improve blood flow are phosphodiesterase-5 inhibitors, such as sildenafil (Viagra). These drugs help improve blood flow. They reduce the heart’s burden of working overtime to pump enough blood to the lungs. Some drugs are administered orally. Others are administered continuously via a pump that puts the drug into your veins or under your skin.
Other medications and treatments
Other medications commonly used to treat PH include digoxin, which helps the heart beat stronger. Digoxin is also used to treat heart failure.
Excess fluid can build up in the feet and ankles of PH patients. Diuretics are prescribed to help bring a person’s fluid levels back to normal. Oxygen therapy may also be required to help increase oxygen levels in the blood.
Exercise and a healthy lifestyle are also essential to long-term PH treatment.
Heart-lung transplants offer the only hope for the most serious cases of PH. A lung transplant is performed on patients who also have lung disease. A heart-lung transplant may be necessary if 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 complicated operation, and there is always a waiting list for healthy organs.
If you have PH, talk with your health care provider 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 provider to deal with these issues. And remember: your prognosis with PH can be improved if you get early treatment.