What is pulmonary hypertension?

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 blood flow. Blood must travel through the lungs for air exchange in order 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 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.

There are five types, or groups, of PH:

Group 1 pulmonary arterial hypertension (PAH)

Group 1 PAH can have a variety of causes including:

  • connective tissue diseases (certain autoimmune diseases)
  • HIV
  • liver disease
  • congenital heart disease
  • sickle cell disease
  • schistosomiasis, a type of parasitic infection
  • certain drugs or toxins, including some recreational drugs and diet medications
  • conditions that affect the veins and small blood vessels of the lungs

PAH can also be inherited genetically. In some cases, PAH appears with no known cause.

Group 2 PH

Group 2 PH is caused by conditions that affect the left side of the heart and carry over to the right side of the heart. This includes mitral valve disease and long-term systemic high blood pressure.

Group 3 PH

Group 3 PH is associated with certain lung and breathing conditions, including:

Group 4 PH

Blood clots in the lungs and other clotting disorders are associated with Group 4 PH.

Group 5 PH

Group 5 PH is caused by various other conditions, which include:

All types of PH require medical attention. Treating the cause of PH can help slow the disease’s progression.

There’s currently 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. If left untreated, the disease can become life-threatening within a couple of years.

However, the disease can be managed. Some people can effectively make lifestyle changes and monitor their health. But for many people, PH can lead to significant heart failure, and their overall health can be in great danger. Medications and lifestyle modifications are intended to slow the progression of the disease.

If you have PH and systemic scleroderma, an autoimmune skin disease that also affects the small arteries and internal organs, your two-year survival odds are estimated to be 40 percent, according to a 2008 study. Survival rates with PH depend on the cause of the condition.

There’s currently no nonsurgical 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 certain 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 (Revatio, Viagra). These drugs help improve blood flow by relaxing the smooth muscle inside the pulmonary arteries, causing them to dilate. This reduces 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 used in some to treat heart failure or other heart disease.

Excess fluid can build up in the feet and ankles of people with PH. Diuretics are prescribed to help bring 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, which can be tailored to one’s specific needs with your doctor’s guidance.

Transplantation

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 heart function 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 complications, and there’s always a waiting list for healthy organs.

If you have PH, talk with your healthcare 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’re diagnosed and treated as early as possible.