Pulmonary hypertension is a disease in which there is an increase in pressure in the blood vessel that connects the heart to the lungs, called the pulmonary artery. Because of this increase in pressure, the heart has to work harder in order to pump blood to the lungs.
There are many causes of pulmonary hypertension.
Connective tissue diseases can be considered a cause of pulmonary hypertension by causing inflammation or thickening, also known as fibrosis, of the pulmonary artery. These diseases include:
Lung diseases can also cause pulmonary hypertension by causing the pulmonary blood vessels to narrow, or constrict. These include:
Some forms of pulmonary hypertension are inherited and caused by a genetic mutation that can run in a family. Some patients can develop “idiopathic” pulmonary hypertension, which means the causes are unknown.
There are many potential symptoms of pulmonary hypertension. These include:
Rheumatologists commonly encounter patients with pulmonary hypertension in their practices.
If you are concerned that you may have pulmonary hypertension, talk to your rheumatologist. He or she can perform the necessary history and physical exam, and refer you to a pulmonary hypertension specialist if necessary. Doctors who specialize in the treatment of pulmonary hypertension tend to be cardiologists and pulmonologists, although there are some rheumatologists who have a particular interest in the care of patients with pulmonary hypertension as well.
Studies have shown that about 8 to 12% of patients with scleroderma develop some degree of pulmonary hypertension.
Your doctor will perform a complete history and physical exam. He or she will likely order some blood tests as well as one or more of the following diagnostic tests:
Yes, there are several different medications for the treatment of pulmonary hypertension. Your doctor will determine what medication or combination of medications is right for you.
Yes. If you have scleroderma and have undergone a right heart catheterization within the prior 2 years, you may be eligible for a study of a 3-minute step test that evaluates how well your lungs work. If you are interested in participating, please contact Dr. Elana Bernstein at 212.774.2788 for more information.
Elana J. Bernstein, M.D.
Rheumatology Fellow, Hospital for Special Surgery