Sonja Bartolome, M.D. Answers Questions On: Pulmonary Hypertension

A lot of people think that because there’s no cure for pulmonary hypertension, there’s nothing you can do for it. I’ve met patients who have been told there was nothing that could be done for them and they should consider hospice. But that’s not true: It is treatable, and it’s worth treating. In the last few years, we’ve made many strides in helping people live longer and better. We can improve their quality of life and the length of their life.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is thought to begin with an acute pulmonary embolus, or a sudden blood clot that goes to the lungs. Acute pulmonary embolus is extremely common, with some studies estimating that it affects one million people in the United States every year. These blood clots in the lungs usually improve with blood thinners, but in a small subset of patients those blood clots don’t go away.  Rather, they “scar” inside the blood vessels of the lungs. Over time, those scars obstruct blood flow in the lungs and can lead to pulmonary hypertension. That is CTEPH. The way we treat CTEPH can be different from other forms of pulmonary hypertension, because select patients can be cured by removing the “scarred in” clots with a specialized surgery or a procedure called balloon pulmonary angioplasty. Our group here at UTSW has trained with the best in the world at these procedures, so that we can bring them to our patients in North Texas.

Anyone who has had an acute pulmonary embolus but remains short of breath three months after starting the blood thinners should be evaluated for CTEPH. There are specialized tests we can do to make sure the blood clots are resolving. Additionally, we screen all of our newly diagnosed pulmonary hypertension patients to make sure they do not have CTEPH, because the treatment plan may be different.

We have many clinical trials in process to either find better treatments for pulmonary hypertension or to understand the disease better. We have clinical trials that help us use existing drugs in better ways – by combining them or trying new ways to deliver them. We also have trials for drugs that are new agents showing promise for the treatment of this disease. Usually those are done in combination with a proven therapy.